Wilcox's Surgical Anatomy of The Heart 4th Ed

Wilcox’s Surgical Anatomy
of the Heart
Fourth edition
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Wilcox’s Surgical Anatomy
of the Heart
Fourth edition
Robert H. Anderson, BSc, MD, FRCPath

Visiting Professor, Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, UK;
Visiting Professor of Pediatrics, Medical University of South Carolina, Charleston, SC, USA
Diane E. Spicer, BS, PA(ASCP)

Pathologists’ Assistant, University of Florida – Pediatric Cardiology, Gainesville, Florida, and
Congenital Heart Institute of Florida, St. Petersburg, FL, USA
Anthony M. Hlavacek, MD
Associate Professor, Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA
Andrew C. Cook, BSc, PhD

Senior Lecturer, Cardiac Unit, Institute of Child Health, University College London, London, UK
Carl L. Backer, MD
A. C. Buehler Professor of Surgery, Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie
Children’s Hospital of Chicago, Chicago, IL, USA
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Fourth edition first published 2013
Third edition first published 2004
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Library of Congress Cataloguing in Publication data
Anderson, Robert H. (Robert Henry), 1942–
Wilcox’s surgical anatomy of the heart. – Fourth edition / Robert H. Anderson, BSc, MD, FRCPath, Diane E. Spicer,
BS, Anthony M. Hlavacek, MD, Andrew C. Cook, BSc, PhD, Carl L. Backer, MD.
pages cm
ISBN 978-1-107-01448-0 (hardback)
1. Heart – Anatomy. 2. Heart – Surgery. I. Title. II. Title: Wilcox’s Surgical Anatomy of the Heart.
QM181.W55 2013
6110 .12–dc23
2012051614
ISBN 978-1-107-01448-0 Hardback
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Every effort has been made in preparing this book to provide accurate and
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Contents
Preface page vii
Acknowledgements viii
Surgical approaches to the heart 1
Anatomy of the cardiac chambers 13
Surgical anatomy of the valves of the heart 51
Surgical anatomy of the coronary circulation 90
Surgical anatomy of the conduction system 111
Analytical description of congenitally malformed hearts 128
Lesions with normal segmental connections 150
Lesions in hearts with abnormal segmental connections 244
Abnormalities of the great vessels 321
Positional anomalies of the heart 363
Index 377
Preface
The books and articles devoted to feedback from those who had used the first Diane Spicer to our anatomical team. She
technique in cardiac surgery are legion. edition was very positive. In the third has contributed enormously by providing
This is most appropriate, as the success of edition, we sought to expand and improve many new and better illustrations of the
cardiac surgery is greatly dependent upon still further on the changes made in the anatomy as seen in the autopsied heart.
excellent operative technique. But second edition. In the second edition, we These advances are complimented by the
excellence of technique can be dissipated had added an entirely new chapter on contributions of our other new editor,
without a firm knowledge of the underlying cardiac valvar anatomy, and greatly Tony Hlavacek. Tony has provided quite
cardiac morphology. This is just as true for expanded our treatment of coronary remarkable images obtained using
the normal heart as for those hearts with vascular anatomy. We retained this format computed tomography and magnetic
complex congenital lesions. It is the in the third edition, as we were gratified resonance imaging, which show that the
feasibility of operating upon such complex that, as hoped, readers were able to find a heart can be imaged with just as much
malformations that has highlighted the particular subject more easily. The third accuracy during life as when we hold the
need for a more detailed understanding of edition also contained still more new specimens in our hands on the autopsy
the basic anatomy in itself. Thus, in recent illustrations, retaining the approach of bench. Recognising the huge contributions
years surgeons have come to appreciate the orientating these illustrations, where of Ben Wilcox, we are also pleased to
necessity of avoiding damage to the appropriate, as seen by the surgeon rename this fourth edition ‘Wilcox’s
coronary vessels, often invisible when working in the operating room, but Surgical Anatomy of the Heart’. As with
working within the cardiac chambers, and reverting to anatomical orientation for most the previous editions, it is our hope that the
particularly to avoid the vital conduction of the pictures of specimens. So as to clarify new edition will continue to be of interest
tissues, invisible at all times. Although the various orientations of each individual not only to the surgeon, but also to the
detailed and accurate descriptions of the illustration, we continued to include a set of cardiologist, anaesthesiologist, and surgical
conduction system have been available axes showing, when appropriate, the pathologist. All of these practitioners
since the time of their discovery, only directions of superior, inferior, anterior, ideally should have some knowledge of
rarely has its position been described with posterior, left, right, apex, and base. All cardiac structures and their exquisite
the cardiac surgeon in mind. At the time accounts were based on the anatomy as it is intricacies, particularly those cardiologists
the first edition of this volume was observed and, except in the case of who increasingly treat lesions that
published, to the best of our knowledge malformations involving the aortic arch and previously were the province of the surgeon.
there had been no other books that its branches, they owe nothing to Our senior anatomist remains active, and
specifically displayed the anatomy of speculative embryology. has been fortunate to be granted access to
normal and abnormal hearts as perceived at A major change was forced upon us as several archives of autopsied hearts held in
the time of operation. We tried to satisfy we prepared this fourth edition, as our the United States of America subsequent to
this need in the first volume by combining original surgical author, Benson Wilcox, his retirement from the Institute of Child
the experience of a practising cardiac died in May of 2010. It is very difficult to Health in London. We remain confident
surgeon with that of a professional cardiac replace such a pioneer and champion of that, in the hands of this new team, and if
anatomist. We added significantly to the surgical education, but we are gratified that supply demands, the book will pass through
illustrations in the second edition, while Carl Backer has assumed the role of still further editions, hopefully continuing
seeking to retain the overall concept, as surgical editor. We are also pleased to add to improve with each version.
Robert H. Anderson, Diane E. Spicer,
Anthony M. Hlavacek,
Andrew C. Cook,
and Carl L. Backer,
London, Tampa, Charleston and Chicago
November, 2012
http://dx.doi.org/10.1017/CBO9781139028561.001
Acknowledgements
A good deal of the material displayed in obtained using computed tomography and Illustrations and Photography, University
these pages, and the concepts espoused, are magnetic resonance imaging. We of North Carolina. As with the third
due in no small part to the help of our continue, nonetheless, to owe a particular edition, we owe an equal debt of gratitude
friends and collaborators. As indicated in debt to Anton Becker of the University of to Gemma Price, who has continued to
our preface, the major change since we Amsterdam, Bob Zuberbuhler of improve our series of cartoons. For both
produced the third edition has been the sad Children’s Hospital of Pittsburgh, the third edition and this edition, she has
passing of our founding surgical editor, Pennsylvania, United States of America, worked over and above the call of duty. We
Benson R. Wilcox. We have renamed this and F. Jay Fricker of University of Florida, also thank Vi Hue Tran, who helped
fourth edition ‘Wilcox’s Surgical Anatomy Gainesville, Florida, United States of photograph the hearts from Great
of the Heart’. We dedicate this edition to America, all of whom permitted us to use Ormond Street. We are again indebted to
his eternal memory. A further change has material from the extensive collections of Christine Anderson for her help during
been the retirement of Robert H. Anderson normal and pathological specimens held in the preparation of the manuscript, and
from the Institute of Child Health at their centres. We also continue to thank the team supporting Carl Backer at
Great Ormond Street Children’s Hospital, acknowledge the debt owed to Siew Yen Lurie Children’s of Chicago, in
London. Retirement, however, has Ho, of the National Heart and Lung particular Pat Heraty and Anne E. Sarwark.
permitted him to establish new Institute, part of Imperial College in Finally, it is a pleasure to acknowledge the
connections, not least with the newest London. Yen produced many of the support provided by Cambridge
additions to our team of authors. This has original drawings from which we University Press, who have ensured that
permitted many new hearts to be prepared our artwork, and photographed all the good parts of the previous
specifically photographed for this new many of the hearts in the Brompton editions were retained. In particular, we
edition, not only of autopsy specimens, but archive. The initial photographs and thank Nicholas Dunton and Joanna
also in the operating room. In addition, it surgical artwork could not have been Chamberlin for all their help
has created the collaboration that permits produced without the considerable help during the preparation of the book for
the inclusion of wonderful images given by the Department of Medical publication.
Surgical approaches
to the heart
2 Wilcox’s Surgical Anatomy of the Heart
When we describe the heart in this chapter, white line, or linea alba, is incised between infant (Figures 1.3, 1.4). It has two lateral
and in subsequent chapters, our account the two rectus sheaths, taking care to avoid lobes, joined more or less in the midline.
will be based on the organ as viewed in its entry to the peritoneal cavity, or damage to Sometimes this junction between the lobes
anatomical position1. Where appropriate, an enlarged liver, if present. Reflection of must be divided, or partially excised, to
the heart will be illustrated as it would be the origin of the rectus muscles in this area provide adequate exposure. The arterial
viewed by the surgeon during an operative reveals the xiphoid process, which is supply to the thymus is from the internal
procedure, irrespective of whether the incised to provide inferior access to the thoracic and inferior thyroid arteries. If
pictures are taken in the operating room, or anterior mediastinum. Superiorly, a divided, these arteries tend to retreat into
are photographs of autopsied hearts. When vertical incision is made between the the surrounding soft tissues, and can
we show an illustration in non-surgical sternal insertions of the produce troublesome bleeding. The veins
orientation, this will be clearly stated. sternocleidomastoid muscles. This exposes draining the thymus are fragile, often
In the normal individual, the heart lies the relatively bloodless midline raphe emptying into the left brachiocephalic or
in the mediastinum, with two-thirds of its between the right and left sternohyoid and innominate vein via a common trunk
bulk to the left of the midline (Figure 1.1). sternothyroid muscles. An incision (Figure 1.5). Undue traction on the gland
The surgeon can approach the heart, and through this raphe gives access to the can lead to damage to this major vessel.
the great vessels, either laterally through superior aspect of the anterior When the pericardial sac is exposed
the thoracic cavity, or directly through the mediastinum. The anterior mediastinum within the mediastinum, the surgeon
mediastinum anteriorly. To make such immediately behind the sternum is devoid should have no problems in gaining access
approaches safely, knowledge is required of of vital structures, so that the superior and to the heart. The vagus and phrenic nerves
the salient anatomical features of the chest inferior incisions into the mediastinum can traverse the length of the pericardium, but
wall, and of the vessels and the nerves that safely be joined by blunt dissection in the are well lateral (Figures 1.2, 1.6). The
course through the mediastinum retrosternal space. Having split the phrenic nerve on each side passes
(Figure 1.2). The approach used most sternum, retraction will reveal the anteriorly, and the vagus nerve posteriorly,
frequently is a complete median pericardial sac, lying between the pleural relative to the hilum of the lung
sternotomy, although increasingly the cavities. Superiorly, the thymus gland (Figure 1.6).
trend is to use more limited incisions. The wraps itself over the anterior and lateral At operation, the course of the phrenic
incision in the soft tissues is made in the aspects of the pericardium in the area of nerve is seen most readily through a lateral
midline between the suprasternal notch exit of the great arteries, the gland being a thoracotomy (Figure 1.7). It is when the
and the xiphoid process. Inferiorly, the particularly prominent structure in the heart is approached through a median
Long axis of body
Obtuse margin
Long axis of heart
Fig. 1.1 The computed tomogram, with the

cardiac cavities delimited subsequent to
injection of contrast material, shows the
relationships of the heart to the thoracic
structures well. Note the discordance between
Acute margin Apex the cardiac long axis and the long axis of the
body.
Thymic veins Brachiocephalic vein Sup.
Right Left
Right phrenic
nerve
Inf.
Superior
caval vein
Left phrenic
nerve
Pulmonary
trunk
Aorta
Left atrial
appendage
Right atrium
Fig. 1.2 This view, taken at autopsy,

Right ventricle Left ventricle demonstrates the anatomical relationships of
the vessels and nerves within the mediastinum.
sternotomy, therefore, with the nerve not pericardial cavity may also lead to phrenic requiring greater degrees of exposure, the
immediately evident, that it is most liable to paralysis or paresis. latissimus dorsi can be partially divided. It
injury. Although it can sometimes be seen A standard lateral thoracotomy provides is rarely necessary, if ever, to divide the
through the reflected pericardium access to the heart and great vessels via the serratus anterior. The intercostal muscles
(Figure 1.8), its proximity to the superior pleural space. Left-sided incisions provide are then divided equidistant between the
caval vein (Figures 1.2, 1.9, 1.10), or to a ready access to the great arteries, left fourth and fifth ribs. The incision is rarely
persistent left caval vein when that pulmonary veins, and the chambers of the carried forward beyond the midclavicular
structure is present (Figure 1.11), is not left side of the heart. Most frequently, the line in a submammary position, and care is
always easily appreciated when these incision is made in the fourth intercostal taken to avoid damage to the nipple and the
vessels are dissected from the anterior space. The posterior extent is through the tissue of the breast. The intercostal
approach. Near the thoracic inlet, it passes triangular, and relatively bloodless, space neurovascular bundle is well protected
close to the internal thoracic artery between the edges of the latissimus dorsi, beneath the lower margin of the fourth rib.
(Figures 1.6, 1.10), exposing it to injury trapezius, and teres major muscles Having divided the musculature as far as
either directly during takedown of that (Figure 1.12). The floor of this triangle is the pleura, the pleural space is entered, and
vessel, or by avulsing the pericardiophrenic the sixth intercostal space. Division of the the lung permitted to collapse away from
artery with excessive traction on the chest latissimus dorsi, and a portion of trapezius the chest wall. Posterior retraction of the
wall. The internal thoracic arteries posteriorly, frees the scapula so that the lung reveals the middle mediastinum, in
themselves are most vulnerable to injury fourth intercostal space can be identified. which the left lateral lobe of the thymus,
during closure of the sternum. The phrenic Its precise identity should be confirmed by with its associated nerves and vessels, is
nerve may be injured when removing the counting down the ribs from above. The seen overlying the pericardial sac and the
pericardium to use as a cardiac patch, or so-called muscle sparing thoracotomy is aortic arch. Intrapericardial access is
when performing a pericardiectomy. designed to preserve the latissimus dorsi usually gained anterior to the phrenic
Injudicious use of cooling agents within the and serratus anterior muscles. In cases nerve. On occasion, the thymus gland may
Sup.
Right Left
Inf.
Thymus
Pericardial sac
Diaphragm
Fig. 1.3 This view, taken at autopsy,
demonstrates the extent of the thymus as it
extends over the anterior and lateral aspects of
the pericardial sac at the base of the heart.
Note the haemorrhagic pericardial effusion.
Left
Left lobe
of thymus
Sup. Inf.
Right
Pericardial
sac
Right lobe
of thymus
Fig. 1.4 This view, taken in the operating
room through a median sternotomy in an
infant, shows the extent of the thymus gland.
Superior caval vein Phrenic nerve Note the right phrenic nerve adjacent to the
superior caval vein.
require elevation when the incision is and descending thoracic aorta, and the which passes around the inferior border of
extended superiorly, precautions being parietal pleura is divided on its mediastinal the arterial ligament, or the duct if the
taken to avoid unwanted damage as aspect. This is usually done posterior to the arterial channel is still patent (Figure 1.13).
discussed earlier. The lung is retracted vagus nerve. In this area, the vagus nerve The recurrent nerve then ascends towards
anteriorly to approach the aortic isthmus gives off its left recurrent laryngeal branch, the larynx on the medial aspect of the
Left
Sup. Inf.
Right
Brachiocephalic vein
Thymic
veins
Aorta in
pericardium
Right lobe of
thymus
Fig. 1.5 This operative view, again taken through a median
sternotomy, shows the delicate veins that drain from the thymus
gland to the left brachiocephalic veins.
Left pericardiophrenic
artery and vein
Left phrenic nerve
Left vagus & recurrent laryngeal nerve
Left internal thoracic artery

Right phrenic nerve
Fig. 1.6 As shown in this cartoon of a median

Right pericardiophrenic sternotomy, the pericardium can be opened in
Right vagus & recurrent laryngeal nerve artery and vein the midline so that the phrenic and vagus
nerves stay well clear of the operating field.
Ant.
Inf. Sup.
Post.
Left pericardiophrenic vein
Left phrenic nerve

Left pericardiophrenic artery
Fig. 1.7 This operative view, taken through a

left lateral thoracotomy, shows the course of
the left phrenic nerve over the pericardium.
Right atrial Left

appendage
Sup. Inf.
Right
Right phrenic nerve

Superior caval Fig. 1.8 This operative view, taken through a
vein median sternotomy, shows the right phrenic
nerve as seen through the reflected
pericardium.
posterior wall of the aorta, running adjacent the phrenic and vagus nerves (Figures 1.11, the duct, which is usually posteriorly
to the oesophagus. Excessive traction of the 1.14, 1.15). This structure, however, is located and runs along the vertebral
vagus nerve as it courses into the thorax rarely of surgical significance, but is column, can be troublesome when
along the left subclavian artery can cause frequently divided to provide surgical dissecting the origin of the left subclavian
injury to the recurrent laryngeal nerve just access to the aorta. The thoracic duct artery.
as readily as can direct trauma to the nerve (Figure 1.16) ascends through this area, A right thoracotomy, in either the
in the environs of the ligament. The draining into the junction of the left fourth or fifth interspace, is made through
superior intercostal vein is seen crossing subclavian and internal jugular veins. an incision similar to that for a left one. The
the aorta, then insinuating itself between Accessory lymph channels draining into fifth interspace is used when approaching
Left
Cut edge of Right phrenic
pericardium nerve
Sup. Inf.
Right
Fig. 1.9 This operative view, taken through a

median sternotomy having pulled back the
Right pulmonary veins
edge of the pericardial sac, shows the right
phrenic nerve in relation to the right
pulmonary veins.
Right internal
thoracic artery
Right phrenic nerve
Superior
caval vein
Ant.
Inf. Sup.
Azygos vein Fig. 1.10 This operative view, taken through
a right thoracotomy, shows the relationship of
Post.
the right phrenic nerve to the right internal
thoracic artery and the superior caval vein.
Left phrenic nerve

Superior
intercostal vein
Arch of aorta
Ant.
Persistent left
superior caval vein
Inf. Sup.
Fig. 1.11 This operative view, taken through a left thoracotomy, shows the
Post.
relationship of the left phrenic nerve to a persistent left superior caval vein.
Note also the course of the superior intercostal vein.
Teres major
Latissimus dorsi
Trapezius
Bloodless triangle Fig. 1.12 The cartoon shows the location of

the bloodless area overlying the posterior
extent of the sixth intercostal space.
Ant.
Left vagus nerve
Inf. Sup.
Post.
Patent arterial
duct
Left recurrent
laryngeal nerve
Fig. 1.13 This operative view, taken through a left lateral thoracotomy in an
adult, shows the left recurrent laryngeal nerve passing around the arterial duct.
Left vagus nerve
Superior
intercostal
vein
Superior Sup.
caval vein
Ant. Post.
Arterial duct
Inf. Fig. 1.14 The anatomical image shows the course of the left superior
intercostal vein.
Left phrenic nerve
Left vagus
nerve
Left superior
intercostal vein
Aorta
Left subclavian
artery
Ant.
Inf. Sup.
Fig. 1.15 This operative view, taken through a left lateral thoracotomy,
Post. shows the course of the left superior intercostal vein. (Compare with
Figure 1.14.)
Aortic isthmus Left subclavian artery
Ant.
Thoracic Inf. Sup.

duct Fig. 1.16 In this operative view, taken through a left
Post. thoracotomy, the thoracic duct is seen coursing below the left
subclavian artery to its termination in the brachiocephalic vein.
Left
Superior
caval vein Sup. Inf.
Left brachiocephalic
vein
Right
Fig. 1.17 This anatomical image, taken at

Trachea autopsy, shows the normal location of the
azygos vein as it extends along the spine,
receives the intercostal veins, and crosses over
Azygos vein Intercostal veins
the root of the right lung to empty into the
Right common
carotid artery Brachiocephalic artery
Right recurrent
laryngeal nerve
Left
Sup. Inf. Fig. 1.18 This operative view, taken through

a median sternotomy, shows the course of the
Right subclavian artery
Right right recurrent laryngeal nerve relative to the
right subclavian artery.
the heart, while the fourth permits access to vagus nerve, the right recurrent laryngeal the same basic anatomical rules apply as
the right-sided great vessels. Access to the nerve taking origin from the vagus and described previously. Thus far, our
pericardium is gained by incising anterior curling around the posteroinferior wall of account has presumed the presence of
to the phrenic nerve, this approach often the artery before ascending into the neck normal anatomy. In many instances, the
necessitating retraction of the right lobe of (Figure 1.18). Also encircling the disposition of the thoracic structures will
the thymus. To reach the right pulmonary subclavian origin on this right side is the be altered by a congenital malformation.
artery, and its adjacent mediastinal subclavian sympathetic loop, the so-called These alterations will be described in the
structures, it is sometimes useful to divide ansa subclavia, a branch of the sympathetic appropriate sections.
the azygos vein near its junction with the trunk that runs up into the neck. Damage
superior caval vein (Figure 1.17). to this structure can produce Horner’s Reference
Extension of this incision superiorly syndrome.
exposes the origin of the right subclavian An anterior right or left thoracotomy is 1. Cook AC, Anderson RH. Attitudinally
branch of the brachiocephalic trunk. occasionally used in treating congenital correct nomenclature. Heart 2002; 87:
Laterally, this artery is crossed by the right malformations. Once the chest is opened, 503–506.
Anatomy of the
2
cardiac chambers
Regardless of the surgical approach, once support the heart within the mediastinum. myocardium, and is the epicardium
having entered the mediastinum, the Free-standing around the atrial chambers (Figure 2.1). The pericardial cavity,
surgeon will be confronted by the heart and the ventricles, the sac becomes therefore, is the space between the inner
enclosed in its pericardial sac. In the strict adherent to the adventitial coverings of parietal serous lining of the fibrous
anatomical sense, this sac has two layers, the great arteries and veins at their pericardium and the surface of the heart
one fibrous and the other serous. From a entrances to and exits from it, these (Figure 2.2). There are two recesses
practical point of view, the pericardium is attachments closing the pericardial cavity. within the cavity that are lined by serous
essentially the tough fibrous layer; the The cavity of the pericardium is limited pericardium. The first is the transverse
serous component forms the lining of the by the two layers of serous pericardium, sinus, which occupies the inner curvature
fibrous sac, and is reflected back onto the which are folded on one another to of the heart (Figure 2.3). Anteriorly, it is
surface of the heart as the epicardium. It is produce a double-layered arrangement. bounded by the posterior surface of the
the fibrous sac, therefore, which encloses The outer or parietal layer is densely great arteries. Posteriorly, it is limited by
the mass of the heart. By virtue of its own adherent to the fibrous pericardium, while the right pulmonary artery and the roof of
attachments to the diaphragm, it helps the inner layer is firmly attached to the the left atrium. There is a further recess
Aorta
Transverse sinus
Right pulmonary
Pericardial cavity artery
Left atrium
Oblique sinus
Ant.
Base
Visceral
Apex Serous pericardium
Fibrous Parietal Fig. 2.1 The cartoon shows the arrangement of the
Post. pericardium pericardial cavity as seen in a parasternal long axis view.
Left appendage Left
Sup. Inf.
Right
*
Pulmonary
trunk *
Right ventricle
*
Aorta
*
*
*
*
Right
appendage *
* * Fig. 2.2 The operative view through a
Fibrous median sternotomy shows the anterior surface
pericardium * * of the heart following a pericardial incision.
* Pericardial cavity
The white asterisks show the extent of the
pericardial cavity.
from the transverse sinus that extends caval vein, provide excellent access to the The second pericardial recess is the
between the superior caval and the right superior aspect of the left atrium and the oblique sinus. This is a blind-ending cavity
upper pulmonary veins, with its right right pulmonary artery. This fold is also behind the left atrium (Figure 2.5), with its
lateral border being a pericardial fold incised when a snare is placed around the upper boundary formed by the reflection of
between these vessels (Figure 2.4). When superior caval vein. Laterally, on each serous pericardium between the upper
exposing the mitral valve through a left side, the ends of the transverse sinus are in pulmonary veins. The right border is the
atriotomy, incisions through this fold, free communication with the remainder of reflection of pericardium around the right
along with mobilisation of the superior the pericardial cavity. pulmonary veins and the inferior caval
Left
Pulmonary trunk Sup. Inf.
Right
Aorta
Right Clamp in transverse

appendage sinus Fig. 2.3 Operative view through a median
sternotomy. The clamp has been passed
through the transverse sinus.
Left
Clamp tenting pericardial fold
Sup. Inf.
Right
Fig. 2.4 Operative view through a median

sternotomy showing the posterior recess of the
transverse sinus limited by a pericardial fold
around the superior caval vein. In this picture,
Superior caval vein the fold is being tented by a right-angled clamp
passed behind the superior caval vein.
vein, while the left border is the reflection midline and retracted laterally, exposing rightward position (Figure 2.2). Should
of pericardium around the left pulmonary the anterior sternocostal surface of the heart the aortic root not be in this expected
veins (Figure 2.6). and great vessels. The pulmonary trunk and relationship, the ventriculoarterial
With the usual surgical approach aorta are seen leaving the base of the heart connections will almost always be abnormal
through a median sternotomy, the fibrous and extending in a superior direction, with (see Chapter 8). The atrial appendages are
pericardium is opened more-or-less in the the aortic root in the posterior and usually seen one to either side of the
Pulmonary trunk Oblique ligament Left pulmonary veins
Left atrial
appendage
Sup.
Right Left
Fig. 2.5 Anatomical view showing the oblique sinus of the
pericardial cavity, which lies behind the left atrium. Note the
Inf. Oblique sinus oblique ligament, which occupies the site during development of
the left superior caval vein.
Coronary sinus Sup.

Right atrium
Right Left
Inf.
Left pulmonary veins
Inferior caval vein Oblique sinus Fig. 2.6 The heart has been reflected superiorly from its
pericardial cradle to show the location of the oblique sinus.
prominent arterial pedicle. The side of the arterial pedicle is an anomaly in superiorly within the pericardial cavity,
morphologically right appendage is more itself, which is called juxtaposition. This with the left atrial appendage located
prominent. It has a blunt triangular shape, arrangement is most often associated with anteriorly and laterally (Figure 2.9).
and possesses a broad junction with the additional malformations within the heart Within the pericardial cavity, it extends
atrial cavity (Figure 2.7). The (see Chapter 8). Inspection of the left border down the posterior aspect of the left atrium,
morphologically left appendage may not be of the heart should always include a search passing through the inferior left
seen immediately. When found at the left for persistence of the left superior caval atrioventricular groove to reach the right
border of the pulmonary trunk, it is a vein. When present, the venous channel atrial orifice of the coronary sinus
tubular structure, having a narrow junction will be found by following the course of the (Figure 2.10).
with the rest of the atrium (Figure 2.8). The left pulmonary artery. The vein crosses The ventricular mass extends from the
presence of the two appendages on the same anterior to the pulmonary artery and is seen atrioventricular grooves to the apex, and
Left
Sup. Inf.
Right
Right ventricle
Aorta
Right atrial
Fig. 2.7 Operative view through a median sternotomy showing
appendage
the typical triangular shape of the morphologically right atrial
appendage.
Left atrial appendage Left
Sup. Inf.
Right
Pulmonary trunk
Subpulmonary infundibulum

the tubular morphologically left atrial appendage.
usually extends into the left hemithorax. malformations (see Chapter 10). In shape, pulmonary surfaces (Figure 2.11). The
An anomalous position of the ventricular the ventricular mass is a three-sided margin between the first two surfaces is
mass, or its apex, is again highly suggestive pyramid, having inferior diaphragmatic, sharp. Because of this, it is described as the
of the presence of congenital cardiac anterior sternocostal, and posterior acute margin. The angulations of the
Left atrial appendage
Pulmonary trunk
Left
Sup. Inf.
Fig. 2.9 The operative view through a median sternotomy shows

Right
the location of a persistent left superior caval vein, snared by the
surgeon in this image.
Left superior caval vein

Left atrial
appendage
Sup.
Post. Ant. Fig. 2.10 The base of the heart has been dissected by removing
the atrial walls. The dissection shows the course of a persistent left
superior caval vein as it passes through the left atrioventricular
Inf. Coronary sinus groove (red dotted lines), emptying into the right atrium through
the enlarged orifice of the coronary sinus.
Sup.
Obtuse margin
Ant Post
Inf.
Sternocostal
Pulmonary
Diaphragmatic
Acute margin Fig. 2.11 The heart has been removed from the chest and is
viewed from its apex, showing the surfaces of the ventricular cone.
margins between the pulmonary and the the right atrium is the terminal groove, or the vestibule of the tricuspid valve. It also
sternocostal surfaces anteriorly, and the sulcus terminalis. This marks the junction has a small body, but the boundaries of this
pulmonary and diaphragmatic surfaces between the appendage and the systemic part usually cannot be distinguished from
posteriorly, are much more obtuse. The venous component of the right atrium the venous sinus. It is separated from the
surgeon encounters these obtuse marginal (Figure 2.12). The sinus node is located left atrium by the septum. The junction of
areas when the apex of the heart is tipped within this groove, usually laterally and the appendage and the systemic venous
out of the pericardium. They are supplied inferiorly relative to the superior cavoatrial sinus is identified externally by the
by the obtuse marginal branches of the junction (Figures 2.13, 2.14), but prominent terminal groove (Figure 2.12).
circumflex coronary artery. The greater occasionally extending over the crest of the Internally, the groove corresponds with the
part of the anterior surface of the appendage (Figure 2.15). The clinically terminal crest, which gives origin to the
ventricular mass is occupied by the significant artery to the sinus node can also pectinate muscles of the appendage
morphologically right ventricle, with its left be seen on occasion, either as it crosses the (Figure 2.17). In shape, the appendage is
border marked by the anterior crest of the right appendage, or as it courses blunt and triangular, having a wide
interventricular or descending branch of behind the superior caval vein to enter the junction to the venous sinus across the
the left coronary artery. This artery curves terminal groove between the orifices of the terminal groove. The venous sinus is much
onto the ventricular surface between the caval veins. Posterior to, and parallel with, smaller when viewed externally, with only
left atrial appendage and the basal origin of the terminal groove is a second, deeper that part extending between the terminal
the pulmonary trunk. The right border of groove, which interposes between the cavity and Waterston’s grooves being visible to
the morphologically right ventricle is of the right atrium and the right pulmonary the surgeon. It receives the superior and
marked by the right coronary artery, which veins. The surgeon can use this interatrial inferior caval veins at its extremities.
runs obliquely in the atrioventricular groove, known as Waterston’s or Superiorly and anteriorly, the appendage
groove. Unusually prominent coronary Sondergaard’s groove, to gain access to the has a particularly important relation with
arteries coursing on the ventricular surface left atrium (Figure 2.16), either by making the superior caval vein. Here, the
should always raise the suspicion of an incision in the floor of the groove, or appendage terminates in a prominent crest
significant cardiac malformations. through the left atrial roof. The latter area is (Figure 2.13). This forms the summit of
The surface anatomy of the heart is seen behind the aorta, to the left of the the terminal groove, and is continuous in
helpful in determining the most appropriate superior cavoatrial junction (Figure 2.12). the transverse sinus behind the aorta with
site for an incision to gain access to a given the interatrial groove (Figure 2.14).
cardiac chamber. For example, the relatively Absence of such a right-sided crest should
MORPHOLOGICALLY RIGHT
bloodless outlet portion of the right ventricle alert the surgeon to the presence of
ATRIUM
just beneath the origin of the pulmonary isomerism of the left atrial appendages (see
trunk affords ready access to the cavity of The right atrium has three basic parts, the Chapter 6). As already discussed, the sinus
the subpulmonary infundibulum appendage, the venous component node almost always lies immediately
(Figure 2.8). The important landmark for receiving the systemic venous return, and subepicardially within the terminal groove.
Sup.
Superior caval vein
Left Right
Systemic venous sinus Inf.
Right Right atrial

pulmonary appendage
veins
Waterston’s
groove
Fig. 2.12 The heart is photographed from the right side to show
the location of the terminal groove (black dotted line) between the
Inferior caval vein
appendage and the systemic venous sinus.
Crest of appendage Left
Sup. Inf.
Right
Appendage
ICV
SCV
Sinus node Terminal groove
Fig. 2.13 The cartoon shows the usual site of the sinus node within
the terminal groove (upper panel). The lower panel shows the
horseshoe arrangement found in about one-tenth of cases. SCV,
‘Horseshoe’ node
superior caval vein; ICV, inferior caval vein.
Cigar-shaped, it usually lies to the right of one-tenth of cases, the node extends across Also of significance is the course of
the crest as seen by the surgeon; in other the crest into the interatrial groove. It is the artery to the sinus node (Figure 2.18).
words, lateral and inferior to the superior then draped across the cavoatrial junction This artery is a branch of the right coronary
cavoatrial junction (Figure 2.13). In about in horseshoe fashion (Figures 2.14, 2.15)1. artery in about 55% of individuals, and a
Crest of appendage
Aorta
Left
Fig. 2.14 Operative view through a median sternotomy
Sup. Inf. demonstrating the usual site of the sinus node. The node can often
Superior be seen as a pale cigar-shaped area located anterolaterally in the
caval vein Right terminal groove. This anticipated site is highlighted in the
photograph by the white cross-hatched area.
Left Roof of left atrium
Sup. Inf.
Right

a sinus node arranged in horseshoe fashion across the crest of the
Sinus nodal artery right atrial appendage, with one limb in the terminal groove and
the other extending towards the interatrial groove. The nodal
Crest of right atrial appendage location is again highlighted by the white cross-hatched area. Note
the course of the artery to the node.
branch of the circumflex artery in the originates from either coronary artery some Irrespective of its origin, as it enters the
remainder2. Irrespective of its origin, it distance from the aorta. If taking origin sinus node, the artery may cross the crest of
usually courses through the anterior from the right coronary artery, it courses the appendage, course retrocavally
interatrial groove towards the superior over the lateral surface of the appendage to (Figure 2.23), or even divide to form an
cavoatrial junction (Figure 2.19), reach the terminal groove (Figure 2.21). If arterial circle around the junction
frequently running within the atrial originating from the circumflex artery, it (Figure 2.24). All these variations should
myocardium. The artery usually takes its crosses the roof of the left atrium be taken into account when planning the
origin from the proximal segment of its (Figure 2.22). Such lateral origin is rare in safest right atrial incision, particularly
parent coronary artery (Figure 2.20). A normal hearts3,4, but more frequent in when the nodal artery crosses the lateral
significant variant is found when the artery association with congenital malformations5. margin of the right appendage, or courses
Superior caval vein Waterston’s groove
Inferior caval vein
Left
Sup. Inf.
Fig. 2.16 Waterston’s groove seen through a median sternotomy.
Right The tissue plane between the atrial chambers has been partially
Pulmonary veins
dissected.
Terminal crest Deficient oval fossa
Left
Fig. 2.17 Operative view through a median sternotomy having
Sup. Inf. opened the right atrium. The terminal crest is seen giving rise to the
pectinate muscles of the right atrial appendage. Note that, in this
Pectinate muscles patient, the floor of the oval fossa is deficient, producing an atrial
Right
septal defect.
over the roof of the left atrium. Although it muscles of the appendage from the smooth appendage and the superior rim of the oval
might seem obvious, care should be taken walls of the systemic venous sinus fossa. The crest continues through the
to ensure that the incision cuts across (Figure 2.17). The cardiomyocytes are superior interatrial groove as Bachmann’s
neither the terminal crest nor the right aligned along the long axis of the crest, bundle, the major route for conduction into
coronary artery (Figure 2.25). which is one of the major routes for the left atrium. On first sight, when
Opening the atrium through the most conduction from the sinus node towards inspecting the right atrium through this
appropriate incision shows that the the atrioventricular node. Anteriorly, the incision, there appears to be an extensive
terminal groove is the external counterpart crest curves in front of the orifice of the septal surface between the openings of the
of a prominent internal muscle bundle, the superior caval vein, with its medial caval veins and the orifice of the tricuspid
terminal crest. This separates the pectinate extension forming the border between the valve (Figure 2.26). The apparent extent of
Anterocaval course
Fig. 2.18 The cartoon, drawn in anatomical
orientation, shows the variations in the origin of
the artery to the sinus node, and the variability
Origin from right coronary artery Origin from circumflex artery
relative to the cavoatrial junction. The left-hand
panels show the usual arrangement with the
origin from the right coronary artery, found in
55% of the population, with the rare variant of a
Retrocaval course
distal origin with coursing across the appendage
(lower left-hand panel). The right-hand panels
show a proximal origin from the circumflex
artery, found in around 45% of the population,
with the rare variant of a distal origin with
coursing across the dome of the left atrium. The
middle panels show the variation relative to the
Arterial circle superior cavoatrial junction. The sinus node is
Distal origin from right coronary artery Distal origin from circumflex artery
shown in green.
Roof of left atrium
Aorta
Left
Sup. Inf.
the artery to the sinus node, which in this case originates from the
Artery to sinus node circumflex coronary artery and extends across the dome of the left
Right
atrium.
this septum is spurious6,7. The true separate the mouth of the superior caval coronary sinus and the orifice of the
septum7,8 is confined to the floor and the vein and the entrance of the pulmonary inferior caval vein (Figure 2.29). These
anteroinferior margin of the oval fossa veins to the left atrium (Figures 2.28). The muscular structures continue anteriorly
(Figures 2.27 and 2.28). The extensive posteroinferior rim is another fold, this within the atrium as the Eustachian ridge.
superior rim of the fossa is produced by the time formed by reflection of the This is seen to advantage when the floor of
folds of the interatrial groove, which musculature forming the mouth of the the oval fossa is itself deficient
Artery to sinus node
Aorta
Left
Sup. Inf.
Right
Crest of appendage
the artery to the sinus node originating proximally from the right
coronary artery.
Left
Sup. Inf. Fig. 2.21 Operative view through a median sternotomy showing
the artery to the sinus node originating distally from the right
coronary artery and coursing over the lateral surface of the right
Right
atrial appendage. The site of the sinus node is shown by the white
cross-hatched area.
Superior caval
vein
Artery to
sinus node
Dome of left atrium
Sup.
Left Right
Retrocaval course Fig. 2.22 In this specimen, seen in anatomical orientation, the
artery to the sinus node originates laterally from the circumflex
Inf. coronary artery and courses over the dome of the left atrium. The
site of the sinus node is shown by the white cross-hatched area.
Crest of appendage
Left
Sup. Inf.
Right Retrocaval nodal artery a retrocaval course of the artery to the sinus node, the site of the
node itself being emphasised by the white cross-hatched area.
(Figure 2.30). Because of the limited extent within the right atrium is the site of the structure formed by the junction of the
of these septal components, it is an easy atrioventricular node. This is contained Eustachian valve, the valve of the inferior
matter for the surgeon to pass outside the within the triangle of Koch9. This caval vein, and the Thebesian valve, the
heart when attempting to gain access to the important landmark is bounded by the valve of the coronary sinus. The fibrous
left atrium through a right atrial approach. tendon of Todaro, the attachment of the continuation of these two valvar structures
In addition to the position of the sinus septal leaflet of the tricuspid valve, and the buries itself in the anterior continuation of
node, and the extent of the atrial septum, orifice of the coronary sinus (Figure 2.31). the Eustachian ridge. It then runs medially
the other major area of surgical significance The tendon of Todaro9 is a fibrous as the tendon of Todaro before inserting
Retrocaval branch Artery to sinus node
Left
Sup. Inf.
Anterocaval branch the artery to the sinus node dividing to form an arterial circle
Right
around the cavoatrial junction.
Right pulmonary veins Systemic venous sinus
Right atrial appendage
Sup.
Post. Ant.
Fig. 2.25 The heart is shown from the right side. The terminal
Waterston’s groove Terminal groove Inf. groove and Waterston’s groove form the boundaries of the
systemic venous sinus.
into the atrioventricular part of the scrupulously avoided during surgical to that of the mitral valve (Figure 2.33).
membranous septum (Figure 2.32). The procedures, the atrioventricular The relationship between the atrial and
entire atrial component of the axis of conduction tissues will not be damaged. ventricular muscular walls within the
atrioventricular conduction tissues is Should the node need to be identified more triangle of Koch is complex. At first sight,
contained within the confines of the precisely, it should be remembered that the because of the off-setting of the
triangle of Koch. If, in hearts with normal attachment of the tricuspid valve is some attachments of the mitral and tricuspid
segmental connections, this area is way down the surface of the septum relative valves, the entire muscular area seems to
Oval fossa Sup.
Post. Ant.
Inf.
Fig. 2.26 In this specimen, viewed in the anatomical position, the

Coronary sinus white dotted circle shows the apparently extensive ‘septal surface’
within the right atrium.
Infolded posterior rim
Sup.
Fig. 2.27 The same specimen as seen in Figure 2.26 has been
Post. Ant.
transected through the oval fossa. The section shows that the rims
of the oval fossa are infoldings of the atrial walls. Note the
Inf. Infolded anterior rim relationship of the anterior fold to the aortic root, the right
coronary artery, and the artery to the sinus node.
interpose between the cavities of the right muscular septum. In the floor of the atrioventricular groove, with the fibrofatty
atrium and the left ventricle. Indeed, in triangle, however, the atrial musculature is tissue in this area insulating the atrial
earliest editions of the book, we described separated from the underlying ventricular from the ventricular muscular layers10.
this area as representing an atrioventricular myocardium by an extension of the inferior The extent of this insulating layer can be
Flap valve
Left atrium
Aortic root
Infolded superior rim
Right atrium
Post.
Fig. 2.28 This heart has been sectioned in the four-chamber
Base Apex plane, showing that the superior rim of the oval fossa is a deep
infolding producing the interatrial groove between the systemic
Ant. Anteroinferior rim venous sinus of the right atrium and the entry of the pulmonary
veins into the left atrium.
Superior infolding
Sup.
Ant.
Fig. 2.29 The heart shown in Figures 2.26 and 2.27 has been
Post. sectioned in the long axis of the venous sinus, again showing that
the superior rim of the oval fossa is an infolding of the interatrial
Inf.
Left atrium groove. The heart is viewed in anatomical orientation from the
back.
demonstrated by dissecting away the formed by the atrial layer of an of allegedly specialised pathways of
superficial atrial musculature, at the same atrioventricular muscular sandwich, rather myocardium in conducting the sinus
time revealing the location of the artery than a true muscular atrioventricular impulse to the atrioventricular node11,12.
supplying the atrioventricular node septum. Molecular biologists are currently showing
(Figure 2.34). The larger part of Koch’s Much was written in the latter part of that, during development, it is possible to
triangle as seen by the surgeon, therefore, is the twentieth century concerning the role recognise areas of the atrial myocardium on
Septal leaflet Coronary sinus
Left
Sup. Inf. Fig. 2.30 The heart has been opened through an atriotomy, and
the interior surface of the right atrium is shown in surgical
orientation. Note the Eustachian ridge separating the mouth of the
Eustachian ridge Right
coronary sinus from the orifice of the inferior caval vein. The floor
of the oval fossa is deficient, producing an atrial septal defect.
Site of membranous septum
Hinge of tricuspid valve
Sup. Left
Right Inf.
Site of tendon of Todaro Coronary sinus Fig. 2.31 This operative view through a right atriotomy shows the
location of the triangle of Koch.
the basis of their genetic lineage. that surgical operations should be specially additionally insulated from the adjacent
Subsequent to birth, however, all of the modified to avoid presumed specialised working ventricular myocardium14. There
atrial myocardium, apart from the nodal internodal tracts13. The anatomical are no such insulated and isolated tracts
components, has achieved a working paradigm of tracts of myocardium modified within the atrial walls15,16. The major
phenotype. There is no anatomical for conduction in the heart is provided by muscle bundles of the atrial chambers,
evidence, therefore, to support suggestions the ventricular conduction system, which is nonetheless, serve as preferential pathways
Tendon of Todaro Membranous septum
Hinge of
tricuspid valve
Oval fossa
Eustachian valve
Sup.
Right Left
Inf.
Fig. 2.32 The endocardium has been removed from this heart,
viewed in anatomical orientation, to demonstrate the boundaries
Thebesian valve Sub-Thebesian pouch
of the triangle of Koch. Note the non-uniform anisotropic
arrangement of the aggregated cardiomyocytes.
Sup. Floor of oval fossa
Mitral valvar attachment

Right Left
Inf.
Anteroinferior rim of oval fossa
Adipose tissue
Fig. 2.33 The heart has been sectioned in the four-chamber plane
to show the off-setting of the hinges of the tricuspid and mitral
Tricuspid valvar attachment Ventricular septum valves. Note the adipose tissue separating the atrial and ventricular
musculatures in the area of off-setting.
of conduction, with the location of these myocardium of the Eustachian ridge, and atrial myocardium interposes between the
preferential pathways dictated by the the superior interatrial fold, should be nodes, providing that the arterial supply to
overall geometry of the chambers preserved during atrial surgery. Even if the nodes, or the nodes themselves, are not
(Figure 2.35). Ideally, therefore, prominent they cannot be preserved, the surgeon can traumatised. The key to avoiding
muscle bundles, such as the terminal crest, rest assured that internodal conduction will postoperative atrial arrhythmias, therefore,
the superior rim of the oval fossa, the continue as long as some strand of viable is the fastidious preservation of the sinus
Artery to AV node
Membranous septum
Right atrium
Left atrium
Fig. 2.34 This dissection, in anatomical orientation having

removed the non-coronary sinus of the aortic valve, shows the
fibrofatty tissue (star) interposed between the atrial and
ventricular muscular layers (yellow and blue dashed lines) of the
Circumflex coronary artery atrioventricular muscular sandwich. Note the artery to the
atrioventricular (AV) node.
Oval fossa Mouth of superior caval vein
Tricuspid valve
Sup.
Ant. Fig. 2.35 The right atrium, seen in anatomical

Post. orientation, has been opened through a
window in the appendage. The muscular walls
Inferior caval vein Coronary sinus Inf. surround several orifices, producing
preferential routes of conduction (arrows).
and atrioventricular nodes and their Much is also written about the fibrous of the four cardiac chambers, but is seen
arteries, rather than concern about non- skeleton of the heart. The strongest part of most clearly by the surgeon when working
existent tracts of purportedly specialised this skeleton is the central fibrous body. from the right atrium (Figure 2.36). Rather
atrial myocardium. This area of fibrous tissue touches on three than being considered as a specific body, it
Atrioventricular membranous septum
Septal leaflet
Left
Sup. Inf.
Fig. 2.36 Operative view through a right atriotomy showing the
tendon of Todaro inserting into the atrioventricular component of
Tendon of Todaro Right the membranous septum, which forms the right margin of the
central fibrous body.
Post.
Right Left
Ant.
Oval fossa Left ventricular

outflow tract
Fig. 2.37 The heart is sectioned to replicate the

echocardiographic four-chamber plane. The section passes through
the membranous part of the septum, which is divided by the hinge
of the septal leaflet of the tricuspid valve into atrioventricular (red
Hinge of septal leaflet double-headed arrow) and interventricular (blue double-headed
arrow) components.
is better conceptualised as an area within components of the aortic root join in surgeon (Figure 2.38). The anatomical
the heart where the membranous septum, fibrous continuity (Figure 2.37). Its extent view shows how the rightward margin of
the hinges of the leaflets of the is best seen from the left side, albeit that the area of continuity between the leaflets
atrioventricular valves, and the fibrous this aspect is rarely, if ever, seen by the of the aortic and mitral valves, also known
Membranous septum Aortic-mitral continuity
Sup.
Post. Fig. 2.38 This specimen, viewed in anatomical orientation, is

opened to show the origin of the aorta from the left ventricle, with
Ant.
the membranous septum in fibrous continuity with the leaflets of
Inf. the aortic and mitral valves. Note the site of the left bundle branch,
marked by the white dots.
Interleaflet triangle Aortic-mitral continuity
Fig. 2.39 This close-up, in anatomical orientation, shows the

relationships between the membranous septum and the aortic
valve having removed the leaflets of the valve itself (black dashed
lines). The membranous septum is in continuity superiorly with the
interleaflet triangle between the right and non-coronary leaflets of
Membranous septum the aortic valve. It is also continuous with the thickened right end of
Right fibrous trigone the fibrous continuity between the leaflets of the aortic and mitral
valves, known as the right fibrous trigone.
as the right fibrous trigone, joins with the fibrous triangle between the hinges of the septal defects, and control of arrhythmias
membranous septum to form the basis of right and non-coronary leaflets of the aortic require the surgeon to understand its
the fibrous body (Figure 2.39). This view valve. The central fibrous body, therefore, anatomical relationships implicitly, from
also emphasises the intimate relationship of serves as an anatomical focal point for the both the obvious right (Figure 2.36), and
the fibrous body to many other important cardiac surgeon. Operations involving also the usually invisible left (Figure 2.38)
structures within the heart, particularly the valvar replacement or repair, closure of sides. Knowledge of the location of the
Anterosuperior leaflet Inferior leaflet
Left
Sup. Inf.
Fig. 2.40 This operative view through a right atriotomy, seen in

Septal leaflet Right surgical orientation, shows the vestibule of the right atrium and the
three leaflets of the tricuspid valve.
atrioventricular component of the possible to recognise the extensive body of much the same access can be gained by
membranous septum is also the key to the left atrium (Figure 2.41). The chamber, approaching through the right atrium, and
understanding the occurrence of the so- of course, is separated from its neighbour incising just superiorly within the fossa. It
called Gerbode septal defect17, which by the septum. The body of the atrium is must be remembered that an extensive
produces a direct shunt from the left confluent with the venous component. incision may take the surgeon out of the
ventricular outflow tract to the right atrium Unlike in the right atrium, the narrow confines of the atrial chambers and into the
(see Figure 2.37). junction between these parts and the pericardial space. Perhaps more
The final atrial component, the vestibule appendage is unmarked by either a terminal importantly, it should be noted that such an
to the right ventricle, surrounds the orifice groove or crest (Figure 2.41). Because of incision may damage the artery to the sinus
of the tricuspid valve, and is contiguous with the posterior position of the left atrium, and node, either in the interatrial groove or on
both the systemic venous component and its firm anchorage by the four pulmonary the roof of the left atrium. The left atrium
the appendage of the right atrium. The veins, it can be difficult for the surgeon to can also be entered superiorly by incising
anterior junction of these two parts of the gain direct access to the left atrium, so directly through its roof. If the aorta is
atrium overlies the peripheral attachment of knowledge of the salient anatomy can help pulled anteriorly and to the left, an extensive
the anteroseptal commissure of the tricuspid best exposure of the cavity. Probably the trough is seen between the two atrial
valve and the supraventricular crest of the most popular route is provided by an appendages (Figure 2.43), with an incision
right ventricle (Figure 2.40). The posterior incision made just posterior to the right through the floor of this trough entering the
junction is at the orifice of the coronary lateral aspect of the interatrial groove left atrium directly. When making such an
sinus, where there is usually an extensive (Figure 2.42). This groove, the extensive incision, it must again be remembered that
inferior trabeculated diverticulum. infolding between the right pulmonary the artery to the sinus node may be coursing
Although usually called the post-Eustachian veins and the venous sinus of the right through this area when it takes origin from
sinus, it is sub-Thebesian when the heart is atrium, produces the superior rim of the the circumflex artery (Figure 2.43). In other
viewed relative to the anatomical position oval fossa (Figure 2.28). A posteriorly instances, this artery may pass through the
(Figure 2.35). directed incision within this groove takes infolding of the interatrial groove to reach
the surgeon directly into the left atrium. If the terminal groove.
necessary, the incision can be extended to Once access is gained to the left atrium,
MORPHOLOGICALLY LEFT the superior aspect of the left atrium by the small size of the opening of the
ATRIUM incising the pericardial fold between the appendage is apparent (Figure 2.44). The
As with the right atrium, the left atrium superior caval vein and the right mouth of the appendage lies to the left of
possesses an appendage, an extensive pulmonary artery (Figure 2.4). Because the the mitral orifice as viewed by the surgeon.
venous component, and a vestibule. Unlike infolding of the interatrial groove also Anatomically, it is positioned above the
the situation with the right atrium, it is also forms the superior border of the oval fossa, superior end of the zone of apposition
Appendage Venous component
Body
Ant.
Sup.
Inf.
Vestibule Septum Post. Fig. 2.41 In this heart, shown in anatomical orientation, the left
atrium is opened to show its components. Note the extensive body.
Left Interatrial groove
Sup. Inf.
Right
Fig. 2.42 This operative view through a median sternotomy

Incision to left atrium shows how an incision through the bottom of Waterston’s groove
(white dotted line) takes the surgeon into the left atrium.
between the valvar leaflets. The greater septal aspect will be anterior, exhibiting the
part of the pulmonary venous sinus, typically roughened flap–valve aspect of its MORPHOLOGICALLY RIGHT
confluent with the smooth-walled atrial left side (Figure 2.46). The large sweep of VENTRICLE
body, will usually be located inferiorly, tissue between the flap valve of the septum The musculature of the right ventricle
away from the operative field. It is the and the opening of the appendage is the extends from the atrioventricular to the
vestibule of the mitral orifice that internal aspect of the deep anterior ventriculoarterial junctions.
dominates the picture (Figure 2.45). The interatrial groove. Understanding of ventricular morphology
Artery to sinus node Dome of left atrium
Aorta
Right atrial
appendage
Left
Sup. Inf. Fig. 2.43 This operative view through a median sternotomy
Right pulmonary shows how retraction of the aorta to the left reveals the deep
artery trough inferior to the right pulmonary artery and between the
Right atrial appendages. Note that, in this heart, the artery to the sinus
node courses through this trough.
Mouth of appendage
Pulmonary
venous component
Body of atrium
Sup.
Fig. 2.44 The left atrium is photographed from behind to show
the relations of the mouth of the left atrial appendage. All
Left Right
components of the atrial chamber can be seen. Note the isthmus
formed by the vestibular myocardium, and the extensive fold
Fenestrated
Vestibular myocardium oval fossa Inf. between the mouth of the appendage and the origin of the left
pulmonary veins from the pulmonary venous component (star).
in general is greatly aided by considering (Figure 2.47). The inlet portion of the right possible to distinguish three leaflets in the
the ventricles in terms of three ventricle contains, and is limited by, the valvar orifice. They lie in anterosuperior,
components17. These are the inlet, apical tricuspid valve and its tension apparatus. septal, and inferior or mural locations
trabecular, and outlet parts, respectively Although not always easy, it is generally (Figures 2.48, 2.49). When seen in the
Ant.
Aortic leaflet
Sup. Inf.
Post.
Fig. 2.45 On gaining access through a right-sided left atriotomy,

Vestibule Mural leaflet the vestibule of the mitral orifice dominates the surgical picture.
Note the location of the leaflets of the mitral valve.
Body of atrium
Atrial septum
Sup.

Ant. Post.
Fig. 2.46 The left atrium is windowed from the left and posterior
Inf. Mitral valvar vestibule aspect, showing its component parts. Note the probe in the patent
foramen.
closed position, their boundaries are clearly not abut directly on the valvar annulus. tricuspid valve is the direct attachments to
marked by their zones of apposition, which The zones of apposition between the the septum of the cords tethering its septal
extend to the centre of the valvar orifice. leaflets in these commissural areas are leaflet (Figure 2.49).
The entirety of the zones of apposition usually tethered by fan-shaped cords The trabecular component of the right
could, logically, be defined as the arising atop prominent papillary muscles. ventricle extends to the apex, where its wall
commissures. It is the peripheral parts of We discuss the details of this valvar and is particularly thin, being especially
the zones of apposition that are usually commissural anatomy in our next chapter. vulnerable to perforation by cardiac
described as the commissures18, albeit that In anatomical terms, the most constant catheters and pacemaker electrodes. When
in the atrioventricular valves these areas do distinguishing morphological feature of the compared to those of the left ventricle, the
Supraventricular crest Septomarginal

trabeculation
Outlet
Inlet
Apical
Base
trabecular
component
Right Left
Apex Fig. 2.47 This specimen, viewed in anatomical orientation, shows

the three components of the morphologically right ventricle.
Medial papillary muscle Sup.
Right Left
Anterosuperior Inf.
leaflet
Septal leaflet
Tricuspid
valve
Fig. 2.48 The right atrioventricular junction is photographed

Inferior leaflet from the ventricular apex, showing the location of the leaflets of
the tricuspid valve.
right ventricular apical trabeculations are of the valve are attached to the infundibular muscle in the bases of the pulmonary valvar
uniformly coarse. The outlet component of musculature in semilunar fashion. These sinuses (Figure 2.50). Another
the right ventricle is a complete muscular hinges cross the circular anatomical distinguishing morphological feature of
sleeve, the infundibulum, which extends ventriculoarterial junction so as to the right ventricle is the prominent
from the ventricular base to support the incorporate triangles of arterial wall within muscular shelf that interposes between the
leaflets of the pulmonary valve. The leaflets the ventricle, and crescents of ventricular tricuspid and pulmonary valvar orifices.
Anterosuperior leaflet Left
Medial papillary muscle Sup. Inf.
Right
Fig. 2.49 Operative view through a right atriotomy showing the

Septal leaflet Direct septal attachments direct cordal attachments to the septum of the septal leaflet of the
tricuspid valve.
Anatomical ventriculoarterial junction

Sup.
Right Left
Inf.
Fig. 2.50 In this heart, shown in anatomical orientation, the

leaflets of the pulmonary valve have been removed. Note how the
semilunar attachment of the pulmonary valvar leaflets crosses the
anatomical ventriculoarterial junction, the dotted lines and cross-
hatchings emphasising the crescents of ventricular muscle
Haemodynamic ventriculoarterial junction incorporated in the valvar sinus and the triangles of arterial wall
within the ventricle.
This is the supraventricular crest. At first the most medial part of the crest can be (Figure 2.53). It is the presence of this
sight (Figure 2.51), it has the appearance removed so as to create a hole between the sleeve that permits the valve itself to be
of a thick muscle bundle, but its larger part subpulmonary and subaortic outflow removed as an autograft for use in the Ross
is no more than the infolded inner heart tracts (Figure 2.52). This part is a true procedure (Figures 2.54, 2.55).
curve (Figure 2.52). Incisions, or deep muscular outlet septum, but cannot be Considered as a whole, the
sutures, through this part run into the distinguished from the remainder of the supraventricular crest inserts between the
transverse sinus and right atrioventricular crest by gross inspection (Figure 2.51). limbs of an equally prominent and
groove, and can jeopardise the right The crest continues distally as the free- important right ventricular septal
coronary artery19. Only a small portion of standing infundular muscular sleeve trabeculation. This structure, described
Supraventricular crest Septoparietal

trabeculations
Medial papillary muscle
Sup.
Left
Fig. 2.51 In this anatomical specimen, viewed in surgical
Right orientation, the prominent muscular supraventricular crest is seen
between the leaflets of the tricuspid and pulmonary valves. Note
Inf. Septomarginal trabeculation the medial papillary muscle arising from the septomarginal
trabeculation.
Sup. Septomarginal
trabeculation
Right Left
Pulmonary valve
Inf.
Right
coronary
artery
Fig. 2.52 Dissection of the specimen shown in Figure 2.43 shows

here that most of the supraventricular crest is the inner curvature of
the heart, or the ventriculoinfundibular fold. Note the location of
Ventriculoinfundibular fold Resected outlet septum the right coronary artery. The site occupied by the outlet septum
has been resected.
by us as the septomarginal trabeculation, limb runs up to the attachment of the and reinforcing the muscular septum. The
but also known as the septal band, has leaflets of the pulmonary valve, while the medial papillary muscle usually arises
anterosuperior and posteroinferior limbs posteroinferior limb extends backwards, from this posteroinferior limb. The body
that clasp the septal attachment of the extending inferior to the interventricular of the septomarginal trabeculation itself
crest (Figure 2.56). The anterosuperior component of the membranous septum, extends towards the apex of the ventricle,
Free-standing infundibulum
Pulmonary valve
Aortic root
Sup.
Right Left
Septomarginal Fig. 2.53 Further dissection of the heart shown in Figures 2.51
Medial papillary muscle trabeculation and 2.52 reveals the free-standing sleeve of infundibular muscle
Inf.
supporting the leaflets of the pulmonary valve.
First septal perforator Anterior interventricular artery

Aortic valve
Left
Post. Ant.
Fig. 2.54 In this heart, the pulmonary trunk

Right
Infundibular sleeve has been turned forwards to show the sleeve of
free-standing infundibular musculature.
breaking up into a sheath of smaller prominent. One becomes the anterior arise from the anterior margin of the
trabeculations. Some of these mingle into papillary muscle, while another extends septomarginal trabeculation. Variable in
the apical trabecular portion, and some from the septomarginal trabeculation to number, these are the septoparietal
support the tension apparatus of the the anterior papillary muscle, being trabeculations (Figure 2.56).
tricuspid valve. Several of these termed the moderator band. Additional It is the uniformly coarse apical
trabeculations are often particularly significant right ventricular trabeculations trabeculations that, for the morphologist,
Left coronary artery First septal perforator
Aortic valve
Left
Sup. Inf. Fig. 2.55 In the heart shown in Figure 2.54, again viewed from
above and from the right, the pulmonary infundibulum has been
Right ventricular
removed from the base of the heart, revealing the free-standing
infundibular sleeve
Right myocardial sleeve that ‘lifts’ the valvar leaflets away from the left
ventricle. Note the site of the first septal perforating artery.
Ventriculoinfundibular fold
Septomarginal
trabeculation
Sup.
Left
Right
Fig. 2.56 The right ventricle has been opened from the front,
Inf. with the heart orientated anatomically, to show the septoparietal
Anterior papillary muscle trabeculations (stars). The most inferior of the trabeculations is the
moderator band.
Left Ant.
Coarse right
ventricular
Post. Right trabeculations
Fig. 2.57 The apical component of the ventricular mass has been
Fine left ventricular trabeculations transected, and is photographed from above to show the different
patterns of the apical trabeculations of the two ventricles.
Sup.
Ant. Post.
Inf.
Outlet
Inlet
Fig. 2.58 In this specimen, viewed from the side in anatomical

Apical trabecular component orientation, the morphologically left ventricle is opened in
clam-shell-like fashion to show its three components.
serve as the most constant anatomical tracts. These features, however, can be
feature of the morphologically right altered or lacking in the congenitally MORPHOLOGICALLY LEFT
ventricle. Taken overall, there are a abnormal heart. In final arbitration of VENTRICLE
number of morphological differences ventricular morphology, therefore, the As with the right ventricle, the musculature
between the ventricles that permit their morphologist relies on the contrast, in the of the left ventricle extends from the
distinction. These include the arrangement same heart, between the coarse atrioventricular to the ventriculoarterial
of the leaflets of the atrioventricular valves trabeculations of the right ventricle, and junctions. It is again conveniently
and their tension apparatus, the ventricular the much finer trabeculations seen in the considered in terms of inlet, apical
shape, the thickness of the ventricular apical part of the left ventricle trabecular, and outlet components
walls, and the configuration of the outflow (Figure 2.57). (Figure 2.58). In the left ventricle, there is
Base
Left Right
Apex
Aortic leaflet
Mural leaflet
Fig. 2.59 This specimen, viewed from behind, and opened

through a cut in the left atrioventricular junction adjacent to the
Tendinous cords septum, demonstrates the marked differences in the arrangement
Papillary muscles
of the aortic and mural leaflets of the mitral valve.
Sup. Posterosuperior muscle
Apex Base
Inf.
Fig. 2.60 This specimen, viewed from behind after the parietal
wall of the left ventricle has been removed, is dissected to show the
Anteroinferior muscle adjacency of the anteroinferior and posterosuperior papillary
muscles of the mitral valve.
marked overlapping of the inlet and outlet atrioventricular junction. Positioned positioned on its apex20. Unlike the
components. The inlet component posteroinferiorly, it is accurately termed tricuspid valve, the leaflets of the mitral
surrounds, and is limited by, the mitral the mural leaflet. Because the aortic leaflet valve have no direct septal attachments,
valve and its tension apparatus. Its two of the mitral valve forms part of the outlet because the deep posteroinferior
leaflets, supported by two prominent of the left ventricle, the boundary between diverticulum of the subaortic outflow tract
papillary muscles and their tendinous the inlet and outlet is somewhat blurred. displaces the aortic leaflet of the valve away
cords, have widely differing appearances The papillary muscles of the valve, located from the muscular ventricular septum
(Figure 2.59). The anterosuperior leaflet is in anteroinferior and posterosuperior (Figure 2.61). The trabecular component
short, squat, and relatively square. This positions, are close to each other at their of the left ventricle extends to the
leaflet, in fibrous continuity with the aortic origin (Figure 2.60). It is incorrect to ventricular apex and has characteristically
valve, is best termed the aortic leaflet. The describe the muscles as being anteroseptal fine trabeculations (Figure 2.57). As in the
other leaflet is narrower, and its junctional and posterolateral, these terms reflecting right ventricle, the myocardium is
attachment more extensive, being the bad habit of morphologists of removing surprisingly thin at the apex. This feature is
supported by the parietal part of the left the heart from the body and describing it as important to the cardiac surgeon, who may
Sup.
Aorta
Post. Ant.
Inf.
Mitral valve
Right atrium
Fig. 2.61 This heart, seen from above and from the right in
anatomical orientation, has been prepared by removing the atrial
musculature and the non-coronary sinus of the aortic valve to
Inferior extension
illustrate the inferior extension of the subaortic outflow tract
separating the mitral valve from the septum.
have reason to place catheters and landmark of its descent is the membranous of opening of the free edge of the leaflets of
electrodes in the right ventricle, or drainage septum immediately beneath the zone of the aortic valve (Figure 2.64). The
tubes in the left side. Immediate apposition between right coronary and ascending aortic trunk runs its short
perforation, or delayed rupture, may occur. non-coronary leaflets of the aortic valve course, passing superiorly, and obliquely to
This may be a particular problem should (Figure 2.62). The bundle descends, the right, and slightly forwards, towards
catheters stiffened by hypothermia be initially, as a relatively narrow solitary the sternum. It is contained within the
pushed against the apical endocardium as fascicle, but soon divides into three fibrous pericardial sac, so its surface is
the heart is manipulated during surgery on interconnected fascicles that radiate into covered with serous pericardium. Its
the coronary arteries21. anterior, septal, and posterior divisions. anterior surface abuts directly on the
The outlet component of the left The interconnecting radiations do not fan pulmonary trunk, which is also covered
ventricle supports the aortic valve. Unlike out to any degree until the bundle itself has with serous pericardium. The two vessels
its right ventricular counterpart, it is not a descended to between one-third and one- together make up the vascular pedicle of
complete muscular structure. The septal half the length of the septum. As with the the heart (Figure 2.65). The ascending
wall is composed largely of muscle, but in pulmonary valve, the leaflets of the aortic aorta is related anteromedially to the right
this area the membranous septum is found, valve are not attached in circular fashion to atrial appendage, and posterolaterally to the
forming part of the central fibrous body in a supporting ring of fibrocollagenous right ventricular outflow tract and the
the subaortic outflow tract (Figure 2.39). tissue. Instead, the leaflets are attached in pulmonary trunk. Extrapericardially, the
The posterolateral portion of the outflow semilunar fashion, with the hinge lines thymus gland lies between it and the
tract is composed exclusively of fibrous crossing the anatomical ventriculoaortic sternum. The medial wall of the right
tissue, namely the fibrous curtain joining junction (Figure 2.63). This arrangement atrium, the superior caval vein, and the
the leaflets of the aortic valve to the aortic again means that crescents of ventricular right pleura relate to its right side. On the
leaflet of the mitral valve. The left lateral muscle are incorporated into the bases of left, its principal relationship is with the
quadrant, continuing around to the two of the aortic sinuses, while three pulmonary trunk. Posterior to the
septum, is a muscular structure triangles of fibrous tissue are created ascending aorta lies the transverse sinus of
representing the lateral margin of the inner beneath the sinutubular junction22. the pericardium (Figure 2.3), which
heart curvature, and separating the cavity separates it from the roof of the left atrium
of the left ventricle from the transverse and the right pulmonary artery. The arch
sinus. The muscular septal surface of the THE AORTA of the aorta begins at the superior
outflow tract is characteristically smooth, The ascending aorta begins at the distal attachment of the pericardial reflection
with the fan-like left bundle branch extremity of the three aortic sinuses, the just proximal to the origin of the
cascading down from the septal crest. The sinutubular junction, which lies at the line brachiocephalic artery. It continues
Right coronary leaflet Non-coronary leaflet Sup.
Right Left
Inf.
Mitral valve
Fig. 2.62 This specimen, orientated in the anatomical position,

demonstrates the relationship of the zone of apposition between
the right and non-coronary leaflets to the membranous septum
Interleaflet triangle Membranous septum and the interleaflet triangle between the right coronary and non-
coronary leaflets of the aortic valve.
Anatomic ventriculoarterial junction
Ant.
Base
Fig. 2.63 This dissection demonstrates that, as in the pulmonary
Apex
valve (see Figure 2.50), the attachments of the leaflets of the aortic
valve (black dotted line) cross the anatomical ventriculoarterial
Post.
junction (black dashed line), incorporating crescents of ventricular
Haemodynamic ventriculoarterial junction tissue into the arterial valvar sinuses, and triangles of arterial tissue
into the ventricular base.
superiorly for a short distance, before if not carefully identified in the presence The descending or thoracic aorta
coursing posteriorly and to the left, of aortic coarctation. The left phrenic and continues from the arch, running an initial
crossing the lateral aspect of the distal vagus nerves run over the anterolateral course lateral to the vertebral bodies, and
trachea, and finally terminating on the aspect of the arch just beneath the reaching an anterior position at its
lateral aspect of the vertebral column. mediastinal pleura. The left recurrent termination. It gives off many branches to
Here, it is tethered by the parietal pleura laryngeal nerve takes origin from the the organs of the thorax throughout its
and the arterial ligament. During its vagus. It curls superiorly around the course, as well as the prominent lower nine
course, it gives off the brachiocephalic, arterial ligament before passing on to the pairs of intercostal arteries. These latter
the left common carotid, and the left posteromedial side of the arch. Here, the vessels are of critical concern for the cardiac
subclavian arteries (Figure 2.66). arch relates to the tracheal bifurcation and surgeon (see Chapter 9). In coarctation of
Bronchial arteries arise from the oesophagus on its medial border, but also the aorta, they serve as primary collateral
underside of the arch (Figure 2.67). to the left main bronchus and the left vessels to bypass the obstructed aorta,
They can be particularly troublesome pulmonary artery inferiorly. accounting for the rib notching seen in
Sinutubular junction
Ascending aorta
Aortic sinus
Transverse sinus
Left atrium
Left ventricle
Ant.
Base
Apex Fig. 2.64 This long axis section shows the
sinutubular junction at the line of peripheral
Post. attachment of the zones of apposition
between the leaflets of the aortic valve.
Detached pericardial reflection Left
Sup. Inf.
Right
Pulmonary trunk

shows the vascular pedicle leading to the beginning of the aortic
arch, which gives rise to the brachiocephalic and left common
carotid arteries just distal to the detached pericardial reflection
Ascending aorta (white dotted line). The origin of the left subclavian artery is
not seen.
older children with this lesion. These Because it is difficult to predict exactly the they serve as a source of pulmonary
vessels, and their branches to the chest site of origin of these vital branches, the vascular supply.
wall, can be a source of troublesome surgeon must make every attempt to
bleeding if not properly secured when protect their origin from permanent
THE PULMONARY ARTERIES
operating on such patients. The surgeon occlusion. The important bronchial arteries
must also remember that the dorsal (Figure 2.67) also arise from the The pulmonary trunk is a short vessel,
branches of the intercostal vessels descending segment of the thoracic usually less than five centimetres in length
contribute a spinal branch that is important aorta. These vessels can become dilated in in the adult (Figure 2.68). It is completely
in supplying blood to the spinal cord. the presence of pulmonary atresia, when contained within the pericardium
Brachiocephalic arteries Ant.
Right Left
Post.
Right pulmonary artery
Pulmonary trunk
Aorta
Left pulmonary artery Fig. 2.66 The arterial trunks have been removed from the base of
the heart, and photographed from the apical aspect, showing their
patterns of branching.
Arterial ligament
Bronchial artery
Aortic arch
Left Trachea
Sup. Inf.
Right pulm. artery Fig. 2.67 This dissection, viewed from the right side in surgical
Right orientation, shows a bronchial artery arising from the aorta in the
midline and dividing to supply both bronchuses. Pulm., pulmonary.
(Figure 2.69) and, similar to its running vascular pedicle. It takes origin from the the pulmonary trunk overlies the aorta and
mate, the ascending aorta, it is covered with most anterior aspect of the heart, lying just left coronary artery, but it soon moves to a
a layer of serous pericardium except where behind the lateral edge of the sternum and side-by-side relationship with the
the two vessels abut each other in the the second left intercostal space. Initially, ascending aorta. The left coronary artery
Pulmonary trunk Infundibulum
Left
Sup. Inf.
Fig. 2.68 This operative view, taken through a median

Right
sternotomy, shows the short course of the pulmonary trunk prior to
its bifurcation.
Pulmonary trunk Pericardial reflection
Ligament of Marshall
Sup.
Ant. Post.
Fig. 2.69 The anatomical specimen is shown from the left side,
Infundibulum Inf. illustrating the short intrapericardial course of the pulmonary
trunk. Note the location of the ligament of Marshall.
turns abruptly anteriorly to lie between the descending aorta and the left main-stem caval vein, the ligament of Marshall
left atrial appendage and the pulmonary bronchus before it sends branches to the (Figure 2.69). The right pulmonary artery
trunk. The arterial ligament extends from hilum of the lung. Posteroinferiorly, the is somewhat longer than the left, having to
the aorta to the very end of the pulmonary left pulmonary artery is connected to the traverse the mediastinum beneath the
trunk as the latter divides into left and right left superior pulmonary vein by a fold of aortic arch, and then behind the superior
pulmonary arteries. The left pulmonary serous pericardium that contains a caval vein, to reach the hilum of the lung
artery then courses laterally in front of the ligamentous remnant of the left superior (Figure 2.70). It lies in a posteroinferior
Superior Right pulmonary Left pulmonary

caval vein artery artery
Aorta
Post.
Right Left
Fig. 2.70 The heart has been sectioned in the short axis and
Ant. Pulmonary trunk Pulmonary valve photographed from above, showing the branching pattern of the
pulmonary trunk.
position relative to the azygos vein, and is 6. Sweeney LJ, Rosenquist GC. The normal J Cardiovasc Electrophysiol 2001; 12:
anterior to the left main bronchus. The anatomy of the atrial septum in the human 1265–1268.
right pulmonary artery often branches heart. Am Heart J 1979; 98: 194–199. 15. Janse MJ, Anderson RH. Internodal atrial
before reaching the lateral wall of the 7. Anderson RH, Webb S, Brown NA. specialised pathways – fact or fiction? Eur
Clinical anatomy of the atrial septum with J Cardiol 1974; 2: 117–137.
superior caval vein posterior to the
reference to its developmental components. 16. Anderson RH, Ho SY, Smith A, Becker
transverse sinus of the pericardium. In
Clin Anat 1999; 12: 362–374. AE. The internodal atrial myocardium.
this situation, a large upper lobar branch Anat Rec 1981; 201: 75–82.
8. Anderson RH, Brown NA. The anatomy of
may be mistaken for the right pulmonary 17. Gerbode F, Hultgren H, Melrose D,
the heart revisited. Anat Rec 1996; 246: 1–7.
artery itself. Osborn J. Syndrome of left ventricular–
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tendon of Todaro as a marker for the right atrial shunt. Ann Surg 1958; 148:
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10. Anderson RH, Ho SY, Becker AE. Anatomy
location and vascular supply of the sinus arterial valves? J Heart Valve Dis 2010; 19:
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11. James TN. The connecting pathways Ochsner JL. Iatrogenic right ventricular
2. James TN. Anatomy of the Coronary
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An Anatomical Atlas for Clinical Diagnosis,
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Davies MJ. The surgical significance of the RH, McGoon DC. Apparent interruption Delayed left ventricular rupture secondary
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5. Barra Rossi M, Ho SY, Anderson RH, Rossi great arteries. Am J Cardiol 1972; 30: 22. Sutton JP 3rd, Ho SY, Anderson RH.
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42: 573–577. responsible for atrioventricular conduction. 419–427.
Surgical anatomy
3
of the valves of
the heart
It is axiomatic that a thorough knowledge of valvar complex are the leaflets, their
valvar anatomy is a prerequisite for THE VALVAR COMPLEXES supporting arterial sinuses, and the
successful surgery, be it valvar replacement When considering the valves, we fibrous interleaflet triangles. In the right
or reconstruction. The surgeon will also distinguish between the atrioventricular ventricle, the arterial sinuses are
require a firm understanding of the valves, which guard the atrioventricular supported at the ventricular base
arrangement of other aspects of cardiac junctions, and the arterial valves, which exclusively by infundibular myocardium.
anatomy to ensure safe access to a diseased guard the ventriculoarterial junctions The junction between the myocardial
valve or valves. These features were (Figure 3.1). The atrioventricular valves walls and the fibroelastic walls of the
described in the previous chapter. are best analysed in terms of the valvar sinuses, the anatomical ventriculoarterial
Knowledge of the surgical anatomy of the complex, made up of the annulus, the junction, forms one of the two true
valves themselves, however, must be leaflets, the tendinous cords, the papillary anatomical rings to be found within the
founded on appreciation of their muscles, and the supporting ventricular pulmonary root (Figure 3.4). The other
component parts, the relationships of the musculature (Figure 3.2). All of these obvious anatomical ring is the sinutubular
individual valves to each other, and their components must work in harmony so as junction. In the left ventricle, only the two
relationships to the chambers and arterial to achieve valvar competence1. The coronary arterial valvar sinuses are
trunks within which they reside. This leaflets of the atrioventricular valves are supported by ventricular musculature,
requires understanding of, firstly, the basic supplied with a complex tension with the sinus and leaflet of the non-
orientation of the cardiac valves, apparatus, as they must withstand the full coronary aortic sinus being contiguous
emphasising the intrinsic features that force of ventricular systole, so as to retain with one of the leaflets of the mitral valve
make each valve distinct from the others. their competence when in their closed (Figure 3.5). It is still possible,
This information must be supplemented by position. The arterial valves are also a nonetheless, to recognise the ring formed
attention to their relationships with other combination of complex anatomical parts. at the junction between the ventricular
structures that the surgeon must avoid, Often named the semilunar valves, it is supporting structures and the fibroelastic
notably the conduction tissues and the the leaflets that are semilunar, being walls of the sinuses, and to recognise the
major channels of the coronary circulation. hinged within the overall valvar complex, fibrous interleaflet triangles extending
For this chapter, throughout our narrative which extends from the proximal distally to the level of the sinutubular
we will presume the presence of a normally ventriculoarterial orifice, a virtual ring junction. As in the pulmonary root, the
structured heart, lying in its usual position, constructed at the nadir of the hinges of sinutubular junction is the other obvious
and without any coexisting congenital the leaflets, to the sinutubular junction anatomical ring to be found within the
cardiac malformations. (Figure 3.3). The components of the aortic root (Figures 3.4, 3.5). In both of
Sup.
Arterial valve
Apex Base
Aorta
Inf.
Left ventricle Left atrium
Fig. 3.1 The heart has been sectioned in the

long axis plane, and orientated so as to
replicate the long axis parasternal section as
obtained by echocardiographers. The cut
Atrioventricular valve clearly shows how, in the left heart, the mitral
valve guards the atrioventricular junction, and
the aortic valve the ventriculoarterial junction.
Surgical anatomy: valves of the heart 53
Sup.
Apex Base
Valvar
leaflets
Inf.
Tendinous
cords
Papillary
muscles
Attachments Fig. 3.2 The close-up view of the mitral valve,

at annulus taken from the long axis section illustrated in
Ventricular musculature Figure 3.1, shows the components of the
atrioventricular valvar complex.
Valvar
sinuses
Arterial
Valvar root
leaflets
Virtual proximal ring Sup.

Fig. 3.3 A comparable close-up of the aortic valve from Figure 3.1
Apex Base
shows the components of the arterial valvar complex, which
Inf. extends from the virtual plane at the nadir of the attachments of
the leaflets (white dashed line) to the sinutubular junction.
the roots, the distal margins of the When comparisons are made between that the leaflets of the atrioventricular
semilunar hinges of the leaflets are the overall arrangement of the valves are suspended in true annular
attached at the sinutubular junctions atrioventricular and arterial valvar fashion at the atrioventricular junctions
(Figures 3.4, 3.5). complexes, one fundamental difference is (Figure 3.2), whereas in the arterial valves,
Circular anatomical ventriculoarterial junction
Distal attachments at sinutubular junction
Fig. 3.4 The ventriculoarterial junction of the

right ventricle has been cut anteriorly, and
opened out. The valvar leaflets have been
removed, their remnants revealing their initial
semilunar attachments. Note how these hinges
cross the circular anatomical junction between
the muscular infundibulum and the walls of the
pulmonary valvar sinuses (white dashed line).
All the leaflets are supported by muscle, which
therefore forms the basal part of each of the
Proximal attachments to infundibulum Semilunar hinges sinuses of Valsalva. The distal attachments,
however, are at the sinutubular junction.
Continuity with mitral valve
Fig. 3.5 The ventriculoarterial junction of the left ventricle has

Proximal muscular attachments been opened and splayed in comparable fashion to that in
Figure 3.4, and again the leaflets of the aortic valve have been
removed, revealing the semilunar attachments. In the left ventricle,
because of the fibrous continuity with the aortic leaflet of the
mitral valve, only two of the aortic valvar leaflets have muscular
Circular anatomic ventriculoarterial junction support. The semilunar hinges, however, still cross the circular
anatomical ventriculoarterial junction.
the leaflets are hinged in semilunar fashion, different ways to describe the components should be considered as cusps. Our
with the attachments crossing the of the various valves has led to much of the preference is to achieve uniformity by
anatomical ventriculoarterial junctions confusion surrounding their specific describing these working parts in both the
(Figures 3.4, 3.5). It is these differences anatomy, as was evidenced in a recent atrioventricular and arterial valves as the
that underscore the problems currently questionnaire answered by cardiac leaflets2. As we have already discussed, it is
existing in the use of the word ‘annulus’ surgeons working in worldwide centres3. the individual leaflets of the arterial valves
when describing the morphology of the Thus, some consider that, while the that are semilunar, reflecting the shape of
cardiac valves2. Indeed, the fashion in atrioventricular valves possess leaflets, the the attachments of the hinges. The overall
which the same words have been used in moving components of the arterial valves anatomical complexes guarding the
Fig. 3.6 The aortic valve has been photographed from above,
with the leaflets in their closed positions. As can be seen, the zones
of apposition between the leaflets (red double-headed arrows)
extend from the peripheral sinutubular junction to the valvar
centroid (white circle). In anatomical terms, these zones are the
commissures between the leaflets. Conventionally, however, it is
the peripheral attachments (stars) that are usually defined as the
commissures.
ventriculoarterial junctions, therefore, are used anatomically, a commissure describes the four cardiac valves, are best appreciated
best considered as being arterial rather than a line of junction between adjacent by examining the short axis cylindrical
semilunar. With regard to the leaflets, structures, as in the eyelids or lips. These section of the heart (Figure 3.8). The
nonetheless, it follows that, because of their structures, with two moving parts, have a leaflets of three of the valves are in fibrous
semilunar shape, they are supported in solitary commissure, which has two ends. continuity with one another within this
crown-like fashion within the arterial roots. An alternative definition for ‘commissure’, cylinder, although descriptions of a
There is often a degree of fibrous however, is the end-point of a zone of fibrous skeleton providing support for the
thickening that reinforces these apposition2. This is the fashion in which leaflets within the atrioventricular
attachments, the fibrous thickenings being ‘commissure’ has been used traditionally junctions are grossly exaggerated. The
more obvious in the aortic than the when describing the cardiac valves, and arterial sinuses of the pulmonary valve are
pulmonary valve (compare Figures 3.4 and such usage is unlikely to change3. But, in supported exclusively by ventricular
3.5), but becoming more marked in the addition to taking note of the ends of the muscle, specifically by the free-standing
pulmonary valve with ageing. When zones of apposition, it is also important to right ventricular infundibulum
considered as a unit, the semilunar acknowledge the structure of the zones of (Figures 3.3, 3.9). More distally, the valvar
attachments are not strictly circular, and apposition themselves, which extend from leaflets are hinged from the arterial wall
hence not annular. The remnants of the the commissures to the valvar centroid4. rather than the infundibular muscle, with
leaflets are often described during surgical There are three such zones of apposition to the distal attachments being at the
procedures as representing the valvar be found in the arterial valves (Figure 3.6), sinutubular junction, where they form the
‘annulus’3. Echocardiographers, in and also in the tricuspid valve. In the mitral commissures, a similar arrangement
contrast, measure the virtual ring marking valve, in contrast, there is a solitary zone pertaining for the aortic valve
the entrance to the valvar complex as the of apposition (Figure 3.7). (Figure 3.10). When considered in
annulus, and report this dimension to the attitudinally appropriate fashion, the
surgeon2. This discrepancy must harbour pulmonary valve is the most anterior and
the potential for confusion4. Consensus is superior valve, the tricuspid valve is the
POSITION AND SUPPORT OF THE
now growing, therefore, that surgeons most inferior, with the mitral valve being
VALVES WITHIN THE HEART
should also consider this virtual ring as the most posterior of the four. The aortic
representing the valvar annulus2–4. It In the left heart, the leaflets of the valve is centrally located relative to its
would be optimal if the entrance to the atrioventricular and arterial valves are in neighbours, and is rightwards and
roots was simply described as the valvar close proximity to each other within the posterior to the pulmonary valve, even
diameter, but this might be too much to atrioventricular and ventriculoarterial though it guards the exit of the left
hope for. junctions (Figure 3.1), but their hinges are ventricle.
Further potential difficulties arise with well separated within the right heart. These The concept of an extensive fibrous
the use of the word ‘commissure’. When differences, and the overall arrangement of skeleton supporting the leaflets of all four
Aortic leaflet
Fig. 3.7 The mitral valve has been

photographed in its closed position, showing
the view obtained from the left atrium. There is
a solitary zone of apposition (white dashed
Mural leaflet
line) between the aortic and mural leaflets, but
conventionally it is the ends of this zone of
apposition (white circles) that are defined as
the valvar commissures. (The photograph was
taken by Dr Van S. Galstyan, from Armenia, and
we thank him for permitting us to use it in
our book.)
Aortic valve Pulmonary valve
Mitral valve
Sup.
Post. Ant.
Inf. Fig. 3.8 This superior view of a cylindrical section of the heart
Tricuspid valve through the atrioventricular and ventriculoarterial junctions shows
the relationship of all four cardiac valves.
valves within the muscular cylinder relationships of the aortic leaflet of the thickened at both its ends, serving to
forming the base of the ventricular mass, mitral valve that underscore the presence of anchor the aortic-mitral valvar unit to the
appealing as it may be, is far from reality. the greatest fibrous support within the ventricular musculature across the short
The pulmonary valvar leaflets have no cardiac base. This leaflet of the mitral valve axis of the left ventricle (Figure 3.11).
direct relationship to the leaflets of the is in direct fibrous continuity with two of These thickenings are called the right and
other valves (Figures 3.9, 3.11). The the leaflets of the aortic valve, with the left fibrous trigones, respectively. The
leaflets of the two atrioventricular valves continuity producing the aortic-mitral right fibrous trigone is itself continuous
have limited fibrous support within the curtain which forms the roof of the left with the membranous septum, with the
atrioventricular junctions, but it is the ventricle. The area of fibrous continuity is conjunction of these two fibrous
Infundibulum
Pulmonary trunk
Aorta
Fig. 3.9 The cut across the heart to replicate the oblique
subcostal echocardiographic section shows the muscular
infundibular sleeve of the right ventricle supporting the leaflets
and sinuses of the pulmonary valve. (See also Figure 3.3.)
Base
Right Left Left fibrous trigone
Apex
Fig. 3.10 The aortic root has been opened to

show the area of the central fibrous body as
seen from the left ventricle in anatomical
orientation. The body is made up of the areas
of continuity between the membranous
septum and the right fibrous trigone. Note that
Interleaflet triangle the fibrous area then extends distally to the
Fibrous continuity level of the sinutubular junction, forming a
triangle between the superior parts of the
Membranous septum Right fibrous trigone semilunar attachments of the right coronary
and non-coronary leaflets of the aortic valve.
components forming the strongest part of membranous septum continues cranially one of the interleaflet fibrous triangles
the fibrous support. The conjoined to the level of the sinutubular junction. (Figure 3.12).
structure is called the central fibrous body. This wall of the aortic root, interposing Included in the central fibrous body,
The fibrous area formed by the between two of the valvar sinuses, is therefore, is this interleaflet fibrous
Fibrous
continuity
Pulmonary
trunk
Infundibulum
Fig. 3.11 To produce this picture, the heart has been dissected to
parallel the parasternal long axis echocardiographic plane
(compare with Figure 3.1). The arterial roots, however, have been
Base left intact in this specimen, as has the mitral valve, albeit with the
arterial roots cleaned to show the free-standing sleeve of
subpulmonary infundibular musculature that lifts the leaflets of
Ant. Post.
Left fibrous trigone the pulmonary valve away from the base of the ventricular mass.
Note the area of fibrous continuity between the leaflets of the
Apex aortic and mitral valves forming part of the roof of the left
Right fibrous trigone ventricle. The ends are thickened to form the right and left fibrous
trigones.
Removed triangle
* *
*
**
Right atrium
Fig. 3.12 To produce this image, the interleaflet triangle

between the right and non-coronary aortic leaflets has been
removed, and the heart has been photographed from the right
Ant. side. The base of the triangle is formed by the interventricular
Base
component of the membranous septum. The white asterisks show
the cut edge of the ventriculoinfundibular fold, which forms the
greater part of the supraventricular crest. The most apical part of
Post. Apex the removed triangle, however, encroaches on the transverse sinus,
showing that this area was initially part of the parietal wall of the
left ventricle.
triangle, which fills the gap between two of plane that runs between the aortic root and pierces the strongest part of the fibrous
the semilunar hinges of the leaflets of the the free-standing right ventricular skeleton, specifically through the
aortic valve, in this instance the space infundibulum (Figure 3.15). Taken overall, atrioventricular component of the
between the non-coronary and right therefore, the fibrous components of the membranous septum. This point is
coronary leaflets (Figure 3.10). The aortic root form a three-pronged crown conveniently marked for the surgeon by the
location of the fibrous triangle can best be extending to the level of the sinutubular attachment to the central fibrous body of
perceived from the right side, the fibrous junction (Figure 3.16). The fibrous tissue another fibrous structure, the tendon of
tissue forming its floor having been forming the crown-like aortic root Todaro. This fibrous cord extends through
removed (Figure 3.12). The triangle is seen continues into the left atrioventricular the muscle of the right atrial wall, attaching
to extend superiorly to the level of the junction, where it supports the hinge of the to the right-sided aspect of the
aortic sinutubular junction, thus separating mural leaflet of the mitral valve membranous septum, and forming the
the most distal part of the left ventricular (Figure 3.17). The extent of this support, atrial boundary of the triangle of Koch
cavity from the right side of the transverse however, varies markedly from heart to (Figure 3.20).
sinus. heart6. It is rare to find a complete cord of It follows from the above discussion that
Similar small triangles of fibrous tissue fibrous tissue (Figure 3.18) encircling the it is incorrect to speak of either an aortic or
form the most distal parts of the spaces mitral valvar orifice. In many places the a pulmonary valvar ‘ring’ in the sense of a
between the other leaflets of the aortic hinge of the leaflet is supported by a fibrous collagenous structure supporting the valvar
valve5. The triangle between the non- shelf, or in some instances only by the leaflets. Rather than being hinged in
coronary and the left coronary leaflets is fibrofatty tissues of the left atrioventricular uniplanar circular fashion, the leaflets of
itself confluent with the area of fibrous groove, which also serve to insulate the the arterial valves have semilunar
continuity between the aortic and mitral atrial from the ventricular myocardium. attachments within the arterial roots
valves (Figure 3.13). When the triangle is The arrangement in which the fibrofatty (Figures 3.4, 3.5). True uniplanar rings,
removed, and the heart photographed from tissues insulate the atrial from the two of them, do exist within the structure
behind, it can be seen how the fibrous ventricular muscle masses (Figure 3.19) is of each arterial root, but neither is
tissue separates the left ventricular outflow the rule around the orifice of the tricuspid confluent with the semilunar attachments
tract from the middle part of the transverse valve. It is very rare to find the valvar of the leaflets, and neither is taken by
sinus (Figure 3.14). The much smaller leaflets supported by a fibrous annulus. surgeons to represent the valvar annulus.
triangle separating the two coronary leaflets Paradoxically, the solitary normal area of The most obvious anatomical ring, the
of the aortic valve separates the left atrioventricular muscular continuity, anatomical ventriculoarterial junction,
ventricular outflow tract from the tissue namely the penetrating bundle of His, becomes evident only when the valvar
Transverse sinus
Apex of interleaflet triangle
Non-coronary leaflet
Sup.
Fig. 3.13 This heart has been sectioned through the interleaflet
Apex Base
Mitral valve triangle between the non-coronary and left coronary aortic
leaflets, showing how the fibrous wall interposes between the left
Inf. ventricular outflow tract and the transverse sinus (white dotted
line).
Non-coronary sinus
Left coronary sinus
Left Ant.
Fig. 3.14 In this heart, the tip of the fibrous triangle between the
Tip of interleaflet triangle left coronary and non-coronary aortic valvar leaflets has been
Post. Right
removed, and the heart photographed from the transverse sinus to
show the location of the apex of the triangle.
Right coronary artery Left coronary artery
Tip of interleaflet triangle
Fig. 3.15 This dissection shows the location of the interleaflet

Base fibrous triangle between the two sinuses of the aortic valve that
give rise to the coronary arteries. The triangle itself has been
Right Left removed, as has the free-standing sleeve of subpulmonary
infundibulum, and the heart is photographed from the right
Apex Septal perforating arteries ventricular aspect. Note the position of the septal perforating
arteries.
leaflets themselves have been removed, There is a third ‘ring’ within the arterial echocardiographers as the valvar annulus2.
being best seen in the pulmonary valve roots, but this one is a virtual rather than an As now suggested by the German Working
(Figure 3.4). The semilunar attachments of anatomical structure. It is formed by Group concerned with surgical treatment
the leaflets cross this ring so that the upper joining together the most proximal parts of of the aortic valve3, the overall situation
extremity of each leaflet is attached to the valvar hinges (Figure 3.3). The virtual would become much clearer if surgeons
another obvious anatomical ring, namely ring, however, has particular clinical also accepted this virtual ring as
the sinutubular junction (Figure 3.3). significance, as this is the ring identified by representing the valvar annulus.
Right fibrous trigone Central fibrous body
Left fibrous trigone
Fig. 3.16 This heart has been dissected by removing the entirety
of the atrial myocardium, and is photographed from behind. The
Sup. Membranous septum walls of the aortic sinuses have been removed to the level of the
semilunar hingepoints of the valvar leaflets, showing the overall
Left Right crown-like arrangement of the fibrous part of the root supporting
the leaflets. Note also the fibrous extensions extending from the
fibrous tissue of the aortic root into the orifices of the mitral and
Inf. Aortic–mitral continuity tricuspid valves. These are the fibrous components of the annuluses
of the atrioventricular valves.
Aortic root
Fig. 3.17 The fibrous skeleton supporting the

aortic–mitral unit has been removed from the
heart shown in Fig. 3.16, and is photographed
from beneath. The fibrous tissue extends
Mitral valvar orifice around the orifice of the mitral valve, albeit
that histological studies show variability in the
firmness of the fibrous support. There is
minimal extension, however, around the
orifice of the tricuspid valve (stars). The red
Central fibrous body dotted line and the white triangles show the
Mural leaflet continuity between the leaflets of the aortic
and mitral valves.
common, which permit their collective earlier, this is appropriately termed the
BASIC MORPHOLOGY OF THE description in terms of a valvar complex annulus of the atrioventricular valves,
ATRIOVENTRICULAR VALVES (Figure 3.1)1. The first feature is the albeit that it is not always a firm and
The mitral and tricuspid valves, as we have attachments of the valvar leaflets to the continuous collagenous cord. A second
seen, have several important features in atrioventricular junctions. As discussed feature is the arrangement of the leaflets.
Atrial myocardium
Fibrous annulus
Mitral valvar leaflet
Fig. 3.18 This histological section is through the mural leaflet of

the mitral valve. Note that, at this particular part of the junction,
Ventricular myocardium there is a cord-like annulus anchoring the leaflet at the
atrioventricular junction. It is the exception rather than the rule,
however, to find such cords supporting the full length of the mural
leaflet6.
Atrial myocardium
Fibroadipose tissue
Ventricular myocardium Fig. 3.19 The histological section across the right
atrioventricular junction shows that it is the fibrofatty tissue of the
atrioventricular groove that, at all points except the penetration of
the bundle of His, insulates the right atrial from the right
Tricuspid valvar leaflet ventricular myocardium. There is no fibrous ‘annulus’ supporting
the hinge of the tricuspid valve.
Histologically, these structures have a musculature may extend into the fibrous muscles, or directly into the ventricular
spongy atrial and a fibrous ventricular layer. In normal valves, blood vessels are myocardium. These cordal attachments are
layer. The atrial myocardium inserts for found only within the segment of leaflet the third feature common to both tricuspid
varying distances between the containing muscular fronds. The leaflets and mitral valves, although there are
endocardium and the spongy layer. On rare are supported by the tendinous cords, fundamental differences in the way each of
occasions, fronds of ventricular which themselves insert into the papillary the cordal units is arranged. This is also the
Hinge of tricuspid valve
Fig. 3.20 This picture, taken in the operating

room, shows how the continuation of the
Eustachian valve, inserting as the tendon of
Todaro into the membranous septum (white
dashed circle), demarcates the atrial border of
the triangle of Koch. The star marks the site of
Tendon of Todaro the atrioventricular node, located at the apex
of the triangle.
case with the fourth feature, namely the have sought to achieve such definition by usually supported by fan-shaped cords, and
papillary muscles. The differences in these identifying the so-called commissures these are defined as commissural cords
various features readily permit the between them. This necessitates defining a (Figure 3.21). In addition to the fan-shaped
morphological differentiation of the valves. commissure. Surgeons usually take the cord, the entire free edges of the leaflets are
Before describing these differences, it is commissures as the peripheral attachments normally uniformly supported by cords
important to emphasise again those of a breach in the skirt of valvar tissue. For (Figure 3.22). As was indicated by Frater11
features of the leaflets and their tension the atrioventricular valves, these breaches when discussing the exquisitely complex
apparatus that are common to both valves. are supported by fan-shaped commissural categorisation of cords proposed by the
Considered as a whole, the leaflets form cords, such cords then inserting into a group from Toronto12, if any of these cords
a continuous skirt that hangs from the major papillary muscle or papillary muscle supporting the free edge are cut, the valve
atrioventricular junction. The skirt itself is group (Figure 3.21). This concept depends may become regurgitant. We agree with
divided into discrete components, with on defining one variable structure in terms Frater11 that, for the purposes of
these individual sections representing the of another, which is itself variable. In terms categorisation, it is sufficient simply to
individual leaflets. Sadly, there is no of philosophy, this is a poor principle. So as distinguish those cords supporting the free
current consensus as to how many leaflets to define commissures in this fashion, the edge (Figure 3.22) from those supporting
there are in each valve, nor as to what valve is also assessed in the opened the rough zone (Figure 3.23). The detailed
constitutes the point of separation of one position. Yet, as all surgeons know, in order classification proposed by the Toronto
leaflet from the other. Traditionally, the to function properly, the leaflets of the group12 has little, if any, surgical utility.
tricuspid valve, as indicated by its name, valve must fit together snugly when closed. The cords from the rough zone are
has been considered to have three leaflets. Because of this, we prefer to define the prominent structures that extend from the
Some have argued that the valve has only extent of the leaflets according to the papillary muscles to the ventricular aspect
two leaflets7, albeit that our studies lend no location of the zones of apposition between of the leaflets. Some of the cords to the
support to this concept8. The alternative them. It is thus the peripheral extents of rough zone are particularly prominent in
name for the mitral valve is the bicuspid these zones of apposition that become the the mitral valve, and are called strut cords
valve. Yet it has been suggested that this commissures, and the areas of the skirt (Figure 3.23). There is a third type of cord
valve is best considered as having four9, or between them that represent the leaflets. that extends from the ventricular wall close
even six leaflets10. These ongoing The leaflets themselves are supported by to the atrioventricular junction, and again
arguments illustrate the difficulties that tendinous cords. The ends of the zones of inserts into the ventricular aspect of the
exist in defining individual leaflets. Some apposition between the adjacent leaflets are leaflet. These are the basal cords
Ant.
Aortic leaflet of Fan-shaped
mitral valve cord
Sup. Inf.
Post.
Fig. 3.21 This surgical view of a rheumatic

mitral valve, taken through a left atriotomy,
Mural leaflet of shows a fan-shaped cord arising from the tip of
mitral valve its papillary muscle. This is the type of cord
traditionally considered to represent a
‘commissural’ cord.
Sup.
Left Right
Inf.
Aortic leaflet of
mitral valve
Fig. 3.22 This anatomical specimen, viewed from the inlet aspect,
Uniform cordal support to free edge shows the aortic leaflet of the mitral valve, with tendinous cords
supporting the entirety of the free edge of the valve.
(Figure 3.24). There is little point in very likely a mechanism leading to prolapse valvar mechanism. The reason that the
characterising the pattern of branching and of a leaflet13,14. atrioventricular valves have such a complex
the generations of these cords further, as The different components of the tension apparatus is that, while in their
long as it is appreciated that, normally, all atrioventricular valve making up the overall closed position, they must withstand the
parts of the leaflets receive good cordal valve complex (Figure 3.1) must function full brunt of systolic ventricular pressure.
support. Lack of uniform cordal support is in concert so as to produce a competent A lesion of any of the valvar components
Base Aortic leaflet

of mitral valve
Right Left
Apex
Strut Fig. 3.23 Some cords to the rough zone of the ventricular
cords aspect of the aortic leaflet of the mitral valve are particularly
prominent, as shown in this illustration, and are defined as
strut cords.
Fig. 3.24 The mural leaflet of the mitral valve has been
reflected upwards, showing the basal cords (arrow), each with
their own muscular belly.
can result in regurgitation. Thus, it is demands an area of overlap. Often, edge gives a margin of safety for dilation of
essential that the atrioventricular junction particularly in aged valves, this point of the valvar orifice. Equally significant to
be of normal size and not overly dilated. closure is marked by a series of nodules. competent closure, however, is the support
The leaflets must coapt snugly. This The point of closure away from the free of the leaflet by cords attached to the free
edge. As discussed previously, this feature (Figure 3.25). The ends of the solitary zone mural, this description accounting for both
is highly significant to the mechanism of of apposition between them, traditionally their morphology and their usual location
prolapse of the valve leaflets. It has been labelled as the commissures, together with within the body.
suggested15 that prolapse of the leaflets of the papillary muscles, are positioned in the The aortic leaflet is attached to only
the mitral valve is one of the most common inferoanterior position adjacent to the one-third of the annular circumference. It
congenital lesions. As yet, there is no septum, and superoposteriorly arising from is trapezoidal, or more semicircular, in
consensus as to the aetiology of such the posterior ventricular wall, although shape than the mural leaflet (Figure 3.27).
prolapse. Morphological observations11,12 they are usually described incorrectly as The mural leaflet, attached to two-thirds of
certainly suggest that lack of cordal support being posteroseptal and anterolateral. The the annulus, is a long, rectangular
to the free edge of the leaflets can advent of computed tomographic imaging, structure, although its middle component
contribute to prolapse, along with which relates the location of the heart to the can also be somewhat semicircular. It is the
lengthening of the persisting cords; coordinates of the body, reveals the segments of the mural leaflet adjacent to the
uniform support of the free edges of the inappropriate nature of the current ends of the zone of apposition with the
leaflets is an integral part of a normal valve. description (Figure 3.26). The aortic leaflet that show the most variation in
Also important in the maintenance of tomographic images also clearly show the morphology. Often these segments are
competence is the correct action of the angle existing between the axis of opening almost completely separate from the central
papillary muscles and the ventricular of the valve and the plane of the inlet component, producing the so-called
myocardium. Not only must the papillary component of the muscular ventricular scallops of the mural leaflet. Carpentier16
muscles be viable, but they must also be in septum (Figure 3.26). The posterior has coded these scallops in alphanumeric
their appropriate position of mechanical extension of the subaortic outflow tract is fashion, along with the facing segments of
advantage relative to the axis of the valve. wedged into this angle. As we will see, this the aortic leaflet, and this convention has
Taken together, all parts of the valvar arrangement is key to the disposition of the widespread surgical use (Figure 3.28).
unit are significant in the normal function axis of atrioventricular conduction tissue Yacoub9 has gone so far as to nominate the
of the valve1. (see Chapter 5). The two leaflets of the scallops as separate leaflets of a
mitral valves themselves are frequently quadrifoliate valve. Others10 point out that
described as being ‘anterior’ and the parts of the leaflet adjacent to the ends
‘posterior’. Strictly speaking, they are of the zone of apposition can also take a
THE MITRAL VALVE
anterosuperior and posteroinferior, again ‘scallop-like’ appearance (Figure 3.29). All
The mitral valve has two major leaflets, because of the oblique axis of opening of these categorisations are artificial. Because
which are supported by paired papillary the valve relative to the anatomical axes of of the marked variability that occurs
muscles. The two leaflets have widely the body (Figure 3.26). We prefer to normally in the valve, the mural leaflet can
dissimilar circumferential lengths describe the leaflets as being aortic and be comprised of four, five, or even more
Fig. 3.25 The mitral valve is viewed from

above in a closed position, as it might be seen
by the surgeon. There is a solitary zone of
apposition between its two major leaflets, with
slits in the mural leaflet producing a number of
potential subcomponents in the mural leaflet
(stars). Note that the ends of the zone of
apposition do not extend to the
atrioventricular junction (bold red braces). (The
original photograph of the mitral valve was
kindly sent to us by Dr Van S. Galstyan, from
Armenia, and we thank him for permitting us
to use it in our book.)
Superoposterior
Subaortic papillary muscle
outflow tract
Fig. 3.26 The computed tomographic image clearly shows the

orientation of the short axis of the ventricular mass relative to the
Left ventricle
bodily coordinates, indicated both by the box seen to the bottom
Right
right-hand corner of the image, and the yellow abbreviations at the
ventricle
margins of the image showing the coordinates of the head (H), feet
(F), anterior (A), and posterior (P). The tomographic section clearly
shows the angle between the line of the zone of apposition
between the leaflets of the mitral valve and the plane of the
Inferoanterior
papillary muscle inferior part of the muscular ventricular septum (white dotted
lines). Note that, contrary to the usual descriptions, the papillary
muscles are positioned inferoanteriorly and superoposteriorly
when considered in attitudinally correct orientation (see
orientation box).
Base
Aortic leaflet
Left Right
Apex
Mural leaflet
Fig. 3.27 Opening the mitral valve through the end of the zone
of apposition between the leaflets closest to the septum, and
spreading the valve, illustrates the widely dissimilar lengths of the
two leaflets at their hingepoints from the atrioventricular junction.
components. It is better to recognise that The supporting cords attach the leaflets muscles then support other cords running
there are a variable number of slits in the either to the papillary muscles, in the form to the free edge, which usually attach
mural leaflet, and that these slits act as of the cords to the free edge and rough uniformly along the leading edge of the
pleats so that the normal valve can close zone, or directly to the wall of the ventricle, leaflets, although, as we have discussed, the
snugly17. In the setting of prolapse, as with the basal cords. The cords at the degree of support can be quite variable.
nonetheless, individual components of ends of the zone of apposition attach The cords supporting the divisions
the divided mural leaflet may be deformed adjacent sides of both the leaflets to the between the scallops of the mural
in isolation, and must then be recognised apex of each of the papillary muscles. leaflet also often have a fan-shaped
as such. Supplementary heads of both papillary appearance. When attached to a prominent
A1
A2
P1 A3
P2
P3 Fig. 3.28 The classification of Carpentier16 recognises three
major scallops in the mural leaflet (P1 to P3), and also names the
facing segments of the aortic leaflet in alphanumeric fashion (A1
to A3). This classification, however, ignores the potential
subcomponents of the leaflet at the ends of the solitary zone of
apposition (see Figure 3.29).
2
A1
A2
A3
P1
3
P2 6
4 P3
Fig. 3.29 Kumar and colleagues10, in their suggested
5
classification, also took into account the components of the mural
leaflet adjacent to the ends of the solitary zone of apposition
between the two major leaflets (stars), giving six rather than four
potential subcomponents for the entire valvar skirt (compare with
Figure 3.28).
supplementary head of a papillary muscle, structures, located on the inferoanterior terms of the conduction tissues. The
they can be markedly similar to the cords and superoposterior aspects of the parietal atrioventricular node and the penetrating
supporting the commissures. The cords to ventricular wall (Figure 3.26). Although atrioventricular bundle are adjacent to
the rough zone run from the papillary there can be considerable variation in the these areas (Figures 3.31, 3.32). The area of
muscles to the ventricular aspect of the precise morphology of either muscle13, the orifice between the two trigones, more
leaflets. As already emphasised, several of neither arises from the septum, in contrast or less the midportion of the aortic leaflet,
these cords, running from each papillary to the attachments of the tricuspid valve is directly related to the commissure
muscle to the undersurface of the aortic within the right ventricle. When the valve between the non-coronary and left
leaflet, are particularly prominent, and are is dissected in its natural position within coronary leaflets of the aortic valve. At this
called strut cords (Figure 3.23). The basal the left ventricle (Figure 3.30), the muscles point, the aortic root is tented up, and an
cords attach to the ventricular aspect of the are seen to be adjacent to each other at the incision apparently through the atrial wall
mural leaflet, running directly from junction of the apical and middle thirds of will extend into the subaortic outflow tract.
miniature muscle heads on the parietal wall the parietal ventricular wall. If the incision is continued superiorly, it
of the ventricle (Figure 3.24). It is the area of the valvar orifice related will pass into the transverse sinus of the
The papillary muscles of the mitral to the right fibrous trigone and central pericardial cavity, which overlies the
valve are almost always prominent paired fibrous body that is most vulnerable in aortic-mitral curtain (Figure 3.13). The
Posterosuperior muscle Sup.
Apex Base
Inf.
Fig. 3.30 The parietal wall of the left

ventricle has been removed and the heart
photographed from behind and beneath. The
Anteroinferior muscle papillary muscles are directly adjacent at their
ventricular origins.
Excised non-coronary aortic sinus
Location of AV node
Mitral
valve
Tricuspid
valve
Ant.
Left Right Fig. 3.31 The atrial chambers have been removed, along with the
non-adjacent sinus and leaflet of the aortic valve, revealing the
deep posteroinferior diverticulum of the left ventricular outflow
Post. tract. Note the position of the atrioventricular (AV) node (red circle)
relative to the mitral valve.
Site of atrioventricular node
Central fibrous body
Coronary sinus
Sinus node
Fig. 3.32 The cartoon shows the components of the mitral valve
Dominant right relative to the structures of surgical significance when the right
Circumflex artery coronary artery coronary artery is dominant, this being the situation in 90% of
individuals.
deep inner curvature of the heart overlies inferior, or mural, leaflet is usually divided, resulting in a discrete cleft
and runs to either side of the curtain, and is described as being posterior. This is yet that extends towards the central
lined by the transverse sinus. Encircling another example of the description of fibrous body.
the mural leaflet of the valve are the cardiac structures as seen in the autopsy or The anterosuperior leaflet descends from
circumflex coronary artery from below and dissecting room, the heart having been the underside of the ventriculoinfundibular
to the left, and the coronary sinus from removed, rather than using the bodily fold, hanging like an extensive curtain
below and to the right (Figure 3.32). The coordinates for reference. The septal leaflet between the inlet and outlet parts of the
atrioventricular nodal artery also runs in is supported between the relatively ventricle. Its medial and superior end is
close proximity to the right side of the inconstant inferior papillary muscle, and supported by cords from the medial
mitral orifice, particularly when arising the much more constant medial papillary papillary muscle (Figure 3.37). There is
from a dominant circumflex artery. Indeed, muscle. Also known as the muscle of more variability in the arrangement of its
the extent of the margin directly related to Lancisi, or the papillary muscle of the lateral and inferior component. On occasion,
the circumflex artery depends on coronary conus, it arises from the posterior limb of its zone of apposition with the inferior leaflet
arterial disposition. When the left coronary the septomarginal trabeculation is supported by the prominent anterior
artery is dominant, an arrangement found (Figure 3.37). The attachment of the septal papillary muscle, which takes origin from
in one-tenth of the population, the entire leaflet crosses the membranous septum, the apical portion of the septomarginal
attachment of the mural leaflet is intimately dividing it into atrioventricular and trabeculation. More often, the anterior
related to the coronary artery, including its interventricular components (Figure 3.38). muscle supports the midportion of the
branch supplying the atrioventricular (AV) The distal extent of the leaflet is attached anterosuperior leaflet. The leaflet is well
node (Figure 3.33). by multiple cords running from the free supported by cords to its free edge, but also
edge directly to the septum (Figure 3.39). by rough-zone and strut cords, in a fashion
It is this feature that serves to comparable with that seen in the aortic
THE TRICUSPID VALVE distinguish the tendinous support of the leaflet of the mitral valve. When the anterior
As judged on the basis of its pattern of tricuspid valve from that of the mitral papillary muscle supports the midpoint of
closure8, the tricuspid valve has three valve, the mitral valvar leaflets lacking any the anterosuperior leaflet, the muscle
major leaflets (Figures 3.34, 3.35).They are septal attachments. In the area where the supporting its zone of apposition with the
positioned septally, anterosuperiorly, and septal leaflet extends across the inferior leaflet is relatively indistinct.
inferiorly (Figure 3.36), albeit that the membranous septum, the leaflet may be Frequently, there are extensions of
Left coronary artery Ant.
Left Right
Post.
Fig. 3.33 The dissection, photographed from

above and behind, with the heart in the
anatomical position, shows the relationship of
the circumflex artery to the mural leaflet of the
Artery to AV node mitral valve when the left coronary artery is
dominant. Note that the inferior
interventricular artery, and the artery to the
Circumflex artery
Inferior interventricular artery atrioventricular (AV) node, take their origin
from the circumflex artery.
Anterosuperior leaflet
Inferior
leaflet
Ant.
Sup. Inf. Fig. 3.34 This picture, taken in the operating room, shows the
arrangement of the three leaflets of the tricuspid valve, which are
positioned septally, anterosuperiorly, and inferiorly or murally. The
Septal leaflet Post. latter leaflet is usually described, incorrectly, as being positioned
posteriorly.
muscle from either the papillary muscles or mural one. It is less constant than the atrioventricular junction, being
the ventriculoinfundibular fold directly other two, as the inferior papillary muscle supported distally by several cords to
into the anterosuperior leaflet. The third is often indistinct and variable. The leaflet the free edge, attached either to small
leaflet of the tricuspid valve is the inferior or is attached to the parietal part of the heads of muscle, or directly into the
Anterosuperior
leaflet Inferior
leaflet
Septal
leaflet
Fig. 3.35 The anatomical specimen has been

photographed to replicate the arrangement as
Sup. Base seen in the operating room, as shown in
Triangle Figure 3.34, confirming that the leaflets are
of Koch
located septally, anterosuperiorly, and
inferiorly. Note the relationship of the septal
Apex Inf.
leaflet to the landmarks of the triangle of Koch
(white dotted lines).
Anterosuperior
leaflet
Septal
leaflet
Fig. 3.36 The computed tomographic image, showing the three

Inferior leaflets of the tricuspid valve, confirms that they are positioned
leaflet septally, anterosuperiorly, and inferiorly when the heart is viewed
in attitudinally appropriate fashion. Note the markers produced by
the computed tomographic software.
ventricular wall. There are usually also basal encircled by the right coronary artery myocardial masses are separated almost
cords supporting the inferior leaflet running in the atrioventricular groove exclusively by the adipose tissue within the
(Figure 3.40). (Figure 3.41). It is rare to find a groove, with the leaflets of the valve hinged
The entire parietal attachments of well-formed collagenous tricuspid from the endocardial surface of the
the leaflets of the tricuspid valve are ‘annulus’. Instead, the atrial and ventricular ventricular wall (Figure 3.20).
Sup. Medial papillary muscle
Right Left
Inf.
Antero-superior Fig. 3.37 The right ventricle is photographed from the

leaflet infundibular aspect in anatomical orientation, showing the medial
Septomarginal papillary muscle, also known as the muscle of Lancisi, supporting
Inferior leaflet Septal leaflet trabeculation the zone of apposition between the septal and the anterosuperior
leaflets.
Atrioventricular membranous septum
Aortic root
Mitral
valve
Tricuspid
Sup. valve
Fig. 3.38 The base of the heart is shown from the atrial aspect,
Left Right the atrial myocardium and the non-coronary sinus of the aortic
valve having been removed. The attachment of the septal leaflet of
Interventricular membranous septum the tricuspid valve (arrow) divides the membranous septum into its
Inf.
atrioventricular and interventricular components.
they do not need an intricate arrangement of semilunar leaflets open into the supporting
BASIC ARRANGEMENT OF THE tension apparatus to ensure their arterial sinuses during ventricular systole,
ARTERIAL VALVES competence, are still best analysed in terms and then collapse together during diastole,
The arterial valves, although much simpler of a valvar complex (Figure 3.3). Their being held in their closed position by the
structures than the atrioventricular valves, as design is simplicity itself. The three hydrostatic pressure of the column of blood
Septal leaflet
Ant.
Oval fossa Sup. Inf.

defect
Fig. 3.39 This operative view through a right atriotomy shows the
Post. cordal attachments (arrows) to the septum of the septal leaflet of
the tricuspid valve. The patient also has a defect in the oval fossa.
Sup.
Right Left
Inf.
Fig. 3.40 The inferior leaflet of the tricuspid valve has been
reflected away from the ventricular wall to show the basal cords
(arrows).
they support (Figure 3.42). In terms of the valve do not close at their free edge. The is of half-moon, or semilunar, shape
histological structure, the leaflets have a line of closure is some distance from the free (Figures 3.4, 3.5). When the three semilunar
fibrous core with an endothelial lining edge, towards the semilunar attachment. Not hinges are considered in terms of their
(Figure 3.43). The fibrous core is thickened infrequently, the leaflets may be perforated overall structure, the seating of the leaflets
at the free edge, particularly at the central beyond the line of closure in this outer takes the form of a coronet (Figure 3.44).
portion short of the free edge where, with component. Such perforations are a normal The attachments of the leaflets at the apex of
age, a distinct fibrous thickening, the nodule finding, and do not affect valvar function. It their zones of apposition are significantly
of Arantius, becomes evident. The leaflets of is the hinge line of the individual leaflets that higher than the attachments at their
Right coronary artery

Ant. Artery to sinus node
Left Right
Post.
Tricuspid valvar orifice
Fig. 3.41 This view of the heart, also shown in

Figure 3.66, with a dominant right coronary artery,
photographed in anatomical orientation from above
Artery to atrioventricular node
and behind, shows the intimate relationship of the
artery to the orifice of the tricuspid valve.
Sup.
Left Right
Inf.
1
2
Fig. 3.42 This image of the closed aortic valve from above shows
the three valvar leaflets (1, 2, 3) coapting snugly, held together by
the diastolic pressure of the column of blood they support. The stars
mark the commissures, which are the peripheral attachments of the
zones of apposition to the sinutubular junction.
midportion. In light of these arrangements, true rings within the extent of the arterial being the plane of attachment of the valvar
it is less than ideal to describe the hinges of root, but paradoxically neither has attracted commissures (Figure 3.45). The most
the valvar leaflets in terms of the annulus, attention from surgeons for description as obvious ring anatomically is the locus over
albeit that many surgeons still consider this the valvar annulus. Perhaps the most which the fibrous walls of the arterial trunks
to be an appropriate term3. There are two obvious is the sinutubular junction, this are attached to the supporting ventricular
Leaflet
Wall of aorta
Ventriculoarterial
junction
Valvar hinge Fig. 3.43 The histological section shows the fibrous core of one leaflet
Ventricular myocardium of the aortic valves, with its endothelial linings on the arterial and
ventricular aspects. Note that the valvar hinge is well below the
anatomical ventriculoarterial junction.
Crown-like configuration Sup.
Left Right
Inf.
Fig. 3.44 The aortic root in this specimen has been dissected by
Mitral valvar orifice removing the valvar sinuses, leaving behind the semilunar
attachments of the leaflets. It shows how the overall arrangement
is crown-like.
Fig. 3.45 The cartoon shows the relationship of the semilunar

hinges of the aortic valvar leaflets (yellow) relative to the rings
within the root. The superiorly located green ring is the sinutubular
junction. The blue ring shows the ventriculoarterial junction, while
the red ring is a virtual structure, formed by joining together the
proximal attachments of the valvar hinges.
Fig. 3.46 The cartoon shows the plane of space measured by

echocardiographers as representing the arterial valvar annulus. It is
the virtual plane constructed by joining together the nadirs of the
hinges of the leaflets (blue double-headed arrow). It is a virtual
anatomical ring, however, rather than a true anatomical entity.
The anatomical rings in the arterial roots are formed by the
sinutubular junction (red double-headed arrow), and the
anatomical ventriculoarterial junction (plane indicated by dotted
line). Note that the widest part of the root is at the midsinusal level
(green double-headed arrow).
structures, thus constituting the haemodynamic junction, the latter arterial trunk (Figure 3.46). Thus, despite
anatomical ventriculoarterial junction, and being marked by the semilunar attachment the simple nature of the valvar structure,
best seen in the right ventricular outflow of the leaflets. By virtue of this the overall arterial roots are complex
tract with the valvar leaflets having been arrangement, part of the arterial wall, structures. The sinuses themselves are
removed (Figure 3.4). There is a marked haemodynamically, is a ventricular arranged in clover-like fashion, permitting
discrepancy between this ring-like structure. Part of the ventricle at the base the valvar leaflets to close in the fashion of
anatomical junction and the of each sinus is, in contrast, within the a Mercedes-Benz sign (Figure 3.42). It is
the nadirs of attachment of the valvar reality, it is no more than the diameter of outlet portion of the left ventricle
leaflets, however, that are taken as the the entrance to the arterial roots. (Figure 3.47). When naming the leaflets of
point of measurement of the the valve, advantage can be taken of the fact
echocardiographic valvar annulus that, almost without exception, the major
(Figure 3.46). This plane, therefore, does THE AORTIC VALVE coronary arteries take origin from two of
not correspond to any anatomical The semilunar leaflets of the aortic valve the aortic sinuses, but not the third. Thus,
structure, albeit that consensus among are attached in part to the area of fibrous the aortic leaflets can accurately be
surgeons is now moving towards accepting continuity with the aortic leaflet of the described as being right coronary, left
it as representing the valvar annulus3. In mitral valve, and in part to the muscular coronary, and non-coronary (Figure 3.48).
Sup.
Left Right
Inf.
Fig. 3.47 The aortic root has been displayed by removing the
leaflets of the aortic valve, the outflow tract between the left
coronary and the non-adjacent aortic leaflets having been opened.
The two sinuses giving rise to the coronary arteries have septal
musculature at their bases (inner red arrows), while part of the left
Left ventricle
coronary sinus, along with the non-coronary sinus, is supported by
a fibrous continuity with the aortic leaflet of the mitral valve (outer
red arrows).
Left coronary artery

Pulmonary valve
Right coronary
Left coronary aortic sinus
aortic sinus
Fig. 3.48 The heart has been photographed from above, the
Sup.
tubular aorta having been removed to reveal the valvar sinuses.
The origins of the coronary arteries permit two sinuses to be named
Non-adjacent Left Right as right and left coronary aortic sinuses. The third sinus is non-
sinus adjacent relative to the pulmonary trunk. In almost all instances, it
Right coronary artery Inf. does not give rise to a coronary artery. In those circumstances, it can
also be called the non-coronary sinus.
Fig. 3.49 The image is an enlargement of the heart shown in

Figure 3.47. It shows how the bases of the sinuses supporting the
coronary aortic leaflets are made up of the musculature of the
ventricular septum (dotted black lines), because the semilunar
hinges extend apically beyond the anatomical ventriculoarterial
junction. The red triangle enclosing a white cross-hatched area
shows the region of aortic wall that forms the distal extent of the
outflow tract between the coronary aortic leaflets, running to the
level of the sinutubular junction.
However, because the so-called non- incorporated in the fibrous skeleton. Half leaflets vary from heart to heart,
coronary sinus can rarely give rise to one of of it, extending from the zone of apposition particularly with regard to that portion of
the coronary arteries, it can more accurately with the right coronary leaflet, is attached the zone of apposition between the non-
be described as the non-adjacent sinus. It is to the area of the membranous septum coronary and left coronary leaflets that is
the right and left coronary aortic leaflets (Figure 3.51). The triangle between these related to the aortic-mitral curtain. The
that have a predominantly muscular origin leaflets has important relationships to both length of the subaortic fibrous curtain also
from the left ventricular wall, the base of right atrium and right ventricle varies from heart to heart. In a small
the myocardium being incorporated within (Figure 3.52). The extensive posterior percentage of normal hearts, the aortic
the supporting aortic sinus (Figure 3.49). extension, or diverticulum, of the left valve in this area is supported by a complete
These are the leaflets that are adjacent to ventricular outflow tract lies beneath the muscular infundibulum or sleeve18. These
the pulmonary trunk. Their more distal non-adjacent leaflet, limited anterolaterally individual variations do not distort the
adjacent parts take origin from the free by the atrioventricular component of the basic anatomical relationships as described
aortic wall, with the small interleaflet membranous septum. Reaching its nadir at earlier.
fibrous triangle between them separating the attachment to the right fibrous trigone, Appreciation of the anatomy of the
the cavity of the outflow tract from the the non-adjacent leaflet then rises to the valvar hinges brings into focus the
tissue plane between the aortic root and the apex of its zone of apposition with the left important surgical danger areas related to
subpulmonary infundibulum coronary leaflet. The adjacent parts of both the aortic valve. The ascending part of the
(Figures 3.15, 3.50). leaflets in this area are attached to the free non-coronary aortic leaflet is positioned
As the attachment of the right coronary aortic wall distally, and proximally are directly above the part of the atrial wall
leaflet is traced from its zone of apposition continuous with the aortic leaflet of the containing the atrioventricular node, while
with the left coronary leaflet, it drops mitral valve. In this way, they form the the zone of apposition with the right
towards the crest of the muscular part of aortic-mitral curtain, which separates the coronary aortic leaflet is above the
the septum in the area of the membranous left ventricular outflow tract not from the penetrating atrioventricular bundle and the
septum. It then rises again to the apex of left atrium, but from the transverse sinus of membranous septum (Figures 3.54, 3.55).
the zone of apposition with the non- the pericardium (Figure 3.53). Beyond this The zone of apposition between the right
adjacent leaflet (Figure 3.51). The area, the left coronary leaflet adjoins a short coronary and left coronary leaflets is
attachment of this posterior part of the segment of parietal myocardium before usually positioned opposite the
right coronary leaflet is an integral part of extending anteriorly to reach the zone of corresponding zone of apposition between
the fibrous skeleton. All of the non- apposition with the right coronary leaflet. the facing leaflets of the pulmonary valve.
adjacent leaflet has a fibrous origin, and is The precise attachments of the aortic The adjacent parts of the two aortic
Pulmonary trunk
Infundibulum
Fig. 3.50 The aortic root in this heart has been dissected from the
Base right side, showing the relationship with the free-standing
muscular subpulmonary infundibulum (white brace). The root has
Right Left been cut along the fibrous triangle separating the two coronary
aortic leaflets (white arrow), showing how this triangle separates
Left coronary aortic leaflet
Apex the aortic outflow tract from the tissue plane between the aortic
root and the infundibulum (star).
Right coronary Non-coronary

aortic sinus aortic sinus
∗ Fig. 3.51 The aortic root shown in Figure 3.50 has been enlarged
to show the triangle between the right coronary aortic leaflet and
the non-adjacent leaflet. The base of the triangle is made up of the
membranous septum (dashed white oval), while the apex of the
Aortic leaflet of mitral valve triangle rises to the sinutubular junction (red triangle). The white
arrow shows the continuity with the aortic leaflet of the mitral
valve, and the black asterisk indicates the right fibrous trigone.
coronary leaflets, therefore, are directly ventricular outflow tract at this site lead modified Konno, procedure. Beyond this
related to the infundibulum of the right directly into the subaortic region point, the lateral part of the left coronary
ventricle, albeit that a discrete extracardiac (Figure 3.56). This is the basis of the leaflet is the only part of the aortic valve
tissue plane interposes between them right ventricular approach for relief of that is not intimately related to another
(Fig. 3.50). Incisions through the right subaortic obstruction, as in the Konno, or cardiac chamber. This is the part of the
Base Fig. 3.52 The dissection through the fibrous triangle between the
right and non-adjacent leaflets of the aortic valve shows the
relations of the aortic outflow tract to the right atrium (black brace)
Right Left
and right ventricle. The black dotted line shows the apex of the
triangle, which separates the left ventricular outflow tract from the
Tricuspid valve Apex
transverse sinus (white asterisk). The white triangle is between the
non-adjacent and left coronary aortic sinuses.
Apex of interleaflet triangle Transverse sinus
Left ventricle
Sup.
Fig. 3.53 The left ventricular outflow tract in this heart is

Apex Base
bisected through the fibrous triangle between the non-adjacent
and left coronary aortic leaflets. The fibrous wall (red dotted line)
Inf. separates the outflow tract from the transverse sinus (white
dotted line).
valve that takes origin from the lateral the infundibulum of the right ventricle
margin of the inner heart curvature. It is in THE PULMONARY VALVE (Figure 3.57). Because of its oblique
relationship externally with the free The pulmonary valve in the normal heart position, there is difficulty in naming the
pericardial space. has exclusively muscular attachments to pulmonary leaflets according to the body
Infundibulum
Aortic root
Tricuspid valve Fig. 3.54 The heart has been prepared by removing the right
coronary aortic sinus and the supporting ventricular septum.
Viewed in surgical orientation, it shows how the atrioventricular
∗ conduction axis (black line) penetrates the membranous septum
(black brace), extending from the atrioventricular node (red
asterisk) to branch on the muscular ventricular septum (blue
dashed lines).
Left bundle branch Right coronary

aortic sinus
Left coronary
aortic sinus
Non-coronary aortic sinus

Fig. 3.55 The cartoon shows the relationships of the
Central fibrous body atrioventricular node and penetrating bundle as they would be
Atrioventricular node
seen by the surgeon working through the aortic valve.
coordinates. It is better to describe them appropriately, as the non-adjacent leaflet. coronary artery. Our preference, therefore,
according to their relationship to the aortic When looked at from the stance of the is to account for their anatomical location.
valve (Figure 3.58). The two leaflets of the observer positioned within the non- The zone of apposition between the two
aortic valve that are attached to the septum adjacent sinus, the two adjacent sinuses are adjacent leaflets has traditionally been
are always adjacent to two leaflets of the to his or her right- and left-hand sides19. considered to be attached to the muscular
pulmonary valve. These two leaflets of the The right-hand leaflet as seen from the outlet septum immediately above the
pulmonary valve, therefore, can simply be pulmonary trunk, however, is positioned anterior limb of the septomarginal
called the right and left adjacent leaflets. leftwards and posteriorly in space, while trabeculation. In reality, it is attached to a
The third leaflet is then described, the left-hand leaflet is adjacent to the right free-standing sleeve of infundibular
Infundibulum
Pulmonary trunk
Aorta
Fig. 3.56 The cartoon shows how, in the Konno operation, an
incision through the posterior wall of the subpulmonary
infundibulum takes the surgeon into the subaortic outflow tract,
Non-coronary aortic sinus the incision in the infundibular wall being carried into the muscular
ventricular septum, and entering the left ventricular outflow tract
Right coronary aortic sinus between the two aortic sinuses giving origin to the coronary
arteries. The two stars show how the wall has been folded open.
Pulmonary valve
Muscular infundibulum
Left
Sup. Inf.
Fig. 3.57 This heart, shown in anatomical orientation, is opened
along the parietal wall of the right ventricle. It shows how all
Right leaflets of the pulmonary valve are supported by infundibular
musculature.
musculature (Figure 3.50). As the right the hinges of the atrioventricular and between the non-adjacent and left adjacent
adjacent leaflet drops down from the apex arterial valves is the ventriculoinfundibular leaflets extends from the parietal
of its zone of apposition with the other fold. The zone of apposition between the attachment back towards the septum, so
adjacent leaflet, it is supported by the inner right and non-adjacent leaflets is thus that the left adjacent leaflet runs from the
heart curve, which separates it from the towards the parietal extent of this fold, with parietal wall to the area of the septum.
tricuspid valve. This muscular mass is the the non-adjacent leaflet being supported by When considered as a whole, the valve can
supraventricular crest of the right the anterior parietal wall of the be liberated from the right ventricle,
ventricle. The particular part separating infundibulum. The zone of apposition together with its infundibular sleeve
Non-adjacent
component
Rightward adjacent
component
Leftward adjacent
component
Developing aortic valve
Developing pulmonary valve
Fig. 3.58 The image shows the components of the developing

Right coronary aortic and pulmonary roots, as seen in the mouse at 12.5 days of
component
embryonic life. The cushions will excavate so as to give rise to the
Sup. valvar leaflets and their supporting sinuses. The components retain
their relationships, so that two of the sinuses of the pulmonary
Right Left valve are always adjacent to the sinuses of the aortic valve that,
Non-adjacent Left coronary eventually, will give rise to the coronary arteries. The other sinus is
component component
Inf. non-adjacent. This provides a logical means of describing the valvar
sinuses and leaflets, as shown by the labels in this figure.
First septal perforator Anterior interventricular artery

Aortic valve
Left Ant. Fig. 3.59 The pulmonary trunk has been

tilted forwards in this heart, showing the sleeve
of infundibular musculature that lifts the
leaflets of the pulmonary valve away from the
Post. Right Infundibular sleeve ventricular base. Note the origin of the first
septal perforating artery.
(Figure 3.59) without damaging any vital cardiac subsystems, namely the
vital structures, although the left adjacent RELATIONSHIPS OF THE VALVES atrioventricular conduction system and the
leaflet is closely related to the first TO OTHER VITAL CARDIAC coronary circulation. It is mandatory to
septal perforating artery (Figure 3.60). STRUCTURES avoid damage to these structures during
Preservation of this artery is one of the Positioned as they are within the surgery, so it is important to know their
features that underscores the success of the ventricular base, the tricuspid, mitral, and precise locations. This must be learned
Ross procedure20. aortic valves are intimately related to two relative to landmarks within the valves
Left coronary artery First septal perforator
Left
Inf. Sup.
Fig. 3.60 The subpulmonary infundibulum has been removed
completely from the heart shown in Figure 3.59, revealing how this
Infundibulum can be achieved without transgressing on the left ventricle. Note
Right
again the location of the first septal perforating artery.
themselves, as the conduction tissues are exclusively within the triangle of Koch. interleaflet fibrous triangle separates the
largely invisible, while the course of the The inferior extent of the triangle is the valvar attachments from the conduction
coronary vessels is likely to be hidden as the junctional attachment of the septal leaflet of axis. When seen from the left ventricle, the
surgeon approaches the valves. Although the tricuspid valve. As the axis penetrates left bundle branch fans out as a continuous
we have mentioned these landmarks when the septum, it immediately enters the sheet on the smooth left septal surface
discussing the individual valves, their subaortic region of the left ventricle beneath the zone of apposition, splitting
importance is such that they justify a (Figure 3.61). The point of penetration is into its three divisions as it approaches the
collective review. related to the anteroinferior end of the zone ventricular apex. The right bundle branch
of apposition between the leaflets of the takes origin from the axis beyond the take-
mitral valve. In this area, the off of the left bundle branches. It courses
THE SPECIALISED MUSCULAR atrioventricular node lies within five to 10 back across the septum as a thin, insulated
AXIS FOR ATRIOVENTRICULAR millimetres of the atrial attachment of the cord, which emerges on the right
CONDUCTION medial scallop of the mural leaflet ventricular aspect in the area of the medial
In the normal heart, the penetrating (Figure 3.32). Having reached the left papillary muscle. It then runs, usually
atrioventricular bundle is the only ventricular outflow tract, the conduction intramyocardially, within the structure of
muscular communication between the axis begins to branch, either on the crest of the septomarginal trabeculation, to ramify
atrial and ventricular muscle masses. The the muscular ventricular septum, at the ventricular apex. When considered
bundle penetrates the membranous septal sandwiched between it and the from the right side, the site of the
component of the central fibrous body. It interventricular membranous septum branching segment of the conduction axis
is, therefore, intimately related to the (Figure 3.52), or on the left ventricular can be imagined as a line joining the apex of
leaflets of the mitral, tricuspid, and aortic aspect of the septum. This area is the triangle of Koch to the medial papillary
valves. The axis takes its origin from the immediately beneath the zone of apposition muscle (Figure 3.62). Care must be taken
atrioventricular node and its atrial zones of between the non-adjacent and right when placing sutures in this area, because
transitional cells. These atrial components coronary leaflets of the aortic valve the overall axis is within five millimetres of
of the conduction axis are contained (Figure 3.54), but the height of the the right ventricular septal surface.
Excised Sup.
membranous
septum Right Left
Aorta
Inf.
Fig. 3.61 This dissection is through the membranous septum

(white dotted line). The atrioventricular conduction axis penetrates
this septum to reach the crest of the muscular ventricular septum.
As can be seen, the point of penetration is directly adjacent to the
right end of the area of continuity with the mitral valve, which is
thickened to form the right fibrous trigone (white dotted area).
Ant. Medial papillary muscle
Sup. Inf.
Post.
Aorta
Fig. 3.62 The heart is shown in surgical orientation, the parietal

walls of the right atrium and ventricle having been removed. The
red line shows the course of the atrioventricular conduction axis as
Triangle of Koch it penetrates from the atrioventricular node (star) to reach the left
ventricular outflow tract. The right bundle branch re-emerges on
the right side beneath the medial papillary muscle.
cardiac vein runs within the left tissue of the inferior atrioventricular
THE VULNERABLE CORONARY atrioventricular groove, receiving the groove to empty into the right atrium
CIRCULATION oblique vein as it turns into the inferior part (Figure 3.65). In its course in an adult, it
On the venous side of the circulation, the of the groove (Figures 3.63, 3.64). This may approach to within five to 15
coronary sinus is the only noteworthy confluence marks the beginning of the millimetres of the medial attachment of the
structure related to the valves. The great coronary sinus, which proceeds in the fatty mural leaflet of the mitral valve. Deeply
Great cardiac vein

Base
Ant. Post.
Apex
Left atrium
Left
ventricle
Fig. 3.63 This cast shows the location of the great cardiac vein in
the left atrioventricular groove (white dotted line). The vein does
Oblique vein
not become the coronary sinus until it receives the oblique vein of
the left atrium.
Great cardiac vein Oblique vein

Coronary
sinus
Left
Base
Apex
Fig. 3.64 The heart has been removed from the body, and the
diaphragmatic surface is photographed to show the course of the
Right
coronary sinus within the inferior left atrioventricular groove. The
great cardiac vein becomes the coronary sinus at the point where it
Middle cardiac vein
receives the oblique vein of the left atrium.
placed sutures in this area during placed mitral valvar prosthesis, care must The coronary arteries are intimately
replacement of the mitral valve may lead to be taken not to enter the sinus with related to both the mitral and tricuspid
damage to the coronary sinus. This can scalpel or suture. The normal anatomy valves, as much of their course is within the
cause extremely troublesome bleeding. In may well have been distorted by the earlier atrioventricular grooves. The main stem of
addition, when removing a previously operation. the left coronary artery branches in the
Coronary
sinus
Diaphragmatic
surface
of left ventricle
Left
Sup. Inf.
sternotomy with the surgeon having reflected the heart, shows the
Right
position of the coronary sinus in the inferior atrioventricular
groove. The cannula is in the inferior caval vein.
Dominant right coronary artery
Mitral valvar
orifice
Ant.
Fig. 3.66 The heart in this image, also shown in Figure 3.41, has a
L R
dominant right coronary artery, which gives rise to the inferior
interventricular artery, and the artery to the atrioventricular node.
Post. As can be seen, the circumflex artery is only marginally related to
Circumflex a. stops at obtuse margin
the mural leaflet of the mitral valve in this setting.
angle of the margin of the left-sided of the pulmonary valve. This important is dominant, that is, gives rise to the inferior
ventriculoinfundibular fold immediately artery could be damaged by extensive interventricular artery, as it does in about
above the left fibrous trigone. The anterior dissection in this area. It is the circumflex 90% of cases, the circumflex artery (a.) is
interventricular artery moves away from branch of the left coronary artery that is related only to the area around the lateral
the valves, although its septal perforating most intimately related to the mitral valve, scallop of the mural leaflet of the mitral
branches extend into the septum particularly when the left coronary artery is valve (Figure 3.66). When the circumflex
immediately beneath the left-facing leaflet dominant. When the right coronary artery branch becomes the inferior
interventricular artery, its entire course is 3. Sievers HH, Hemmer G, Beyersdorf F, 13. Becker AE, de Wit APM. Mitral valve
intimately related to the entirety of the et al. The everyday used nomenclature of apparatus. A spectrum of normality
mural leaflet (Figure 3.33). the aortic root components: the Tower of relevant to mitral valve prolapse. Br Heart J
The right coronary artery always runs a Babel? Eur J Cardiothorac Surg 2012; 41: 1979; 42: 680–689.
478–482. 14. Van der Bel-Kahn J, Duren DR, Becker
circumferential course around the mural
4. Anderson RH. Demolishing the Tower of AE. Isolated mitral valve prolapse: chordal
attachments of the tricuspid valve. The
Babel. Eur J Cardiothorac Surg 2012; 41: architecture as an anatomic basis in older
initial course of the artery is through the 483–484. patients. J Am Coll Cardiol 1985; 5: 1335–
right atrioventricular groove (Figure 3.41), 5. Sutton JP 3rd, Ho SY, Anderson RH. The 1340.
where it lies on the epicardial aspect of the forgotten interleaflet triangles: a review of 15. Roberts WC. The 2 most common
ventriculoinfundibular fold. It can be the surgical anatomy of the aortic valve. congenital heart diseases. (Editorial) Am J
damaged by deeply placed sutures in this Ann Thorac Surg 1995; 59: 419–427. Cardiol 1984; 53: 1198.
area21. The artery encircles the attachment 6. Angelini A, Ho SY, Anderson RH, Davies 16. Carpentier A, Branchini B, Cour JC, et al.
of the mural leaflet of the tricuspid valve MJ, Becker AE. A histological study of the Congenital malformations of the mitral
before, in the majority of cases, turning to atrioventricular junction in hearts with valve in children. Pathology and surgical
become the inferior interventricular artery. normal and prolapsed leaflets of the mitral treatment. J Thorac Cardiovasc Surg 1976;
valve. Br Heart J 1988; 59: 712–716. 72: 854–866.
Just prior to its descent, the right coronary
7. Victor S, Nayak VM. The tricuspid valve is 17. Victor S, Nayak VM. Definition and
artery, when dominant, takes a prominent
bicuspid. J Heart Valve Dis 1994; 3: 27–36. function of commissures, slits and scallops
U-loop beneath the floor of the coronary 8. Sutton JP 3rd, Ho Sy, Vogel M, Anderson of the mitral valve: analysis in 100 hearts.
sinus, giving off the artery to the RH. Is the morphologically right Asia Pacific J Thorac Cardiovasc Surg 1994;
atrioventricular node at the apex of the atrioventricular valve tricuspid? J Heart 3: 10–16.
loop. In cases where the circumflex artery Valve Dis 1995; 4: 571–575. 18. Rosenquist GC, Clark EB, Sweeny LJ,
gives rise to the inferior interventricular 9. Yacoub M. Anatomy of the mitral valve McAllister HA. The normal spectrum of
artery, the atrioventricular nodal artery chordae and cusps. In: Kalmason D (ed). mitral and aortic valve discontinuity.
originates from the circumflex artery The Mitral Valve. A Pluridisciplinary Circulation 1976; 54: 298–301.
(Figure 3.33). Whether arising from a Approach. London: Edward Arnold, 1976; 19. Dodge-Khatami A, Mavroudis C, Backer
dominant left or right coronary artery, this pp 15–20. CL. Congenital heart surgery nomenclature
10. Kumar N, Kumar M, Duran CM. A and database project: anomalies of the
small but important vessel is related to the
revised terminology for recording surgical coronary arteries. Ann Thorac Surg 2000;
inferior aspect of the annular attachment of
findings of the mitral valve. J Heart Valve 69: S270–279.
the septal leaflet of the tricuspid valve. Dis 1995; 4: 76–77. 20. Merrick AF, Yacoub MH, Ho SY,
11. Frater R. Anatomy and physiology of the Anderson RH. Anatomy of the muscular
References normal mitral valve. (Discussion) In: subpulmonary infundibulum with regard to
Kalmanson D (ed). The Mitral Valve. A the Ross procedure. Ann Thorac Surg 2000;
1. Perloff JK, Roberts WC. The mitral Pluridisciplinary Approach. London: 69: 556–561.
apparatus. Functional anatomy of mitral Edward Arnold, 1976; p 41. 21. McFadden PM, Culpepper WS
regurgitation. Circulation 1972; 46: 227–239. 12. Lam JHC, Ranganathan N, Wigle ED, 3rd, Ochsner JL. Iatrogenic right
2. Frater RWM, Anderson RH. How can we Silver MD. Morphology of the human ventricular failure in tetralogy of Fallot
logically describe the components of the mitral valve. I. Chordae tendineae: a repairs: reappraisal of a distressing
arterial valves? J Heart Valve Dis 2010; 19: new classification. Circulation 1970; 41: problem. Ann Thorac Surg 1982; 33:
438–440. 449–458. 400–402.
Surgical anatomy
4
of the coronary
circulation
Surgical anatomy: coronary circulation 91
The coronary circulation consists of the (Figure 4.2). When described in this particularly valuable when considering
coronary arteries and veins, together with fashion, the terms ‘right’ and ‘left’ refer to coronary arterial origins in malformed
the lymphatics of the heart. Because the the aortic sinuses giving rise to the right hearts (see Chapter 8).
lymphatics, apart from the thoracic duct, and left coronary arteries, rather than to Irrespective of the specific sinus from
are of very limited significance to operative the position of the sinuses relative to the which they arise, the coronary arteries
anatomy, they will not be discussed at any right-to-left coordinates of the body usually take their origin beneath the
length in this chapter. The veins, relatively (Figure 4.3). In the normal heart, the sinutubular junction (Figure 4.5). The
speaking, are similarly of less interest. In aortic root is situated obliquely, while in junction is the discrete transition between
this chapter, therefore, we concentrate malformed hearts, the root is frequently the aortic root and the tubular component
upon those anatomical aspects of arterial positioned abnormally. Whatever the of the ascending aorta, and is the most
distribution that are pertinent to the position of the aortic root, however, the obvious annular structure within the aortic
surgeon, concluding with a brief discussion two coronary arteries, when two are root. Deviations of origin of the coronary
of the cardiac venous drainage and the present, almost always take origin from arteries relative to the junction are not
cardiac lymphatics. those aortic sinuses that are adjacent to the uncommon2. They are considered
sinuses of the pulmonary trunk. Because abnormal, in adults, when arising more
of this, it is more convenient, and more than one centimetre distal to the
THE CORONARY ARTERIES accurate, to consider these sinuses as sinutubular junction, a feature said to occur
being to the left-hand and the right-hand in almost one-twentieth of normal hearts3.
The coronary arteries are the first side of the observer standing, figuratively The arterial opening can be deviated either
branches of the ascending portion of the speaking, within the non-adjacent sinus towards the ventricle, so that the artery
aorta. They take their origin from the and looking towards the pulmonary trunk arises deep within the aortic sinus, or
sinuses within the aortic root, immediately (Figure 4.4). This approach to towards the aortic arch, so that the origin is
above its attachment to the heart distinguishing the aortic sinuses giving outside the sinus (Figure 4.6). The
(Figure 4.1). There are three sinuses rise to the coronary arteries, introduced by displacement is of greater significance
within the aortic root, but only two the group from Leiden1, holds true when combined with the artery taking an
coronary arteries. The sinuses can be irrespective of the relationships of the oblique course through the aortic wall and
named, therefore, according to whether arterial trunks. It is now conventional to originating above or within an
they give rise to an artery, the normal describe the right-hand sinus as ‘no. 1’, inappropriate aortic sinus. This
arrangement being a right coronary, left and to distinguish the left-hand sinus as arrangement, now known as an anomalous
coronary, and non-coronary aortic sinus ‘no. 2’. The convention becomes aortic origin of a coronary artery4, is
Main stem of left

Base coronary artery
Right Left
Apex
Aorta
Fig. 4.1 The heart has been dissected by removing the

subpulmonary infundibulum. It is photographed in anatomical
orientation to show the origin of the coronary arteries from the
Right coronary artery Removed infundibulum sinuses of the aortic root that lie adjacent to the pulmonary root
(stars). The black dashed line shows the sinutubular junction.
Left coronary sinus

Ant.
Base
Apex
Post.
Fig. 4.2 The heart is photographed from above in anatomical

orientation after removal of the atrial myocardium and the
arterial trunks. Two sinuses of the aortic valve give rise to coronary
arteries, permitting them to be named as the right and left aortic
Non-coronary sinus Right coronary sinus coronary sinuses. The other sinus is the non-coronary sinus, which
is also non-adjacent relative to the pulmonary trunk.
Right coronary sinus

Left coronary sinus
Left
Post. Ant. Fig. 4.3 The software producing computed tomographic images
also produces markers showing the orientation of structures
relative to the anatomical position. These markers show that the so-
Right Non-adjacent sinus called right and left aortic sinuses are not strictly right-sided and
left-sided.
typically associated with the proximal part coronary artery arising from the left individuals5. Both variants introduce the
of the artery crossing the attachment of the coronary aortic valvar sinus (Figures 4.7, potential for luminal narrowing, and may
zone of apposition between adjacent 4.8), an arrangement which occurs in one of provoke disturbances in myocardial
leaflets, and is also considered to represent every 500 individuals, or the left coronary perfusion6.
one variant of the intramural arrangement artery arising from the right coronary aortic The left coronary artery almost always
(Figure 4.7). It can involve either the right valvar sinus, found in one of 2000 takes origin from a single orifice within the
Left hand
No.2
Right hand
No.1
Fig. 4.4 The cartoon demonstrates the basis of the so-called

Leiden convention. The surgeon stands, figuratively speaking, in
the non-coronary sinus, and looks towards the pulmonary trunk. Of
the sinuses adjacent to the pulmonary trunk, one is thus to the left-
hand side. This is sinus no. 2. The other sinus, to the right-hand side,
Left coronary artery is sinus no. 1. Almost without exception, the coronary arteries take
Right coronary artery origin from one or both of these sinuses. In the usual arrangement,
the right coronary artery arises from sinus no. 1, and the main stem
of the left coronary artery from sinus no. 2.
Left coronary artery Right coronary artery
Tubular aorta
Sup.
Right coronary aortic sinus

Post. Ant.
Fig. 4.5 The computed tomogram shows the right and left aortic
sinuses as viewed from behind. It shows the usual situation in
Inf. Left coronary aortic sinus which the coronary arteries arise within the sinuses, proximal to the
sinutubular junction (red dashed line).
left-hand facing sinus. In contrast, in about sinus node. In one large series7, two orifices in the same sinus, of course, is of far greater
half of all hearts, there are two orifices were found in the right-hand facing sinus significance in the setting of an anomalous
within the right coronary aortic sinus in almost half the cases, three orifices in aortic origin of a coronary artery, when one
(Figure 4.9). In such instances, the orifices 7%, and four orifices in 2%. In contrast, of the orifices gives rise to an artery taking
are unequal in size, the larger giving rise to multiple orifices in the left coronary aortic an intra-arterial and intramural course, as
the main trunk of the right coronary artery, sinus (Figure 4.10) are considerably more shown in Figures 4.7 and 4.8.
while the considerably smaller second rare7. If unrecognised, they may create Although rare, the coronary arteries, on
orifice usually gives rise to an infundibular problems in the interpretation of coronary occasion, can arise from a solitary orifice in
artery, or rarely to the artery supplying the angiograms. The presence of dual orifices the aortic root. This is usually within the
Sup.

Right Left
Inf.
Fig. 4.6 In this heart, the aortic valve has been opened and the
aortic root photographed from behind to show the right and left
aortic sinuses. The right coronary artery arises well above the
Left coronary artery sinutubular junction.(Reproduced by kind permission of Professor
Anton Becker, University of Amsterdam.)
Left coronary artery - Intramural course of

right coronary artery
Fig. 4.7 The computed tomogram, in the same orientation as in

Figure 4.5, shows an intramural course of the right coronary artery.
The artery crosses the peripheral attachment of the zone of
apposition between the valvar leaflets guarding the right and left
Left coronary sinus Right coronary sinus aortic sinuses, and takes its origin, along with the left coronary
artery, from the left aortic sinus (no. 2).
right coronary aortic sinus. The artery into anterior interventricular and transverse sinus. It is the variants involving
originating from the solitary orifice can take circumflex branches. The solitary artery a course between the great arterial trunks
one of two patterns. It can divide can also arise from the left coronary aortic that are of most significance, as they can be
immediately into right and left coronary sinus, branching so that the right coronary harbingers of sudden cardiac death6,8.
arteries. The left artery then passes either artery passes in front of the pulmonary There are subtle variations in the course
in front of the pulmonary trunk trunk (Figure 4.12), runs between the taken by the artery as it passes between the
(Figure 4.11), between it and the aorta, or arterial trunks, or extends behind the trunks9. There are three possibilities
behind the arterial pedicle, before dividing arterial pedicle, running through the (Figure 4.13). In the first, the artery runs
Sup.
Fig. 4.8 The tubular aorta has been removed at the level of the
sinutubular junction to show an anomalous origin of the right
coronary artery from the left aortic valvar sinus, which also gives
Left Right rise to the left coronary artery. Note that the right coronary artery
crosses the points of attachment of the valvar leaflets to the
Inf. Right aortic sinus sinutubular junction. This is the essence of the intramural
arrangement.
Orifice of infundibular artery Orifice of right coronary artery
Sup.
Ant. Post.
Fig. 4.9 In this heart, the right coronary artery has a separate
Inf. Left aortic sinus origin from the right coronary aortic sinus relative to the
infundibular artery.
between the aorta and the pulmonary trunk pulmonary infundibular sleeve and the is likely that compression on the arterial
at the level of the aortic sinutubular aortic root (Figure 4.15). This course is lumen will be greatest when the artery is
junction, as shown in Figures 4.7 and 4.8. below the level of the hinges of the buried within the musculature of the
The second possibility is for the artery to pulmonary valvar leaflets, yet not buried septum, an important consideration when
extend deeply within the musculature of within the muscular ventricular septum. assessing the need for surgical correction of
the ventricular septum (Figure 4.14). The The fact that the artery is not within the the anomalous course. A still further
third possibility is for the artery to track septum may not readily be appreciated when alternative course for a solitary coronary
within the tissue plane between the seen from the lateral aspect (Figure 4.16). It artery, although less frequent, is when
Left coronary sinus Sup.
Ant. Post.
Inf.
Anterior interventricular artery Fig. 4.10 The computed tomogram shows the separate origin of
Circumflex artery the interventricular and circumflex arteries from the left coronary
aortic sinus.
Left coronary artery Left
Sup. Inf.
Right
Pulmonary trunk
Aorta
Fig. 4.11 This operative view, seen through a median sternotomy,

shows a solitary coronary artery taking origin from the right
coronary aortic sinus. It divides immediately into right and left
branches, with the main stem of the left coronary artery crossing
Right coronary artery the subpulmonary infundibulum. The left coronary artery then
divides into the circumflex and anterior interventricular arteries.
the single artery initially follows the path through the territory usually supplied by The right and left coronary arteries,
of the normal right coronary artery. It the circumflex artery, before terminating having taken origin from the aortic root,
then continues beyond the crux, as the anterior interventricular coronary extend subepicardially within the
encircling the left atrioventricular junction artery. atrioventricular and interventricular
Sup. Main stem of solitary

coronary artery
Right Left
Inf.
Circumflex artery Fig. 4.12 In this heart, there is a solitary coronary artery arising
from the left aortic valvar sinus. It divides into circumflex and
anterior interventricular (ant. intervent.) arteries, but also gives rise
to the right coronary artery, which courses across the
Ant. intervent. artery
Right coronary artery subpulmonary infundibulum to reach the right atrioventricular
groove.
Infundibulum
Aortic root
Sup. Fig. 4.13 The long axis section replicating the parasternal
echocardiographic cut shows the tissue plane that exists between
Ant. Post. the aortic root and the subpulmonary infundibulum. As shown,
arteries can run between the trunks at the level of the sinutubular
junction (red oval), within the tissue plane between the
Inf.
subpulmonary infundibulum and the aortic root (blue oval), or
within the substance of the ventricular septum (yellow oval).
grooves, the left-sided artery giving rise to atrioventricular groove (Figure 4.17). It nine-tenths of cases, the right coronary
two major branches. The right coronary then encircles the tricuspid orifice, running artery gives rise to an inferior
artery emerges from the right aortic sinus in the right atrioventricular groove interventricular artery at the crux, albeit
and immediately enters the right (Figure 4.18). In approximately that the artery is usually said to be
Right coronary aortic sinus Sup.
Right Left
Inf.
Fig. 4.14 The computed tomogram shows the main stem of the
left coronary artery arising from a solitary coronary artery, which
itself arises from the right coronary aortic sinus. The left coronary
artery extends deeply within the crest of the ventricular septum
Left coronary artery (black double-headed arrow) before dividing into its anterior
interventricular and circumflex branches.
Right coronary aortic sinus Sup.
Right Left
Inf.
Fig. 4.15 This reconstructed computed tomogram shows the

anterior interventricular artery arising from the right coronary
aortic sinus, and extending between the aortic root and the
subpulmonary infundibulum. Unlike the situation shown in
Figure 4.14, however, the artery does not burrow within the crest of
Anterior interventricular artery
the muscular septum, but runs in the tissue plane separating the
aortic root from the free-standing infundibular musculature.
posterior. Computed tomographic images supplies downgoing branches to the related to the origin of the leaflets of the
now leave no doubt that the artery is diaphragmatic surface of the left ventricle tricuspid valve near the take-off of its acute
inferior and interventricular (Figure 4.19). (Figure 4.20). This is right coronary marginal branch. Other important
In a good proportion of these cases, the arterial dominance. As the artery encircles branches also take origin from this
artery continues beyond the crux, where it the tricuspid orifice, it is most closely encircling segment of the artery.
Sup.
Ant. Post.
Inf.
Aortic root
Infundibulum
Fig. 4.16 The computed tomogram shows the course of the

anterior interventricular artery between the infundibulum and the
aortic root previously shown in the reconstruction as Figure 4.15. It
is difficult from the lateral projection to appreciate that the artery
runs within the adipose tissue plane between the infundibulum
and the aortic root, rather than within the musculature of the
ventricular septum (see Figure 4.14).
Left
Apex
Infundibulum
Base
Right
Aorta
Fig. 4.17 The operative view, seen through a median sternotomy,

Right coronary artery shows the right coronary artery as it emerges from its sinus into the
right atrioventricular groove.
Immediately after its origin, the artery lies cases, the right coronary artery gives rise The left coronary artery has a short
within the rightward extent of the to the artery supplying the sinus node confluent stem, usually called the left main
transverse sinus, with the adjacent (Figure 4.21). This artery typically arises artery by the surgeon. It emerges from the
muscular wall representing the from the proximal part of the right left coronary aortic sinus, and enters the
ventriculoinfundibular fold of the coronary artery, but on occasion the left margin of the transverse sinus, being
supraventricular crest. In this course, the nodal artery can arise more distally, positioned behind the pulmonary trunk
right coronary artery gives rise to coursing over the lateral margin of the and beneath the left atrial appendage. It is a
downgoing infundibular branches, which appendage to reach the terminal groove. very short structure, rarely extending
may also arise by separate orifices within This is of major surgical significance beyond one centimetre in length before
the right aortic sinus. In just over half the (Figure 4.22)10. bifurcating into its anterior interventricular
Aorta
Sup.
Right Left
Fig. 4.18 The computed tomographic image shows the right
Inf. coronary artery, having emerged from its aortic valvar sinus,
encircling the tricuspid valvar orifice.
Right Right coronary artery
Post. Ant.
Left
Inferior interventricular artery Fig. 4.19 The computed tomographic image leaves no doubt that
the artery supplying the diaphragmatic surface of the heart is
inferior and interventricular (note the orientation marker in the
lower right corner; F, foot).
and circumflex branches (Figure 4.23). In margins of the left ventricle. The anterior important perforating branches that pass
some hearts, the left main artery trifurcates, interventricular or descending artery runs inferiorly into the septum (Figures 4.26,
with an intermediate branch present inferiorly within the anterosuperior 4.27). The first septal perforating branch
between the two major branches interventricular groove, giving off diagonal (Figure 4.26) is particularly important, as it
(Figure 4.24). The intermediate branch branches to the pulmonary surface of the is at major risk when the pulmonary valve is
supplies the pulmonary surface and obtuse left ventricle (Figure 4.25), and the removed for use as a homograft. The
Sup. Right coronary artery
Left Right
Inf.
Fig. 4.20 The diaphragmatic surface of the heart has been

photographed from beneath, with the heart positioned on its apex.
The right coronary artery, having supplied the inferior
interventricular artery, continues to give branches to the
Supply to left ventricle Inferior interventricular artery diaphragmatic surface of the left ventricle. This is right coronary
arterial dominance.
Aorta
Superior
caval vein
Left
Sup. Inf.
Right sternotomy, shows the artery to the sinus node arising from the
right coronary artery.
interventricular artery then continues relation to the mitral orifice. Its relationship coronary artery, however, is found in only
towards the apex, frequently curving under to the orifice is most extensive when it gives about one-tenth of cases. When the left
the apex onto the diaphragmatic surface of rise to the inferior interventricular artery at coronary is not dominant, the circumflex
the ventricles. the crux. In this circumstance, the left artery usually terminates by supplying
The circumflex branch of the left coronary artery is said to be dominant branches to the obtuse margin of the left
coronary artery passes backwards to run in (Figures 4.28, 4.29). A dominant left ventricle. In almost half of normal

Surgical incision
Ant.
Fig. 4.22 In this specimen, photographed to replicate the view
Apex
seen by the surgeon working through a median sternotomy, the
Base artery to the sinus node takes a lateral origin from the right
coronary artery. It courses over the lateral margin of the right atrial
Post.
Artery to sinus node appendage to reach the terminal groove. It has been divided by
the standard atriotomy.
Sup.
Main stem of left coronary artery
Ant. Post.
Inf. Aorta
Circumflex artery
Fig. 4.23 The computed tomographic image shows the left

coronary artery branching into its anterior interventricular and
Anterior interventricular artery circumflex branches. Note the diagonal branch (star) arising from
Obtuse marginal branch
the anterior interventricular artery.
individuals, the circumflex artery also tissue. In some hearts, the myocardium impediment to the surgeon in efforts to
gives rise to the artery that supplies the itself may form a bridge over segments isolate the artery.
sinus node. of the artery (Figure 4.30). The role of We have already emphasised the
Throughout much of their epicardial these myocardial bridges in the significance of the origin of the important
course, the arteries and their accompanying development of coronary arterial disease is artery supplying the sinus node. This, the
veins are encased in epicardial adipose not clear. They certainly can be an largest of the atrial arteries, originates
Anterior interventricular artery Left main stem
Circumflex artery
Sup.
Ant. Post.
Fig. 4.24 The computed tomographic image shows the left
coronary artery giving rise to three, rather than two, branches. The
Inf.
Intermediate artery intermediate branch supplies the obtuse margin of the left
ventricle.
Base Left main stem
Ant. Post.
Apex
Fig. 4.25 This dissection, photographed from the front in

anatomical orientation, shows the course and branches of the
Anterior interventricular artery Diagonal branch anterior interventricular artery. Note the location of the first septal
perforating branch (arrow).
from the right coronary artery in just right coronary artery, with a course across (Figure 4.31). Although rare in normal
over half of individuals, and from the the appendage, is an obvious potential individuals, our experience suggests that
circumflex artery in the remainder danger for the standard atriotomy these variants are more frequent in
(Figure 4.31). There are, however, rare (Figure 4.22). The artery to the sinus node, congenitally malformed hearts11. In
variants that must also be recognised rarely, may also take a lateral or terminal addition, the artery to the sinus node takes
when present10. A lateral origin from the origin from the circumflex artery a variable course relative to the cavoatrial
Sup.
Base
Apex
Inf.
Aorta
Excised pulmonary root

Fig. 4.26 This close-up view of a specimen, in anatomical
orientation subsequent to excision of the pulmonary valve, shows
the origin of the first septal perforating artery (arrow). Note its
proximity to the subpulmonary infundibular area, putting it at risk
when the pulmonary valve is removed for use as a homograft.
Left ventricle
Sup.
Ant. Post.
Inf. Fig. 4.27 The computed tomographic reconstruction shows the

course of the septal perforating arteries (arrows).
junction. There are three possibilities it runs deeply within Waterston’s groove, branch, forming a circle around the
(Figure 4.31). Usually, the artery courses and passes retrocavally. It is thus intimately cavoatrial junction.
anterocavally across the crest of the related to the superior rim of the oval fossa. The arterial supply to the ventricular
appendage to reach the node. Alternatively, The third possibility is for the artery to conduction tissues is also of surgical
Left coronary artery Ant.
Left Right
Post.
Fig. 4.28 This specimen, dissected and photographed to show the

Artery to AV node base of the heart in anatomical orientation, has a dominant
circumflex branch passing behind the mitral orifice and giving rise
Circumflex artery to the inferior interventricular artery at the crux. AV,
Inferior interventricular artery
atrioventricular.
Base
Left Right
Apex
Circumflex artery
Fig. 4.29 The computed tomographic reconstruction shows a

Inferior interventricular artery dominant circumflex artery, which gives rise to the inferior
interventricular artery.
significance. The atrioventricular nodal it perforates the fibrous atrioventricular

artery arises from the dominant coronary junction to supply a good part of the THE CORONARY VEINS
artery at the crux, usually from a U-turn of branching atrioventricular bundle. The The coronary veins drain blood from the
this artery beneath the floor of the coronary septal perforating arteries from the anterior myocardium to the right atrium. The
sinus. The nodal artery then passes towards interventricular artery (Figures 4.26, 4.27) smaller anterior, and the smallest cardiac
the central fibrous body, running within always supply the anterior parts of the veins, drain directly to the cavity of the
the fibrofatty plane forming the ‘meat’ in ventricular bundle branches. Occasionally, atrium. They are not of surgical
the atrioventricular muscular sandwich. they also supply the greater part of the significance. The larger veins accompany
Having traversed the node in some hearts, inferior ventricular conduction tissues. the major arteries, and drain into the
Sup.
Right Left
Inf.
Anterior
interventricular
artery
Fig. 4.30 In this specimen, photographed with the heart

positioned on its apex, there is extensive myocardial bridging across
the anterior interventricular artery (black brace).
Anterocaval course
Origin from right Origin from

coronary artery circumflex artery Fig. 4.31 The cartoon, drawn in anatomical orientation, shows
the variations in the origin of the artery to the sinus node, and the
variability relative to the cavoatrial junction. The left-hand panels
Retrocaval course
show the usual arrangement with the origin from the right
coronary artery, found in 55% of the population, with the rare
variant of a distal origin with coursing across the appendage (lower
left-hand panel). The right-hand panels show a proximal origin
from the circumflex artery, found in around 45% of the population,
with the rare variant of a distal origin with coursing across the
Arterial circle dome of the left atrium. The middle panels show the variation
Distal origin from right Distal origin from relative to the superior cavoatrial junction. The sinus node is shown
coronary artery circumflex artery in green.
coronary sinus (Figure 4.32). The great (Figure 4.33), lying between the left atrial interventricular artery, and the small
cardiac vein runs alongside the anterior wall and the ventricular myocardium cardiac vein, which has encircled the
interventricular artery. It becomes the (Figure 4.34), before draining into the right tricuspid orifice in company with the
coronary sinus as it encircles the mitral atrium between the sinus septum and the right coronary artery. Occasionally, these
orifice to enter the inferior and leftward sub-Eustachian sinus. At the crux, the latter two veins drain directly to the
margin of the atrioventricular groove. The sinus receives the middle cardiac vein, right atrium. The orifice of the coronary
coronary sinus then runs within the groove which has ascended with the inferior sinus is guarded by the Thebesian valve
Sup.
Left Right
Oblique
vein
Inf.
Great
vein
Small vein
Fig. 4.32 The cartoon shows the diaphragmatic surface of the

Middle vein
heart seen from behind, in anatomical orientation. It illustrates the
arrangement of the coronary veins that drain into the coronary
Coronary sinus
sinus.
Coronary
sinus
Diaphragmatic surface
of left ventricle

sternotomy, with the apex of the heart being lifted, shows the
coronary sinus running through the left atrioventricular groove.
(Figure 4.35), which, on very rare in patients suffering with problems in

occasions, may be imperforate. A THE CARDIAC LYMPHATICS lymphatic drainage subsequent to their
prominent valve is also found in the great Little is known about the surgical conversion to the Fontan circulation. The
cardiac vein where it turns around the implications of the lymphatic drainage of thoracic duct originates within the
obtuse margin of the left ventricle. This is the heart itself, although lymphatic abdomen in the confluence of lymphatic
the valve of Vieussens12. Some consider structures exist as superficial, myocardial, channels known as the cysterna chyli, this
that the valve marks the transition from the and subendocardial networks13. The most structure lying on the second lumbar
great cardiac vein to the coronary sinus. An important lymphatic channel within the vertebra. It enters the right paravertebral
alternative view is that the coronary sinus thorax, however, is both well recognised gutter of the thorax through the aortic
commences at the site of drainage of the and of particular importance. This is opening of the diaphragm, and runs within
oblique vein of the left atrium because, on occasion, the surgeon may the gutter to the level of the fourth thoracic
(Figure 4.36). need to ligate this vessel, the thoracic duct, vertebra. Within the lower part of the
Sup.
Post. Ant.
Inf.
Great cardiac vein
Mitral valve
Fig. 4.34 In this heart, the musculature of the atrial walls has
Coronary sinus
been removed to show the course of the coronary sinus within the
left atrioventricular groove. It opens into the right atrium at the
base of the triangle of Koch (black double-headed arrow).
Thebesian valve
Left
Sup. Inf.
Right Orifice of coronary sinus Fig. 4.35 This operative view, through a right atriotomy, shows
the Thebesian valve guarding the orifice of the coronary sinus.
Great cardiac vein
Oblique vein
Coronary sinus
Middle cardiac vein
Sup.
Fig. 4.36 This preparation was made by casting the coronary sinus
with silastic, the heart then being photographed from behind in
Apex Base
anatomical orientation. The great cardiac vein is seen entering
the coronary sinus. The location of the site of drainage of the
Inf. oblique vein marks the point at which the great vein becomes the
coronary sinus.
Left subclavian artery

Aorta
Sup.
Ant. Post.
Thoracic duct
Fig. 4.37 This operative view, taken through a left thoracotomy,
Inf. shows the location of the thoracic duct in the vicinity of the left
subclavian artery and descending thoracic aorta.
thorax, it lies on the vertebral column gutter, running beneath the arch of the terminate in either the left subclavian or
between the descending thoracic aorta and aorta (Figure 4.37). It then continues internal jugular vein as these structures join
the azygos vein. Crossing the midline superiorly and anteriorly, curving over the to form the left brachiocephalic vein. The
obliquely at the level of the fourth thoracic aortic arch between the left common duct has a fibromuscular coat, and contains
vertebra, it enters the left paravertebral carotid and subclavian arteries, to several valves along its course, with a
bifoliate valve characteristically present at origin of the coronary artery. Ann Thorac the evaluation of septal vs interarterial
its termination in the brachiocephalic vein. Surg 2011; 92: 986–992. course of anomalous left coronary arteries.
5. Yamanaka O, Hobbs RE. Coronary artery J Cardiovasc Comput Tomogr 2010; 4:
anomalies in 126,595 patients undergoing 246–254.
References cited coronary arteriography.Cathet Cardiovasc 10. Busquet J, Fontan F, Anderson RH, Ho
Diagn 1990; 21: 28–40. SY, Davies MJ. The surgical significance of
1. Gittenberger-de Groot AC, Sauer U, 6. Taylor AJ, Rogan KM, Virmani R. Sudden the atrial branches of the coronary arteries.
Oppenheimer-Dekker A, Quaegebeur J. cardiac death associated with isolated Int J Cardiol 1984; 6: 223–234.
Coronary arterial anatomy in transposition of congenital coronary artery disease.J Am 11. Barra Rossi M, Ho SY, Anderson RH,
the great arteries: a morphologic study. Coll Cardiol 1992; 20: 640–647. Rossi Filho RI, Lincoln C. Coronary
Pediatr Cardiol 1983; 4(Suppl 1): 15–24. 7. Engel HJ, Torres C, Page HL Jr. Major arteries in complete transposition: the
2. Neufeld HN, Schneeweiss A. Coronary variations in anatomical origin of the significance of the sinus node artery. Ann
Artery Disease in Infants and Children. coronary arteries: angiographic Thorac Surg 1986; 42: 573–577.
Philadelphia, PA: Lea & Febiger 1983; pp observations in 4,250 patients without 12. Zawadzki M, Pietrasik A, Pietrasik K,
73–75. associated congenital heart disease.Cathet Marchel M, Ciszek B. Endoscopic study of
3. Bader G. Beitrag zur Systematic und Cardiovasc Diagn 1975; 1: 157–169. the morphology of Vieussen’s valve.Clin
Haufigkeit der Anomalien der 8. Sharbaugh AH, White RS. Single coronary Anat 2004; 17: 318–321.
Coronararterien des Menschen.Virch Arch artery. Analysis of the anatomic variation, 13. Walmsley T. The Heart. In: Sharpey-
Path Anat 1963; 337: 88–96. clinical importance, and report of five cases. Schafer E, Symington J, Bryce TH (eds).
4. Kaushal S, Backer CL, Popescu AR, et al. JAMA 1974; 230: 242–246. Quain’s Elements of Anatomy. Eleventh
Intramural coronary length correlates with 9. Torres FS, Nguyen ET, Dennie CJ, et al. edition Vol IV, Part III. London:
symptoms in patients with anomalous aortic Role of MDCT coronary angiography in Longmans, Green and Co., 1929; p 110.
Surgical anatomy
5
of the conduction
system
The disposition of the conduction atrioventricular bundle. Although this anteriorly, the axis reaches the crest of the
system in the normal heart has been sometimes occurs inadvertently, it can be muscular septum, immediately beneath the
emphasised already (see Chapter 2). In surprisingly difficult to divide this fibrous membranous septum (Figure 5.5).
that earlier chapter, we pointed to the structure intentionally. The landmark to It divides here, with the branches of the left
importance, during surgical procedures, penetration of the atrioventricular bundle cascading down the smooth left
of avoiding the cardiac nodes and conduction axis through the fibrous surface of the muscular ventricular septum.
ventricular bundle branches, and insulating plane is the apex of the triangle When viewed from the left, the branching
scrupulously protecting the vascular of Koch (Figure 5.1). The apex is marked bundle is intimately related to the subaortic
supply to these structures. In this by the point at which the tendon of Todaro outflow tract, with the fibrous triangle
chapter, we will consider the anatomy inserts into the central fibrous body separating the non-coronary and right
of these tissues relative to the treatment (Figure 5.2). Just inferior to the apex of this coronary leaflets of the aortic valve marking
of intractable problems of cardiac triangle, the components of the the take-off of the left bundle branches
rhythm. The abnormal dispositions of atrioventricular node gather themselves (Figure 5.6). Sometimes, the branching
the conduction tissue to be found in together, and enter the insulating tissues of bundle lies below the septal crest, being
congenitally malformed hearts, the fibrous body (Figures 5.3, 5.4). Once carried on the left ventricular aspect of the
features of obvious significance to the insulated from the atrial myocardial mass, septum1. Having given rise to the left
congenital cardiac surgeon, will be the conduction axis becomes the bundle branches, the right bundle branch
discussed in the sections devoted to penetrating atrioventricular bundle, better takes origin from the axis, burrowing
those lesions in the chapters that follow. known as the bundle of His. This part of intramyocardially to reach the right side of
In this chapter, nonetheless, we the overall atrioventricular conduction axis the septum, and surfacing beneath the
will discuss surgical procedures is short, but extends leftwards as it pierces medial papillary muscle. The axis itself
performed to treat arrhythmias that the fibrous body. As the axis passes continues beyond the origin of the right
develop in the setting of the Fontan through the fibrous tissue, and divides into bundle branch, entering the aortic root as
circulation. its branches, the fibrous tissue, which the dead-end tract2. Often unrecognised,
forms the membranous septum, is crossed this rudimentary structure may prove to be
by the septal leaflet of the tricuspid valve, significant as a substrate for outflow tract
LANDMARKS TO THE dividing it into atrioventricular and tachycardias. Taken together, the site of the
ATRIOVENTRICULAR interventricular components. The conduction axis can be determined by
CONDUCTION AXIS interventricular component of the septum taking a line from the apex of the triangle of
In patients with intractable tachycardia, it is often occupied by the non-branching Koch to the medial papillary muscle
may be necessary to ablate the component of the conduction axis. Further (Figure 5.7). In terms of the atrial
Septal leaflet of tricuspid valve Left
Sup. Inf.
Right
Fig. 5.1 The operative photograph shows the surgeon’s view

through a right atriotomy in a patient with an atrial septal defect in
the oval fossa (star). The landmarks of the triangle of Koch (white
dashed lines; red circle marks its apex) are obvious. Tension has
Tendon of Todaro
Coronary sinus been placed on the Eustachian valve to bring the tendon of Todaro
into prominence.
Surgical anatomy: conduction system 113
Atrioventricular membranous septum Septal leaflet
Left
Sup. Inf.
Fig. 5.2 Another operative view, in the same orientation as in

Tendon of Todaro Right Figure 5.1, shows the tendon of Todaro inserting into the
atrioventricular part of the membranous septum.
Atrial Mitral
septum valve
Ventricular
Bundle of His septum
Tricuspid
valve

orientation, shows the location of the
conduction tissues within the triangle of Koch,
Tendon and the mechanism of penetration of the axis
of Todaro
of conduction tissue into the central fibrous
body to form the bundle of His, the point of
Compact node
penetration serving to delimit the junction of
the atrioventricular node with the penetrating
bundle.
components of the axis, the compact when all, or part, of the ventricular
atrioventricular node, located within the VENTRICULAR PRE-EXCITATION myocardium is excited earlier than would
triangle of Koch, is positioned some Ventricular pre-excitation is a frequent be expected had the impulse reached the
distance posterior to the attachment of the problem of cardiac rhythm that necessitates ventricles by way of the normal
septal leaflet of the tricuspid valve. It is well knowledge of the pertinent anatomy for its atrioventricular conduction system3. There
superior to the orifice of the coronary sinus. optimal treatment. The arrhythmia occurs are various anatomical pathways, proven
Atrial myocardium Central fibrous body
Penetrating atrioventricular bundle
Left
Base Apex
Fig. 5.4 The histological section shows the location of the
penetrating atrioventricular bundle as an insulated structure
Right Ventricular myocardium within the central fibrous body. The section corresponds to the
upper right-hand panel of Figure 5.3.
Central fibrous body Left bundle branch

Left
Base Apex
Right
Ventricular septum
Fig. 5.5 The histological section, in the same anatomical

orientation as in Figure 5.4, shows the branching component of the
atrioventricular conduction axis. It lies astride the crest of the
Septal leaflet of muscular ventricular septum, sandwiched between the septum and
Branching atrioventricular bundle tricuspid valve the central fibrous body. (Courtesy of Professor Anton Becker,
University of Amsterdam.)
and hypothetical, that can produce this taken for the impulse to traverse the arrangement first being described by
phenomenon. Essentially, they are ventricular bundle branches, as these Mahaim as paraspecific connections4.
pathways that short circuit part, or all, of structures are insulated from the septal These are not amenable to surgical
the normal delay induced within the myocardium. Accessory pathways can exist division. In contrast, the accessory
atrioventricular conduction axis. Most of between the atrium and the atrioventricular atrioventricular pathways that produce the
this delay occurs within the atrioventricular bundle, producing atrio-Hisian tracts, Wolff–Parkinson–White syndrome,
node and its zones of transitional cells, but and between the conduction axis and the probably the most common form of
an increment of delay reflects the time crest of the ventricular septum, the latter pre-excitation, are very much amenable to
Right coronary aortic leaflet Non-coronary aortic leaflet
Left bundle branch Membranous septum Fig. 5.6 The diagram is a reproduction of the original drawing
made by Tawara. It shows the location of the left bundle branch (in
red) relative to the aortic root.

Ant.
Sup. Inf.
Post.
Aorta
Fig. 5.7 The dissection was made by removing the parietal walls
of the right atrium and ventricle. It is viewed in surgical orientation,
Triangle of Koch
showing the location of the atrioventricular conduction axis (red
line). The star shows the position of the atrioventricular node.
surgical division5. Now, however, if arrhythmia, nonetheless, constituted an bundles of Kent5. The atrioventricular
division is necessary, the pathways will important step in the evolution of cardiac muscular strands that are part of the circuit
almost certainly be ablated in the catheter surgery. When treatment was first mooted, responsible for the abnormal rhythm join
laboratory. Surgical treatment of this the bundles were called, inappropriately, together the atrial and ventricular
myocardial muscle masses outside the area node-like remnants described by Kent, and animals such as the mouse or rat7, and the
of the specialised conduction tissues inappropriately considered by him to be remnants can be found by careful
(Figure 5.8). The best initial description of part of the normal conduction system examination of human hearts8. Only in
the bundles was given by Ohnell6. His (Figure 5.10). In reality, they are abnormal situations, however (see later), do
illustration (Figure 5.9) shows that the remnants of a ring of specialised the node-like remnants give rise to
structures, which typically extend through conducting tissue that surrounds the anomalous muscular atrioventricular
the fat pad on the epicardial aspect of the atrioventricular canal of the developing connections. The abnormal accessory
valvar annulus, bear no resemblance to the heart. They are found as complete rings in muscular bundles that are the substrate for
Atrial wall Accessory atrioventricular connection
Ventricular wall
Base
Right Left Annulus

Fig. 5.8 The histological section across the atrioventricular
junction shows the arrangement of a typical left-sided accessory
Apex muscular atrioventricular connection. (Courtesy of Professor Anton
Becker, University of Amsterdam.)
Fig. 5.9 The original drawing made by Ohnell shows the

arrangement of the typical left-sided accessory muscular
atrioventricular connections. Comparison with Figure 5.8 shows the
accuracy achieved by Ohnell when making his drawing.
Atrium
Node
Fig. 5.10 The illustration is taken from one of Kent’s

Ventricle demonstrations to the Society of Physiology, and relabelled by us. It
shows the structure of remnants of conduction tissue found
adjacent to the right atrioventricular junction. The node-like
structure bears no resemblance to the accessory connections that
produce Wolff–Parkinson–White syndrome (see Figures 5.8 and 5.9),
Valve but can rarely give rise to an anomalous connection (see
Figure 5.16).
the Wolff–Parkinson–White syndrome same patient9. If approached from within coexist with left-sided pathways. They are
(Figure 5.8) can be found anywhere around the atrium, incisions that divide the atrial frequently associated with Ebstein’s
the atrioventricular junctions. They are myocardium, or ablative lesions placed malformation, and may need to be treated
best described as being left-sided, right- above the origin of the leaflets of the mitral concomitantly with repair or replacement
sided, and paraseptal. The anatomy of each valve, are unlikely to divide the accessory of the abnormal tricuspid valve. They can
group shows significant differences. muscle bundles themselves. In order to be found at any point within the parietal
Left-sided pathways are found at any ablate the accessory connection surgically, aspect of the tricuspid orifice, from the
point around the mural component of the it was usually necessary to dissect within site of the membranous septum to the
mitral valvar orifice. Pathways can extend the fat pad on the epicardial aspect of the mouth of the coronary sinus
through the area of aortic-mitral valvar annulus, or to approach the pathway from (Figure 5.11). The same rules for their
fibrous continuity, but are exceedingly the epicardium. If approached ablation apply as discussed for left-sided
rare. The pathways almost always cross endocardially, it was necessary to reflect the connections.
from the atrial to the ventricular muscle coronary vessels to expose the accessory It is connections in the paraseptal
masses outside a well-formed fibrous muscle bundles. When treated in the position that constitute the greatest
annulus, with the fibrous structure catheter laboratory, lesions are usually clinical challenge10. When viewed from
supporting the hinge of the mural leaflet of placed on the ventricular aspect of the the right atrium, they can cross from the
the mitral valve (Figure 5.8). The atrial hingepoint of the mitral valve. atrial to the ventricular myocardium
origins of the connections are very close to Right-sided accessory pathways may (Figure 5.12) at any point between the
the fibrous junction9. The bundles also pass through the fat pad to connect mouth of the coronary sinus and the
themselves usually skirt very close to the the atrial and ventricular myocardial supraventricular crest. They present
fibrous tissue, often branching into several masses. More frequently, bundles on the problems for ablation, firstly, because
roots, which then insert into the ventricular right side are found some distance away they may run deep within the floor of the
myocardium. The bundles are rarely from the attachment of the leaflets of the triangle of Koch as viewed from the right
thicker than one to two millimetres in tricuspid valve, which is rarely a firm and atrium. The second problem is that the
diameter, and are made up of ordinary well-formed fibrous junction, as is usually atrioventricular node and bundle are also
working cardiomyocytes. On occasions, the case on the left side. Right-sided found within this area. The anatomy of
there may be more than one bundle in the connections can be multiple, and can the area is best illustrated by dissections
Left Ant.
Aorta
Post. Right
Tricuspid
valvar
orifice
Fig. 5.11 The dissection, photographed in anatomical orientation

Potential sites of right-sided from above and behind, shows the potential sites of
atrioventricular connections atrioventricular connections (black dashed line) in the parietal
aspect of the right atrioventricular junction.
Left
Base Apex Ventricular septum
Right
Atrial myocardium
Fig. 5.12 The histological section, in anatomical orientation,

Accessory AV connection
shows a muscular accessory atrioventricular (AV) connection
crossing the fibrofatty insulating plane of the right atrioventricular
junction. The strand takes its origin from the distal insertion of the
atrial myocardium into the area of attachment of the septal leaflet
of the tricuspid valve, and crosses the groove to attach to the
ventricular myocardium. (Courtesy of Professor Anton Becker,
Lumen
University of Amsterdam.)
that have removed the atrial walls from a layer of fibroadipose tissue between the within this tissue plane (Figure 5.13). The
the base of the ventricular mass atrial and ventricular muscle masses, atrioventricular node itself occupies the
(Figure 5.13). Such dissections reveal the reaching the central fibrous body when superior part of the atrial layer of this
cranial continuation of the inferior traced cranially. The artery to the triangular sandwich, with the
atrioventricular groove, which extends as atrioventricular node courses forwards fibroadipose tissue representing the
Artery to AV node
Membranous septum
Left atrium Right atrium
Fig. 5.13 The dissection, shown in anatomical orientation,

illustrates the plane occupied by adipose tissue that runs superiorly
and anteriorly beneath the mouth of the coronary sinus. The right
and left atrial walls have been resected (blue and yellow dotted
lines). The star shows the apidose tissue occupying the inferior
Circumflex coronary artery atrioventricular groove. The artery to the atrioventricular (AV)
node runs within the adipose tissue, in this case from a dominant
left coronary artery.
‘meat’ between the muscular layers of the catheter ablation, it should be (Figure 5.15). Experience has also shown
sandwich. Accessory muscular remembered that the triangle of Koch that muscular connections running to the
connections may cross through this contains both the atrioventricular node most lateral margin of the supraventricular
insulating layer at any point from the and its nutrient artery. Unless performed crest at the acute margin of the right
attachment of the mitral and tricuspid with care, there is always the danger that ventricle can produce the
valves at either side of the muscular intervention can produce complete electrocardiographic pattern initially
ventricular septum. Indeed, the only atrioventricular dissociation. attributed to the so-called Mahaim
connection that has been identified Surgeons and catheter ablationists, when connections13. Such connections, when
morphologically within this area was attempting to treat arrhythmias, have removed surgically from the acute margin,
located at the insertion of the tricuspid conventionally referred to the floor of the were shown to resemble the tissues of the
valve (Figure 5.12)9. If necessary, the triangle of Koch as being septal. They also atrioventricular node histologically. We had
fibroadipose tissue plane can be entered describe an anterior septum in the region previously identified such a pathway
surgically from the cavity of the right cranial to the membranous septum12. By running across the right atrioventricular
atrium, or can be reached by dissection this, they mean the area of the right junction (Figure 5.16)9. The atrial
from the epicardial aspect. Incisions atrioventricular junction that lies adjacent to component of these connections is
within the atrial component of this the supraventricular crest. It is a mistake to remarkably reminiscent of the illustrations
atrioventricular sandwich, interrupting describe this part of the right provided by Kent (Figure 5.10). Kent had
the muscular approaches to the atrioventricular junction as being septal, just argued, incorrectly, that the nodal remnants
atrioventricular node, have also been as it is incorrect to consider the atrial aspect were pathways for normal atrioventricular
shown to interrupt reciprocating of the triangle of Koch as representing a conduction14. Under abnormal
atrioventricular nodal tachycardias11. septal structure. In reality, the so-called circumstances, nonetheless, these node-like
Treatment of these latter arrhythmias is anterior septum is the medial margin of the remnants are able to function as the atrial
also accomplished now with efficiency and ventriculoinfundibular fold. If muscular origin of specialised muscular accessory
safety using catheter ablation atrioventricular connections exist in this connections15. It is these connections that
(Figure 5.14). When treating these area, they will join the atrial wall to the now are known to produce ventricular pre-
arrhythmias, either surgically or by supraventricular crest of the right ventricle excitation of the Mahaim type.
Triangle of Koch
Oval fossa
Sup.
Fig. 5.14 The photograph shows the site of ablation at the base of
Post. Ant. the triangle of Koch, which cured a patient with atrioventricular nodal
re-entry tachycardia. It is outside the area occupied by the specialised
Site of ablation tissues of the atrioventricular node (star) and its zones of transitional
Inf. cells.(Reproduced by kind permission of Dr Wyn Davies, St Mary’s
Hospital, London.)
Non-coronary leaflet of aortic valve

Sup.
Right Left
Inf.
Fig. 5.15 The dissection, in anatomical orientation, illustrates

how the area anterior to the membranous septum, which was
previously considered to represent the anterior septum, is part of
Ventriculoinfundibular fold
the parietal wall of the right atrioventricular junction.
substrates of other supraventricular counter-clockwise fashion (Figure 5.17),

SUBSTRATES FOR OTHER tachycardias, particularly atrial flutter and running down the terminal crest before
SUPRAVENTRICULAR fibrillation. Atrial flutter of the commonest ascending through the septal isthmus of the
TACHYCARDIAS type has shown itself to be especially tricuspid valvar vestibule16. The most
The attention of both surgeons and amenable to interventional therapy. The inferior part of the circuit passes through
interventionists has focused on the usual flutter circuit is known to pass in another muscular isthmus, this time
Node of Kent Accessory AV connection
Fig. 5.16 The section is taken across the right atrioventricular (AV)
Left junction, and comes from the heart of a patient who had ventricular
pre-excitation. The tricuspid valve was deformed by Ebstein’s
Ventricular myocardium malformation. An accessory muscular connection was identified
Base Apex
taking origin from a nodal remnant as identified by Kent, and
crossing the insulating plane to run within the muscularised leaflet
Right of the tricuspid valve.(Section reproduced by kind permission of
Professor Anton Becker, University of Amsterdam.)
Terminal crest Sup.
Right Left
Inf.
Septal isthmus
Fig. 5.17 The right atrium has been opened and photographed in
anatomical orientation to show the circuit (arrows) known to be
responsible for the common variant of atrial flutter. The flutter
wave descends the terminal crest, crosses through the inferior
cavotricuspid isthmus, and then ascends through the septal isthmus
Cavotricuspid isthmus to reach the superior aspect of the terminal crest before
recommencing the circuit.
limited by the orifice of the inferior caval are able to construct lines of block with is the reasoning behind the surgical
vein and the hinge of the septal leaflet of the great facility so as to divide the circuit. Care manoeuvres performed as part of the
tricuspid valve (Figure 5.18). The isthmus should be taken in the vestibular area to treatment of patients with the Fontan
has three discrete areas, containing various avoid damage to the right coronary artery circulation, known as the Fontan
combinations of fibrous and muscular (Figure 5.19). Such lines obviously would conversion18, to be discussed in greater
tissue (Figure 5.19)17. Interventionists now be made surgically with equal facility. This detail later.
Coronary sinus Sup.
Post. Ant.
Inf.
Oval fossa
Tricuspid valve
Fig. 5.18 The right atrium has been opened through the parietal
wall, and photographed in anatomical orientation to show the
Posterior Anterior structure of the inferior cavotricuspid isthmus. It has posterior,
Middle
middle, and anterior components (black braces).
Posterior-Fibrous
Anterior-Smooth
Lumen of right atrium
Fig. 5.19 This histological section,

prepared by Professor Siew Yen Ho, shows
the structure of the three components of the
inferior cavotricuspid isthmus. Note the
adjacency of the right coronary artery to the
Inf. caval vein vestibular musculature in the anterior
Middle-Pectinated Right coronary artery Tricuspid valve
compartment.
The other arrhythmia now becoming designed by Cox when considering distances along the pulmonary veins from
increasingly amenable to interventional treatment of postoperative arrhythmias the venoatrial junctions (Figure 5.20).
therapy is atrial fibrillation. It has long been (see later). More recently, interventionists These sleeves of myocardium contain
known that surgical techniques that create have shown that it is feasible to construct bundles of cardiomyocytes running in
mazes19 or corridors20 can certainly lines of block within both the left and right different directions, along with separating
ameliorate, if not cure, this troublesome atriums, lesions which can provide sheaths of fibrous tissue, which set the
entity. The operative procedures are successful treatment for atrial scene for the focal triggering that produces
complex and time consuming. The maze fibrillation22,23. It has also been shown that the fibrillation25,26. It has been suggested
procedure, for example, has undergone a proportion of cases of fibrillation can be that the cardiomyocytes within the sleeves
many modifications21, details of which are cured by making focal lesions in the mouth are histologically specialised27. This is a
beyond the context of our description, of the pulmonary veins24. This is because spurious claim. The cardiomyocytes are all
although we describe the third iteration the atrial myocardium extends for variable of working myocardial origin, with the
Dome of left atrium
Fig. 5.20 The dissection, made by Professor Damien Sanchez-

Quintana, shows the dome of the left atrium, the epicardium
having been removed to illustrate the organisation of the
myocardial fibres. Sleeves of myocardium (arrows) can be seen to
extend onto the pulmonary veins for varying distances. These
sleeves are now known to be the sources of focal activity in some
variants of atrial fibrillation.
Fig. 5.21 The pulmonary root has been opened, and the valvar
Semilunar hinges of leaflets removed, to show the muscular crescents (stars)
pulmonary valvar incorporated at the base of the three pulmonary valvar sinuses.
Proximal attachments to infundibulum leaflets
These muscular crescents can be the site of substrates for outflow
tract tachycardias.
pulmonary venous myocardium never ventricular muscle, as the semilunar hinges crescent within the non-adjacent sinus. In
having had the characteristics of of the valvar leaflets cross the anatomical the absence of such musculature, however,
conduction tissues28. ventriculoarterial junctions (Figures 5.21, it must be presumed that the abnormal
5.22). It is most likely that the abnormal rhythm has an extraventricular origin. It is
rhythms producing the outflow tract also known that, sometimes, the outflow
tachycardias take their origin within these tract tachycardias are ablated by lesions
SUBSTRATES FOR OUTFLOW myocardial crescents. It is also known, placed distal to the ventriculoarterial
TRACT TACHYCARDIAS however, that abnormal rhythms can rarely junction31. During their development, the
Experience shows that some ventricular be cured by ablative lesions placed in the arterial roots are encased within turrets of
tachycardias can be cured by placing non-adjacent sinus of the aortic root29. It is outflow tract myocardium, which then
lesions in the region of the bases of the rare to find myocardium within the base of regress as part of the normal developmental
arterial valvar sinuses29. Such lesions are this non-adjacent sinus. Sometimes the process32. Persistence of parts of this sleeve
placed most frequently in the adjacent aortic valve, like the pulmonary valve, can of outflow tract myocardium provides the
sinuses of the aorta and pulmonary trunk. be supported by a complete muscular best explanation for the origins of those
As we have shown in Chapter 3, these infundibulum30. If present, such arrhythmias originating distal to the
sinuses, at their bases, contain crescents of infundibular musculature would produce a ventriculoarterial junctions.
Continuity with mitral valve
Proximal muscular attachments
Fig. 5.22 The aortic root has been opened, and the valvar leaflets
removed. There is muscle at the base of the two aortic valvar sinuses
giving rise to the coronary arteries (stars), but not in the non-
adjacent sinus.
Amputated
right appendage Tricuspid valve
Coronary sinus
Oval fossa
Fig. 5.23 The cartoon shows the lesions made within the right
atrium during the Fontan conversion procedure so as to avoid
Superior caval vein Inferior caval vein
postoperative problems with rhythm.
implantable cardiac defibrillators have also with functionally univentricular hearts, and
ARRHYTHMIA SURGERY IN contributed to improved treatments33, but those who have undergone surgical
PATIENTS WITH CONGENITAL such details are beyond the scope of our treatment of tetralogy of Fallot or
HEART DISEASE current discussion. Knowledge of the transposition. The reasoning behind the
As patients get older, subsequent to surgical anatomical background for the created therapeutic approach is to transform areas of
correction of congenital cardiac lesions, therapeutic lines of block, nonetheless, now slow conduction to areas of no conduction34.
increasing numbers present with arrhythmic achieves increasing significance. The This is achieved by interrupting myocardial
problems33. The enhanced understanding of commonest arrhythmias requiring treatment corridors or isthmuses between obstacles or
the macro-re-entrant circuits responsible for are macro-re-entrant atrial tachycardia, atrial scars, while preserving sinus rhythm and
the abnormal rhythms has led to fibrillation, and ventricular tachycardia. normal atrioventricular conduction.
modifications of surgical techniques. Such problems are themselves encountered The commonest operations are the
Advances in the design of pacemakers and most frequently in postoperative patients right atrial maze, typically performed for
Atrial septal defect Right
Sup. Inf.
Left
Cryothermy lesion
Fig. 5.24 The cryoprobe is photographed in position to produce a

lesion from the atrial septal defect to the coronary sinus during a
Fontan conversion procedure, in a patient with tricuspid atresia.
Coronary sinus
Aorta Tricuspid atresia
Cryocath probe
Pulmonary trunk
Cannula in superior Fig. 5.25 The cartoon shows the lesions producing the right
caval vein Cannula in inferior atrial maze procedure as part of the Fontan conversion
caval vein procedure.
those with macro-re-entrant tachycardia, then created from the edge of the resected of the inferior caval vein, and from the
and the Cox maze III procedure for appendage to the oval fossa, from the inferior caval vein to the hinge of the
patients with atrial fibrillation. The right oval fossa across the terminal crest, and septal leaflet of the tricuspid valve. The
atrial maze procedure involves the then a series of lines to divide the last lesion is obviously not possible in
resection of the tip of the right atrial cavotricuspid isthmus (Figure 5.23). The those with tricuspid atresia due to
appendage, and part of its anterior surface lines dividing the isthmus run from the absence of the right atrioventricular
if the appendage is significantly enlarged, edge of the oval fossa to the margin of the connection, but the other lines can
as is often the case in patients with failed coronary sinus (Figure 5.24), from the easily be created in this setting
Fontan circulations. Lines of block are mouth of the coronary sinus to the mouth (Figure 5.25).
Amputated left
Tricuspid valve
appendage
Aorta
Mitral valve
Pulmonary trunk
Fig. 5.26 The cartoon shows the lesions produced in the third
Cannula in superior Cannula in inferior iteration of the Cox maze procedure for treatment of atrial
caval vein caval vein
fibrillation.
In the third iteration of the series of 2. Kurosawa H, Becker AE. Dead-end tract of 10. Sealy WC, Gallagher JJ. The surgical
procedures designed by Cox for treatment the conduction axis. Int J Cardiol 1985; 7: approach to the septal area of the heart
of patients with atrial fibrillation 13–20. based on experience with 45 patients with
(Figure 5.26)21, the lines of block can be 3. Durrer D, Schuilenburg RM, Wellens HJJ. Kent bundles. J Thorac Cardiovasc Surg
Pre-excitation revisited. Am J Cardiol 1970; 1980; 79: 542–551.
produced either by surgical incisions, or
25: 690–698. 11. Johnson DC, Ross DL, Uther JB. The
by cryoablation. An extensive line is
4. Mahaim I. Maladies Organiques du Faisceau surgical cure of atrioventricular junctional
created within the roof of the left de His-Tawara. Paris: Masson et Cie., 1931. reentrant tachycardia. In: Zipes DP, Jalife J
atrium, encircling the orifices of all 5. Sealy WC, Gallagher JJ, Pritchett ELC. The (eds). Cardiac Electrophysiology from Cell to
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then created to the mouth of the left electrophysiological mapping and surgical 921–923.
atrial appendage, which can be excised, exploration. J Thorac Cardiovasc Surg 1978; 12. Guiraudon GM, Klein GJ, Sharma AD,
or alternatively encircled by another line. 76: 804–815. et al. Surgical approach to anterior septal
A linear lesion is then made from the line 6. Ohnell RF. Preexcitation, a cardiac accessory pathways in 20 patients with the
encircling the pulmonary veins to the abnormality. Pathophysiological, Wolff–Parkinson–White syndrome. Eur J
annulus of the mitral valve in the region of pathoanatomical and clinical studies of an Cardiothorac Surg 1988; 2: 201–206.
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the third scallop of the mural leaflet. The
Scand 1944; 152: 14–167. “Nodal ventricular” Mahaim pathway:
final lesion is placed epicardially across
7. Yanni J, Boyett MR, Anderson RH, histologic evidence for an accessory
the coronary sinus as it runs within the Dobrzynski H. The extent of the atrioventricular pathway with an AV
left atrioventricular groove. If using specialized atrioventricular ring tissues. node-like morphology. Circulation 1988; 78
cryoablation, the final lesion is maintained Heart Rhythm 2009; 6: 672–680. (Suppl 2): 40.
for two minutes, while the other requires 8. Anderson RH, Davies MJ, Becker AE. 14. Kent AFS. The structure of the cardiac
only one minute of freezing at minus Atrioventricular ring specialized tissue in tissues at the auriculo-ventricular junction.
160 degrees Celsius. the normal heart. Eur J Cardiol 1974; 2: J Physiol 1913; 47: 17–18.
219–230. 15. Anderson RH, Ho SY, Gillette PC, Becker
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Wellens HJJ. The anatomical substrates atrioventricular conduction. Cardiovasc Res
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Medina A, Anderson RH. The architecture 23. Pappone C, Oreto G, Rosanio S, et al. Atrial the invasive electrophysiologist:
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1186–1195. atrial fibrillation. Circulation 2001; 104: McAllister HA. The normal spectrum of
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Analytical description
6
of congenitally
malformed hearts
Congenitally malformed hearts 129
Systems for describing congenital cardiac basic framework within which all other possessing the appendage in which the
malformations have frequently been based associated malformations can be pectinate muscles extend to the crux is
upon embryological concepts and theories. catalogued4. right-sided, while the one with a smooth
As useful as these systems have been, they inferoposterior vestibule is left-sided. This
have often had the effect of confusing the usual arrangement is often called ‘situs
clinician, rather than clarifying the basic solitus’. Rarely, the appendages can be
ATRIAL ARRANGEMENT
anatomy of a given lesion. As far as the disposed in mirror-image fashion, so-called
surgeon is concerned, the essence of a The first step in analysing any malformed ‘situs inversus’. More common than the
particular malformation lies not in its heart is to determine the arrangement of mirror-imaged topological arrangement,
presumed morphogenesis, but in the the chambers within the atrial mass. When but still relatively rare, is the situation in
underlying anatomy. An effective system distinction is based on the anatomy of the which the appendages of both chambers in
for describing this anatomy must be based appendages, which are the most constant the atrial mass have the same morphology.
upon the morphology as it is observed. At components of the atriums, and specifically This can occur in two forms, with either
the same time, it must be capable of on the extent of the pectinate muscles morphologically right (Figure 6.8) or
accounting for all congenital cardiac relative to the atrial vestibules5, atrial morphologically left (Figure 6.9)
conditions, even those that, as yet, might chambers can be of only morphologically appendages on both sides. These bilaterally
not have been encountered. To be useful right or morphologically left type. The symmetrical topological patterns, or
clinically, the system must be not only morphologically right appendage is broad isomeric arrangements, have traditionally
broad and accurate, but also clear and and triangular (Figure 6.2), whereas the been named according to the arrangement
consistent. The terminology used, morphologically left appendage is finger- of the abdominal organs, particularly the
therefore, should be unambiguous. It like, and has a much narrower neck spleen. This is because they usually
should be as simple as possible. The (Figure 6.3). In most instances, it is exist with a jumbled up abdominal
sequential segmental approach provides possible to identify the appendages simply arrangement, an arrangement also termed
such a system1, particularly when the on the basis of their shape (Figure 6.4). visceral heterotaxy6. It is far more
emphasis is placed on its surgical Only in circumstances of uncertainty will it convenient, as well as more accurate, to
applications2. The basis of the system is, in prove necessary to inspect the extent of the designate them in terms of their own
the first instance, to analyse individually pectinate muscles (Figures 6.5, 6.6). This intrinsic morphology8, particularly as this
the architectural make-up of the atrial feature, of course, is readily visible to the can be determined readily by the surgeon
chambers, the ventricular mass, and the surgeon once the atrial chambers have been in the operating room. Isomerism of the
arterial segment3. Emphasis is thus given to opened. right appendages is usually, but not always,
the nature of the junctional arrangements When judged on the extent of the found with absence of the spleen. It is most
(Figure 6.1). Still further attention is pectinate muscles, there are only four often associated with right bronchial
devoted to the interrelationships of the topological ways in which the appendages isomerism. Isomerism of the left
cardiac structures within each of the can be arranged within the atrial mass appendages is typically found, but again
individual segments. This provides the (Figure 6.7). Almost always, the atrium not always, with multiple spleens. The
lar
Atriums tr icu
Ve
en
iov
ntr
Atr
icu
Arterial
loa
trunks
rte
ria
l
Fig. 6.1 The cartoon shows the three segments of the heart. These
are the atriums, the ventricular mass, and the arterial trunks. The
segments are joined together at the atrioventricular and
Ventricles ventriculoarterial junctions.
Triangular appendage Sup.
Right Left
Inf.
Right coronary artery Fig. 6.2 The computed tomogram shows the typical triangular
shape of the morphologically right atrial appendage.
Hooked and tubular appendage
Sup.
Ant. Post.
Fig. 6.3 As shown in this computed tomogram, the

Inf. morphologically left atrial appendage has a characteristic narrow
and hooked shape (compare with Figure 6.2).
association with left bronchial isomerism is degree of accuracy by studying the appendages is of value in two additional
more constant. relationships of the abdominal great vessels ways. Firstly, it alerts the surgeon to
The anticipated topological as determined with cross-sectional unusual dispositions of the sinus node11. In
arrangement of the atrial appendages can ultrasonography10. When identified, right isomerism, the sinus node, being a
be predicted preoperatively with a high knowledge of isomerism of the atrial morphologically right atrial structure, is
Morphologically left appendage
Left
Sup. Inf.
Right
Fig. 6.4 This operative view, taken through a median sternotomy,

shows the differences between the broad triangular
Morphologically right appendage morphologically right atrial appendage, and the narrow finger-like
morphologically left atrial appendage.
‘Septum spurium’ Post.

Sup.
Ant. Inf.
Extensive pectinate muscles
Fig. 6.5 The computed tomogram shows the extensive pectinate

muscles found in the roof of the morphologically right atrium.
When assessed relative to the vestibule of the atrioventricular
junction, they extend to the crux of the heart.
duplicated. A node is found laterally in a constant site. Usually the node is found in arrangements are known to be harbingers
each of the terminal grooves12. In left the anterior interatrial groove, close to the of complex intracardiac lesions. Hearts
isomerism, there are no terminal grooves atrioventricular junction12. with isomerism of either type tend to have
(Figure 6.9). In this situation, the sinus The second advantage of recognising bilateral superior caval veins, an effectively
node is a poorly formed structure, without isomeric appendages is that the common atrial chamber, albeit with two
Pulmonary veins
Sup.
Post. Ant.
Fig. 6.6 As shown in this computed tomogram, the pectinate
muscles of the morphologically left appendage are confined within
Pectinate muscles
Inf. the tubular component, which has a very narrow junction with the
body of the atrium (black double-headed arrow).
Usual Mirror-imaged
Fig. 6.7 The cartoon shows the four possible arrangements of the
atrial appendages, which cannot always be distinguished on the
basis of their shape. The best means of distinguishing between
them is to establish the extent of the pectinate muscles. These
muscles extend all the way to the crux in the morphologically right
atrial appendage, but are confined around the mouth of the
appendage in the morphologically left atrial appendage, leaving a
smooth posterior vestibule. Using this criterion, all congenitally
malformed hearts have appendages fitting within one of the four
Isomeric right Isomeric left
groups shown in the cartoon.
isomeric appendages, and common inferior caval vein, with continuation of the junctions. For this, the surgeon needs to
atrioventricular valves. Right isomerism is venous drainage from the abdomen know how the atrial chambers are, or are
always associated with a totally anomalous through the azygos system of veins. not, connected to the chambers present
pulmonary venous connection, even if the within the ventricular mass. Most often,
pulmonary veins are joined to one or other there are two such chambers, which can be
atrium. It is also seen most frequently with THE ATRIOVENTRICULAR of only right or left morphology. The
pulmonary stenosis or atresia, and in JUNCTIONS morphological distinction is based on the
association with a univentricular nature of the apical trabeculations. In the
atrioventricular connection, typically a Having established the arrangement of the morphologically right ventricle, these
double-inlet ventricle through a common atrial appendages, the next step in trabeculations are coarse, in contrast to the
valve. Left isomerism, in the majority of sequential analysis is to determine the fine criss-crossing trabeculations that
cases, is associated with interruption of the morphology of the atrioventricular characterise the morphologically left
Left-sided morphologically right appendage
Left
Fig. 6.8 This surgical view through a median sternotomy
shows a left-sided atrial appendage of morphologically right
pattern. The right-sided appendage is also of right morphology,
Sup. Inf.
so the patient has isomerism of the right atrial appendages. Note
the crest of the appendage in relation to the left superior caval vein
Right and the terminal groove. When examined internally, pectinate
muscles encircled both atrioventricular junctions.
Left
Sup. Inf.
Right
Fig. 6.9 This surgical view through a median sternotomy shows a

right-sided atrial appendage of morphologically left pattern. The
left-sided appendage is also of left morphology, so the patient has
isomerism of the left atrial appendages. Note the absence of any
Right-sided morphologically left appendage terminal groove. Internal inspection confirmed the presence of
smooth bilateral posterior vestibules.
ventricle (Figure 6.10). It is the septal surface of the morphologically right morphology, it is possible to determine the
interrelationships between the two ventricle such that the thumb is within the way in which the atrial chambers are
ventricles that permit the description of inlet component, and the fingers in the connected, or not connected, to the
ventricular topology. When analysed ventricular outlet, the patterns reflect ventricular mass. In most instances, there
according to the way that the palmar either right-hand or left-hand topology are two atrioventricular junctions, although
surfaces of the hands can be placed on the (Figure 6.11). Having assessed ventricular one of the junctions can be absent. Also
Ant. Coarse right

Left
ventricular
trabeculations
Post. Right
Fig. 6.10 The apical part of the normal ventricular mass has been
amputated, and is viewed from above. It shows the marked
difference between the fine apical trabeculations of the
Fine left ventricular trabeculations morphologically left ventricle when compared to the coarse right
ventricular apical trabeculations.
Fig. 6.11 The cartoon shows how the

patterns of ventricular topology can be
described, figuratively speaking, in terms of
the way that the palmar surface of the hands
can be placed on the septal surface of the
morphologically right ventricle. The fingers
point up the outlet, and the thumb lies in the
inlet, giving right-hand and left-hand patterns.
In the arrangements shown, the
atrioventricular connections are concordant,
but the ventriculoarterial connections can also
be discordant, with the aorta arising from the
morphologically right ventricle (see
Right-hand topology Left-hand topology Figure 6.29).
important is the morphology of the valves intriguing further variation. Most often, represents discordant connections, each
that guard the atrioventricular junctions, the atrial chambers are connected to their atrium is connected with a morphologically
because the paired junctions can be morphologically appropriate ventricles. inappropriate ventricle.
guarded by a common atrioventricular This pattern is called concordant Concordant and discordant
valve. This shows that junctional and valvar atrioventricular connections. When each connections can exist with either the usual
morphology are separate and independent atrium is connected in this way to its own or mirror-imaged arrangement of the
features. ventricle, there is rarely any difficulty in atrial appendages (Figure 6.12), but not
There are five distinct and discrete ways distinguishing the morphology of the with isomeric appendages. When the
in which the atrial chambers may be ventricles, even when the ventricles appendages are isomeric, and each atrium
connected to the ventricular mass, the final themselves are unusually related one to the is connected to its own ventricle, then of
one having two subtypes, along with an other. In the second pattern, which necessity one junction will be
Usual arrangement Mirror-imaged pattern
Concordant Concordant
Fig. 6.12 The cartoon shows how the atrial chambers can be
connected to the ventricles in concordant or discordant fashion,
Discordant Discordant with each pattern existing in usual and mirror-imaged variants.
Right isomerism Left isomerism
Right-hand topology Right-hand topology
Fig. 6.13 The cartoon demonstrates the mixed and biventricular

atrioventricular connections found when there are isomeric
atrial appendages, and each atrium is connected to its own
ventricle. In each pattern, half of the heart is concordantly
connected, and the other half is discordant. It is essential in these
settings, therefore, to describe both the type of isomerism, and the
Left-hand topology Left-hand topology specific ventricular topology.
concordantly connected, but the other ventricular mass (see later). The In the three connections described
junction will be discordantly connected arrangement produces a third discrete thus far, each atrium is connected to its
(Figure 6.13). This will occur irrespective pattern, namely biventricular and mixed own ventricle. This means that the
of the topological pattern of the atrioventricular connections. atrioventricular connections themselves
are biventricular. The essential feature in of the atrioventricular connections is described as having univentricular hearts,
the remaining two types of absent, be it right-sided or left-sided, the ventricular mass contains more than
atrioventricular connection is that the namely when the atrioventricular valve one chamber. By focusing on the fact that
atrial chambers, with one exception, guarding the solitary connection straddles the atrioventricular connection is, in
connect to only one ventricle. In one of the septum, being attached in both reality, joined to only one ventricle, we are
these patterns, both atrial chambers ventricles. The end result is a uniatrial, able to achieve a satisfactory solution for
connect to the same ventricle. This is a but biventricular, atrioventricular this dilemma. Thus, the hearts can logically
double-inlet atrioventricular connection connection (Figure 6.17). and accurately be described in terms of
(Figure 6.14). In the other variant, one of There has been much controversy being functionally univentricular. It
the atrial chambers is connected to a concerning the description of the hearts follows that, in some patients with
ventricle, but the other atrium has no in which the atrial chambers connect to biventricular atrioventricular connections,
connection with the ventricular mass. only one ventricle. It became conventional imbalance between the ventricles can again
This latter arrangement can be divided to describe them in terms of single produce a functionally univentricular
into two subtypes, depending on whether ventricle, common ventricle, or arrangement13. In those with
absence of the connection is right-sided univentricular hearts. It is exceedingly rare, univentricular atrioventricular
(Figure 6.15) or left-sided (Figure 6.16). however, to find patients with solitary connections, however, one of the ventricles
An intriguing variation is seen when one ventricles. Almost always, in patients must be incomplete, while the other
Left atrium
Right atrium
Dominant left ventricle
Sup.
Right Left
Fig. 6.14 When both atriums are connected to only one ventricle,
the atrioventricular connection is univentricular. In this heart,
showing a four-chamber section in anatomical orientation, there is
Inf.
a double inlet to a dominant left ventricle. The black braces show
the segments of atrial vestibular myocardium, connected to the
dominant left ventricle through separate atrioventricular valves.
Sup.
Right Left
Left atrium
Inf.
Fig. 6.15 This anatomical specimen, seen in

four-chamber orientation, shows the absence
Right atrium of the right atrioventricular connection, with
the fibroadipose tissue of the right
atrioventricular (AV) groove interposing
between the right atrial floor and the base of
the ventricular mass. This arrangement
produces another form of univentricular
atrioventricular connection. In this case, the
morphology is that of classical tricuspid atresia,
with the left atrium connected to a dominant
Right AV groove left ventricle. Note the presence of the base of
the incomplete right ventricle (arrow), which
has no connection with the atrial chambers.
Left atrium
Right atrium
Sup. Dominant left ventricle
Fig. 6.16 This anatomical specimen, again seen in four-chamber

Right Left orientation (compare with Figures 6.14 and 6.15), shows the
absence of the left atrioventricular connection (red dotted line),
giving the third variant of univentricular atrioventricular
Inf.
connection. In this example, the right atrium is connected to a
dominant left ventricle through a right-sided atrioventricular valve.
ventricle is dominant. The dominant apical trabecular component, the dominant incomplete right ventricles are always
ventricle, which supports the ventricle will be morphologically left. found anterosuperiorly relative to the
atrioventricular junction or junctions, can There will be a complementary right dominant left ventricle, irrespective of
take one of three morphologies: right, left, ventricle, perforce incomplete because it whether there is a double inlet, or an absent
or indeterminate (Figure 6.18). Most will lack its atrioventricular connection, right or absent left atrioventricular
frequently, as judged from the pattern of its and hence its inlet component. Such connection. They can, however, be
Straddling & overriding AV valve
LA
RA
RV
LV Fig. 6.17 This cartoon illustrates the arrangement when there is an

absence of the right atrioventricular (AV) connection, but with the
solitary atrioventricular valve straddling the ventricular septum. This
produces a uniatrial but biventricular atrioventricular connection,
Absent AV connection shown here in the setting of a usual atrial arrangement with right-
hand ventricular topology. RA, LA, right and left atriums; RV, LV,
right and left ventricles.
Usual Mirror-imaged Right isomerism Left isomerism
Fig. 6.18 This cartoon shows the multiple possibilities for

univentricular atrioventricular connection that can be produced
by combining the variations in atrial morphology, atrioventricular
connection, and ventricular morphology. It takes no account of
the further variations possible according to ventricular
Dominant left with Solitary and Dominant right relationships, ventriculoarterial connections, etc. The arrows in
incomplete RV indeterminate with incomplete LV the middle row emphasise the absence of one of the
ventricle atrioventricular connections. LV, RV, left and right ventricles.
positioned either to the right (Figure 6.19), connection (Figure 6.21), but can be found connection and its inlet portion. The
or to the left (Figure 6.20) relative to the with a double inlet or, rarely, with an incomplete left ventricle will always be
dominant ventricle. absence of the right atrioventricular found in a posteroinferior position. Usually
More rarely, the atrial chambers can be connection. When only the right ventricle it is left-sided, although rarely it can be
connected to a dominant right ventricle. is connected to the atrial chambers, and right-sided.
This happens most frequently in the hence dominant, it is the left ventricle that The third morphological
absence of the left atrioventricular is incomplete, lacking its atrioventricular configuration found with a double inlet
Sup.
Aorta
Right Left
Inf.
VSD
Fig. 6.19 In this anatomical specimen with a double-inlet left

ventricle, the incomplete right ventricle is positioned
Incomplete right ventricle anterosuperiorly and to the right of the dominant left ventricle. VSD,
ventricular septal defect.
Sup.
Right Left
Aorta
Inf.
VSD
Fig. 6.20 This anatomical specimen, again with a double-inlet left

ventricle, has the incomplete right ventricle in an anterosuperior
Incomplete right ventricle and leftward position relative to the dominant left ventricle. VSD,
or, exceedingly rarely, with an absence of ventricles have indeterminate apical univentricular atrioventricular
either atrioventricular connection, is trabecular morphology (Figure 6.22). connection, in which the only septal
when the atrial chambers connect to a Incomplete second ventricles are structure in the ventricle is that
solitary ventricle. Such solitary never found in this variant of separating the outflow tracts.
Incomplete left ventricle

Left
atrium
Right
atrium
Dominant right ventricle
Sup. Fig. 6.21 This section through a specimen,

shown in four-chamber orientation, illustrates
Left the absence of the left atrioventricular
Right
connection (red dotted line) with the right
atrium connected to a dominant right
Inf. ventricle. This produces one of the variants of
the hypoplastic left heart syndrome.
To pulmonary trunk Coarse apical

trabeculations
Left AV
valve
To aorta
Sup.
Right AV
valve Fig. 6.22 This heart, opened in
Right Left clam-shell-like fashion, has a double inlet to,
and double outlet from, a solitary and
indeterminate ventricle, the ventricle itself
Inf. having very coarse apical trabeculations. AV,
atrioventricular.
feature of the atrioventricular junctions. guarded by two separate atrioventricular

VALVAR MORPHOLOGY When there are concordant, discordant, valves (Figure 6.23), or by a common valve
The atrioventricular valvar morphology is mixed, or double-inlet connections, then (Figure 6.24). When there are two valves,
independent of the way in which the atrial both atrial chambers are connected, actually either of them can be imperforate, blocking a
chambers connect with the ventricles. Valvar or potentially, to the ventricular mass. The potential atrioventricular connection. An
morphology, therefore, constitutes a separate two atrioventricular junctions can be imperforate valve, therefore, needs to be
Base
Right Left Fig. 6.23 This normal heart, sectioned in

four-chamber orientation, shows the right and
left atrioventricular junctions (black dotted
Apex
lines with double-headed arrows) guarded by
separate atrioventricular valves.
Left
atrium
Right
atrium
Left
ventricle
Sup. Right
ventricle
Right Left Fig. 6.24 This section, again in four-chamber orientation

(compare with Figure 6.23), shows that the right and left
atrioventricular junctions (black dotted line with double-headed
Inf.
arrow) are guarded by a common atrioventricular valve in the
setting of an atrioventricular septal defect.
distinguished from absence of an the atrioventricular connection has formed, atrium involved is completely separated
atrioventricular connection, as either can but is blocked by the conjoined valvar from the ventricular mass by the
produce atrioventricular valvar atresia. leaflets (Figure 6.25). In the setting of fibroadipose tissue of the atrioventricular
The essence of the imperforate valve is that absence of the connection, the floor of the groove (see Figures 6.15, 6.16).
Sup.
Right Left
Left Inf.
atrium

Right
atrium
Fig. 6.25 This section of a heart, cut in four-chamber orientation,

shows an imperforate right atrioventricular valve connecting to
a hypoplastic right ventricle. The atrioventricular connections
Hypoplastic right ventricle remain concordant, even though the right valve (arrow) is
imperforate.
Left atrium
Right atrium
Base
Fig. 6.26 This section of a heart, seen in four-chamber
Right Left orientation, shows how the tension apparatus of the right
atrioventricular valve (arrows) is attached to both sides of the
ventricular septum (star), while the orifice of the right
Apex atrioventricular junction (white brace) is overriding the septal
crest (star).
Either of two valves, or a common attached to both sides of the ventricular in the presence of overriding, the overriding
valve, can also straddle a septum within the septum (Figure 6.26). Overriding is present valve is assigned to the ventricle
ventricular mass. Straddling of the tension when the junction is connected to both connected to its greater part (Figure 6.27).
apparatus of a valve should be distinguished ventricles. The degree of override, which The possible arrangements are much
from overriding of its supporting usually coexists with straddling, determines more limited when one atrioventricular
atrioventricular junction. Straddling exists the precise atrioventricular connection connection is absent. In this situation, the
when the valvar tension apparatus is present. So as to adjudicate the connection solitary valve can either be committed in its
Fig. 6.27 In the presence of an

overriding junction, whether it is a straddling
RA LA RA LA tricuspid valve as shown in Figure 6.26 or
ventriculoarterial, the overriding junction
(black braces) is assigned to the ventricle
supporting its greater part. In the situation
illustrated with the straddling tricuspid
valve, the atrioventricular connections are
RV
defined accordingly. There is a spectrum
between the illustrated extremes (double-
RV LV headed arrow). The left-hand panel shows the
LV
situation with the junction connected primarily
to the right ventricle (large arrow), the lesser
part joining the left ventricle (small arrow).
Hence, the atrioventricular connections are
Straddling Straddling tricuspid deemed to be concordant. In the right-hand
tricuspid valve valve with double- panel, the larger part of the overriding
concordant AV inlet left ventricle junction is committed to the left ventricle, so
connections that the connection is deemed to be a double
inlet. This is the essence of the 50% rule. RA,
LA, right and left atriums; RV, LV, right and left
ventricles.
entirety to one ventricle, or it can straddle or left appendage, can be connected to conduction tissues11. When the atrial
and override. When the valve straddles and either a morphologically right or a chambers connect to only one ventricle, the
overrides in this setting, then the morphologically left ventricle (see morphology of that ventricle must always
atrioventricular connection itself is Figure 6.13). When there is right be described. This is because the dominant
uniatrial but biventricular (Figure 6.17). isomerism, and the right-sided atrium is ventricle can be of left ventricular, right
connected to a morphologically right ventricular, or solitary and indeterminate
ventricle, the ventricular mass typically is pattern. It is also necessary to describe the
seen as in hearts with concordant relationships of the dominant and
VENTRICULAR MORPHOLOGY
atrioventricular connections and the usual incomplete ventricles.
AND TOPOLOGY
atrial arrangement. In contrast, when there
The nature of the atrioventricular is right isomerism, and the right-sided
connections is inextricably linked with the atrium is connected to a morphologically
VENTRICULAR RELATIONSHIPS
architectural arrangement of the left ventricle, the ventricular mass is
ventricular mass. Biventricular usually found in the presence of discordant Ventricular relationships, as opposed to
atrioventricular connections, for example, atrioventricular connections and the usual ventricular topology, generally should be
cannot be diagnosed without knowledge of atrial arrangement. described as a separate feature of the heart.
ventricular morphology. Double-inlet and As we have discussed already, these two Where each atrium is connected to its own
absent connections can all be identified basic patterns of ventricular topology can ventricle, the relationships are almost
without mention of ventricular conveniently be described according to the always in harmony with both the
morphology, although in this setting it is way in which the hands, figuratively connection and topology present. When
always necessary to give more information speaking, can be placed palm downwards the atrial chambers are in their usual
concerning the arrangement of the upon the septal surface of the position with concordant atrioventricular
ventricular mass. In the case of mixed and morphologically right ventricle. The other connections, the relationships described in
biventricular atrioventricular connections, hand will then fit in the morphologically the setting of the heart within the chest are
it is important to describe the pattern in left ventricle in similar fashion, but it is the almost always for the morphologically right
which the morphologically right ventricle arrangement of the morphologically right ventricle to be right-sided, anterior, and
is structured relative to the ventricle that is chosen for the purposes of inferior to the morphologically left
morphologically left ventricle. This feature description (Figure 6.11). In the hearts ventricle. In mirror-imaged atrial
can take only one of two topological with biventricular and mixed arrangement, with concordant
arrangements. This is because, when the atrioventricular connections, it is this atrioventricular connections, the
connections are mixed, the right-sided ventricular topology that determines the morphologically right ventricle is almost
atrium, with either a morphologically right disposition of the atrioventricular invariably left-sided and relatively anterior,
Sup.
Aorta
Ant. Post.
Inf.
Outlet
Inlet Fig. 6.28 The left side of the heart is shown from a patient with
discordant atrioventricular and ventriculoarterial connections; in
other words, congenitally corrected transposition. It is the palmar
surface of the left hand only that can be placed on the septal
surface of the morphologically right ventricle so that the fingers
Morph. right apical trabeculations occupy the ventricular outlet, and the thumb goes in the tricuspid
valve guarding the inlet component.
Morph. left atrium

Aorta
Outlet
Inlet
Fig. 6.29 The computed tomogram shows the right side of the
Apical trabecular component heart from a patient having congenitally corrected transposition in
Sup.
the setting of a mirror-imaged atrial arrangement. The
morphologically left atrium is right-sided, but connects to a
Post. Ant. morphologically right ventricle with right-hand topology, the
palmar surface of the right hand fitting on the septal surface so
Inf. that the fingers are in the subaortic outlet and the thumb in the
inlet component.
but frequently more side-by-side relative to right ventricle to be left-sided (Figure 6.28). tend to be more side-by-side. When the
its neighbour. When discordant atrioventricular relationships are as anticipated, it is
With the atrial chambers in their usual connections accompany mirror-imaged atrial unnecessary to describe them. Very
arrangement, and discordant atrioventricular arrangement, there is usually right-hand occasionally, the relationships of the
connections, there is almost always the left- topology (Figure 6.29). The ventricular ventricles are not as anticipated for the
hand pattern of ventricular topology. The relationships are thus similar to those seen in connections present. This disharmony
usual relationship is for the morphologically the normal heart, although the two chambers between connections and relationships
Fig. 6.30 This cartoon shows the patterns

that identify the morphology of the arterial
trunks. When there is absence of the
intrapericardial pulmonary arteries, there is no
way of knowing if, had there been an atretic
pulmonary trunk, it would have originated
from the base of the heart or from the aorta.
Aorta Pulmonary Common Solitary This pattern, therefore, is best described as a
trunk arterial trunk arterial trunk solitary arterial trunk.
underscores the anomaly known as the criss- ventricular relationships. All are of arteries, or it may be an aortic or pulmonary
cross heart14. With these hearts, and also importance to the surgeon because they trunk when the complementary arterial
those with superoinferior ventricles, influence the disposition of the trunk is atretic, and its connection to a
connections and relationships must be atrioventricular conduction axis. known ventricle cannot be established
described separately, using as much detail as (Figure 6.30). Rarely, in the absence of
is necessary to achieve unambiguous intrapericardial pulmonary arteries, it may
categorisation. The essence of the criss-cross VENTRICULOARTERIAL be more accurate to describe an arterial
heart, and those with superoinferior JUNCTIONS trunk as solitary rather than common
ventricles, is that the ventricular (Figure 6.30).
relationships are not as expected for the Analysis of the ventriculoarterial
atrioventricular connection present. Even junctions proceeds as described for the
atrioventricular junctions, with the Arterial valvar morphology
more rarely, the ventricular topology may be
disharmonious with the atrioventricular morphology of the connections, the The morphological arrangement of the
connection15. All features must then be valvar morphology, and the relationships arterial valves is limited because they have
described. of the arterial trunks being different no tension apparatus. Furthermore, a
In hearts with a univentricular facets requiring separate description in common valve can exist only with a
atrioventricular connection, it is the mutually exclusive terms. It is also common trunk. The different patterns,
relationship of the incomplete ventricle to necessary to take account of infundibular therefore, involve one or two arterial
the dominant ventricle that must be morphology. valves. Usually both valves are perforate,
described. When the left ventricle is but either or both may override the
dominant, the incomplete right ventricle is ventricular septum. When a valvar orifice is
always anterosuperior, but can be right- or
Ventriculoarterial connections overriding, the valve is assigned to the
left-sided. The sidedness of the ventricle There are four discrete ways in which the ventricle supporting its greater part, thus
does not affect the basic disposition of the arterial trunks can take their origin from avoiding the need for intermediate
atrioventricular conduction tissues in these the ventricular mass; namely, in categories. The other pattern of valvar
hearts. With a dominant right ventricle, the concordant, discordant, double-outlet, and morphology is when one of the arterial
incomplete and rudimentary left ventricle, single-outlet fashion. Concordant valves is imperforate. As with the
if present, is always posteroinferior, but ventriculoarterial connections exist when atrioventricular junctions, an imperforate
again can be right- or left-sided. In this the arterial trunks arise from arterial valve must be distinguished from
case, the sidedness of the incomplete morphologically appropriate ventricles. absence of one ventriculoarterial
ventricle will affect the disposition of the Discordant connections account for the connection, as both can produce arterial
atrioventricular conduction tissue. trunks being connected with valvar atresia.
When considering the atrioventricular morphologically inappropriate ventricles.
junctions, therefore, there are four Double-outlet connections exist when both
Infundibular morphology
different features to take into account. great arteries take origin from the same
These are, firstly, the way the atrial ventricle, which may be of right, left, or Describing the morphology at the
myocardium is connected to the ventricular indeterminate morphology. A single-outlet ventriculoarterial junctions also involves
mass; secondly, the morphology of the arrangement is seen when only one arterial the arrangement of the musculature within
atrioventricular valves guarding the trunk is connected to the heart. This may the ventricular outflow tracts. This is
junctions; thirdly, the ventricular be a common trunk, directly supplying the infundibular morphology. Although the
morphology and topology; and finally, the systemic, pulmonary, and coronary outlet regions are integral parts of the
Outlet Pulmonary
septum valve
Aortic
valve
SMT
Sup.
Fig. 6.31 This view of a heart with a double-outlet right ventricle,
seen in anatomical orientation, and with a non-committed
Ventriculoinfundibular fold Right Left
interventricular communication, shows the muscular components
of the ventricular outflow tracts. The hinges of both arterial valves
Interventricular communication Inf. are completely surrounded by outflow musculature. SMT,
septomarginal trabeculation.
Outlet septum Pulmonary valve

Parietal
attachment
Septal
attachment
Sup.
Fig. 6.32 The image shows the outflow tracts in a heart with
Post. Ant. tetralogy of Fallot, viewed from the apex of the right ventricle,
Ventriculoinfundibular fold with the aortic valve (star) overriding the crest of the muscular
ventricular septum, which is reinforced by the septomarginal
Inf. trabeculation, or septal band (white Y). The septal and parietal
attachments of the muscular outlet septum are well seen.
ventricular mass, it is traditional to which has parietal and septal components, anterior free wall of the outflow tracts. The
consider them in concert with the great along with a component adjacent to the septal component is the outlet, or
arteries. The two outflow tracts together atrioventricular junction (Figure 6.31). infundibular, septum. This has a body,
make a complete cone of musculature, The parietal components make up the with septal and parietal insertions, best
Outlet
septum
Aortic
valve
Sup. Fig. 6.33 In this heart with tetralogy of Fallot, the

ventriculoinfundibular fold (red dotted line) interposes between
Post. Ant. the leaflets of the aortic and mitral valves in the roof of the
Ventriculoinfundibular fold interventricular communication (star), producing a completely
muscular subaortic infundibulum. SMT, septomarginal
Inf. SMT
trabeculation.
Outlet Pulmonary
Aortic septum valve
valve
Tricuspid
valve
Sup.
Post. Ant. Fig. 6.34 This heart has the ventriculoarterial connection of a
double-outlet right ventricle, but with fibrous continuity between
Inf. the leaflets of the aortic and tricuspid valves (red double-headed
arrow).
seen in the setting of tetralogy of Fallot give arterial-atrioventricular valvar fibrous band. This latter structure is part of the
(Figure 6.32). The component adjacent to continuity (Figure 6.34). ventricular septum, reinforcing its right
the atrioventricular junction is the The infundibular structures never ventricular aspect. It has a body that
ventriculoinfundibular fold. It separates contain or overlie conduction tissue17. continues apically as the moderator band,
the leaflets of arterial from atrioventricular They should be distinguished from and two limbs (Figure 6.35). The
valves (Figure 6.33). It is the inner heart another structure, namely the anterocephalad limb, in the normal
curvature16. The fold may be attenuated to septomarginal trabeculation, or septal heart, extends to the pulmonary valve,
Supraventricular crest Pulmonary

valve
Tricuspid
Sup. valve Fig. 6.35 The septal surface of the normal right ventricle is
photographed to show the extent of the septomarginal
trabeculation, or septal band, as shown by the white Y. In the
Right Left
normal heart, the supraventricular crest inserts between the limbs
of the trabeculation. The star shows the moderator band, one of
Inf. the series of septoparietal trabeculations that take origin from the
anterior surface of the marginal trabeculation.
overlying the outlet septum. The In the presence of a common arterial

posterocaudal limb runs beneath the trunk, there may be a complete muscular POSITION OF THE HEART
interventricular membranous septum. subtruncal infundibulum, but more often The system discussed in this chapter
This posterocaudal limb usually overlies there is truncal-atrioventricular valvar establishes the cardiac template.
the branching part of the atrioventricular continuity. Irrespective of the internal architecture, it
bundle, with the right bundle branch is well known that the heart itself can
passing down to the apex of the right occupy many and varied positions (see
Arterial valvar and truncal
ventricle within the body of the Chapter 10), particularly when there
relationships
trabeculation. are complex intracardiac malformations.
Although each outflow tract is The final feature of consideration at the To describe the position of the heart in
potentially a complete muscular structure, ventriculoarterial junctions is the unambiguous fashion, account should
in most hearts it is only the outflow tract of relationship of the arterial valves and be taken separately of its site within
the right ventricle that is a complete arterial trunks. Valvar relationships are the thorax, and the orientation of its
muscular cone. This is because, within the independent of both ventriculoarterial apex. We describe the heart as being in
left ventricle, part of the connections and infundibular the left or right side of the chest, or in
ventriculoinfundibular fold is usually morphology. Of the many methods of the midline. Apical orientation is described
attenuated to permit fibrous continuity description, our preference is to describe as being to the left, to the middle, or to
between the leaflets of the arterial and the aortic relative to the pulmonary valve the right.
atrioventricular valves. In the normal as viewed from below in right–left,
heart, therefore, there is a muscular anteroposterior and, when necessary,
subpulmonary infundibulum in the right superoinferior coordinates. This can be
CATALOGUE OF
ventricle, and fibrous valvar continuity in done as precisely as required. In our
MALFORMATIONS
the roof of the left ventricle. In experience, eight coordinates combining
congenitally malformed hearts, three lateral and anterior to posterior positions Having described the template of the heart,
other patterns may be found. These are, suffice (Figure 6.36). When describing the and its position, finally it is necessary to
firstly, a muscular subaortic infundibulum relationship of the arterial trunks, it is catalogue all intracardiac malformations. In
with pulmonary-atrioventricular sufficient to account for trunks that spiral most cases, it is these lesions which will
continuity; secondly, a bilaterally around each other as they ascend, and to require surgical attention. Any lesion,
muscular infundibulum; and thirdly, distinguish them from trunks that ascend nonetheless, cannot be presumed to be the
bilateral deficiency of the infundibulums. in parallel fashion18. only lesion present until the rest of the
anterior
right left
anterior anterior
right left
side-by-side side-by-side
Pulmonary trunk
right
left
posterior
posterior
‘normal’
Fig. 6.36 The cartoon shows the combination of anterior to

posterior, and right to left coordinates that are used to describe the
interrelationships of the aortic and pulmonary valves at their
posterior origins from the ventricular mass.
heart has been established as normal. It is venoatrial connections in hearts with 11. Smith A, Ho SY, Anderson RH, et al. The
these associated lesions that will be patients with visceral heterotaxy. Ann diverse cardiac morphology seen in hearts
emphasised in subsequent chapters, taking Thorac Surg 1995; 60: 561–569. with isomerism of the atrial appendages
particular note, as before, of the features of 6. Van Mierop LHS, Gessner IH, Schiebler with reference to the disposition of the
GL. Asplenia and polysplenia syndromes. specialized conduction system. Cardiol
surgical significance.
In: Bergsma D (ed). Birth Defects: Original Young 2006; 16: 437–454.
Article Series, Vol VIII, No 5. The Fourth 12. Ho SY, Seo J-W, Brown NA, et al.
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Lesions with
7
normal segmental
connections
Normal segmental connections 151
triangle of Koch (Figure 7.3). In the past, element, specifically the interventricular
SEPTAL DEFECTS we considered this component of the atrial part of the membranous septum, and a much
Understanding the anatomy of septal wall, which overlaps the upper part of the larger muscular part. The muscular part,
defects is greatly facilitated if the heart ventricular musculature between the which is significantly curved, is more
is thought of as having three distinct attachments of the leaflets of the tricuspid complex geometrically than the other septal
septal structures: the atrial septum, and mitral valves, as the muscular structures, which lie almost completely in
the atrioventricular septum, and the atrioventricular septum. As we discussed in the coronal plane. At first sight, it seems
ventricular septum (Figure 7.1). The Chapter 2, we now know that it is better possible to divide the muscular septum into
normal atrial septum is relatively small. It is viewed as a sandwich1. This is because, inlet, apical trabecular, and outlet
made up, for the most part, by the floor throughout the floor of the triangle of components, each of these parts seemingly
of the oval fossa. When viewed from the Koch, the fibroadipose tissue of the inferior corresponding with the components of the
right atrial aspect, the fossa has a floor, atrioventricular groove separates the layers right ventricle, and abutting centrally on the
surrounded by rims. The floor is derived of atrial and ventricular myocardium membranous septum (Figure 7.5). Closer
from the primary atrial septum, or septum (Figure 7.4). From the stance of inspection shows that such analysis is
primum. Although often considered to understanding septal defects, nonetheless, simplistic. By virtue of the deeply wedged
represent a secondary septum, or septum it is helpful to consider the entire area location of the subaortic outflow tract, much
secundum, the larger parts of the rims, comprising the fibrous septum and the of the septum delimited on the right
specifically the superior, anterosuperior, muscular sandwich as an atrioventricular ventricular aspect by the septal leaflet of
and posterior components, are formed by separating structure, as it is absent in the the tricuspid valve separates the inlet of the
infoldings of the adjacent right and left hearts we describe as having right ventricle from the outlet of the left
atrial walls. Inferoanteriorly, in contrast, atrioventricular septal defects. (Figure 7.6). The muscular wall forming the
the rim of the fossa is a true muscular The ventricular septum is usually seen by back of the subpulmonary infundibulum is,
septum (Figure 7.2). This part of the rim is the surgeon only from its right ventricular at first sight, an outlet septum. Only a
contiguous with the atrioventricular aspect. For this and other reasons we will small part of this wall, however, interposes
septum, which is the superior component discuss, holes between the ventricles are best between the cavities of the right and left
of the fibrous membranous septum. In the considered in terms of their right ventricular ventricles. This is because most of the
normal heart, this fibrous septum is also landmarks. Taken overall, the ventricular subpulmonary infundibulum is a free-
contiguous with the atrial wall of the septum is made up of a small fibrous standing muscular sleeve, which forms part
Atrial septum
Ventricular septum Fig. 7.1 The four-chamber section through the heart, in
anatomical orientation, shows the atrial and ventricular septal
components, along with the location of the muscular structures
that separate the cavities of the right atrium and left ventricle. We
had previously considered the latter area to be an atrioventricular
Sup.
(AV) muscular septum. We now recognise it to be a sandwich of
fibroadipose tissue, located between the layers of atrial and
Right Left ventricular musculature (see Figure 7.3). It is this area that is
deficient in the setting of a common atrioventricular junction. Note
AV separating structures
Inf. also that the superior rim of the oval fossa (arrow) is an infolding
between the right and left atrial walls.
Floor of oval fossa
Sup. Fig. 7.2 The heart has been sectioned in the four-chamber plane,
showing that the superior rim of the oval fossa is a deep
Right Left infolding (arrow) between the origin of the superior caval vein
from the right atrium (red star), and the entry of the right superior
Inf. pulmonary vein into the left atrium (white star). It is the floor of
Anteroinferior muscular buttress the oval fossa, along with the anteroinferior muscular buttress,
which are the components of the atrial septum.
Triangle of Koch Tricuspid valve
Apex
Sup. Inf.
Base
Defect in oval fossa Fig. 7.3 The surgical view, through a right atriotomy, shows the
landmarks of the triangle of Koch (triangle). This area is the atrial
Coronary sinus aspect of the muscular atrioventricular sandwich. Note that this
patient also has a defect within the oval fossa.
of the supraventricular crest (Figure 7.7). ventriculoinfundibular fold, or inner heart septum. This part extends to the apex in
The small septal component interposing curvature (Figure 7.8). It is the muscular curvilinear fashion, reflecting the
between the ventricular outlets is wall separating the apical trabecular interrelationships of the banana-shaped
inextricably linked with the more extensive components, therefore, which forms the right ventricle and the conical left ventricle.
component of the crest, the greater part of the muscular ventricular Reinforcing the right ventricular aspect of
Floor of oval fossa

Anteroinferior buttress
Sup.
Right Left
Inf.
Adipose tissue
Fig. 7.4 This four-chamber section of a normal heart, taken across

the floor of the triangle of Koch, illustrates the differential
attachments of the atrioventricular valves (arrows). Note the
adipose tissue interposed between the right atrial wall and the
crest of the ventricular septum, which forms the ‘meat’ in the
atrioventricular muscular sandwich.
Base
Ant. Post.
Outlet component
Apex
Apical trabecular
component
Fig. 7.5 The dissection, seen in anatomical
orientation, shows how the ventricular septum
can be separated from the remainder of the
Inlet component
heart, and divided into fibrous and muscular
parts. The muscular part has been further
divided into segments corresponding to the
components of the right ventricle. Apart from
the apical part, however, these parts of the
right ventricle do not correlate with
Membranous septum comparable components on the left side of
the heart.
this part of the septum is the septomarginal supraventricular crest. A series of moderator band, is particularly prominent.
trabeculation, or septal band. This muscular septoparietal trabeculations extend from its It crosses from the septomarginal
strap has a body and limbs, the latter anterocephalad surface and reach the trabeculation to join the anterior papillary
extending to the base of the heart to clasp the parietal ventricular wall. One of these, the muscle (Figure 7.9).
Left ventricular outlet
Base
Fig. 7.6 The section, simulating the oblique

Right Left subcostal echocardiographic cut, shows how
the inferoposterior part of the muscular
Right ventricular inlet septum separates the right ventricular inlet
Apex from the left ventricular outlet (double-headed
arrow).
Infundibular sleeve
Pulmonary trunk
Base
Ant. Post. Fig. 7.7 The dissection of the ventricular outflow tracts, in
anatomical orientation, shows the free-standing sleeve of
infundibulum that supports the leaflets of the pulmonary valve.
Aortic root Apex This is not a septal structure. Note the extensive tissue plane that
separates the infundibular sleeve from the aortic root (arrow).
Infundibular sleeve
Pulmonary valve
Aortic root
Sup.
Post. Ant.
Inf.
Fig. 7.8 The dissection, seen in anatomical orientation, shows

how most of the supraventricular crest is formed by the
ventriculoinfundibular fold. Only a small part of crest, where it
inserts between the limbs of the septomarginal trabeculation
(star), can be removed so as to provide a communication with the
left ventricle. The area has no obvious anatomical boundaries.
Ventriculoinfundibular fold Septomarginal trabeculation Note that the distal part of the crest becomes continuous with the
free-standing muscular infundibular sleeve.
Supraventricular crest Septoparietal

trabeculations
Moderator band
Fig. 7.9 The septal surface of the right ventricle has been
displayed by making a window in the anterior wall to show the
septomarginal trabeculation, or septal band (black Y). The
Base
supraventricular crest inserts between its basal limbs. The
moderator band takes origin from the apical part of its body,
Ant. Post.
crossing the cavity of the ventricle to become continuous with the
Apex anterior papillary muscle. The band is but one of a series of
Anterior papillary muscle septoparietal trabeculations that arise from the anterior margin of
the major septomarginal trabeculation.
Interatrial communications
the normal atrial septum1. Only the deficiencies of the septal components
There are several lesions that permit holes within the floor of the oval fossa2, (Figure 7.2). The ostium primum
interatrial shunting (Figure 7.10). and the much more rare vestibular defect is the consequence of deficient
Although collectively termed atrial septal defects found within the muscular atrioventricular septation. Its cardinal
defects, not all are within the confines of anteroinferior buttress3, are true feature is the commonality of the
Vestibular
Atrioventricular defect
septal defect
Apex
Sup. Inf. Coronary

sinus defect
Base
Superior sinus
Fig. 7.10 The cartoon shows the various holes
Inferior sinus that permit interatrial shunting. Only the holes
venosus defect
venosus defect
in the oval fossa and the rare vestibular defects
Oval fossa defect
are true deficiencies of atrial septal structures.
atrioventricular junction4. It will be to permit direct suture. Now, all but very to ensure continuity of the inferior caval
considered in our next section. Sinus large defects within the oval fossa are likely vein and right atrium following placement
venosus defects, representing an to be closed by the interventional of a patch used to close a deficiency of the
anomalous connection of a pulmonary cardiologist. If attempts are made to close, floor of the oval fossa.
vein, which has retained its left atrial directly, defects large enough to justify Sinus venosus defects are more rare
connection5, are found at the mouths of surgical intervention, the results may so than defects within the oval fossa, and
the caval veins5–8. The rare defect found distort atrial anatomy as to result in present greater problems in repair. The
at the mouth of the coronary sinus is dehiscence. Irrespective of the size of the defect adjacent to the inferior caval vein
the consequence of the disappearance of the septal deficiency, it is always possible to (Figure 7.17) is relatively rare5. It opens
muscular walls that usually separate the secure a patch to the margins of the oval into the mouth of the inferior caval vein
component of the coronary sinus running fossa. When placing sutures, the likeliest posterior to the confines of the oval fossa.
through the left atrioventricular junction potential danger relative to the rims is to Usually the fossa itself is intact, but it can
from the cavity of the left atrium9. the artery supplying the sinus node be deficient or probe patent. The essence of
Defects within the oval fossa are often (Figure 7.14). This artery can course the defect is an anomalous connection of
called secundum defects. Because they intramyocardially through the anterior the right inferior pulmonary vein to the
represent persistence of the secondary margin of the oval fossa, or lie deep within inferior caval vein, the pulmonary vein
atrial foramen, rather than deficiencies of the superior interatrial fold. There is also a retaining its left atrial connection5. It is
the secondary atrial septum, they should remote chance of damaging the aorta when much more frequent to find sinus venosus
properly be called ostium secundum placing stitches anteriorly, as this part of defects adjacent to the mouth of the
defects. We prefer to consider them as the rim is related on its epicardial aspect to superior caval vein6,8. These defects, again,
defects within the oval fossa. They are, by the aortic root (Figures 7.14, 7.15). On are due to an anomalous connection of
far, the most common type of interatrial occasion, deficiency of the posteroinferior one or more of the right pulmonary veins to
communication. They can be caused by rims of the oval fossa permits holes within the superior caval vein, the pulmonary
deficiency (Figure 7.11), perforation the fossa to extend into the mouth of the veins retaining their left atrial connection6.
(Figure 7.12), or absence (Figure 7.13) of inferior caval vein (Figure 7.16). In these The defects are outside the confines of the
the floor of the fossa. The floor is formed by circumstances, care must be taken not to oval fossa, and hence are interatrial
the flap valve of the oval foramen, itself mistake a well-formed Eustachian valve for communications rather than atrial septal
derived from the primary atrial septum. the posteroinferior margin of the defect. defects (Figure 7.18)1,2.
When the haemodynamics of the shunt A patch attached to the Eustachian valve When sinus venosus defects are found
across such a defect dictate surgical closure, would connect the inferior caval vein to the in relation to the superior caval vein, the
the hole is rarely likely to be small enough left atrium. It is always prudent, therefore, orifice of the vein usually overrides the
Apex
Sup. Inf.
Base
Fig. 7.11 The surgical view through a right atriotomy shows a

deficiency in the flap valve of the oval fossa (star).
Apex
Sup. Inf.
Base
Fig. 7.12 The surgical view through a right atriotomy shows three
perforations (arrows) in the flap valve of the oval fossa.
superior rim of the oval fossa (Figures 7.19, associated with anomalous connections of retaining their left atrial connection
7.20). More rarely, such defects can be the right superior pulmonary veins, which (Figure 7.20). The difficulty encountered
found when the caval vein is committed drain into the superior caval vein during surgical repair reflects the need to
exclusively to the right atrium (Figures 7.22, 7.23), often through more reconstruct the anatomy so as to reroute the
(Figure 7.21)6. All the defects are than one orifice (Figure 7.19), while venous return and, at the same time, close
Apex
Sup. Inf.
Base Fig. 7.13 The surgical view through a right atriotomy shows the
absence of the entire flap valve of the oval fossa.
Sup.
Post. Ant.
Inf.
Fig. 7.14 The dissection, made by transecting the atrial

chambers, and illustrated in anatomical orientation, shows the
relationship of the artery supplying the sinus node to the
anterosuperior rim of the oval fossa. Note also the proximity of the
Floor of oval fossa Aortic root rim to the aortic root. The arrow shows the infolded anterior rim of
the fossa.
the interatrial communication. This must subepicardially within the terminal groove node when rerouting the pulmonary
be done without obstructing venous flow (Figure 7.23). Its location should be venous return, or if there is need to enlarge
or, in the case of a superior defect, considered both when making the the orifice of the caval vein. The former risk
damaging the sinus node. The sinus node is atriotomy, and when placing sutures in the can be minimised with judicious superficial
related to the anterolateral quadrant of the atrial walls. Problems arise should it be placement of the sutures. The latter
cavoatrial junction. It lays immediately necessary either to suture in the area of the problem is much greater. Because the
Infundibulum
Aortic root
Right atrium
Left atrium
Fig. 7.15 The computed tomogram shows the adjacency of the

anterosuperior margin of the oval fossa (yellow brace) and the
adjacent atrial walls to the aortic root.
Apex
Sup. Inf.
Base

defect within the oval fossa (large star) extending into the
mouth of the inferior caval vein (arrow). Note the location of the
triangle of Koch (small star).
artery to the sinus node may pass either in itself. Should it be deemed necessary to cut Another defect that permits interatrial
front of or behind the caval vein, the entire across the junction, a much better option is shunting, but which is outside the confines
cavoatrial junction is a potentially to perform the Warden operation10. This of the true atrial septum, is part of a
dangerous area. Incisions across the involves detaching the superior caval vein, constellation of lesions termed unroofing of
cavoatrial junction carry a high risk of and reattaching it to the excised tip of the the coronary sinus (Figure 7.24)9. In this
damaging the artery, or even the node right atrial appendage11. setting, a persistent left superior caval vein
Superior caval vein Sup.
Post. Ant.
Inf.
Fig. 7.17 The specimen, viewed in anatomical orientation, shows

the features of an inferior sinus venosus defect. The margins of
the oval fossa are intact. The defect is due to an anomalous
connection of the middle and inferior right pulmonary veins to the
Right pulmonary veins Sinus venosus defect Oval fossa inferior caval vein. The anomalously connected veins retain their
left atrial connection.
Intact oval fossa Sinus venosus defect Coronary sinus
Apex
Sup. Inf. defect at the mouth of the inferior caval vein. There is anomalous
drainage of the right inferior pulmonary vein, which retains its
Base Pulmonary venous orifice left atrial connection. The oval fossa is intact. This is a typical
example of the inferior sinus venosus defect.
usually drains directly to the left atrial roof interatrial communication (Figure 7.26). Sometimes the mouth of the coronary sinus
(Figure 7.25), entering the chamber between Evidence is frequently seen of the walls of can seem to open normally to the right
the appendage and the left pulmonary veins. the coronary sinus and left atrium along the atrium, but in the absence of its component
Because of the unroofing of the coronary anticipated course of the left superior caval usually occupying the left atrioventricular
sinus into the cavity of the left atrium, the vein into and through the left groove, and without persistence of a left
mouth of the coronary sinus functions as an atrioventricular groove (Figure 7.24). superior caval vein draining to the left atrial
Sup.
Post. Ant.
Inf.

a superior sinus venosus defect with overriding of the orifice of
the superior caval vein (SCV). The probe (stars) has been passed
through the fibroadipose tissue occupying the intact superior
margin of the oval fossa. There is anomalous drainage of the two
Overriding orifice of SCV Intact oval fossa right upper pulmonary veins, which have retained their left atrial
connection.
Superior caval vein
Right pulmonary vein
Fig. 7.20 The computed tomogram shows how, most frequently,

the mouth of the superior caval vein overrides the crest of the
atrial septum in the setting of a superior sinus venosus defect. Note
that the right pulmonary veins drain anomalously to the superior
Atrial septum
Left atrium caval vein while retaining their left atrial connection (white double-
headed arrow).
roof. In this setting, the left ventricular fenestration of the walls of the coronary and connections, of the left superior caval
coronary veins drain directly into the cavity sinus, providing communications with the vein. If it is present, and in free
of the left atrium. In these circumstances, the cavity of the left atrium (see Chapter 9). communication with the right superior
mouth of the coronary sinus again functions Surgical treatment of interatrial caval vein, or if there is no left-sided
as an interatrial communication. This lesion communications through the mouth of the superior caval vein, the mouth of the
is the extreme form of the spectrum of coronary sinus is dictated by the presence, coronary sinus can simply be closed. If the
Right upper pulm. veins

Sup.
Post. Ant.
Inf.
Sinus venosus defect

a superior sinus venosus defect without overriding of the orifice
of the superior caval vein. As in the specimen shown in Figure 7.18,
there is anomalous drainage of the right upper pulmonary
Oval fossa (pulm.) vein, which has retained its left atrial connection. Note the
distance between the defect and the oval fossa (double-headed
arrow). Note also the intact rims of the oval fossa.
Sinus venosus defect Intact oval fossa

Apex
Sup. Inf.
Base
Fig. 7.22 This surgical view through a right atriotomy shows a

Right pulmonary vein superior sinus venosus defect, recognised because it is outside the
confines of the intact oval fossa.
right atrial mouth of the sinus is to be channel has no anastomoses with the right the left-sided vein with the mouth of the
closed, decisions must be made concerning side, consideration should be given to coronary sinus.
the treatment of the left superior caval vein. construction of a left-sided cavopulmonary
In the presence of an adequate venous anastomosis, the Glenn shunt.
Atrioventricular septal defects
channel communicating with the Alternatively, a channel can be constructed
brachiocephalic vein, the left caval vein can along the posteroinferior wall of the left It is becoming increasingly frequent for the
be ligated. If, in contrast, the left-sided atrium, connecting the left atrial opening of anomalies variously described as
Superior caval vein Sinus node Apex
Sup. Inf.
Base
Fig. 7.23 This surgical view, through a median sternotomy of

the heart shown in Figure 7.20, illustrates the anomalous
Right pulmonary vein Pericardium connection of the right superior pulmonary vein. Note the site of
the sinus node lying in the terminal groove.
Base
Ant. Post.
Apex
Fig. 7.24 The heart is shown from the left side in anatomical
Unroofed coronary sinus orientation. There is unroofing of the coronary sinus as it courses
Mitral valve
through the left atrioventricular groove, draining a persistent
left superior caval vein, with filigreed remnants (stars) showing the
site of the walls that initially separated the vein from the left
atrium.
endocardial cushion defects, defects12. This is entirely apppropiate atrioventricular septum, but also the
atrioventricular canal defects, or a because, in anatomical terms, the overlapping region of atrial and ventricular
persistent atrioventricular canal, to be malformations are due to not only the musculatures that normally forms the floor
described as atrioventricular septal absence of the membranous of the triangle of Koch. The structures are

Left atrial
appendage
Left atrium
Fig. 7.25 The computed tomogram shows a persistent left

superior caval vein draining to the roof of the left atrium, in the
absence of the walls that usually separate the course of the vein
through the left atrioventricular groove to the mouth of the
coronary sinus.
Sup. Orifice of SCV
Post. Ant.
Oval fossa
Inf.
Fig. 7.26 The right atrium from the heart illustrated in Figure 7.24
is shown. Because of the unroofing of the walls of the persistent
Eustachian valve Interatrial communication
left superior caval vein (SCV), the mouth of the coronary sinus
through mouth of coronary sinus
functions as an interatrial communication.
absent because the unifying feature of the because, on rare occasions, defects of the defects, can take two forms. The more
group is the commonality of the membranous atrioventricular septum can frequent variant exists when shunting
atrioventricular junction (Figure 7.27)4. be found in the setting of separate right and across a ventricular septal defect enters the
The optimal title for the group, therefore, left atrioventricular junctions. These right atrium through a deficient tricuspid
would be atrioventricular septal defect with defects, first described by Gerbode and valve (Figure 7.28). The more rare variant
common atrioventricular junction. This is colleagues13, and often called Gerbode is a true deficiency of the atrioventricular
Sup.
Right Left
Inf.
Right atrium Left atrium
Fig. 7.27 The section of a specimen, cut in the four-chamber

orientation, shows a common atrioventricular junction (double-
headed arrow) guarded by a common atrioventricular valve in a
Right ventricle heart with deficient atrioventricular septation. The black brace
Left ventricle shows the atrioventricular septal defect, between the leading edge
of the atrial septum and the crest of the muscular ventricular
septum (stars).
Apex
Sup. Inf. Shunting through

perimembranous VSD
Base
Intact atrioventricular Atrioventricular Fig. 7.28 The cartoon shows how, when there is a deficiency of
membranous septum conduction axis
the septal leaflet of the tricuspid valve, shunting across a
ventricular septal defect (VSD) can also produce left ventricular to
right atrial shunting. This is the so-called indirect Gerbode defect.
component of the membranous septum septum is to demonstrate the competence in the setting of separate right and left
(Figures 7.29–7.32)14. The key to of the tricuspid valve (Figure 7.32). atrioventricular junctions, now it is usual to
differentiating the true defects from those Although atrioventricular septal defects presume the presence of a common
involving passage via a deficient ventricular do exist in the form of the Gerbode defect atrioventricular junction when considering
Aortic root
Right atrium Left ventricle
Sup. Fig. 7.29 The section through the heart, replicating the
echocardiographic four-chamber cut, shows how the septal leaflet
Right Left of the tricuspid valve divides the membranous septum into
interventricular and atrioventricular components. It is deficiency of
Inf. the atrioventricular component (white double-headed arrow) that
underscores the existence of the direct Gerbode defect.
Apex
Intact interventricular
Sup. Inf. membranous septum
Base
Shunting through deficient Atrioventricular Fig. 7.30 The cartoon shows how a deficiency of the
atrioventricular conduction axis
membranous septum atrioventricular component of the membranous septum
produces the setting for direct shunting from the left ventricle to
right atrium. This is the direct Gerbode defect.
deficient atrioventricular septation. The right and left atrioventricular junctions there is no septal atrioventricular junction.
presence of the common junction (compare Figures 7.27 and 7.33). Instead, the leading edges of the atrial
fundamentally distorts the overall anatomy Because of the lack of the membranous septum and the ventricular septum, the
when compared to the normally separate and muscular atrioventricular structures, latter usually covered by the
Apex
Sup. Inf.
Right atrium
Base
Tricuspid valve
Fig. 7.31 The image, taken in the operating room, shows a

Deficient atrioventricular membranous septum deficiency of the atrioventricular component of the membranous
septum.
Apex
Competent tricuspid valve
Sup. Inf.
Base
Fig. 7.32 In the heart of the patient illustrated in Figure 7.31,

insufflations of saline in the right ventricle reveals a competent
tricuspid valve, showing that the shunting from the left ventricle to
Shunting through deficient
atrioventricular membranous septum right atrium is across a deficiency of the atrioventricular part of the
membranous septum; in other words, a direct Gerbode defect.
atrioventricular valvar leaflets, meet at the the septal structures relative to the junction analogue of the triangle of Koch can be seen
superior and inferior margins of the produces ventricular imbalance, or a within the leading edge of the atrial
common atrioventricular junction double-outlet atrium. septum15. Because of the common
(Figure 7.34). These meeting points of the It is the overall anatomy produced by atrioventricular junction, however, the
septal structures typically divide the the common atrioventricular junction that atrial septal myocardium makes contact
common junction into more-or-less equal distorts the disposition of the with the ventricular myocardium only
right and left sides. An eccentric location of atrioventricular conduction axis. An superiorly and inferiorly (Figure 7.34).
Sup.
Right Left
Inf.
Fig. 7.33 This normal heart has also been sectioned in four-
chamber orientation. It shows the right and left atrioventricular
junctions (double-headed arrows with red borders) guarded by
separate atrioventricular valves (compare with Figure 7.27). The
white double-headed arrow shows the separating atrioventricular
structures.
Atrioventricular septal defect Conjoined bridging leaflets
Fig. 7.34 The specimen, seen in anatomical orientation, has been

prepared to show the right side of the heart with deficient
atrioventricular septation and separate right and left
atrioventricular orifices, the lesion also known as an ostium primum
defect. It is the conjoined bridging leaflets that produce the
separate valvar orifices. The septal defect occupies the site of the
Sup.
normal atrioventricular separating structures. Note that the atrial
septum itself is virtually normal, and the oval fossa is intact,
Post. Ant. although the leading edge of the septum is bowed from the
atrioventricular junction in the margin of the septal defect. The
Normally formed atrial septum Inf. stars show how the meeting points of the septal structures divide
the common junction into its right and left sides.
Usually the atrioventricular node is effectively common atrioventricular valve (Figure 7.37). The proximity of the
displaced inferiorly. It typically lies in a to the right-hand side (Figures 7.35, 7.36). coronary sinus to the apex of the nodal
nodal triangle, but not the triangle of Koch. The atrioventricular conduction axis triangle is pertinent to surgical closure of
When seen by the surgeon, the nodal penetrates the apex of the triangle. It then the septal defect. Ideally, the surgeon will
triangle has the coronary sinus at its base, courses on the crest of the muscular place a patch so as to leave the coronary
with the atrial septum to the left-hand side, ventricular septum, covered by the inferior sinus draining to the systemic venous
and the attachment of the leaflets of the bridging leaflet of the atrioventricular valve atrium. The best option is to deviate the
Apex Inferior bridging leaflet
Sup. Inf.
Base
Fig. 7.35 This view through a right atriotomy shows the surgeon’s
view of the atrial septal surface in a heart with an
atrioventricular septal defect and separate right and left
atrioventricular orifices, or the ostium primum defect. Koch’s
triangle is well formed (red star), but no longer contains the
atrioventricular node. The node is displaced posteroinferiorly to
the apex of a new nodal triangle (white star). The three dashed
lines show the options for placement of sutures so as to avoid
traumatising the atrioventricular conduction axis. The green line
runs directly from the inferior bridging leaflet to the inferior
margin of the leading edge of the atrial septum. The yellow line
courses from the inferior bridging leaflet to the right side of the
atrioventricular node and skirts the margins of the coronary sinus,
keeping the orifice of the sinus to its right side. The blue line
Coronary sinus courses to the right side of the atrioventricular node, but is placed
so as to leave the coronary sinus draining to the left atrium.
Conjoined bridging leaflets Atrioventricular septal defect
Fig. 7.36 In this heart, again shown as seen by the surgeon

through a right atriotomy, there is less room around the coronary
Apex sinus to place a patch so as to leave the sinus draining to the right
side (yellow dashed line). The site of the atrioventricular node is
Sup. Inf. shown again by the white star (compare with Figure 7.35). Note
that it is not within the well-formed triangle of Koch (red star). The
Leading edge of normally formed atrial septum Base green and blue lines show the alternate options for placement of
the suture line, as indicated in Figure 7.35.
suture line towards the left-hand side of the but place the suture line within the mouth mouth of the coronary sinus and the
inferior bridging leaflet at its junction with of the coronary sinus so as to reach the edge apex of the nodal triangle (compare
the atrial septum, using superficial surgical of the atrial septum (yellow line in Figures 7.35 and 7.36). A further
bites (green line in Figure 7.35)15. An Figure 7.35). The safety of this approach alternative is to keep the suture line to the
alternative is to stay on the right-hand side, depends on the margin between the right of the mouth of the coronary sinus,
Branching AV bundle
Analogue of triangle of Koch-
does not contain AV node
Atrioventricular (AV) node
Coronary sinus
Atrial septum Fig. 7.37 The cartoon shows the usual disposition of the
atrioventricular (AV) conduction axis in hearts with an
atrioventricular septal defect and common atrioventricular
junction. The variant with a common valvar orifice is illustrated.
Anterosuperior leaflet Right mural leaflet
Bridging leaflets
Apex
Sup. Inf. Fig. 7.38 In this atrioventricular septal defect with separate right
and left valvar orifices, the atrial septum is grossly deficient. The
Left mural leaflet Base atrioventricular node is found at the point where the muscular
ventricular septum joins the inferior atrioventricular junction (star).
placing the sinus to the left of the atrial atrial wall. The conduction axis, however, The feature that distinguishes between
patch (blue lines in Figures 7.35 and 7.36). follows the course of the muscular the various forms of atrioventricular septal
Occasionally, the inferior portion of the ventricular septum, with the node defects is the morphology of the leaflets of
atrial septum itself can be deficient. The formed at the site of its union with the the atrioventricular valve that guard the
coronary sinus then opens more inferior atrioventricular junction common atrioventricular junction,
posteriorly and medially through the left (Figure 7.38)16. together with their relationship to the
Anterosuperior leaflet Sup.
Superior bridging leaflet

Left Right
Inf.
Fig. 7.39 This atrioventricular septal defect, positioned

anatomically and viewed from above, and with a common valvar
Left mural leaflet orifice, has been dissected to show the arrangement of the five
leaflets that guard the common atrioventricular junction. The
Inferior bridging leaflet Right mural leaflet white double-headed arrow shows the zone of apposition between
the two bridging leaflets.
septal structures bordering the defect17. In extensive mural leaflet guards two-thirds of leaflet to be divided, with the two
any heart that lacks the separating the circumference of the valvar orifice. The components both attached to the
atrioventricular structures, the common left ventricular outflow tract then ventricular septum. They considered their
atrioventricular valve has five leaflets. The interposes between the aortic leaflet of the Type B variant again to have a divided
arrangement is seen most readily when the mitral valve and the ventricular septal anterior common leaflet, but with both
valve itself has a common orifice surface. In atrioventricular septal defects parts attached to an anomalous papillary
(Figure 7.39). Two of the leaflets extend with a common atrioventricular junction, muscle in the right ventricle. In their
across the ventricular septum, with their in contrast, the left ventricular papillary Type C malformation, they interpreted the
tension apparatus attached in both muscles are deviated laterally, being arrangement in terms of an undivided
ventricles. These are the superior and positioned superiorly and inferiorly12,17. common anterior leaflet, which was free-
inferior bridging leaflets. Two other leaflets Because of this, the mural leaflet is floating and attached in the right ventricle
are contained entirely within the right relatively insignificant, and guards much to an apical papillary muscle. In reality, the
ventricle. They are the anterosuperior and less than one-third of the circumference of purported division of the anterior common
inferior mural leaflets. The fifth leaflet is the left atrioventricular orifice. The left leaflet is the site of coaptation between the
contained exclusively within the left orifice, in effect, is guarded by a valve right ventricular part of the superior
ventricle, and is also a mural leaflet. possessing three leaflets, these being the bridging leaflet and the anterosuperior
Although the two leaflets found within the small mural leaflet, and the more extensive leaflet of the right ventricle (Figure 7.43).
right ventricle are comparable to similar left ventricular components of the superior In the Rastelli Type A variant, the point of
leaflets of the tricuspid valve seen in the and inferior bridging leaflets (Figure 7.42). coaptation is supported by the medial
normal heart, the left ventricular leaflets of It had originally been suggested by papillary muscle of the right ventricle. The
the common atrioventricular valve Rastelli and colleagues18 that the common variation noted by Rastelli and his
(Figure 7.40) bear no resemblance to a valve possessed four rather than five colleagues18 is then readily explained on
normal mitral valve. In the normal mitral leaflets. They argued that the differing the basis of increased commitment of the
valve, found with separate atrioventricular morphology to be found in the setting of a superior bridging leaflet to the right
junctions, the ends of the solitary zone of common valvar orifice reflected the ventricle, with concomitant diminution in
apposition between the leaflets, and the morphology of the anterior common leaflet, size of the anterosuperior leaflet of the right
papillary muscles supporting them, are which is now usually described as the ventricle (Figure 7.44). As the superior
situated inferoanteriorly and superior bridging leaflet. In the bridging leaflet becomes increasingly
superoposteriorly within the left ventricle arrangement they described as Type A, committed to the right ventricle, its site of
(Figure 7.41). With this arrangement, the they considered the anterior common coaptation with the anterosuperior leaflet
Superior bridging leaflet Inferior bridging leaflet
Zone of apposition
Fig. 7.40 This operative view through a right atriotomy shows the
Apex typical trifoliate formation of the left atrioventricular valve in a
heart with a deficient atrioventricular septation and common
Sup. Inf. atrioventricular junction. It bears no resemblance to the formation
of the leaflets as seen in the normal mitral valve. Note the extensive
Left mural leaflet Base zone of apposition between the two leaflets that bridge the
ventricular septum (dashed white double-headed arrow).
moves towards the right ventricular apex of the remaining variability in ostium primum variant. The bridging
(Figures 7.45, 7.46)17. A further difference atrioventricular septal defects with a leaflets are joined to each other along the
between the hearts at either end of the common atrioventricular junction. The crest of the ventricular septum by a
spectrum identified by Rastelli and overall valvar morphology is comparable in connecting tongue of leaflet tissue
colleagues18 is that, with minimal bridging, all hearts with this specific phenotype. The (Figure 7.47). The essence of the ostium
the superior bridging leaflet is tethered by variability depends on the relationship of primum defect, therefore, is the presence
cords to the crest of the ventricular septum the two bridging leaflets to each other, or of separate valvar orifices for the right and
(Figure 7.43). With extreme commitment their relationships, on the one hand, to the left ventricles within the common
of the supporting papillary muscle to the lower edge of the atrial septum and, on the atrioventricular junction. Because the heart
right ventricle, in contrast, the superior other hand, to the crest of the muscular of necessity possesses a common
bridging leaflet is always free-floating. The ventricular septum. If these two features atrioventricular junction, the left valve
variation noted by Rastelli and colleagues are described separately, there is no need to has three leaflets, with an extensive
reflected the changing morphology of the use terms such as ‘complete’, ‘partial’, and zone of apposition between the left
superior bridging leaflet. There is also ‘intermediate’ when seeking to subdivide ventricular components of the superior
variation in the arrangement of the inferior the group. It is the use of these terms that, and inferior bridging leaflets. This area, in
bridging leaflet. This does not, however, in the past, has created most confusion in the past, was frequently described as a cleft.
reflect its commitment to the two description. From the surgical stance, if it It has no morphological similarity to the
ventricles, which is usually balanced. The is possible to visualise a bare area at the cleft found in the aortic leaflet of the
leaflet is often divided along the inferior midportion of the ventricular septal crest; mitral valve in hearts with normal
edge of the ventricular septum, with the this would constitute a complete lesion. If atrioventricular septation and separate
attachments of the two components the crest of the septum is covered by a right and left atrioventricular junctions19.
providing a relatively clear zone of tongue of tissue that joins the bridging It is necessary surgically to close part, or all,
separation. The edges are most frequently leaflets together, then the lesion is of this zone of apposition when repairing
attached to the septal crest, but there can be considered either partial or intermediate. ostium primum defects (Figure 7.48). The
a ventricular component to the defect The intermediate variant is characterised resulting closure, nonetheless, does not
through intercordal spaces. Such potential by the presence of shunting at both atrial recreate a leaflet comparable to the aortic
for shunting beneath the inferior bridging and ventricular levels, but with separate leaflet of the normal mitral valve
leaflet is almost always found when the valvar orifices within the common (Figure 7.49).
superior bridging leaflet is free-floating. atrioventricular junction. The options for haemodynamic
It is the morphology of the bridging The partial variant, with no ventricular shunting across the atrioventricular septal
leaflets themselves that accounts for much shunting, is also well described as the defects, which largely colour the clinical
Aorta
Mitral valve
Fig. 7.41 This view of the left ventricle, taken from the apex with
the ventricular mass sectioned in its short axis, shows the
Sup. interrelationship of the normal mitral valve and the outflow tract
to the aorta. Note the oblique position of the papillary muscles
Right Left supporting the solitary zone of apposition between the leaflets of
the mitral valve, along with the extensive space between the aortic
Inf. leaflet of the mitral valve and the septal surface of the left
ventricle (star).
Zone of apposition
Sup.
Right Left
Inf.
Fig. 7.42 This view of the left atrioventricular valve and the
outflow tract in a heart with an atrioventricular septal defect is
taken in comparable fashion to the normal heart seen in
Figure 7.41, showing the short axis of the left ventricle. Note that
the left valve in the heart with deficient atrioventricular septation
has three leaflets, with papillary muscles positioned directly
superiorly and inferiorly, rather than being obliquely positioned as
in the normal heart. There is an extensive zone of apposition
between the bridging leaflets (dashed white double-headed
arrow). Note also the way that the outflow tract is squeezed
Inferior bridging leaflet Left mural leaflet between the superior bridging leaflet and the superior margin of
the left ventricle (star).
Inferior leaflet
Fig. 7.43 The image shows the location of the line of coaptation
between the superior bridging and anterosuperior leaflet of the
common atrioventricular valve as seen from the atrial aspect in an
atrioventricular septal defect with a common valvar orifice (white
arrow). This arrangement, with minimal bridging of the superior
leaflet, produces the so-called ‘Rastelli Type A’ malformation. The
star shows the atrial component of the septal defect. Note that
there is the potential for multiple shunts through intercordal
spaces to occur at the ventricular level beneath the superior
Sup. bridging leaflet. Note also the presence of anterosuperior and
inferior leaflets in the right ventricle. The white arrow shows the
Post. Ant. zone of apposition between the leftward margin of the
anterosuperior leaflet and the right ventricular component of the
Inferior bridging leaflet Inf. superior bridging leaflet. It is a mistake to consider this
arrangement to represent a divided common anterior leaflet.
Rastelli Type A
Medial papillary muscle Anterior papillary muscle
Sup.
Left Right
Inf.
Anomalous apical muscle Bifid anterior muscle
Fig. 7.44 The cartoon is shown as seen in

anatomical orientation and viewed from above
Anterior muscle (compare with Figure 7.39). It shows the spectrum
of bridging of the superior leaflet in hearts with
an atrioventricular septal defect and common
orifice that underlies the classification introduced
by Rastelli and his colleagues18. As the superior
bridging leaflet (pink) extends further into the
right ventricle, there is concomitant diminution in
size of the anterosuperior leaflet (pink, cross-
hatched), and fusion of the anterior and medial
papillary muscles of the right ventricle, which
increasingly are attached towards the right
Rastelli Type B Rastelli Type C
ventricular apex.
features, depend upon the relationship of leaflets to be attached directly to the crest of lack direct attachments to the septal
the bridging leaflets to the septal the ventricular septum, although the extent structures, the potential exists for shunting
structures4. When there is a common of their tethering by tendinous cords can at both atrial and ventricular levels, the
valvar orifice, it is extremely rare for the vary markedly. When the bridging leaflets magnitude of the shunts depending on the
Inferior leaflet
Fig. 7.45 The image is taken in the same orientation as in

Figure 7.43. It shows how, in the Rastelli Type B variant of the
common valvar orifice, the line of coaptation between the superior
bridging leaflet and the anterosuperior leaflet of the right ventricle
Sup. (white arrow) is displaced into the right ventricle. The papillary
muscle supporting the zone of coaptation has moved down the
Post. Ant. ventricular septum towards the apex of the right ventricle. The
anterosuperior leaflet itself is smaller than in the Rastelli Type A
Inf. variant, but there is also an inferior leaflet in the right ventricle,
Inferior bridging leaflet
displaced upwards because of the opened right atrioventricular
junction. The star shows the atrioventricular septal defect.
Superior bridging leaflet Anterosuperior leaflet
Fig. 7.46 The image, taken to parallel the views seen in

Figures 7.43 and 7.45, but showing only the right ventricular
component of the superior bridging leaflet, illustrates the Rastelli
Type C arrangement. Its line of coaptation with the anterosuperior
leaflet of the right ventricle has moved even further into the right
Sup. ventricle (white arrow), with a concomitant decrease in size of the
anterosuperior leaflet. The papillary muscle (PM) supporting the
Post. Ant. line of coaptation is further towards the apex of the right ventricle.
Anomalous apical PM The star shows the location of the muscular ventricular septum.(The
Inf. Inferior leaflet image is reproduced by kind permission of Mr. Bill Devine,
Children’s Hospital of Pittsburgh.)
prevailing haemodynamic conditions, confined at atrial level. This is the the common junction, but with both
together with the extent of the tethering. If arrangement found most frequently in the bridging leaflets firmly fused to the crest of
the leaflets are firmly attached to the typical ostium primum defect, with the ventricular septum (Figure 7.50).
ventricular septum, shunting will be separate right and left valvar orifices within Much less frequently, the bridging leaflets
Superior bridging leaflet Anterosuperior leaflet
Left mural leaflet
Fig. 7.47 This heart with an atrioventricular septal defect and

separate right and left valvar orifices is viewed in anatomical
orientation from above, after removal of the atrial chambers and
the arterial trunks (compare with Figure 7.39). The common
Sup. atrioventricular junction is guarded by a valve with separate
orifices for the right and left ventricle. This is because a tongue of
Left Right leaflet tissue (star) joins together the facing surfaces of the
bridging leaflets (compare with Fig. 7.39). The white double-
Inf. Inferior bridging leaflet Right mural leaflet headed arrow shows the zone of apposition between the left
ventricular components of the bridging leaflets.
Superior bridging leaflet Inferior bridging leaflet
Apex Fig. 7.48 This view, taken through a right atriotomy, shows the
trifoliate configuration of the left atrioventricular valve
Sup. Inf. subsequent to repair of the zone of apposition between the left
ventricular components of the bridging leaflets (dashed black
Left mural leaflet Base double-headed arrow). Even after the surgical repair, the valve has
no similarity to the normal mitral valve (see Figure 7.49).
can be firmly attached to the underside of produces the true ventricular septal defect characteristics of a common
the atrial septum (Figure 7.51). This of atrioventricular canal variety, as the atrioventricular valve, rather than tricuspid
arrangement will confine the potential for valve guarding the common and mitral valves (Figures 7.51–7.55). The
shunting at the ventricular level. It atrioventricular junction will have the potential also exists, nonetheless, for the
Aortic leaflet
Sup.
Left Right
Fig. 7.49 This view through a right atriotomy shows a normal
Mural leaflet
Inf. mitral valve. The structure bears no comparison to the trifoliate
valve shown in Figure 7.48.
Superior bridging leaflet Sup.
Right Left
Inf.
Fig. 7.50 This heart with a common atrioventricular junction and

separate right and left valvar orifices is shown from the left side in
anatomical orientation. The bridging leaflets are firmly fused to
the crest of the ventricular septum (red dotted line), confining
shunting through the atrioventricular septal defect at the atrial
level. The white double-headed arrow shows the zone of
Inferior bridging leaflet Left mural leaflet apposition between the left ventricular components of the
bridging leaflets. This is the essence of the ostium primum defect.
leaflets to be free-floating even in the intermediate defect. If the variability in valvar orifice or separate right and left
presence of separate valvar orifices terms of attachment of the bridging leaflets orifices, there is no need to introduce the
(Figure 7.56). Most would consider the is described, along with information concept of intermediate or transitional
lesion shown in Figure 7.56 as an concerning the presence of a common variants. We recognise, nonetheless, the
Right atrium Left atrium
Inferior bridging leaflet Fig. 7.51 The four-chamber section is through an anatomical
Sup. specimen from a patient with deficient atrioventricular septation
and a common atrioventricular junction (dashed white double-
Right Left headed arrow). The inferior bridging leaflet is firmly fused to the
undersurface of the atrial septum (star), confining shunting
Inf. through the atrioventricular septal defect at the ventricular level
(solid white double-headed arrow).
Ventricular septal defect Superior bridging leaflet
Oval
fossa
Sup.
Fig. 7.52 The specimen is viewed in anatomical orientation from
Right Left the right side. The defect permits shunting at the ventricular level
only, because although the leaflets of the atrioventricular valve
Inferior bridging leaflet Inf. form a bridge across the crest of the ventricular septum into the left
ventricle, the atrial septum is intact (star).
value of the shorthand terms of partial, Thus, in many patients with so-called beneath the tongue that joins together the
intermediate, and complete variants, partial defects, with separate right and left bridging leaflets, or otherwise through
providing that all working on the same valvar orifices within the common junction, intercordal spaces tethering the bridging
team understand the definitions used for echo Doppler interrogation reveals the leaflets themselves. Such patients are
the different types. potential for interventricular shunting well described as having separate valvar
Sup.
Right Left
Inf.
Fig. 7.53 The image shows the left-sided aspect of another heart
with deficient atrioventricular septation and a common
Mural leaflet
atrioventricular junction. This picture clearly shows the
attachments of the bridging leaflets to the leading edge of the
Inferior bridging leaflet Ventricular septal defect atrial septum, confining shunting at the ventricular level. Note the
typical configuration of the left ventricular outflow tract (star).

Apex
Sup. Inf.
Base
Fig. 7.54 This picture, taken in the operating room, shows the
right atrial aspect of an atrioventricular septal defect with a
common atrioventricular junction, in which shunting is confined at
the ventricular level because the bridging leaflets are firmly
Intact atrial septum Inferior bridging leaflet attached to the underside of the atrial septum. In this image, the
septal defect itself is not obvious.
orifices, with predominantly atrial defects with a common atrioventricular common atrioventricular junction,
shunting, but with the potential for junction12. As already emphasised, it is coupled with the absence of the
minimal ventricular shunting. The basic this disposition that determines the atrioventricular muscular and membranous
morphology of the deficient ventricular course of the ventricular conduction separating structures, there is also
septum, therefore, is comparable in all pathways (Figure 7.34)13,20. Although the hypoplasia of the muscular ventricular
patients having atrioventricular septal hallmark of the malformation is the septum to a greater or lesser extent. This
Superior bridging leaflet Apex
Sup. Inf.
Base
Ventricular
septal defect
Fig. 7.55 This image shows how, when the surgeon separated
the right ventricular components of the bridging leaflets of the
heart shown in Figure 7.54, the common atrioventricular junction is
Intact atrial septum Inferior bridging leaflet seen, with shunting at the ventricular level across the
atrioventricular septal defect.
Atrial component Conjoined bridging leaflets
Sup. Fig. 7.56 In this heart from a patient with an atrioventricular

septal defect (white double-headed arrow), with separate valvar
Post. Ant. orifices and shown in anatomical orientation, the bridging leaflets
and the connecting tongue float free of both atrial and ventricular
Ventricular component Inf. septal structures so that there are both atrial and ventricular
defects.
involves disproportion between the inlet regard to the extent of scooping of the greater in hearts with a common valvar
and outlet dimensions of the ventricular septum (Figure 7.58). The scooping is orifice, thus increasing the likelihood of
septum when compared to the normal heart greater in hearts with a common valvar there being a ventricular component to the
(compare Figures 7.57 and 7.58). The orifice than in those with separate right and defect, variability is still found among these
degree of septal hypoplasia also varies with left orifices. Although the scooping is hearts. When there is minimal scooping, it
Sup.
Ant. Post.
Inf.
Fig. 7.57 A normal heart is shown from the left side, the parietal
wall of the left ventricle having been removed. There is equality in
the inlet (blue double-headed arrow) and outlet (red double-
headed arrow) dimensions of the ventricular septum, along with
the midseptal dimension (yellow double-headed arrow). (Compare
with Figure 7.58.)
Sup.
Ant. Post.
Inf.
Fig. 7.58 The valvar leaflets have been removed from the
ventricular mass in this heart with deficient atrioventricular
septation and a common atrioventricular junction. The heart is
viewed from the left side and shown in anatomical orientation.
Because the leaflets have been removed, there is no way of
knowing whether, originally, there was a common atrioventricular
orifice, or separate valvar orifices for the right and left ventricles.
Note the scooping of the ventricular septum (yellow double-
headed arrow), and the disproportion between inlet (blue double-
headed arrow) and outlet (red double-headed arrow) dimensions
of the ventricular mass.
is certainly possible to close the ventricular convinced of its utility. Thus, the use of the If the technique is used, care must be
component of the septal defect by attaching modified single patch approach remains taken not to damage the exposed
the bridging leaflets directly to the right controversial. Some consider that the conduction tissues along the septal crest.
ventricular aspect of the ventricular technique creates potential narrowing of The non-branching bundle runs down the
septum21. This manoeuvre, in fact, is the left ventricular outflow tract. There crest of the scooped-out septum, and is
feasible in all patients with a common may be subtle technical differences that covered by the inferior bridging leaflet.
valvar orifice22,23, although not all are produce the different results. The inferior leaflet itself is often divided by
a midline raphe immediately above the valvar orifices. It is exposed in the Although not readily evident to the
vulnerable non-branching bundle. The presence of a common orifice and free- surgeon during operation, the left ventricular
branching component of the conduction floating leaflets. The right bundle branch outflow tract in atrioventricular septal defects
axis is found astride the midportion of then runs towards the medial papillary is intrinsically narrow24. It is much longer in
the septal crest. This is usually covered muscle. Anterior to this point, the septum hearts with separate orifices. This is because
by the connecting tongue and leaflet is devoid of conduction tissue of the attachment of the superior bridging
tissue in hearts with separate right and left (Figures 7.59, 7.60)15. leaflet to the ventricular septal crest (compare
Atrioventricular septal defect Sup.
Post. Ant.
Inf.
Fig. 7.59 This heart came from a patient with an atrioventricular

Right bundle branch septal defect with a common atrioventricular junction and
shunting confined at the atrial level; in other words, an ostium
primum defect. The disposition of the atrioventricular conduction
Atrioventricular node Atrioventricular conduction axis axis has been superimposed in red on the picture, as seen from the
right side.
Sup. Atrioventricular septal defect
Ant. Post.
Inf.
Fig. 7.60 The photograph shows the left ventricular aspect of the
heart illustrated in Figure 7.59, again with the location of the
atrioventricular conduction axis superimposed on the picture. Note
Left bundle branch Long non-branching bundle that the left ventricular outflow tract (star) is distant from the
conduction tissues.
Sup. Atrioventricular septal defect
Ant. Post.
Inf.
Fig. 7.61 The heart, shown in anatomical orientation from the

left side, has been dissected to show the extent of the narrowed
outflow tract in an atrioventricular septal defect with separate
right and left atrioventricular valves, and with the potential for
atrial shunting only. Because the superior bridging leaflet is firmly
Bridging leaflets attached to the crest of the ventricular septum, the outflow tract
has considerable length (black double-headed arrow).
Sup. Superior bridging leaflet
Ant. Post.
Inf.
Fig. 7.62 In this heart, again shown in anatomical orientation

from the left side (compare with Figure 7.61), the outflow tract
(black double-headed arrow) is much shorter in the presence of a
Atrioventricular septal defect common atrioventricular valvar orifice. The star shows the zone of
apposition between the bridging leaflets.
Figures 7.61 and 7.62). The area is insertion of a model with low profile, Further variability is found in the
prone to postoperative obstruction, and otherwise resection of the shelf that exists commitment of the common
surgical enlargement may be necessary. between the hingepoint of the superior atrioventricular junction to the
Obstruction may be due to naturally bridging leaflet and the attachment of the ventricular mass. Usually it is shared
occurring lesions25, or to injudicious aortic valve24. The superior bridging leaflet equally, giving a balanced arrangement
placement of a prosthesis used to replace the can also be liberated from the septal crest, (Figure 7.27). Should the common
left atrioventricular valve. If a prosthesis inserting a gusset to enlarge the outflow tract junction favour one or other ventricle,
must be employed, the anatomy dictates (Figures 7.63 and 7.64). producing so-called right or left ventricular
Sup.
Right Left
Inf.
Fig. 7.63 This heart has an atrioventricular septal defect with a

common atrioventricular orifice, but with the superior bridging
leaflet tethered across the subaortic outflow tract (crossed black
Trifoliate left AV valve arrows). It is photographed in anatomical orientation from the
left side.
Sup.
Right Left
Inf.
Fig. 7.64 An incision has been made in the superior bridging

leaflet of the heart shown in Figure 7.63, at the same time
detaching the leaflet from the crest of the ventricular septum. The
Disattached superior bridging leaflet
incision makes it possible to insert a patch so as to widen the
outflow tract (crossed black arrows).
dominance, the other ventricle is often In the setting of right ventricular ventricular dominance, in contrast, is
severely hypoplastic. This can have a major dominance, there is usually alignment malalignment between the atrial septum
influence on the outcome of surgery, and between the atrial and ventricular septal and the muscular ventricular septum
should always be assessed preoperatively. structures at the crux. The essence of left (Figure 7.65). This produces an
Plane of Superior bridging leaflet Sup.

atrial septum
Left Right
Inf.
Left
ventricle
Fig. 7.65 In this specimen from a patient with an atrioventricular
septal defect and common atrioventricular junction, shown in
anatomical orientation, there is gross malalignment between the
muscular ventricular septum (red dotted double-headed arrow)
and the atrial septum (black double-headed arrow). As a
consequence, the atrioventricular conduction axis originates from
Cut-back coronary sinus Bifid inferior bridging leaflet an anomalous node (star) in the inferior aspect of the right
atrioventricular junction, rather than at the crux of the heart.
Malaligned muscular ventricular septum
Anomalous inferolateral node
Fig. 7.66 The cartoon shows how, when there is malalignment

Atrial septum comes to crux between the atrial septum and the muscular ventricular septum,
with left ventricular dominance, the atrioventricular node is
formed at the point where the ventricular septum meets the
inferior atrioventricular junction.
arrangement analogous to straddling of the at the point where the malaligned muscular axis. Septal malalignment, therefore,
tricuspid valve26. As with straddling the ventricular septum meets the should be excluded in all cases of
tricuspid valve, this has major consequence atrioventricular junction (Figure 7.66). atrioventricular septal defect with left
for the disposition of the atrioventricular This particular arrangement must be ventricular dominance. This arrangement
conduction axis27. Because of the septal identified preoperatively, as it can be should also be distinguished from those
malalignment, the connecting exceedingly difficult to recognise during hearts in which the atrial septum is absent,
atrioventricular node is no longer to be surgery. If unrecognised, it is likely that a and the coronary sinus terminates in the
found at the crux. It continues to be formed standard repair will damage the conduction left atrium16.
Ventricular septal defects third group were unified because part of surgical considerations (Figure 7.70).
their right ventricular borders was made up When using this system, nonetheless, the
When asked to close a clinically significant
of fibrous continuity between the leaflets of borders of the holes between the
hole between the ventricles, the primary
the aortic and pulmonary valves ventricles must be assessed as seen by the
concern of the surgeon is to ensure that the
(Figure 7.69), with holes of this third type surgeon working through the
task can be achieved in a safe and secure
showing additional variability depending morphologically right ventricle
fashion. The important anatomical
on whether the fibrous continuity extended (Figures 7.67–7.69).
considerations reflect the location of the
to include the leaflet of an atrioventricular The essence of the largest group of
defect relative to the landmarks of the right
valve. The defects in the patients making defects requiring surgical closure is that
ventricle. These features determine the
up the third group, of necessity open part of the central fibrous body, specifically
proximity of the defect to the
between the outflow tracts of the ventricles. the area of fibrous continuity between the
atrioventricular conduction axis, and to the
Defects within the other groups, however, leaflets of the mitral, aortic, and tricuspid
leaflets of the atrioventricular and arterial
need further description depending on valves, that forms a direct part of the rim of
valves. One categorisation of the defects28
whether they open primarily to the inlet, to the defect as seen from the right ventricle
was designed specifically to focus the
the apical, or to the outlet components of (Figure 7.68). This fibrous area
attention of the surgeon on these pertinent
the right ventricle. There is thus an incorporates the atrioventricular
features. The essence of the system was
additional feature that always requires component of the membranous septum,
that, according to the anatomical features of
description, if present, namely which retains its integrity when ventricular
the margins of the defects as seen from the
malalignment between the septal septation is incomplete, being an integral
morphologically right ventricle, all the
components. part of the central fibrous body. The
holes fitted into one of three groups.
There are, of course, other defects, therefore, surround the
The first group included all those holes
categorisations available for distinguishing membranous part of the septum, and are
that, when viewed from the right ventricle,
between types of holes between the described, justifiably, as being
had exclusively muscular borders
ventricles. One time-honoured system perimembranous. In many instances, the
(Figure 7.67). The phenotypical feature of
identified four variants, and grouped them interventricular component of the
the second group was that part of the right
in numerical fashion29. Another popular membranous septum is found as a fold of
ventricular border was composed of fibrous
system used developmental fibrous tissue in the posteroinferior margin
continuity between the leaflets of an
considerations so as to distinguish between of the defect (Figure 7.71).
atrioventricular valve and an arterial valve
the different holes30. We prefer the system The defects themselves represent the
(Figure 7.68). The patients falling in the
designed specifically to emphasise the unclosed embryonic interventricular
Apex
Sup. Inf.
Base
Fig. 7.67 This view through a right atriotomy, and across the
orifice of the tricuspid valve, shows a hole between the
ventricles that is enclosed by the walls of the muscular ventricular
septum (star).
Ventricular septal defect

Fibrous continuity
Apex Fig. 7.68 This view, again through a right atriotomy with
retraction of the leaflets of the tricuspid valve, shows the fibrous
Sup. Inf. tissue of the atrioventricular septum forming part of the right
Coronary ventricular border, as viewed by the surgeon. This is the criterion
Base sinus that permits the defect to be categorised as being
perimembranous.
Pulmonary valve Limbs of septomarginal

trabeculation
Base. Fig. 7.69 This surgical view, through a right ventriculotomy,

shows a hole between the ventricles that, at its cranial border, has
Left Right fibrous continuity between the leaflets of the aortic and pulmonary
valves (black dotted line). The defect, opening into the ventricular
Apex Aortic valve outlets between the limbs of the septomarginal trabeculation, is
doubly committed and directly juxta-arterial.
communication (Figure 7.72). They always be considerably larger than the area of the axis of atrioventricular conduction
presumably result from a deficiency of the occupied by the interventricular tissue31. In the normally formed heart, the
muscular ventricular septum forming the membranous septum of the normal heart. axis penetrates the atrioventricular
apical and cranial rims of the persisting The degree of septal deficiency has membranous septum to reach the crest of
hole. Defects requiring surgical closure will important consequences for the disposition the muscular septum. Having penetrated, it
Muscular defects
Septomarginal
Perimembranous defects trabeculation
Juxta-arterial defects
Trabecular
Outlet
Doubly committed
juxta-arterial defect
Trabecular
Inlet
Inlet Fig. 7.70 The cartoon, shown in surgical

orientation, illustrates the categorisation used
Outlet for differentiating the phenotypical variations
for holes between the ventricles. It combines
Central the phenotypical features shown in
fibrous Triangle of Koch
Figures 7.67 to 7.69 with the location of the
body and atrioventricular
node hole relative to the components of the right
Sinus node
ventricle.
Remnant of
membranous
ventricular septum
Septal leaflet of
tricuspid valve
Apex
Sup. Inf. Fig. 7.71 In this heart, viewed through a right atriotomy and
through the orifice of the tricuspid valve, a remnant of the
Base interventricular membranous septum is present in the
posteroinferior margin of the perimembranous defect.
is sandwiched between the muscular muscular ventricular septum, the axis atrioventricular bundle (Figure 7.74). If
septum and the interventricular penetrates the area of continuity between such a remnant is seen at operation
component of the membranous septum the leaflets of the aortic and tricuspid valves (Figure 7.71), and is substantial, it may
(Figure 7.73). In perimembranous defects, (Figure 7.74). When a remnant of the safely be used for anchorage of sutures
when the atrioventricular connections are interventricular membranous septum is placed superficially to anchor a surgical
concordant, so as to reach the crest of the present, it lies immediately on top of the patch.
Developing aortic valve Muscularising infundibulum
Sup.
Right Left
Inf.
Fig. 7.72 The image is a section taken from a dataset prepared

from a mouse embryo at embryonic day 13.5. It shows the margins
of the closing interventricular foramen (white brace), formed
dorsally by continuity between the cushions forming the leaflets of
the aortic and atrioventricular valves. Perimembranous defects
Developing tricuspid valve Closing interventricular foramen reflect failure to close this embryonic interventricular
communication.
Aortic root
Left
atrium
Atrioventricular
membranous
septum
Right
atrium
Left
ventricle
Sup.
Right Left Fig. 7.73 This four-chamber section, shown in anatomical

Septal leaflet orientation, reveals the position of the penetrating atrioventricular
of tricuspid valve
Right Inf. bundle (star) in the normal heart. It is sandwiched between the
ventricle central fibrous body and the crest of the muscular ventricular
septum.
The location of the medial papillary perimembranous, in other words the holes associated with straddling and overriding
muscle, together with the apex of the bordered posteroinferiorly by fibrous of the tricuspid valve27. The proximity of
triangle of Koch, provides the guide for continuity between the leaflets of the aortic the conduction tissues to the leaflets of the
predicting the location of the conduction and tricuspid valves (Figure 7.75). The aortic and atrioventricular valves, however,
axis in almost all holes that are only exceptions to this rule are the defects varies depending upon the precise area of
Aortic root Ventricular septal defect
Fig. 7.74 This ‘four-chamber’ section, again seen in anatomical

orientation, shows the position of the penetrating
atrioventricular bundle (star) in a heart with a perimembranous
Septal leaflet ventricular septal defect (black brace). The black dotted line shows
of tricuspid valve Membranous flap the area of fibrous continuity between the leaflets of the aortic
and tricuspid valves.
Turned back Ventricular septal defect

septal leaflet
Fig. 7.75 This hole between the ventricles, shown in anatomical

orientation, is viewed from its right ventricular aspect. The
posteroinferior margin of the defect is made up of a fibrous
continuity between the leaflets of the aortic and tricuspid valves
(black dotted line). In this setting, which makes the defect
Sup.
perimembranous, the course of the conduction axis, shown by the
red dotted line originating from the triangle of Koch (white
Right Left
triangle), is always positioned posteroinferiorly. Note that the
Membranous flap Inf. axis lies directly beneath a membranous flap. The septal leaflet of
the tricuspid valve has been retracted.
deficiency of the muscular septum. It is open mostly into the inlet of the right fibrous continuity also extends to involve
likely that all parts are deficient to a certain ventricle, its atrial margin, as viewed the leaflets of the mitral valve (Figure 7.75).
extent. It can usually be determined, through the tricuspid valve, is an area of The apex of the triangle of Koch is usually
nonetheless, which part is most affected. fibrous continuity between the leaflets of deviated inferiorly towards the coronary
When a perimembranous defect extends to the aortic and tricuspid valves, but the sinus. It is thus to the right-hand side of the
surgeon working through the atrium. The related to the atrial margin. The septal non-coronary and right coronary leaflets of
axis of atrioventricular conduction tissue leaflet of the tricuspid valve is often cleft the aortic valve are much more closely
penetrates this corner of the defect. or deficient, an arrangement that may related to the left-hand margin
Usually, the non-branching and branching permit shunting from the left ventricle to (Figure 7.79), and may prolapse towards
bundles are carried on the left ventricular right atrium, and hence producing an the right ventricle32.
aspect of the muscular septum as they indirect Gerbode defect (see The essential feature of muscular
descend the right-hand margin of the Figure 7.28)14. If the cleft in the septal defects is that, when viewed from the right
defect. The right bundle branch then leaflet of the tricuspid valve requires ventricle, they have exclusively muscular
courses intramyocardially, surfacing surgical closure, it should be remembered borders (Figure 7.67). They can open into
beneath the medial papillary muscle, which that the penetrating atrioventricular bundle the inlet, apical, or outlet parts of the right
is usually at the left-hand margin of the is located at its apex. ventricle. Always, nonetheless, ventricular
defect. The non-coronary leaflet of the The third type of perimembranous musculature will interpose between the
aortic valve is more to the left, and usually defect extends mostly so as to open into the edges of the defect and the attachments of
distant from the rim of the defect, although outlet of the right ventricle. The outlet the leaflets of the valves. When a muscular
it often maintains fibrous continuity with septum, along with the free-standing defect opens into the inlet of the right
the septal leaflet of the tricuspid valve subpulmonary infundibulum, can thus be ventricle (Figure 7.80), it is inferior to the
(Figure 7.76). recognised separating the leaflets of the atrioventricular axis of conduction tissue.
When perimembranous defects aortic and pulmonary valves, being When viewed by the surgeon through the
requiring surgical closure are located so as malaligned relative to the rest of the septum tricuspid valve (Figures 7.81, 7.82), the
to open mostly towards the ventricular (Figure 7.77). It is this type of defect that conduction axis is located on the left-hand
apex, they are large. They typically open others describe as being conoventricular30. margin of the defect. The proximity of the
additionally towards the inlet and outlet In the presence of anterocephalad axis to the edge depends upon the
components, and hence are confluent malalignment of the muscular outlet adjacency of the defect to the intact
defects. The triangle of Koch is not septum, the aortic root overrides the crest membranous septum. The basal margin of
deviated as far towards the coronary sinus of the muscular ventricular septum the muscular septum, interposing between
in such defects as when they open primarily (Figures 7.78, 7.79). The medial papillary the edge of the defect and the atrial septum,
to the right ventricular inlet, but the right- muscle is on the right-hand margin of the separates the septal leaflet of the tricuspid
hand rim is still the major area at risk. The defect, and the axis of atrioventricular valve from the mitral valve, preserving the
medial papillary muscle tends to be at the conduction tissue is more distant from the off-setting of the valvar hinges. Its size will
apex of such defects, and the non-coronary edge, being carried well down on the left determine whether it is suitable to be an
leaflet of the aortic valve is more closely surface of the ventricular septum. The anchorage for sutures.
Tricuspid valve (through defect) Central fibrous body
Sup. Fig. 7.76 This illustration, seen in anatomical orientation, shows

the left ventricular aspect of the perimembranous defect that
Ant. Post. opened primarily to the inlet of the right ventricle shown in
Figure 7.75. The black dotted line shows the fibrous continuity
Inf. between the non-coronary leaflet of the aortic valve and the
Membranous flap
tricuspid valve, via the membranous flap.
Malaligned outlet septum

Pulmonary trunk
Aorta
Fig. 7.77 This heart, shown in anatomical orientation, has been

Sup. sectioned to replicate the subcostal oblique long axis
echocardiographic projection. The perimembranous defect (star)
Right Left opens towards the outlet of the right ventricle. The muscular outlet
septum is now recognised in its own right. It is malaligned
Aortic-tricuspid valvar continuity Inf. relative to the muscular ventricular septum, and attached
anterocephalad relative to the septomarginal trabeculation.
Overriding aortic valve
Sup.
Fig. 7.78 This is the heart shown in Figure 7.77 prior to making
Right Left
the section to correlate with the echocardiographic plane. The
Perimembranous defect ventricular septal defect opens into the outlet of the right
Inf.
ventricle, with the aortic valve overriding the crest of the
ventricular septum.
Defects opening through the apical part conduction tissue axis, but may be related coarse apical trabeculations. Multiple
of the septum can be single (Figure 7.83), to ramifications of the distal bundle defects, if small, may not be visible through
double, or multiple (Figure 7.84). They are branches. The right ventricular aspect of a right ventriculotomy. The septal
unrelated to the proximal parts of the such defects is frequently obscured by the deficiencies are much more readily
Apex
Sup. Inf.
Base
Fig. 7.79 This view is through a right atriotomy, subsequent to

making an incision at the junction of the septal and anterosuperior
Incised leaflet leaflets of the tricuspid valve. It shows a perimembranous
of tricuspid valve defect opening into the outlet of the right ventricle. The leaflets of
Aortic valvar leaflet
the aortic valve are overriding the ventricular septal crest to the
left-hand margin of the defect.
Tricuspid
valve
Sup.
Fig. 7.80 This heart, photographed from the right side in
Left anatomical orientation, has a muscular ventricular septal defect
Right
opening into the inlet of the right ventricle. The atrioventricular
Inf. conduction axis (black dotted line) runs anterocephalad relative to
the defect. In this instance, it is well removed from the superior
margin of the hole (star).
identified from the left ventricular aspect. ventricular openings (Figure 7.84) simply involve the entirety of the apical ventricular
Inspection from the left side will often reflect crossing of the solitary defect by the septum. This produces the ‘swiss-cheese’
demonstrate that the defect is a solitary right ventricular apical trabeculations33. septum, an arrangement that can be
hole (Figure 7.85). The multiple right Multiple small defects, nonetheless, can notoriously difficult to close surgically.

Apex
Sup. Inf.
Base
Fig. 7.81 This surgical view shows a muscular defect opening into
the inlet of the right ventricle, viewed through the orifice of the
tricuspid valve. The conduction axis (black dotted line), arising
Inlet muscular defect from the apex of the triangle of Koch (cross-hatched triangle), is
seen to the left-hand side of the surgeon working through the
tricuspid valve.
Apex
Sup. Inf.
Base
Fig. 7.82 This view of the heart shown in Figure 7.81, again seen
through a right atriotomy and the orifice of the tricuspid valve,
shows the musculature of the ventricular septum interposing
between the basal margin of the defect (star) and the hinge of the
septal leaflet of the tricuspid valve. The triangle of Koch (cross-
Basal muscular rim
hatched triangle) is to the left-hand side of the surgeon relative to
the defect.
Muscular defects opening into the defects opening into the right ventricular muscular posteroinferior rim to the defect,
outlet of the right ventricle are relatively outlet typically show malalignment of the thus distinguishing the hole from a
rare in patients with concordant muscular outlet septum (Figure 7.87). perimembranous defect with outlet
atrioventricular and ventriculoarterial Close inspection will show whether the extension. When present, the fusion of
connections. If small, the endocardium posterocaudal limb of the septomarginal these muscle bars separates the edge of the
may appear to be heaped up at the edges to trabeculation is fused with the defect from the axis of atrioventricular
produce a fibrous rim (Figure 7.86). Larger ventriculoinfundibular fold to form a conduction tissue. The superior rim is
Apex
Sup. Inf.
Base
Fig. 7.83 This defect is seen through a right atriotomy with the
tricuspid valve retracted. It shows a muscular defect (star) opening
into the outlet of the right ventricle.
Pulmonary infundibulum
Tricuspid
valve
Sup.
Fig. 7.84 In this heart, viewed in anatomical orientation, it
Right Left seems that there are three muscular defects (arrows) opening
anteriorly into the apical trabecular component of the right
Inf. ventricle. Note, however, the presence of the septoparietal
trabeculations (stars).
the muscular outlet septum, combined with latter attached to its left ventricular The third type of ventricular septal
the free-standing subpulmonary surface. If this superior muscular rim is defect is the one that is doubly committed
infundibulum. These tissues separate the attenuated, the leaflet of the aortic valve and juxta-arterial. The phenotypical
leaflets of the pulmonary valve from the may again prolapse through the defect feature of this defect is absence of both the
right coronary leaflet of the aortic valve, the (Figure 7.87). muscular outlet septum, and the posterior
Sup. Aorta
Ant. Post.
Inf.
Left ventricle
Fig. 7.85 The left ventricular view, seen in anatomical

orientation, shows the left ventricular aspect of the heart
illustrated in Figure 7.83. In reality, there is a solitary defect (star) in
the muscular septum but it is crossed by the septoparietal
trabeculations on the right side, giving the spurious impression of
multiple defects.
Pulmonary valve
Sup.
Tricuspid valve
Right Left
Fig. 7.86 The heart is photographed in anatomical orientation,
Inf. showing a muscular defect (star) opening into the outlet of the
right ventricle. Note the accretion of fibrous tissue at the edges of
the defect, reducing its size.
aspect of the free-standing subpulmonary continuity, producing a fibrous raphe that some hearts part of the aortic sinus may
infundibulum. Because of the absence of forms the superior rim of the defect interpose between them, producing valvar
these structures, the facing leaflets of the (Figures 7.69, 7.88). The leaflets can be off-setting. In either event, sutures can be
aortic and pulmonary valves are in fibrous attached at the same level, albeit that in secured in the region of fibrous continuity.
Prolapse of aortic leaflet
Pulmonary valve
Sup.
Right Left
Muscular outlet defect Fig. 7.87 In this heart, viewed in anatomical orientation, the right
Inf.
coronary leaflet of the aortic valve prolapses minimally through a
muscular defect that opens into the outlet of the right ventricle.
Muscular posteroinferior rim
Fig. 7.88 In this heart, the defect opening into the outlet of the
Sup.
right ventricle (star) is doubly committed and juxta-arterial, due to
failure of muscularisation of the subpulmonary infundibulum.
Right Left
The roof of the defect is made up of a fibrous continuity between
the leaflets of the aortic and pulmonary valves (black dotted
Inf.
line). Note the extensive muscular posteroinferior rim to the defect
that protects the atrioventricular conduction axis.
The doubly committed defect can also be similar to that found in the muscular defect ventriculoinfundibular fold (Figure 7.88).
found with overriding of the orifice of the that opens into the outlet part of the right When present, this muscular rim separates
aortic valve (Figure 7.89). In most ventricle, the posterocaudal limb of the the axis of atrioventricular conduction
instances, the inferior rim of the defect is septomarginal trabeculation fusing with the tissue from the edge of the defect.
Overriding of
aortic valve
Fig. 7.89 This heart, photographed in anatomical orientation,

Sup. also has a doubly committed and juxta-arterial ventricular septal
defect opening into the outlet of the right ventricle in the absence
Right Left of muscularisation of the muscular subpulmonary infundibulum.
There is fibrous continuity between the leaflets of the arterial
Inf. valves (blue dotted line). Note the extensive overriding of the
orifice of the aortic valve.
Aortic-pulmonary fibrous continuity

Sup.
Right Left
Inf.
Fig. 7.90 This doubly committed and juxta-arterial ventricular

septal defect (star), shown in anatomical orientation, extends
so that its posteroinferior margin is formed by a fibrous continuity
between the leaflets of the aortic and tricuspid valves, making
it additionally perimembranous. The pathognomonic feature is the
Fibrous continuity with tricuspid valve fibrous continuity between the leaflets of the aortic and pulmonary
valves.
Occasionally, the muscular bundles do not doubly committed (Figure 7.90). The the aortic valve32. Such defects are also
fuse. The defect then extends to be conduction axis, therefore, is much closer much more frequent in oriental as opposed
bordered by fibrous continuity between the to its inferior corner. It is the doubly to occidental populations34.
leaflets of the aortic and tricuspid valves, committed variant that is particularly prone Although the descriptions thus far have
making it perimembranous as well as to set the scene for prolapse of the leaflets of related to ventricular septal defects in
patients having concordant atrioventricular atrioventricular junction can exist with amenable to surgical repair. We will
and ventriculoarterial connections, this shunting exclusively at the ventricular level concentrate on features of immediate
topology, and the guidance it gives to the (Figure 7.52). Patients with this surgical relevance.
site of the conduction axis, is equally valid morphology, however, need to be The anatomy of atrioventricular valves
for patients with concordant distinguished from those with either themselves indicates that problems may be
atrioventricular connections, but abnormal perimembranous defects opening into the encountered at the atrioventricular
ventriculoarterial connections (see right ventricular inlet, or those associated junction, which is contiguous with the
Chapter 8). The only exception to the rules with straddling and overriding of the valvar annulus, in the leaflets, or in
we have described for recognition of the tricuspid valve, as both the latter variants the tension apparatus. Sometimes all the
location of the atrioventricular conduction are found in the setting of separate components of the valve, along with the
axis is produced by overriding and atrioventricular junctions. All of these entire atrioventricular connection, are
straddling of the tricuspid valve (see later). variants must be distinguished from totally absent. This produces the
This arrangement results in a particular muscular defects opening into the inlet of commonest variant of atrioventricular
type of defect that opens into the inlet of the right ventricle (Figure 7.80). There is valvar atresia, which is discussed in
the right ventricle. As we have described, similar phenotypical variability among Chapter 8. The lesions to be considered in
defects with markedly different defects opening into the right ventricular this section can involve either the
phenotypical features, and with markedly outlet, but the major feature of surgical morphologically tricuspid or mitral valves.
different dispositions of the significance in this latter setting is to Because the tricuspid valve usually
atrioventricular conduction axis, can open determine whether a muscular bar is functions in an environment of low
towards the inlet of the morphologically interposed between the caudal rim of the pressure, the lesions are more frequently
right ventricle. This is why it is insufficient defect and the atrioventricular conduction manifest when affecting the mitral valve.
simply to describe them as inlet defects35. axis (compare Figures 7.88 and 7.90). We will deal with the respective lesions in
The commonest defects opening into the turn, indicating their proclivity towards
right ventricular inlet are probably the ones one or the other valve.
that are perimembranous (Figure 7.74). Of considerable surgical significance is
These, and the ones associated with
MALFORMATIONS OF THE overriding of the atrioventricular
straddling and overriding of the tricuspid
ATRIOVENTRICULAR VALVES junction. This means that the valvar
valve, have been described as being of the The pathological lesions that affect orifice is looking into both ventricles,
atrioventricular canal type. It is certainly atrioventricular valves, both acquired and positioned astride a septal defect
the case that hearts with a common congenital, are legion. Not all are (Figure 7.91)27. Almost always, this is
Left atrium
Right atrium
Fig. 7.91 This heart has been sectioned to replicate the ‘four-
Sup.
chamber’ echocardiographic view, and is shown in anatomical
orientation. There is overriding of the orifice of the tricuspid valve
Right Left
(white double-headed arrow), and straddling of its tension
apparatus (white arrows). Note the gross malalignment between
Inf.
the atrial and ventricular septal structures, the star marking the
crest of the muscular ventricular septum.
associated with straddling of the valvar arrangement does not produce malaligned relative to the atrial septum,
tension apparatus, with the tendinous atrioventricular septal malalignment, so no longer inserting at the crux
cords attached to both sides of the that the muscular ventricular septum is (Figure 7.91). The conduction axis,
muscular ventricular septum normally related to the atrial septum at the which is carried on the muscular
(Figure 7.91)27. Although it is the mode crux, and the atrioventricular conduction ventricular septum, originates from an
of insertion of the tension apparatus axis is normally disposed. It is the superior anomalous node in the inferolateral
across the septum that determines the papillary muscle of the mitral valve that is margin of the right atrioventricular
surgical options, the degree of override is abnormally attached within the right junction27, found at the point where the
also important. Overriding of the ventricle. It typically arises from the muscular septum comes into contact
tricuspid valve typically is associated septomarginal trabeculation alongside, but with the junction (Figures 7.94, 7.95).
with malalignment between the atrial separate from, the anterior papillary muscle There is a rudimentary node found within
and ventricular septums, and this has of the tricuspid valve. This arrangement, the regular triangle of Koch, but it makes
major consequences in terms of seen with either discordant no contact with the ventricular
arrangement of the conduction tissues. ventriculoarterial connections, or a double- musculature. The septal leaflet of the
Straddling and overriding, nonetheless, outlet right ventricle with a subpulmonary tricuspid valve is usually tethered to the
can affect either valve, and can occur in defect, producing the Taussig–Bing enlarged inferoseptal papillary muscle of
various segmental combinations27. malformation, can seriously compromise the mitral valve (Figure 7.96). A
Straddling in the setting of a double-inlet surgical repair. We discuss the problems in miniseptation procedure is often
ventricle, and with discordant greater detail in Chapter 8. necessary for complete ventricular
atrioventricular connections, is Straddling of the tricuspid valve is repair, which carries a high risk of
considered in Chapter 8. Here, the found when the ventricular septal defect producing heart block36. It is possible,
concern is with straddling valves opens into the inlet of the right ventricle nonetheless, to place a patch by sewing
coexisting with concordant (Figure 7.93)27. It can exist as an isolated the stitches exclusively in the straddling
atrioventricular connections. lesion, or be found with tetralogy of leaflet of the tricuspid valve (Figures 7.97,
When the mitral valve straddles, it does Fallot, or other abnormal 7.98). This ensures avoidance of the
so through an interventricular ventriculoarterial connections such as conduction tissues. An alternative option
communication that opens into the outlet transposition. The phenotypical feature is is to convert the patient to the Fontan
of the right ventricle (Figure 7.92)27. This that the muscular ventricular septum is circulation.
Straddling mitral valve

Sup.
Right Left
Inf.
Tricuspid valve
Fig. 7.92 This heart has concordant atrioventricular and

discordant ventriculoarterial connections. It is viewed in anatomical
orientation, showing straddling of the tension apparatus of the
mitral valve through a defect opening into the outlet of the right
ventricle.
Fig. 7.93 The heart is photographed in anatomical orientation. It

shows the right atrial aspect of a straddling and overriding tricuspid
Sup. valve in the setting of concordant atrioventricular connections.
There is gross malalignment between the muscular ventricular
Left septum, which carries the atrioventricular conduction axis (black
Right
dotted line), and the atrial septum. Because of this, the
connecting atrioventricular node is formed inferiorly in the
Inf.
atrioventricular junction (star), and is not in the regular triangle of
Koch (triangle).
Apex
Sup. Inf.
Base
Fig. 7.94 The image shows an operative picture through a right

atriotomy in a patient with concordant atrioventricular
connections complicated by straddling and overriding of the
tricuspid valve with basically concordant atrioventricular
connections. Note the malalignment of the muscular ventricular
septum, which carries the atrioventricular conduction axis (white
Straddling tension apparatus dotted line). The axis arises from an anomalous posteroinferior
atrioventricular node (star).
Dilation of the atrioventricular myocarditis. When surgical narrowing of replacement of the valve. Dilation of the
junction occurs almost exclusively as an the orifice is indicated, it can be tricuspid valvar orifice is seen most
acquired lesion. A dilated mitral valvar accomplished using various annuloplasty frequently as a result of right heart
orifice is most frequently secondary to techniques, without resorting to failure.
Malaligned ventricular septum
Apex
Fig. 7.95 The cartoon shows the anomalous location of the
Inf. atrioventricular conduction axis, as seen by the surgeon operating
Sup.
through a right atriotomy in the setting of straddling and
overriding of the tricuspid valve. The rudimentary node found at
Base
the apex of the regular triangle of Koch does not make contact
with the ventricular musculature.
Aorta
Mitral valve
Sup.
Ant. Post. Fig. 7.96 This picture, taken in anatomical orientation, shows
the left ventricular aspect of the heart shown in Figure 7.93. Note
Straddling tricuspid valve Inf. the straddling part of the tension apparatus supporting the
tricuspid valve.
More of a challenge surgically is the inlet and apical trabecular components of around the area of the central fibrous body.
dilation that accompanies Ebstein’s the right ventricle, rather than at the The anterosuperior leaflet is less affected in
malformation37. The crucial feature of this atrioventricular junction (Figure 7.99)38. terms of its junctional attachment, but shows
anomaly is the attachment of the hingepoint The displacement is usually described as important variations in its distal
of the septal and mural leaflets of the being downwards. In reality, there is attachments39. These can be focal
tricuspid valve towards the junction of the rotational displacement of the valvar orifice (Figure 7.100). In more severe cases, the
Sup.
Ant. Post.
Inf.
Mitral valve
Fig. 7.97 In this heart, from a patient with straddling and

overriding of the tricuspid valve, and shown here from the left
ventricle in anatomical orientation, the surgeon placed a patch to
close the malalignment of the ventricular septal defect, keeping his
sutures in the leaflets of the overriding valve, and avoiding the
abnormally located atrioventricular conduction axis (white dotted
Sutures securing patch line [axis] and cross-hatched area [node] superimposed on the
photograph). (Compare with Figure 7.96.)
Sup.
Right Left
Inf.
Fig. 7.98 The image shows the right atrial aspect of the heart
seen also in Figure 7.97. It is photographed in anatomical
orientation, demonstrating how the surgeon was able to close
the ventricular septal defect successfully, at the same time avoiding
the atrioventricular conduction axis (black dotted line). The site of
the abnormal atrioventricular node is shown by the star.
leading edge of the leaflet is attached in setting of Ebstein’s malformation (see also right ventricle is both dilated and thinned.
linear fashion, severely restricting antegrade Chapter 8). Reparative operations often require
flow into the pulmonary trunk Ebstein’s malformation requires surgical placement of sutures in the area of thinning.
(Figure 7.101). In the most severe form, the treatment when there is significant dilation Particular care should be taken to avoid the
anterosuperior leaflet completely blocks this of the true atrioventricular junction, and right coronary artery and its branches. In the
junction, producing tricuspid atresia in the when the wall of the inlet component of the septal area, the triangle of Koch remains
Septal leaflet
Anterosuperior
leaflet
Atrialised Fig. 7.99 The heart is photographed from the right side in
inlet
anatomical orientation. The hinge line of the septal leaflet of the
Apex tricuspid valve has been displaced rotationally (red dotted line)
away from the atrioventricular junction, shown by the black dotted
Sup. Inf. line. This feature, incorrectly described as ‘downwards
displacement’, is the hallmark of Ebstein’s malformation. Note also
the marked dysplasia of the septal leaflet. The black double-
Base
headed arrow shows the thinned wall of the atrialised component
of the right ventricular inlet.
Sup.
Right Left
Inf.
Fig. 7.100 This picture, photographed in anatomical orientation

from the right ventricle, shows the ventricular aspect of the heart
demonstrated in Figure 7.99. The anterosuperior leaflet of the
abnormal tricuspid valve is tethered in focal fashion, with normal
attachments to the medial and anterior papillary muscles. The
Inferior leaflet rotational displacement has produced a bifoliate valve hinged at
Septal leaflet the junction of the inlet and apical trabecular components of the
right ventricle.
the guide to the atrioventricular conduction connections are discordant is discussed of the mural leaflet that is displaced away
axis (Figure 7.102). Ebstein’s malformation in Chapter 8. Rarely, the normally from the atrioventricular junction40,41.
involving the left-sided morphologically located morphologically mitral valve can Malformations of the leaflets can be
tricuspid valve when the atrioventricular show Ebstein’s malformation. It is the hinge summarised in terms of dysplasia, prolapse,
Fig. 7.101 This heart also has Ebstein’s malformation, and is

Sup. photographed in anatomical orientation to show the ventricular
aspect of the abnormal tricuspid valve. In this example, the
Right Left anterosuperior leaflet has grossly abnormal linear attachments
towards the apex of the right ventricle. The valvar orifice is a mere
Linear attachment Inf. keyhole (white double-headed arrow), opening directly into the
subpulmonary infundibulum.
Septal leaflet Anterosuperior leaflet Sup.
Post. Ant.
Inf.
Fig. 7.102 This heart with Ebstein’s malformation, viewed in

anatomical orientation as seen from the right atrium, shows
how the triangle of Koch (cross-hatched triangle) remains the
Atrialised
inlet guide to the atrial components of the atrioventricular conduction
tissue axis, despite the abnormal attachments of the septal
leaflet. Note again the rotational displacement of the hinge of the
Inferior leaflet septal leaflet (red dotted line) relative to the atrioventricular
junction (black dotted line).
and clefting. Dysplastic valves show malformation42. Isolated dysplasia particularly elongation of the tendinous
thickening and heaping up of the substance (Figure 7.103) is exceedingly rare except in cords (Figure 7.105). It may be sufficiently
of the leaflets, usually with obliteration of the neonatal life43, when it is often a fatal lesion. severe to warrant valvar replacement, but the
intercordal spaces. A dysplastic valve may Prolapse occurs more frequently, and prolapsing leaflets can be repaired by various
pose a significant surgical problem. It is seen usually involves the mitral valve techniques, including cordal shortening
frequently with atresia of the outflow tract, (Figure 7.104). It is usually associated (Figure 7.106), and insertion of annular rings
and is an integral part of Ebstein’s with deficiency of the tension apparatus44, (Figure 7.107).
Sup.
Right Left
Septal leaflet
Inf.
Fig. 7.103 The right ventricle in this heart is opened in

clam-shell-like fashion and photographed from the ventricular
apex. The tricuspid valve, although grossly dysplastic, has normal
Inferior leaflet junctional attachments (black dotted lines). This is not Ebstein’s
malformation.
Prolapsing aortic leaflet
Sup.
Left Right
Fig. 7.104 This surgical view of the mitral valve, taken

Inf. through the dome of the left atrium, shows prolapse of the aortic
leaflet.
The true cleft of the aortic leaflet of the atrioventricular septal defects19. The latter guard the left side of the common
mitral valve can be repaired simply by entity (Figure 7.109) is the zone of atrioventricular junction in trifoliate
reconstituting its edges. Such an isolated apposition between the left ventricular fashion.
cleft of the aortic leaflet (Figure 7.108) has components of the bridging leaflets of the We have already discussed some of the
to be distinguished from the lesion often common atrioventricular valve. Along with abnormalities of the tension apparatus that
described as a cleft in the setting of the short mural leaflet, the bridging leaflets accompany malformations of the junction
Sup.
Ant. Post.
Inf.
Fig. 7.105 In this specimen, photographed in anatomical

orientation, there is gross elongation of the cords supporting the
middle scallop of the mural leaflet of the mitral valve (arrow),
which is prolapsed (star).
Sup.
Fig. 7.106 As shown in Fig. 7.105, when the leaflets of the mitral
Left Right valve are prolapsed, the cords supporting them are usually
elongated. This view shows how the surgeon has shortened the
Inf. elongated cords by incising the papillary muscle and suturing the
cords within the muscle (arrow).
or leaflets, such as straddling tension anomalies such as arcade lesions45. Some (Figure 7.110), so that all the cords insert
apparatus. Of those which remain, the confusion exists about the definition of a into a common muscle mass46. In the
so-called parachute deformity is probably parachute valve. It is considered logically as original description47, in contrast, the
the most worrisome lesion, apart from rare fusion of the papillary muscle groups parachute lesion was defined on the basis of
Sup.
Left Right
Inf.
Fig. 7.107 Having shortened the cords of the prolapsed mitral

valve, as shown in Figure 7.106, the surgeon has completed the
repair by inserting an annular ring to support the atrioventricular
junction, which has been reduced by annuloplasty.
Sup.
Left Right
Inf.
Fig. 7.108 This specimen, seen from the inlet aspect of the left
ventricle in anatomical orientation, has a cleft (arrow) in the
aortic leaflet of an otherwise normally structured mitral valve. This
lesion should be distinguished from the space between the left
ventricular bridging leaflets, or the so-called ‘cleft’, found in the
left atrioventricular valve of hearts with deficient
atrioventricular septation and a common atrioventricular junction
(see Figure 7.109).
gross hypoplasia of one end of the zone of difficult, and valvar replacement is likely to coarctation of the aorta. The combinations
apposition between the leaflets, together be necessary48. Parachute deformity of are known as Shone’s syndrome47.
with an absence of its supporting papillary the mitral valve may be complicated Parachute malformation of the tricuspid
muscle. Irrespective of how the lesion is further by other lesions, such as a valve can occur, but is rarely of clinical
defined, surgical reconstruction is supravalvar left atrial stenosing ring, and significance49.
Sup.
Ant. Post.
Inf.
Fig. 7.109 This heart with deficient atrioventricular septation, a

common atrioventricular junction, and shunting exclusively at the
ventricular level, the so-called ‘ostium primum’ defect, is
photographed from the left side in anatomical orientation. Note
the difference between the zone of apposition between the left
ventricular components of the bridging leaflets (arrow), the so-
Inferior bridging leaflet Mural leaflet
called ‘cleft’, and the true cleft of the aortic leaflet of an otherwise
normal mitral valve shown in Figure 7.108.
Fig. 7.110 This specimen, removed at surgery, shows the so-

called ‘parachute’ arrangement of the mitral valve. There is fusion
of the papillary muscles, along with thickening and fusion of the
tendinous cords.
across the arterial valves fully, it is essential column of blood that stenosis or
MALFORMATIONS OF THE to have a firm grasp of the arrangement of regurgitation occurs within the valvar
ARTERIAL VALVES AND the valvar leaflets at the ventriculoarterial complex.
OUTFLOW TRACTS junction. As described in Chapters 2 and 3, Valvar aortic stenosis may occur with
In this section, we consider the surgical the arterial valves do not possess an annulus unicuspid, bicuspid, tricuspid and, rarely,
aspects of subvalvar obstruction of the in the sense of a circular ring of collagen even quadricuspid configurations.
ventricular outflow tracts, valvar stenosis, that supports the leaflets in the fashion of a Dysplastic lesions are also seen in the aortic
and atresia of the outflow. In the normally circle. The only ring within the valvar valve, but only rarely can surgery provide
connected heart, obstruction in the left complex, aside from the sinutubular the answer to this problem. The unicuspid
ventricular outflow tract produces junction, is the circular area over which the valve has two raphes at the ends of abortive
subaortic stenosis. It must be remembered fibrous wall of the great arterial trunk is zones of apposition (Figure 7.114). The
that the same anatomical lesions will supported by the underlying ventricular leaflets are also abnormally attached in
produce subpulmonary obstruction in the structures50. Surgeons do not define this linear rather than semilunar fashion
patient with discordant ventriculoarterial ring, however, as the annulus51. The (Figure 7.115). Surgical opening of the
connections. Similarly, obstruction of the junctions are partly muscular and partly raphes in the conjoined leaflets typically
right ventricular outflow tract produces fibrous in the left ventricle results in valvar incompetence. The
subpulmonary obstruction in the heart (Figure 7.111)52, but exclusively muscular creation of new interleaflet triangles,
with normal segmental connections, but at the right ventriculoarterial junction supplementing the leaflet tissue so as to
subaortic stenosis when the (Figure 7.112)50. The attachments of the create new semilunar hinges, is likely to
ventriculoarterial connections are leaflets are arranged as half moons, with the produce greater clinical success.
discordant. When both outflow tracts are bases of the leaflets attached to ventricular A valve with two effective leaflets is seen
connected to the same ventricle, the muscle, but the apex of the leaflets attached most frequently in the adult patient.
anatomical problems are more discrete. to the fibrous wall of the arterial trunk at Perhaps this is because such a bicuspid
These are considered separately in the sinutubular junction. When seen in the valve, in itself, is not usually intrinsically
Chapter 8. closed position, the three leaflets coapt stenotic. It is only with the effects of time
Stenosis of the aortic valve can occur at snugly along their zones of apposition, and turbulence that the bifoliate valves
valvar, subvalvar, and supravalvar levels. which extend from the circumferential become manifestly obstructive. If the
Aortic regurgitation is ultimately a valvar margins of the arterial wall to the centre of valvar morphology has not been totally
problem, and the perivalvar anatomy is the valve (Figure 7.113). It is on the basis of obscured by calcific deposits, a bicuspid
often of great importance. To understand perturbation of this coaptation of the valve seen at operation will take one of two
the substrates for stenosis and regurgitation leaflets under pressure of the diastolic forms. Occasionally the two leaflets are of
Right coronary aortic sinus Muscular support
Sup.
Ant. Post.
Inf.
Fig. 7.111 The aortic outflow tract has been spread open and is
Fibrous support photographed from the ventricular aspect in anatomical
orientation. The arterial valvar leaflets are attached in semilunar
Non-coronary aortic sinus Left coronary aortic sinus fashion, taking their origin in part from fibrous tissue, and in part
from the muscular ventricular septum.
Anatomical ventriculoarterial junction
Fig. 7.112 In this anatomical specimen, the subpulmonary

infundibulum has been opened and photographed in anatomical
orientation, the leaflets of the pulmonary valve having been
removed. Note that the semilunar attachments of the leaflets cross
the circular anatomical ventriculoarterial junction. This is more
Muscular support obvious than in the subaortic outflow tract (see Fig. 7.111), but the
basic arrangement is comparable.
Fig. 7.113 In this specimen, the aortic valve is photographed from

above in its closed position. The three zones of apposition between
the leaflets (red double-headed arrows) extend from the
sinutubular junction at the periphery (stars) to the centre of the
valvar orifice (red oval). The leaflets are closed by the hydrostatic
pressure of the column of blood they support.
equal size, the solitary zone of apposition conjoined, leaflet usually exhibiting a sinuses giving rise to the coronary arteries,
between them bisecting the aortic root raphe, which can be eccentrically placed then both arteries will arise from the
(Figure 7.116). This type of valve is (Figure 7.117) or centrally positioned conjoined sinus. Alternatively, when
frequently found in patients with (Figure 7.118) within the conjoined leaflet. the conjoined sinus is formed from the
coarctation of the aorta. In the other form, If the conjoined leaflet is produced by right coronary and non-coronary aortic
the leaflets are unequal, with the large, or fusion of the leaflets guarding the aortic sinuses, the coronary arteries will be
Fused zones of apposition

Sup.
Left Right
Inf.
Fig. 7.114 This view of an abnormal aortic valve seen through an

aortotomy shows the so-called unicuspid and unicommissural
arrangement. Fusion of two of the putative zones of apposition
Solitary patent zone of apposition during development leaves the persisting zone of apposition as the
eccentric valvar orifice.
Fig. 7.115 This picture of the aortic valve, taken in a specimen

with the left ventricular outflow tract having been opened, shows
the unicuspid and unicommissural arrangement as demonstrated
Sup. in Fig. 7.114. There is loss of the semilunar suspension of the
leaflets (black dotted line), so that paradoxically they are
Ant. Post.
Mitral valve attached in true annular fashion. The solitary zone of apposition
(red arrow) points backwards towards the mitral valve. Note the
Inf. marked reduction in height of the remaining interleaflet triangles
(white arrows).
positioned so that one coronary artery follow-up. When the conjoined leaflet is calcification, some relief from the stenosis
arises from the conjoined sinuses, and the made up of the right and non-coronary can be obtained by careful enlargement of
other from the third sinus53. It is aortic leaflets, it is common to find the ends of the solitary zone of apposition.
increasingly recognised that the two degenerative disease of the aortic walls. If Careful follow-up is essential, especially
phenotypes carry significant differences valves with two effective leaflets are seen when the phenotype points towards
with regard to anticipated problems during before they become rigid and distorted by degenerative disease.
Sup.
Left Right
Inf.
Fig. 7.116 This picture, taken in the operating room through

an aortotomy, shows an aortic valve with two leaflets of
comparable size.

Sup.
Left Right
Inf.
Fig. 7.117 This picture, taken in the operating room through

Non-coronary aortic sinus Right coronary aortic sinus an aortotomy, shows a bicuspid aortic valve with a raphe (star) in
one of the leaflets.
Aortic stenosis also occurs in patients in the aortic root, may lead to the partly muscular and partly fibrous. The
with valves having three leaflets, but is not development of calcification and stenosis in muscular portion comprises the
usually seen until later in life. A possible the elderly (Figure 7.119)54. ventriculoinfundibular fold anterolaterally,
cause of such stenosis is the unequal size of Subvalvar stenosis may be fibrous, the small outlet component of the muscular
the leaflets in the otherwise normal aortic fibromuscular, or muscular, reflecting the septum anteriorly, and the upper edge of
valve. This, coupled with the high pressure fact that the left ventricular outflow tract is the apical part of the muscular septum
Sup. Left coronary aortic sinus
Left Right
Inf.
Fig. 7.118 In this specimen, the aortic valve is photographed

from behind in anatomical orientation, the left ventricular outflow
tract through the mitral valve having been opened. The two
leaflets arising from the sinuses giving rise to the coronary
Non-coronary aortic sinus arteries have fused, with the line of fusion represented by the
Right coronary aortic sinus
raphe (star).
Sup.
Left Right
Inf.
Fig. 7.119 This abnormal aortic valve, photographed from the

aortic aspect, has fusion of two of the zones of apposition
(black dotted lines) between the leaflets, with calcification also
present. This is the typical substrate of aortic stenosis as seen in the
elderly.
posteriorly. The fibrous part comprises the Of the variants producing fixed circular. This is not always the case. A
central fibrous body, the area of continuity stenosis, a subvalvar fibrous shelf is relatively thin shelf of tissue sometimes
between the leaflets of the aortic and mitral perhaps most easily approached runs from beneath the non-coronary
valves, and the left fibrous trigone. surgically. It appears circular when leaflet of the aortic valve, originating over
Subvalvar stenoses may also be either fixed viewed through the usually normal aortic the site of the penetrating bundle, then
or dynamic in nature. valve (Figure 7.120). Indeed, it can be extending to the septal musculature,
Subvalvar fibrous shelf

Sup.
Left Right
Inf.
Fig. 7.120 This view, taken in the operating room through the
Non-coronary aortic sinus aortic valve, shows a circular fibrous shelf producing subaortic
stenosis.
Sup.
Left Right
Inf.
Mitral valve
Fig. 7.121 This specimen is opened anteriorly through the

subaortic outflow tract, and is photographed in anatomical
orientation. Note the extensive shelf-like lesion producing
subvalvar stenosis, and extending onto the aortic leaflet of the
mitral valve (arrows).
eventually coursing over the particular care where the shelf intimately may lead to detachment of that structure.
ventriculoinfundibular fold. The shelf can overlies the conduction tissues, a In cases where the ventricular septum
also extend laterally to involve the aortic circumferential lesion can be removed appears to be playing a part in causing the
leaflet of the mitral valve (Figure 7.121). If completely (Figure 7.122). Too vigorous stenosis, it may be prudent to remove a
dissection is performed carefully55, taking an attack on the side of the mitral valve segment of muscle (Figure 7.123).
Fig. 7.122 This specimen is the subaortic shelf shown in

Figure 7.120 subsequent to its surgical removal. Note the horseshoe
configuration.
Sup.
Left Right
Inf.
Fig. 7.123 This view, taken in the operating room through the
aorta, shows how a segment of ventricular muscle (arrow) can
be removed safely to relieve shelf-like fibrous obstruction of the
left ventricular outflow tract.
In cases where complete removal proves producing a fibromuscular tunnel. Surgical A much more rare form of fixed
difficult, interruption of the fibrous shelf in correction, however, may be less successful subaortic obstruction is produced by
the safe area over the ventriculoinfundibular than with a simple shelf, because the tunnel hypertrophy of the usually inconspicuous
fold will result in a safe and satisfactory relief extends farther into the left ventricle, anterolateral muscle bundle56. This muscle
of the stenosis. The same rules apply when making the obstruction it produces more runs down the outflow tract from the
resecting the variant of aortic stenosis difficult to relieve. ventriculoinfundibular fold to the
ventricular septum. In its course over the with atrioventricular septal defect. The isolated lesion in the normally connected
parietal wall, it would not be expected to latter occurs only in the presence of a heart57. They can produce significant
involve the conduction tissues. ventricular septal defect (Figure 7.124). obstruction of the left ventricular outflow
Anomalous attachment of the left The fixed type of subaortic obstruction is tract in hearts with an atrioventricular
atrioventricular valve can also cause fixed also produced by so-called tissue tags. These septal defect (Figure 7.125), or with
obstruction, as can a deviated muscular can herniate from any adjacent fibrous tissue discordant ventriculoarterial connections
outlet septum. The former is usually seen structure, but are exceedingly rare as an (see Chapter 8).
Muscular outlet ventricular septal defect
Deviated muscular outlet septum
Sup.
Fig. 7.124 This anatomical specimen shows the left ventricle
Ant. Post. opened in clam-shell-like fashion, and viewed from the apex of the
left ventricle. There is a fixed subaortic obstruction produced by the
Inf. Narrowed subaortic outflow tract posterior deviation of the muscular outlet septum through a
Sup.
Ant. Post.
Fig. 7.125 This heart is opened through the subaortic outflow
tract, and photographed in anatomical orientation. There is an
Inf. atrioventricular septal defect with a common atrioventricular
junction, with subaortic obstruction due to tissue tags (arrows).
Dynamic subvalvar obstruction is a sinuses and the coronary arteries. The critical importance of the anatomy
result of thickening of the septal Secondly, the circumferential narrowing at of this region perhaps is best demonstrated
musculature abutting the aortic leaflet of the sinutubular junction (Figure 7.127) by the problems exhibited by patients with
the mitral valve during ventricular systole. tends to tether the three aortic leaflets at the endocarditis of the aortic valve61. Because
This usually creates a ridge of thickened ends of their zones of apposition in such a the valve is the keystone to all the other
endocardium, easily seen through the way that it is rarely enough to perform a valves and chambers of the heart
aortic valve. If an operation becomes simple aortoplasty60. If possible, all three (Figure 7.136), an eroding abscess in the
necessary, the hypertrophied muscle sinuses should be opened to release the aortic root may lead to formation of a fistula
bundle can be resected, offering tethering of the leaflets. This can be involving any of these adjacent structures.
satisfactory relief of the obstruction58. accomplished by resecting the thickened The patient may present with findings of
Again the surgeon must scrupulously avoid sinutubular junction, and inserting left heart failure, left-to-right shunting,
the conduction tissue as it emerges beneath pericardial patches in each sinus complete heart block, or any combination
the zone of apposition between the right (Figures 7.128–7.131). of these, in addition to the usual signs of
and non-coronary leaflets and descends on Aortic valvar insufficiency may be due sepsis. Surgical management requires a
the muscular ventricular septum to congenital malformation of the valve detailed knowledge of this area, as the
(Figure 7.126). Interventionists are now (Figure 7.132), its supporting structures surgeon may be faced with virtual
able to offer alternative therapy by injecting (Figure 7.133), or both. It may also be disruption of the ventriculoarterial
alcohol into the first septal perforating secondary to an infectious process in the connection61. A similar problem can occur
artery. aortic root (Figure 7.134), or to when the aortic root, or the fibrous coronet,
Supravalvar aortic stenosis occurs in degenerative disease of the aortic walls. is severely damaged by dissection or
hourglass, membranous, and more diffuse Occasionally, aortic insufficiency may be marked degeneration of its fibrous
tubular variants. All forms are rare. due to trauma. Its frequent association structure.
Fortunately, the severe tubular type is with the doubly committed and juxta- As with aortic stenosis, stenosis of the
extremely unusual. Two problems are arterial ventricular septal defect suggests right ventricular outflow can occur at the
shared by all three varieties because of that deficiency in the structures valvar, supravalvar, or subvalvar levels.
narrowing of the aorta at the junction of the supporting the leaflets plays some role in The latter is discussed in association with
sinuses with the ascending tubular aorta59. these problems. Prolapse of the leaflets, tetralogy of Fallot (see later). Dysplasia of
Firstly, the aortic sinuses, which usually and insufficiency, may occur with other the valvar leaflets is most often seen as
contain the coronary arteries, may be types of ventricular septal defect marked distortion and thickening, although
converted into high pressure zones, in (Figure 7.135). Prolapse can be found three discrete leaflets can sometimes be
which the arteries provide the only run-off even when the ventricular septum is recognised (Figure 7.137). It can be
other than through the distal stenosis. This intact, the latter situation usually being associated with insufficiency as well as
can produce marked dilation of both the associated with a bicuspid aortic valve. stenosis.
Central fibrous body
Mitral
valve
Sup.
Left bundle
Left Right branch
Tricuspid Fig. 7.126 The cartoon shows the location of the atrioventricular
valve
Inf. Atrioventricular node conduction axis as it would be visualised by the surgeon working
through the aortic valve.
Sup.
Ant. Post. orientation, there is severe narrowing at the level of the
sinutubular junction (arrows). Although usually termed
Inf. ‘supravalvar’, the obstruction involves the attachment of the valvar
leaflets (stars) at the sinutubular junction.
Aortic root
Pulmonary trunk
Tubular aorta
Left
Sup. Inf.
Fig. 7.128 This picture, taken in the operating room through a
Right median sternotomy, shows narrowing (arrows) of the aorta at the
level of the sinutubular junction.
Isolated pulmonary stenosis typically the leaflets are attached to the wall of the opening (Figure 7.139).The arterial root
is found in the form of a dome-shaped pulmonary trunk along the peripheral is narrowed at the sinutubular junction.
valve with three well-developed but fused ends of the zones of apposition between This is an integral part of the valvar
commissures (Figure 7.138)50. Typically, them, leaving only a restricted central mechanism, although such narrowing is
Left coronary aortic leaflet
Left
Fig. 7.129 In the patient shown in Figure 7.128, the surgeon has
made an extensive vertical incision into the non-coronary sinus or
Sup. Inf.
the aortic root, revealing the marked constriction at the
sinutubular junction, with a particularly narrow entry to the left
Right Non-coronary aortic leaflet coronary aortic sinus, which is guarded by the edge of the valvar
leaflet.
Left
Sup. Inf. Fig. 7.130 The attachments of the left and non-coronary leaflets
at the thickened sinutubular junction, as shown in Figure 7.129,
Right have been liberated (arrow), thus releasing the left coronary leaflet
so as to allow its full excursion.
often described as being supravalvar. satisfactory relief of the obstruction. The obstruction at the subvalvar level may
These areas of tethering can be effectiveness of surgery is best measured maintain a pressure gradient across the
dissected from the arterial wall and six to nine months after operation, as outflow tract. This muscular hypertrophy
incised, providing a particularly significant secondary muscular will almost always regress with time62.
Left
Sup. Inf.
Fig. 7.131 In the patient shown in Figures 7.129 and 7.130, a
helical pericardial patch (white double-headed arrow) has been
Right inserted in the incision to the non-coronary sinus, thus enlarging
the sinutubular junction and the ascending aorta.
Sup.
Left Right
Fig. 7.132 This view, taken in the operating room through an
Inf. aortotomy, shows a regurgitant aortic valve as the consequence of
tethering of one of its leaflets (arrow).
Despite the excellent results of surgery, doubtful, however, whether inflation of a True supravalvar stenosis usually
treatment of congenital pulmonary valvar balloon will ever rival the anatomical takes the form of a waist-like narrowing of
stenosis has become largely the province precision achieved by the competent the pulmonary trunk distal to the
of the interventional cardiologist. It is surgeon. sinutubular junction (Figure 7.140),
Sup.
Left Right
Inf.
Fig. 7.133 This anatomical specimen is photographed from

above, showing failure of central coaptation of the leaflets of the
aortic valve because of dilation at the sinutubular junction.
Sup.
Left Right
Fig. 7.134 This operative view, taken through an aortotomy,

Inf. shows a perforation (arrow) in one leaflet of a bicuspid aortic valve,
due to infective endocarditis.
Pulmonary
trunk
Left
Sup. Inf.
Fig. 7.135 This operative view, seen through a right
ventriculotomy, shows prolapse of the leaflets of the aortic valve
Right (star) in the setting of a perimembranous ventricular septal defect
opening into the outlet of the right ventricle.
Pulmonary
root
Mitral
valve
Tricuspid
Sup. valve
Post. Ant.
Fig. 7.136 The short axis of the ventricular mass has been
displayed by removing the atrial musculature and the arterial
Inf. trunks, showing the ‘keystone’ location of the centrally positioned
aortic valve (star).
Left
Sup. Inf.
Fig. 7.137 This operative view, taken through an incision in the
Right pulmonary trunk, shows gross dysplasia of the leaflets of the
pulmonary valve.
Fig. 7.138 This pulmonary valve is viewed from above in

anatomical orientation. There is extensive fusion of the zones of
apposition between the leaflets, leaving a domed membrane with
a central orifice the size of a pinhole. Note the tethering of the
domed valvar tissue to the walls of the pulmonary trunk at the sites
of fusion of the leaflets (arrows).
although it may occur at the arteries supplying the lung directly more akin to a segment of
sinutubular junction, or anywhere at from the aorta in cases of tetralogy of tubular hypoplasia. These lesions, if
one or more locations within the Fallot with pulmonary atresia63. Very anatomically accessible, are
pulmonary arterial tree. Narrowing has rarely, the obstructions may be amenable to enlargement using a simple
also been reported within collateral membrane-like, but the usual lesion is patch.
Left
Sup. Inf.
Right
Fig. 7.139 This operative view, taken through an incision in the

pulmonary trunk, shows fusion of the zones of apposition between
the leaflets of the pulmonary valve, with tethering of the fused
leaflets to the wall of the pulmonary trunk (arrows), leaving a
constricted central opening (star).
Pulmonary root
Left
Sup. Inf.
Right
Fig. 7.140 The view, taken in the operating room, shows the
Pulmonary trunk external narrowing (arrows) at the sinutubular junction of the
pulmonary valve demonstrated in Figure 7.139.
When there is extreme overriding of the hallmark is anterocephalad deviation of

TETRALOGY OF FALLOT aortic valvar orifice, it can also show the the insertion of the muscular or fibrous
One form of obstruction of the right abnormal ventriculoarterial connection of a outlet septum, combined with
ventricular outflow tract is so clearly double-outlet right ventricle, but it is subpulmonary obstruction due to
demarcated that it constitutes an entity in convenient to discuss the entity at this hypertrophy of septoparietal
its own right, namely tetralogy of Fallot. point of our narrative. Its anatomical trabeculations64. In the normal heart
Sup. Supraventricular crest
Post. Ant.
Inf.
Aorta
Fig. 7.141 The normal right ventricle is photographed in

anatomical orientation, showing the septal aspect. The
supraventricular crest inserts between the limbs of the
septomarginal trabeculation. Although when, using dissection, it is
possible to create a direct communication with the left
Tricuspid valve ventricular outflow tract through the muscle inserted between the
limbs of the septomarginal trabeculation, it is not possible, in
Septomarginal the normal heart, to distinguish where the septal component
trabeculation finishes, and where the musculature becomes that of the parietal
ventricular wall.
(Figure 7.141), the muscular outlet the leaflets of the aortic and tricuspid The cases in which the posterior
septum is inconspicuous, being the small valves in the inferocaudal quadrant of the limb of the septomarginal trabeculation
area of the supraventricular crest inserted defect thus defined, making it has fused with the ventriculoinfundibular
between the limbs of the septomarginal perimembranous (Figure 7.143). fold superior to an intact interventricular
trabeculation. In this position, it is fused Alternatively, the inferocaudal rim membranous septum (Figure 7.144),
with the much more extensive can be muscular when the thus protecting the atrioventricular
ventriculoinfundibular fold, the crest itself ventriculoinfundibular fold fuses with the conduction tissues, account for about
supporting the distal free-standing caudal limb of the septomarginal one-fifth of the overall population with
infundibular sleeve. trabeculation (Figure 7.144)65. These this lesion. In this setting, superficial
In tetralogy of Fallot, the outlet features have the same implications for sutures can be placed along the entire
part of the septum is malaligned protection of the axis of atrioventricular muscular margin of the right
anterocephalad to the anterior limb of the conduction tissue as they do in isolated ventricular aspect of these defects
septomarginal trabeculation, becoming ventricular septal defects (see earlier). without fear of traumatising the
recognisable in its own right. The deviated When the inferocaudal margin is fibrous, conduction axis.
position of the septum, combined with the atrioventricular conduction axis In rare cases, more frequently
hypertrophy of the septoparietal penetrates beneath the atrioventricular encountered in Asia or South America69,70,
trabeculations, serves to narrow the membranous septal component of this the outlet septum is fibrous rather than
subpulmonary outflow tract. At the area (Figure 7.145). Often this is overlain muscular, with failure of formation of the
same time, it leaves an interventricular by the membranous flap, or pseudoflaps posterior aspect of the free-standing
communication that is overridden by derived from the tricuspid valve66. subpulmonary infundibular sleeve. The
the leaflets of the aortic valve Usually, in tetralogy of Fallot, the non- defect is thus doubly committed and
(Figure 7.142). branching and branching components of juxta-arterial (Figure 7.148). The
The interventricular communication, the atrioventricular bundle are carried significant surgical feature in these cases is
therefore, is positioned beneath the down the left ventricular side of the again the relationship of the conduction
ventricular outlets in association with septum, being positioned some distance axis in the inferior border of the defect,
malalignment of the muscular outlet from the septal crest. In a minority of which may be either muscular or
septum. Therefore, it is the rightwards cases, the bundle can branch directly perimembranous.
margin of the cone of space subtended by astride the septum67,68. It can then be While it is clearly important to close
the overriding valvar leaflets that should traumatised (Figure 7.146) by sutures the ventricular septal defect securely in
be defined as the ventricular septal defect. placed directly through the septal crest patients with tetralogy of Fallot,
There can be fibrous continuity between (Figure 7.147). patching the aorta into the left ventricle,
Outlet septum Pulmonary valve
Ventriculoinfundibular
fold
Aortic valve
Sup.
Post. Ant.
Fig. 7.142 This view of the outlet of the right ventricle of the
heart from a patient with tetralogy of Fallot, taken in anatomical
Inf. Septomarginal trabeculation orientation, shows the divorce of the muscular structures that
combine to form the normal subpulmonary outflow tract.
Aortic valve Pulmonary valve
Fibrous
continuity
Tricuspid valve
Fig. 7.143 This view of an anatomical specimen, photographed in

anatomical orientation, shows the usual variant of the ventricular
Sup. septal defect in tetralogy of Fallot, in which the posteroinferior
border is formed by a fibrous continuity between the leaflets of the
Post. Ant. aortic and tricuspid valves, thus making it perimembranous. The
star shows the deviated muscular outlet septum, which supports a
Inf. sleeve of free-standing subpulmonary infundibular musculature
(arrow).
probably the most important feature success is the size of the pulmonary successfully without incising across the
for a successful surgical outcome, is trunk. Tables are available for ventriculoarterial junction71,72. Such
relief of the subpulmonary obstruction. preoperative evaluation to select those incisions are usually described as being
One of the major determinants of this patients who can be corrected transannular.
Outlet septum
Ventriculoinfundibular
fold
Sup.
Post. Ant. Fig. 7.144 In this specimen with tetralogy of Fallot,

photographed in anatomical orientation, the defect (star), as seen
Septomarginal trabeculation from the right ventricle, has exclusively muscular borders, because
Inf.
of fusion posteroinferiorly between the caudal limb of the
septomarginal trabeculation and the ventriculoinfundibular fold.
Membranous flap
Sup.
Post. Ant.
Inf.
Fig. 7.145 This anatomical specimen with tetralogy of Fallot,

photographed in anatomical orientation, has been prepared by
removal of the septal leaflet of the tricuspid valve. It shows the site
of the remnant of the interventricular membranous septum,
known as the membranous flap, and illustrates the relationships of
the atrioventricular conduction axis (red dotted line) when the
ventricular septal defect is perimembranous. The star shows the
Patch
Sup.
Fig. 7.146 In this heart, viewed in anatomical orientation from

Ant. Post.
the left ventricle, the defect in a patient with tetralogy of Fallot was
repaired by placing sutures directly through the crest of the
Inf. Haematoma on septal crest muscular ventricular septum. Note the haemorrhage produced at
the septal crest.
Haemorrhage
in AV bundle
Sup.
Fig. 7.147 The histological section is taken across the ventricular
Right Left.
septum in the heart shown in Figure 7.146. The atrioventricular
(AV) conduction tissue axis in this heart branched directly astride
Inf. the septum. The atrioventricular bundle was traumatised by a
suture (arrow) securing the patch, producing atrioventricular block.
An understanding of the precise the subpulmonary infundibulum, with its structure is always the hypertrophied
anatomy of the subpulmonary outflow tract parietal segment produced by hypertrophy septal insertion of the outlet septum, which
is also vital if the surgeon is to plan a of free-standing septoparietal can be incised without fear of damaging
reproducible operation for successful relief trabeculations. Knowledge of this feature is vital structures. At the same time, any
of the muscular obstruction accurately. important to the surgeon when deciding free-standing septoparietal trabeculations
This is the consequence of formation of a which muscle to resect so as to widen the should be identified and removed
constrictive muscular ring at the mouth of narrowed outflow tract. The major limiting (Figures 7.149, 7.150). They, too, never
Pulmonary
trunk
Aorta
Fig. 7.148 This specimen, viewed in anatomical orientation from

Sup.
the apex of the right ventricle, shows tetralogy of Fallot with a
doubly committed and juxta-arterial defect due to failure of
Right Left.
formation of the muscular subpulmonary infundibulum. Note the
Aortic-pulmonary fibrous continuity between the leaflets of the aortic and pulmonary
Inf. Perimembranous VSD continuity valves, showing that the defect is also perimembranous. VSD,
Outlet septum Septoparietal

Left trabeculation
Base Apex
Right
Fig. 7.149 This view, taken in the operating

room through a right infundibulotomy, shows
the stenotic orifice of the subpulmonary
infundibulum in a patient with tetralogy of
Fallot. The stenosis is formed in part by the
Pulmonary valve Infundibular orifice
hypertrophied outlet septum, and also by
septoparietal trabeculations.
contain vital structures. The body of the aortic valve arising from its left ventricular fold, which is the inner curvature of the
outlet septum usually contributes to aspect. heart. Care must be taken not to perforate
obstruction, and it is tempting to resect this It is also usual to resect the parietal to the right-sided atrioventricular groove in
structure. Excessive resection, however, insertion of the outlet septum (Fig. 7.151). this region. Dissection, or injudicious
may lead to damage to the leaflets of the This fuses with the ventriculoinfundibular placement of sutures, can damage the
Septoparietal trabeculations
Left
Base Apex
Right
Fig. 7.150 In the patient shown in

Figure 7.149, the surgeon was able to liberate
Infundibular orifice the septoparietal trabeculations. They could
then be excised.
Outlet septum
Resected
septoparietal
bands
Left
Base Apex Fig. 7.151 In the patients shown in Figures 7.149 and 7.150, the
Resected parietal attachment obstruction at the mouth of the infundibulum was
of septal insertion Right completely relieved by resecting the parietal extension of the
outlet septum.
right coronary artery73. It is very unusual hypertrophied, particularly when the latter muscle of the tricuspid valve often arises
for the septomarginal trabeculation itself structure has a high take-off. Severe from the inlet aspect of the obstructing
to contribute to the subpulmonary hypertrophy produces a two-chambered shelf. Care must be taken, therefore, not to
obstruction. Thus, it is usually unnecessary right ventricle74, when the intervening damage this muscle during resection.
to resect its limbs. Its body, and the muscle band may require resection The final variable in tetralogy of Fallot is
moderator band, nonetheless, may be (Figure 7.152). The anterior papillary the connection of the leaflets of the
Sup.
Post. Ant. Fig. 7.152 In this heart from a patient with tetralogy of Fallot,
shown in anatomical orientation, there is hypertrophy of the body
of the septomarginal trabeculation (star), separating the apical
Inf.
trabecular component into two parts (arrows). This is the so-called
‘two-chambered right ventricle’.
overriding aortic valve. Depending on this Patients with tetralogy of Fallot can window, or coronary arterial fistulas77. In
feature, the aorta can be connected mostly to have multiple associated lesions, such as most instances, nonetheless, the lungs are
the left ventricle, making the straddling and overriding of the tricuspid supplied either through a persistently
ventriculoarterial connection concordant, or valve, or deficient atrioventricular patent arterial duct (Figure 7.154), or
connected mostly to the right ventricle, and septation. The end point is then a through major aortopulmonary collateral
hence producing a double-outlet combination of the anatomical lesions arteries78. It is rare to find such collateral
connection. The degree of override should already discussed. One of the most arteries supplying the pulmonary
not affect the surgical procedure markedly. important associated lesions is the circulation except when the intracardiac
With greater commitment of the aorta to the presence of pulmonary atresia rather than anatomy is that of tetralogy of Fallot.
right ventricle, nonetheless, the placement pulmonary stenosis. This combination is Additionally, only very rarely, will a
of the patch tunnelling the aorta to the left often described as pulmonary atresia duct and collateral arteries supply the
ventricle, and closing the ventricular septal with ventricular septal defect, which is same lung, although these two sources of
defect, becomes more important. The not an incorrect designation. The variant flow can supply separate lungs
internal conduit constructed from the left found with the intracardiac anatomy of (Figure 7.155). When a duct is present,
ventricle to the aorta may complicate relief the tetralogy, however, is so distinctive and the pulmonary arteries are confluent,
of the obstruction to the right ventricular that it should be described as tetralogy of the arteries supply the entirety of the
outflow tract further, because it is always Fallot with valvar or infundibular lung parenchyma (Figure 7.155),
necessary to ensure an adequate outlet from pulmonary atresia75. The intracardiac although they may be variably
the left ventricle. anatomy includes deviation of the developed. The degree of hypoplasia
Although the primary obstruction in muscular outlet septum sufficient to determines whether total correction is
tetralogy of Fallot is at the infundibular block the subpulmonary infundibulum feasible.
level, the pulmonary valve is frequently completely (Figure 7.153). The anatomy When the right and left pulmonary
stenotic, being bifoliate in the majority of the ventricular septal defect can vary as arteries are not confluent, or when major
of patients. Any stenosis must be in tetralogy of Fallot, with the same aortopulmonary collateral arteries are
relieved during operative repair. The surgical connotations. The feature that present, the situation is more complex.
sequels of postoperative pulmonary dominates the surgical options is the Non-confluent pulmonary arteries can be
regurgitation only now are becoming morphology of the pulmonary arteries. supplied independently by bilateral ducts,
evident. It could be that, in the long term, Exceedingly rarely, the pulmonary or one lung can be supplied by a duct and
they will be just as troublesome as residual arteries may be supplied through a the other through collateral arteries
pulmonary stenosis, and less easy to relieve. persistent fifth arch76, an aortopulmonary (Figure 7.156). It is more usual for
Pulmonary
Sup. atresia
Post. Ant.
Inf.
Aorta
Fig. 7.153 In this heart from a patient with tetralogy of Fallot

with pulmonary atresia, viewed in anatomical orientation, there
Blind-ending are confluent pulmonary arteries supplied by an arterial duct. Note
subpulmonary the extreme anterocephalad deviation of the muscular outlet
infundibulum septum (star), with muscular atresia at the ventriculoarterial
junction.
Arterial duct
Aorta
Sup.
Right Left Fig. 7.154 The computed tomographic angiogram from a

patient with tetralogy of Fallot and pulmonary atresia shows
Confluent pulmonary arteries confluent pulmonary arteries fed through a persistently patent
Inf.
arterial duct.
collateral arteries to supply both lungs with collateral arteries (Figure 7.157). It is arteries. It is known that collateral arteries
no duct being present. important to establish how much of the can anastomose with the pulmonary
Well-developed confluent pulmonary pulmonary parenchyma is connected to the arteries at the hilum (Figure 7.158), or
arteries usually coexist within the intrapericardial pulmonary arteries, and extend into the parenchyma to supply lobar
pericardial sac even when there are how much is supplied directly by collateral or segmental arteries directly.
Sup.
Fig. 7.155 In this specimen of tetralogy of Fallot with pulmonary
atresia, viewed from behind, the pulmonary arteries are
Left Right
discontinuous. The left pulmonary artery was initially supplied by
an arterial duct, which has become ligametous (star). The right lung
Inf. is supplied through systemic-to-pulmonary collateral arteries
(arrows).
Sup.
Right Left
Inf.
Aorta
Fig. 7.156 The pulmonary arteries in the heart shown in

Figure 7.153, fed by the arterial duct (star), have a normal
segmental distribution.
Intersegmental anastomoses also occur. this is the ultimate determinant of the trunk is best described as a solitary
The object of preoperative evaluation, success of any attempted total correction. structure (see later).
therefore, should be to establish precisely Cases can be found with supply In contrast to tetralogy of Fallot with
how much of each lung is supplied by the exclusively from collateral arteries pulmonary atresia, where initial survival is
intrapericardial pulmonary arteries, as (Figure 7.159). In this setting, the arterial good, and the results of surgery are
Sup.
Aorta
Right Left
Inf.
Collateral arteries
Fig. 7.157 The computed tomographic angiogram from a patient

with tetralogy of Fallot and pulmonary atresia shows the presence
Atretic pulmonary trunk Confluent pulmonary arteries of both systemic-to-pulmonary collateral arteries and pulmonary
arteries.
Sup.
Left
Fig. 7.158 In this heart from a patient with tetralogy of Fallot and
Right
pulmonary atresia, shown in anatomical orientation, the
intrapericardial arteries extending to enter the right lung (arrow)
Inf. are supplied by a large systemic-to-pulmonary collateral
artery (star).
continually improving, attempted surgical The atresia can be due either to an leaflets of the pulmonary valve
correction of patients with pulmonary atresia imperforate pulmonary valvar membrane (Figure 7.161). The cavities of the outlet and
and an intact ventricular septum continues to (Figure 7.160), or to muscular infundibular trabecular component parts of the right
be disappointing. The anatomy of the lesion atresia. In the latter situation, the pulmonary ventricle are more-or-less completely
itself accounts for the dismal outcome79–81. trunk is blind-ending, with no vestiges of obliterated by gross hypertrophy of the
Sup.
Aorta
Right Left
Inf.
Collateral arteries
Fig. 7.159 The computed tomographic angiogram from a patient

with tetralogy of Fallot and pulmonary atresia shows the systemic-
to-pulmonary collateral arteries arising from the descending aorta
that supply the entirety of the pulmonary parenchyma. In this
patient, there was absence of the intrapericardial pulmonary
arteries.
Ant.
Left Right
Fig. 7.160 In this heart from a patient with pulmonary atresia and
an intact ventricular septum, viewed from above, dissection of the
Post. ventriculopulmonary junction shows an imperforate pulmonary
valvar membrane.
ventricular wall. In consequence, the cavity is useful function, particularly when fistulous treatment. When there is a pulmonary valve
effectively represented by only the communications extend to the coronary present, but its leaflets are imperforate, a
hypoplastic inlet portion (Figure 7.162). arteries. The right ventricle probably should spectrum is seen in terms of the size of the
This cavity is unlikely ever to perform a be disregarded when deciding surgical right ventricular cavity82. In some hearts,
Ant.
Left Right
Fig. 7.161 In this heart from a patient with pulmonary atresia
and an intact ventricular septum, there is no evidence of valvar
Post.
tissue in the blind-ending pulmonary root (compare with
Figure 7.160).
Sup.
Right Left
Inf.
Inlet
Fig. 7.162 In this heart from a patient with pulmonary atresia and
an intact ventricular septum, viewed anatomically, the cavity of the
right ventricle is effectively represented by the inlet component
alone, due to gross hypertrophy of the walls of the apical trabecular
and outlet components. Note the fistulous communication
extending through the wall to the anterior interventricular
coronary artery (white arrow).
hypertrophy of the right ventricular rehabilitate the ventricle83. In the most correction, although increasingly they
myocardium obliterates the apical trabecular favourable situation, the cavity is less are treated by the interventional cardiologist,
part of the cavity (Figure 7.163). It is hypoplastic, and has well-developed who will perforate the valvar membrane
questionable if these ventricles will ever grow inlet, apical, and outlet components before dilating it with balloons.
and become useful, although attempts have (Figure 7.164). These cases are those Whatever the intracardiac anatomy, it is
been made to resect apical trabeculations and which are most amenable to total operative rare that one finds the thread-like
Outlet Obliterated apex
Inlet
Sup.
Right Left Fig. 7.163 In this heart, again from a patient with pulmonary
atresia and an intact ventricular septum, mural hypertrophy has
Inf. effectively obliterated the apical trabecular component of the
cavity, while leaving narrowed inlet and outlet components.
Mild mural hypertrophy
Outlet
Inlet
Sup.
Fig. 7.164 This heart is once more from a patient having
Right Left pulmonary atresia with an intact ventricular septum. It shows the
most favourable variant, in which the pulmonary valve is
Inf. Apical trabecular component imperforate (arrow), but the mural hypertrophy has barely
obliterated any of the right ventricular cavity.
pulmonary arteries seen so frequently with construction of a systemic-pulmonary secondary to surgical intervention or
tetralogy of Fallot and pulmonary atresia. shunt. Other options, such as the need for pulmonary hypertension. Congenital
Furthermore, the flow of pulmonary blood pulmonary valvotomy, should be decided pulmonary valvar insufficiency may be
is almost always duct dependent. With after assessment of the precise anatomy of associated with marked deformity of the
prostaglandins now available to improve the individual case. valvar tissue, as in valvar dysplasia, or with
ductal flow, the pulmonary arteries are Pulmonary valvar insufficiency may be purported absence of the valvar tissue
almost always of sufficient size to permit congenital or acquired, the latter usually altogether. In reality, rudimentary leaflets
Sup.
Right Left
Fig. 7.165 In this specimen, seen in anatomical orientation,
from a patient with tetralogy of Fallot, there is rudimentary
Inf. formation of the leaflets of the pulmonary valve (arrow), with gross
dilation of the pulmonary trunk and pulmonary arteries (stars).
Left
Base Apex
Right
Fig. 7.166 This operative view shows the right

ventriculopulmonary arterial junction in a patient with tetralogy of
Fallot. Note the grossly abnormal leaflets of the pulmonary valve
(arrows).
are almost always present in the so-called combination with a ventricular septal While gross pulmonary valvar
absent pulmonary valve syndrome. This defect (Figure 7.165). It is another of the insufficiency may be relatively well
can rarely be seen with an intact ventricular associated lesions found in combination tolerated by the right heart, it can
septum84,85, but more usually in with tetralogy of Fallot. result in marked enlargement of the
Left
Base Apex
Right
Pulmonary trunk
Aorta
Fig. 7.167 In the patient shown in Figure 7.166, there was gross
dilation of the pulmonary trunk and the pulmonary arteries.
Sup.
Left
Right
Inf.
Fig. 7.168 This posterior view of the hilum of the left lung of the
heart from the patient seen in Fig. 7.165 shows the obstruction
produced by the gross dilation of the left pulmonary artery (star) as
it crosses the narrowed bronchus (double-headed arrow).
pulmonary trunk and arteries, and (Figures 7.166–7.169) results in efficacy of replacement of the valve with
usually is associated with an absence most patients presenting with or without arterial plication has not
of the arterial duct85. Compromise of symptoms of respiratory distress. been proved. Perhaps fortunately, so-
the tracheobronchial tree by these Because only a limited number of cases called absence of the pulmonary valvar
grossly enlarged vessels have come to surgical correction, the leaflets is a rare condition.
Left pulmonary artery Pulmonary trunk
Aorta
Left
Base Apex
Fig. 7.169 This operative view of the patient shown in
Right Figures 7.166 and 7.167 shows the grossly enlarged left pulmonary
artery, which is as large as the aorta.
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Lesions in hearts
8
with abnormal
segmental
connections
Abnormal segmental connections 245
In the previous chapter, we paid attention the lesions in terms of single ventricles, or between dominant and incomplete
to those hearts in which the associated univentricular hearts, despite the fact that ventricles, which are no more and no less
cardiac malformation existed in the setting almost all patients with a double-inlet than ventricular septal defects.
of normal connections between the cardiac atrioventricular connection have two In basic terms, hearts exhibit a double-
segments. All those associated lesions, of chambers within their ventricular mass, inlet atrioventricular connection whenever
course, can also be found in hearts with one being large and the other small1–3. The the greater parts of both atrioventricular
abnormal segmental connections. It is these semantic problems with description can junctions, which belong to the right-sided
abnormal connections that will be our focus now be resolved by the simple expedient of and left-sided atrial chambers, are
in this chapter, emphasising the associated describing functionally univentricular connected to the same ventricle. This
anomalies that are particularly frequent hearts4, this approach also accounting for definition holds good irrespective of
with a given abnormal segmental the other lesions dominated by ventricular whether the connections are guarded by
arrangement. We conclude the chapter imbalance. At this point, we describe the two separate atrioventricular valves, as is
with a brief discussion of those hearts in relevant anatomical characteristics of all usually the case (Figure 8.1), or by a
which the relationships of the arterial those hearts unified by the presence of the common valve (Figure 8.2). The double-
trunks are abnormal in the setting of double-inlet atrioventricular connection, inlet connection can also be found in the
concordant ventriculoarterial connections, irrespective of whether they contain one or presence of overriding and straddling
as these combinations still produce two ventricles, although almost all do have atrioventricular valves, but only when the
problems in understanding and one big and one small ventricle. The degree of overriding is such as to leave the
description. previous problems with description greater part of both junctions connected to
centred on whether the small chamber in the same ventricle, again either through
hearts with ventricular imbalance deserved two valves (Figure 8.3) or a common valve.
DOUBLE-INLET VENTRICLE ventricular status. We now describe all When there is a straddling common valve,
Over the years, hearts with a double-inlet chambers possessing characteristic apical it becomes moot as to whether the hearts
ventricle have represented one of the components as ventricles, recognising should also be considered as possessing
greater challenges to surgical correction. them as being complete or incomplete atrioventricular septal defects with gross
They have also posed significant problems depending on their component make-up. ventricular imbalance. In all these
in adequate description and categorisation. This form of analysis greatly simplifies the instances, it is the precise atrioventricular
Even these days, many continue to describe description of the communications connections that determine the surgical
Left
atrium
Right
atrium
Dominant
left ventricle
Fig. 8.1 This specimen, prepared by slicing the heart in four-chamber

projection, shows the essence of the double-inlet atrioventricular connection.
Base Note that the cavity of only one ventricle is visible. It possesses fine criss-
crossing apical trabeculations, identifying it as a dominant left ventricle.
Right Left There is a second ventricle, albeit incomplete because it lacks its inlet
component, positioned anterosuperiorly, and thus is not sectioned in this cut.
Apex In this heart, both atrioventricular junctions (black braces) are connected to
the dominant ventricle through separate atrioventricular valves.
Left
atrium
Right
atrium
Dominant
right ventricle
Base
Fig. 8.2 This specimen is also prepared by making a section in the four-
chamber plane. It shows a double inlet to a dominant right ventricle, as both
Right Left
Incomplete atrioventricular junctions (white braces) drain to the dominant ventricular
left ventricle chamber (compare with Figure 8.1). Note, however, that there is a common
Apex atrioventricular valve (white double-headed arrow). The section also
demonstrates the incomplete left ventricle, which lacks its inlet component.
Base
Left Right Left

atrium
Right Apex
atrium
Incomplete left ventricle Dominant

right ventricle Fig. 8.3 This specimen, windowed and viewed in anatomical
orientation, has a double inlet (white braces) to a dominant right
ventricle, but through two separate atrioventricular valves. The
inset, photographed from behind, shows that one papillary muscle
of the left atrioventricular valve (arrowed) retains its position
within the incomplete left ventricle.
approach. Whenever patients are found surgical therapeutic option will, almost It will be exceedingly rare for patients to be
with a double-inlet connection as defined certainly, be to create the Fontan encountered with a truly solitary ventricle.
here, their hearts will perforce be circulation although, rarely, it can be Most usually, there will be two ventricles in
functionally univentricular. The chosen possible to septate the dominant ventricle5. the setting of a double-inlet, one being
dominant and the other incomplete, the septate the dominant or solitary ventricle5. ventriculoarterial connections being
smaller ventricle lacking at least the greater The overall morphological arrangement, concordant, compared to the more usual
part of its inlet component4. however, usually conspires to defeat this arrangement with discordant
Although the surgical options are the option. The most frequent surgical tactic, ventriculoarterial connections when the
same for all patients with the double-inlet therefore, is to use the Fontan procedure or aorta arises from the incomplete ventricle
atrioventricular connection5, the hearts one of its modifications8–10. We will (Figures 8.4, 8.7, 8.8). In all instances,
themselves can show marked anatomical concentrate, therefore, on those anatomical nonetheless, the incomplete chambers
variation. They can exist with any features influencing the Fontan procedure, possess an apical component of
arrangement of the atrial chambers, with although we will discuss the morphologies morphologically right ventricular pattern.
one of three ventricular morphologies, with that lend themselves to, or compromise, They are separated from the dominant
the variations in valvar morphology septation. ventricle by the hypoplastic muscular
discussed above, with any ventriculoarterial In most instances, both atrioventricular ventricular septum (Figure 8.4), which
connection, and with varied associated junctions are connected to a dominant left carries the atrioventricular conduction axis,
malformations6. From the anatomical ventricle, in the presence of an incomplete and which is nourished by septal
stance, and probably from the stance of morphologically right ventricle perforating arteries11.
long-term follow-up, the most important (Figure 8.4). The chamber lacks its inlet The crucial feature in terms of surgical
differentiating feature is the morphology of component, either completely when the correction is the dimensions of the
the dominant ventricle. There are three atrial chambers are exclusively connected ventricular septal defect, as this must be of
possibilities7. The first, and most common, to the dominant left ventricle (Figures 8.1, adequate size to support the systemic
is the arrangement in which the atrial 8.4, 8.5), or for the greater part when there circulation irrespective of the precise
chambers are connected to a dominant left is overriding of one (Figure 8.6), or rarely surgical tactics to be adopted. If it is
ventricle in the presence of an incomplete both, atrioventricular junctions. The restrictive (Figure 8.4), it may need to be
right ventricle. The second group comprises incomplete ventricle can be positioned to surgically enlarged, although more
those with a dominant right and an the right (Figure 8.7) or the left frequently the option is to by-pass the
incomplete left ventricle. The final small set (Figure 8.8) of the dominant ventricle, but restrictive area by opting for the Norwood
is made up of those patients having a solitary it is always located anterosuperiorly, with approach, or performing a Damus–Kaye–
ventricle of indeterminate morphology. the atrioventricular connections opening Stansel procedure. Should the surgeon opt
These three variants must be distinguished posteriorly to the hypoplastic ventricular to enlarge the ventricular septal defect, the
from other hearts that, in essence, represent septum (Figures 8.4, 8.9). The morphology major anatomical feature of concern is the
huge ventricular septal defects. of the incomplete ventricle is markedly course of the axis of atrioventricular
A potential means of correcting these different when it supports the pulmonary conduction tissue12. Although always
hearts with a double inlet surgically is to trunk (Figures 8.6, 8.10), the originating from an anomalously located
Aorta
Incomplete
Pulm. right ventricle
trunk
Fig. 8.4 The reconstructed computed tomographic angiogram

shows, in exemplary fashion, the essential anatomy of a double-
Dominant inlet left ventricle. Both atrioventricular junctions (white arrows)
Sup. open into the dominant left ventricle, which gives rise to the
left ventricle
pulmonary (pulm.) trunk. The aorta arises from the incomplete
Right Left right ventricle, which is separated from the dominant ventricle by
the apical ventricular septum (star). The hole between the
ventricles (double-headed black arrow) is a ventricular septal
Inf.
defect, which is restrictive in this example.
Pulmonary trunk
Base Sup.
Right Left Right Left
VSD
Apex
Inf.
Pulmonary
valve
Infundibulum
Right LAVV
atrium
RAVV
Dominant
left ventricle
Straddling tension apparatus

Fig. 8.5 This heart is an example of the most common form of
double-inlet left ventricle. The dominant left ventricle has been
opened in clam-shell-like fashion, and the specimen is shown in Fig. 8.6 In this specimen with a double-inlet left ventricle, the
anatomical orientation. The abnormal course of the conduction incomplete right ventricle is photographed from the front with the
axis has been superimposed on the picture as the red cross-hatched heart positioned anatomically. The ventriculoarterial connections
area. LAVV and RAVV, left and right atrioventricular valves; VSD, are concordant, with the pulmonary trunk seen supported by an
ventricular septal defect. infundibulum and arising from the incomplete right ventricle. The
right atrioventricular valve straddles and overrides, but its greater
part remains connected within the dominant left ventricle, so the
connection remains one of a double inlet.
atrioventricular node (see later), In a small proportion of patients rules pertain should it require surgical
irrespective of its relationship relative to with a double-inlet left ventricle, the enlargement. This is unlikely to be
the dominant left ventricle, the axis always ventriculoarterial connections may be performed, however, unless a septation is
extends posteroinferior to the defect when concordant7, or there can be a double outlet also attempted. The more likely approach
viewed from the incomplete right ventricle, from the dominant left or incomplete right will be to perform a Fontan procedure. The
being carried on the left ventricular aspect ventricle. The variant with concordant incomplete right ventricle will then be
of the septal crest (Figure 8.5). When the ventriculoarterial connections is of excluded from the circulation.
ventriculoarterial connections are significant anatomical interest. The lesion Taken overall, the Fontan procedure, or
discordant, the incomplete right ventricle is described eponymously as the Holmes one of its modifications, will be the likely
usually has a very short outlet portion heart13. In this setting, the incomplete right operation of choice for most patients with a
(Figures 8.7, 8.8), making it difficult to ventricle, when seen in isolation, can be double-inlet left ventricle. It can be used
remove any tissue cephalad to the defect. virtually indistinguishable from the successfully for all the various anatomical
The safest way to enlarge the defect incomplete right ventricle seen in hearts variants, providing that the haemodynamic
surgically, therefore, is to remove a wedge with tricuspid atresia (compare Figures 8.9 criterions are satisfactory14. Almost always,
of the muscular ventricular septum closest and 8.12). The ventricular septal defect is the procedure includes connecting the
to the obtuse margin of the heart often restrictive in this form of double-inlet systemic venous returns, in one way or
(Figure 8.11)12. left ventricle (Figure 8.9), and the same another, to the pulmonary arteries. This is
VSD
Aorta
Aorta
VSD
Base
Base
Right Left
Right Left
Incomplete right ventricle Apex Incomplete right ventricle

Apex
Fig. 8.7 In this specimen with a double-inlet left ventricle and Fig. 8.8 In this specimen with a double-inlet left ventricle, again
discordant ventriculoarterial connections, viewed anatomically, with discordant ventriculoarterial connections, and viewed
the incomplete right ventricle, supporting the aorta, is right-sided. anatomically, the incomplete rudimentary right ventricle,
VSD, ventricular septal defect. supporting the aorta, is left-sided. VSD, ventricular septal defect.
Incomplete right ventricle Outlet septum
Apical septum
Aorta
RAVV
Dominant LV LAVV
Fig. 8.9 This specimen with a double-inlet left ventricle and
discordant ventriculoarterial connections has been sectioned to
Sup. replicate the parasternal long axis view, and is photographed in
anatomical orientation. Note that the apical ventricular septum is
positioned between the apical components of the dominant left
Apex Base
and incomplete right ventricles. This septum is the hypoplastic
muscular ventricular septum, as it carries the conduction axis, and is
To pulmonary trunk Inf. nourished by the septal perforating arteries. LV, left ventricle;
RAVV and LAVV, right and left atrioventricular valves.
Pulm. trunk
Long
Aorta infundibulum
VSD
Fig. 8.10 In this specimen, again with a double-inlet left ventricle,

but with concordant ventriculoarterial connections, as with the
Sup.
heart shown in Figure 8.6, the right-sided incomplete right
ventricle (RV) supports the pulmonary (pulm.) trunk, with the aorta
Right Left arising from the dominant left ventricle. This is the so-called
‘Holmes Heart’. Note the length of the infundibulum of the
incomplete ventricle when it supports the pulmonary trunk,
Apical RV trabeculations Inf. compared to those supporting the aorta (see Figures 8.7 and 8.8).
VSD, ventricular septal defect.
Restrictive VSD
Aorta
Fig. 8.11 This specimen, photographed in surgical orientation,

shows the relationship of the axis of the atrioventricular
conduction tissue to the ventricular septal defect in a double-inlet
left ventricle with discordant ventriculoarterial connections when
Left viewed from the incomplete right ventricle. The ventricular septal
defect (VSD) is restrictive. The course of the conduction axis is
Sup. Inf. superimposed in red, with the site of the anomalous
atrioventricular node shown by the star, while the cross-hatched
Incomplete right ventricle oval shows the area that can be removed without inflicting trauma
Right
to the conduction tissues.
usually achieved now by either creating a vein to the pulmonary arteries, combining atriopulmonary connection still remains a
conduit within the right atrium to produce this with a Glenn procedure16. In the past, surgical option. When there is
a so-called total cavopulmonary the roof of the right atrium at its junction juxtaposition of the atrial appendages, a not
connection15, or inserting an extracardiac with the appendage was often connected infrequent associated malformation with a
conduit that connects the inferior caval directly to the pulmonary trunk. Such an double-inlet left ventricle, this anastomosis
Pulm. trunk Long

infundibulum
Aorta
Right atrium
Fig. 8.12 This specimen has classical tricuspid atresia with

Sup.
Sup.
concordant ventriculoarterial connections, and is photographed in
VSD anatomical orientation. Note the similarity in the morphology of
Right Left
the incomplete right ventricle (RV) in this setting with its long
infundibulum, with that seen in a double-inlet left ventricle when
Apical RV trabeculations Inf. the ventriculoarterial connections are concordant (see Figure 8.9).
Pulm., pulmonary; VSD, ventricular septal defect.
Aorta
Superior caval vein
Left
Fig. 8.13 In this heart, photographed in the operating room, the
Sup. Inf. artery to the sinus node, arising from the right coronary artery, is
seen coursing through the interatrial groove to cross the cavoatrial
Right Sinus node junction at the crest of the right atrial appendage. The sinus node is
visible as a pale area within the terminal groove.
becomes even simpler. If the atrium is to be the artery to the sinus node irrespective of atriopulmonary connection. If this has to be
connected to the pulmonary arteries, either its arterial origin (Figure 8.13). Use of an done, there are at least two options open to
directly or by construction of an internal internal or external conduit obviously the surgeon. It can be done by securing a
conduit, it is crucial for the surgeon to obviates the need to isolate the systemic patch across the vestibule of the valve. It
avoid the sinus node and its arterial supply. venous return from the right can also be achieved by deviating the atrial
In this respect, the key area is the superior atrioventricular valve surgically, an added septum to the parietal border of the right
interatrial groove, through which courses manoeuvre that is essential if attempting an atrium, thus leaving both valves draining to
the dominant ventricle. With both of these sinus node and its arterial supply. Such dictate the insertion of a conduit from the
options, it is important to avoid the problems are avoided when inserting an dominant ventricle to the pulmonary
atrioventricular node, which in a double- extracardiac conduit. arteries. Remarkably few patients will
inlet left ventricle is no longer located at the Very few surgeons now choose septation match all these criterions, and become
apex of the triangle of Koch17. This is for patients with a double-inlet left suitable candidates for septation. In those
because the muscular ventricular septum, ventricle, even though it is perhaps the that are, the major consideration during
carrying the ventricular conduction tissues, most logical surgical option5. Relatively few surgery will be to avoid the axis of
does not reach the crux of the heart, rising patients, however, have anatomy suitable atrioventricular conduction tissue, thus
instead to the acute margin of the for successful septation. Both avoiding iatrogenic heart block. It is also
ventricular mass (Figure 8.5). The atrioventricular valves must be competent necessary, of course, to preserve the sinus
atrioventricular node, therefore, is located structures, and the relationships of node and its arterial supply (Figure 8.13).
within the quadrant of the junction related dominant and incomplete ventricles must The atrioventricular conduction axis in
to the mouth of the right atrial appendage permit the construction of a patch to hearts with a double-inlet left ventricle
(Figure 8.14). This area is the major site of channel flow from the left atrioventricular takes its origin from an anomalous node
danger. Irrespective of its precise location, valve to the ventricular septal defect. This located anterocephalad within the right
nonetheless, the node can be avoided by means that the incomplete right ventricle atrioventricular junction, beneath the
securing the patch at least one centimetre should be left-sided (Figures 8.4, 8.8) or, at mouth of the atrial appendage
above the attachments of the leaflets of the worst, directly anterior. The dominant (Figure 8.14). From this anterior node, the
right atrioventricular valve (Figure 8.14). ventricle also needs to be of good size, atrioventricular bundle penetrates the
The features discussed above, however, which means that septation is unlikely to be lateral end of the area of fibrous continuity
are less pertinent to the modern-day attempted in infancy. If the pulmonary between the leaflets of the pulmonary and
surgeon, as it is now the rule to insert an circulation is not protected by naturally the right atrioventricular valves. When the
extracardiac conduit so as to direct the occurring stenosis, this must be achieved incomplete right ventricle is left-sided, as it
inferior caval venous blood to the lungs or immediately after birth by banding the will be in most cases suitable for septation,
to the superior caval vein. Like an internal pulmonary trunk. Banding itself tends to the non-branching atrioventricular bundle
baffle, this procedure produces the total promote hypertrophy of the ventricular encircles the anterior quadrants of the
cavopulmonary connection16 with the myocardium, and narrowing of the pulmonary orifice to reach the rightwards
extracardiac conduit also having ventricular septal defect. It may also be margin of the muscular ventricular septum
advantages in terms of the pattern of flow necessary, therefore, to combine (Figure 8.5). The left bundle branch then
into the pulmonary arteries. Should it be enlargement of the defect (Figure 8.11) cascades down the left ventricular aspect of
decided to construct an internal baffle, the with septation. The presence of naturally the septum, the axis of conduction tissue
major areas to be avoided are again the occurring subpulmonary stenosis may itself being located anterocephalad to the
Morphologically left ventricle

Apex
Site of suture line

Sup. Inf.
within right atrium
Base
Fig. 8.14 This cartoon, drawn in surgical orientation, shows the

danger area of the right atrioventricular orifice (red double-headed
arrow) in hearts with a double-inlet left ventricle, and the way the
Connecting node not
conduction tissue can be avoided by suturing above the level of the
in triangle of Koch
atrioventricular junction (blue dashed line).
ventricular septal defect when viewed relatively narrow as it emerges from the conduit, or involuntary induction of
either through a fish-mouth incision in the area of fibrous continuity between the atrioventricular dissociation. Septation can
dominant left ventricle (Figure 8.15), or pulmonary and right atrioventricular be a potentially feasible option for patients
through the right atrioventricular valve valves. If this site is known with precision, with a double-inlet left ventricle and
(Figure 8.16). Unless a conduit is also the sutures can be placed so as to avoid the concordant ventriculoarterial connections.
placed from the dominant left ventricle to axis. All of these considerations, taken If attempted, the same rules hold good for
the pulmonary arteries, the line of sutures together, indicate that septation is a the disposition of the axis of conduction
used to septate the dominant ventricle must formidable surgical procedure if the tissue, but a potential caveat is that the
cross this course of the axis of conduction pathways through the dominant ventricle ventricular septal defect is most frequently
tissues (Figure 8.15). The axis itself is are to be separated without insertion of a restrictive or obstructed (Figures 8.4, 8.9).
LAVV Fig. 8.15 The cartoon shows the dominant left ventricle with a
double inlet as seen through a fish-mouth incision in the apex of
the ventricle. The conduction axis is marked in green (see also
RAVV Figure 8.5). The potential line for complete septation, placing the
pulmonary trunk in communication with the systemic venous
return (blue dashed line), crosses the conduction axis. To avoid the
conduction axis (green dashed line), it is necessary to deviate the
Conduction tissue site of septation so that both outflow tracts remain in
Line of suture for complete septation communication with the pulmonary venous return through the left
Line of suture for double-outlet left atrioventricular valve. The star shows the location of the ventricular
ventricle septal defect. LAVV and RAVV, left and right atrioventricular
valves.
Branching bundle
Penetrating bundle
Atrioventricular ring tissue
Transitional cell zone
Fig. 8.16 This cartoon, again drawn in surgical orientation, shows

the view of the dominant left ventricle in the setting of a double-
inlet left ventricle with discordant ventriculoarterial connections,
Pulmonary valve Ventricular septal defect and the course of the conduction axis, in green, as it would be seen
by the surgeon working through the right atrioventricular valve.
Subaortic outlet Subpulmonary outlet
LAVV
RAVV
Sup.
Fig. 8.17 This specimen with a double-inlet and double-outlet
Right Left right ventricle is photographed in anatomical orientation. Both
atrioventricular valves connect exclusively to the dominant right
ventricle, while the extensive outlet septum (white double-headed
Inf. arrow) separates the origins of the two arterial trunks. RAVV and
LAVV, right and left atrioventricular valves.
The other variants of the double-inlet usual position in the triangle of Koch. The remains a formidable undertaking. In these
ventricle are much more rare, and hardly exception is when the incomplete left rare patients, it is the persisting rim of
ever are suitable for septation. The double- ventricle is right-sided, the conduction axis muscular septum separating the apical
inlet right ventricle (Figure 8.17) is seen arising from an anomalous node19, as in trabecular components, together with the
most frequently with a double outlet from congenitally corrected transposition (see inlet septum rising to the crux, that provide
the dominant right ventricle, often with later). the anatomical landmarks. The axis of
straddling and overriding of the left Those rare hearts with a double inlet to a conduction tissue descends from the
atrioventricular valve (Figure 8.3)18. The solitary and indeterminate ventricle usually regular atrioventricular node when the
incomplete left ventricle, usually found in coexist with isomerism of the atrial atrioventricular connections are
the left-sided position, although sometimes appendages, when the multiple associated concordant in this setting (Figure 8.22).
found to the right, is nothing more than a malformations, particularly the anomalous
pouch with left ventricular apical venoatrial connections, dominate the
trabeculations (Figure 8.18). It is always picture. Rare examples can be found with
TRICUSPID AND MITRAL ATRESIA
found posteroinferiorly, and is dorsal to the the usual arrangement of the atrial In most instances, tricuspid atresia is
atrioventricular valves, in the appendages. In this setting, the crossing of produced by complete absence of the right
diaphragmatic surface of the ventricular the tension apparatus from the atrioventricular connection (Figure 8.23).
mass. The Fontan procedure is the most atrioventricular valves, along with the Many hearts with mitral atresia exist
likely therapeutic surgical option, in which particularly coarse indeterminate apical because of the absence of the left
case the landmarks within the atrial trabeculations, tend to conspire against atrioventricular connection (Figure 8.24).
chambers, together with the surgical septation (Figure 8.21). Patients with In these hearts, because of the absence of
caveats, are as discussed for the double- solitary ventricles, therefore, are also most one of the atrioventricular connections, and
inlet left ventricle. Sometimes, a double- likely to undergo repair by means of the hence with only one of the atrioventricular
inlet right ventricle can be found with Fontan option, and the same rules apply as junctions making contact with the
concordant ventriculoarterial connections discussed earlier. ventricular mass, the ventricular
(Figure 8.19), usually with a common valve Hearts with huge ventricular septal morphology is comparable to that seen in
guarding the atrioventricular junctions defects (Figure 8.22) can sometimes be those with a double inlet (see Figure 8.2).
(Figures 8.3, 8.20). The axis of confused with a double inlet to a solitary All three atrioventricular connections are
atrioventricular conduction in this setting and indeterminate ventricle. Even in those univentricular. The solitary
descends from a node located within its hearts with huge septal defects, septation atrioventricular valve, nonetheless, can
Sup. Left atrioventricular valve
Left atrium
Ant. Post.
Inf.
Fig. 8.18 This picture, again viewed in anatomical orientation,

shows the location of the left-sided incomplete left ventricle in the
heart shown in Figure 8.17 with a double-inlet right ventricle. The
incomplete ventricle is no more than an outpouching from the
Incomplete left ventricle dominant ventricle. The black double-headed arrow shows the
hypoplastic apical ventricular septum.
Common AVV to RV
Aorta
Incomplete
left ventricle Sup.
Ant. Post. Fig. 8.19 In this picture, again in anatomical orientation, an

example is seen of a double-inlet right ventricle in which the
incomplete left ventricle gives rise to the aorta. There is a common
Inf. atrioventricular valve (AVV) joining both atriums to the dominant
right ventricle (RV), shown in cross-section in Figure 8.2.
Sup.
Pulm. trunk
Right Left
Inf.
Dominant
right ventricle
Fig. 8.20 This is another picture of the dominant right ventricle

from the heart shown in Figure 8.19. Both atriums drain to the
dominant ventricle through the common atrioventricular (AV)
Common AV valve
valve. The ventriculoarterial connections are concordant, with the
pulmonary (pulm.) trunk arising from the dominant ventricle.
Subpulmonary outlet Coarse apical trabeculations
Left
AV valve
Fig. 8.21 This specimen, seen in anatomical

Right orientation, with the ventricle opened in
AV valve clam-shell-like fashion, has a double inlet to
Sup.
and double outlet from a solitary ventricle of
indeterminate morphology, which has very
Right Left coarse apical trabeculations. Note that the
tension apparatus of both atrioventricular (AV)
Subaortic outlet
Inf. valves has a common origin, making septation
exceedingly difficult, if not impossible.
straddle and override (Figure 8.25). This the same as for the double-inlet while drawing attention to the less
produces a uniatrial, but biventricular, ventricle, most examples fall into what familiar formats.
atrioventricular connection20. can be considered to represent classical In tricuspid atresia, it is the
Although the variations in terms of types of atrioventricular valvar atresia. morphologically right atrium that is blind-
ventricular morphology when one We will concentrate our attention, ending, usually with no vestige of the
atrioventricular connection is absent are therefore, on these anticipated variants, tricuspid valve in its floor (Figure 8.26).
Sup. Aorta Left AV valve
Ant. Post.
Inf.
RAVV
Fig. 8.22 This view, in anatomical orientation, shows the left

ventricle in a heart with concordant atrioventricular connections
and a huge ventricular septal defect. The site of the axis of
atrioventricular conduction tissue has been marked with a red
dotted line on the left ventricular aspect of the hypoplastic
ventricular septum, which runs to the apex (star), where it separates
Left ventricular the apical ventricular components. The right atrioventricular valve
apical trabeculations (RAVV) is connected within the right ventricle, hence the
concordant nature of the atrioventricular connections.
Left-sided inlet
Left atrium
Right atrium
Dominant LV
Fig. 8.23 The heart has been sectioned to replicate the four-
chamber echocardiographic view. It shows complete absence of the
right atrioventricular (AV) connection, the atrioventricular groove
being filled with adipose tissue, and containing the right coronary
artery. This is the essence of the usual variant of tricuspid atresia.
The section passes mostly through the dominant left ventricle (LV)
Absent AV connection (compare with Figure 8.1), but the posterior part of the incomplete
right ventricle is just visible (star).
Because of the absent connection, the right inlet left ventricle (Figures 8.4, 8.10), is ventricle, the apical ventricular septum does
ventricle is incomplete, lacking its inlet separated from the dominant ventricle by the not extend to the crux, but rather extends to
component21. The apical trabecular part of apical muscular ventricular septum the acute margin of the ventricular mass
the incomplete ventricle, as with the double- (Figure 8.27). As in the double-inlet left (Figure 8.28). Surgical palliation involves the
Absent left
AV connection
Left atrium
Slit-like
left ventricle
Right atrium
Fig. 8.24 This heart with classical mitral

Dominant right ventricle atresia, from a patient with hypoplasia of the
left heart, has been sectioned to replicate the
Sup.
four-chamber echocardiographic view
(compare with Figure 8.23). There is complete
Right Left absence of the left atrioventricular (AV)
connection. The section has also transected the
ventricular septum, showing the site of the
Inf.
posteroinferior and slit-like incomplete left
ventricle (compare with Figure 8.2).
Sup.
Right Left
Inf.
Left atrium
Right atrium
Dominant LV
Fig. 8.25 This heart, with the absence of the right atrioventricular
connection (white dotted lines), is sectioned to simulate the
echocardiographic four-chamber view. The solitary atrioventricular
Incomplete
junction (white double-headed arrow) is shared between the
right ventricle
dominant left (LV) and the incomplete right ventricles because of
straddling of the solitary atrioventricular valve (arrow head). This
arrangement produces a uniatrial but biventricular connection.
Superior caval vein Dimple Sup.
Post. Ant.
Inf.
Fig. 8.26 This view of the opened right atrium from a specimen
with tricuspid atresia, seen in anatomical orientation, shows the
muscular floor due to complete absence of the right
atrioventricular connection. The site of the atrioventricular node
Oval fossa Pectinate muscles has been superimposed in green, and is closely related to the
Coronary sinus
‘dimple’.
Sup.
Right Left
Left atrium Inf.
Right atrium
Fig. 8.27 This specimen with classical tricuspid atresia due to

Dominant left ventricle absence of the right atrioventricular connection (white dashed
lines) and concordant ventriculoarterial connections, has been
sectioned in a parasternal long axis fashion, and is photographed
positioned on its apex. The hypoplastic ventricular septum
separates the apical parts of the dominant left and incomplete
Incomplete right ventricle right ventricles (compare with Figure 8.10). The communication
between the ventricles is a ventricular septal defect (star).
Fontan operation, or one of its modifications. incomplete right ventricle into the be constructed within the right atrium, the
In hearts with concordant ventriculoarterial pulmonary circulation22. Presently, most architecture of its muscular walls, and the
connections, attempts were often made surgeons have shifted to constructing total arterial supply, should be disturbed as little
during the early period of evolution of the cavopulmonary connections, now mostly as possible. Preservation of the sinus node
Fontan procedure to incorporate the using external conduits. If the conduit is to and its blood supply is particularly
Right coronary artery Ant. intervent. artery
Aorta
VSD
Fig. 8.28 This dissection of a specimen with tricuspid atresia with

Sup. concordant ventriculoarterial connections, viewed in anatomical
orientation, shows that the right delimiting coronary artery
Right Left descends at the acute margin of the ventricular mass from the right
Acute marginal artery coronary artery, rather than the crux. The other delimiting artery is
the anterior interventricular (ant. intervent.) artery. Note the
Incomplete right ventricle Inf. anterosuperior location of the incomplete right ventricle. VSD,
important, as atrial arrhythmia is a defect provides the interventricular the aortic pathways rather than the
recognised life-threatening postoperative communication (Figure 8.29). ventricular morphology, with the key
complication, particularly in the long term. In very rare cases, tricuspid atresia may features being the relations of the arterial
The rules for avoidance of these structures be produced because an imperforate valvar duct (Figures 8.33, 8.34), and the size of
are as described earlier for the double-inlet membrane is interposed between the right the aortic root (Figures 8.35–8.38). In some
ventricle (Figure 8.9). Whenever a well- atrium and ventricle. This can be seen as patients having atresia of the left
formed Eustachian valve is encountered, it part of pulmonary atresia with an intact atrioventricular valve, however, the aortic
should be preserved. Such a valve can be ventricular septum (Figure 8.30). An outflow tract is patent. Mitral atresia may
incorporated in an internal baffle. Creation of imperforate valve can also be found when not be the best term for description of all
such an internal baffle requires the surgeon there are concordant atrioventricular these patients. This is because, in many,
to make a direct and wide connection connections and a ventricular septal defect the morphology suggests that, had the left
between the right atrium and the pulmonary (Figure 8.31), albeit very rarely, or as part atrioventricular connection been formed, it
arteries. In such circumstances, the artery to of Ebstein’s malformation (Figure 8.32)23. would have been guarded by a
the sinus node can be at risk as it traverses We are unaware of any patient in whom the morphologically tricuspid valve. The right
the interatrial groove. This potential junction blocked by the imperforate atrium in this setting is connected to a
complication is avoided when inserting an membrane has been of sufficient size to morphologically left ventricle, with the
extracardiac conduit. permit resection of the valve, and incomplete right ventricle being anterior
When the Fontan procedure is performed replacement with a valvar prosthesis. and left-sided, usually with discordant
in hearts with discordant ventriculoarterial Operative treatment of mitral atresia is ventriculoarterial connections
connections, it may be necessary to resect the complicated because of its usual association (Figure 8.39). In this setting, there is no
margins of the ventricular septal defect with aortic atresia. This, of course, is one of direct route for the pulmonary venous
should it be restrictive. This must be done in the typical combinations producing return to reach the dominant left ventricle.
a way that avoids the atrioventricular hypoplasia of the left heart24. Direct The blood must cross an atrial septal defect
conduction axis on the left ventricular aspect surgical correction involves reconstruction and traverse the right-sided
of the septum12. When seen from the right of the arterial pathways as an initial atrioventricular valve. Initial survival in
ventricle, this runs posteroinferior to the procedure, employing the Norwood these cases, therefore, depends on the state
septal defect as in the double-inlet left protocol25 or one of its modifications. This of the atrial septum. Should the septum be
ventricle (Figure 8.29). The rare exception is is followed by a subsequent modified restrictive, it will need to be enlarged.
when the typical muscular ventricular septal Fontan procedure. The options in these Some sort of modified Fontan procedure
defect is atretic, and an apical trabecular procedures are dictated by the anatomy of will then be the final option, usually a
Sup.
Apical muscular defect
Regular muscular defect
Right Left
Inf.
Right
atrium
Imperforate
Right atrium tricuspid valve
Apex Hypoplastic right ventricular inlet
Fig. 8.30 This specimen is sectioned across the right atrioventricular

Sup. Inf.
junction. There is pulmonary atresia with an intact ventricular septum, with
mural hypertrophy obliterating the apical (star) and outlet ventricular
Base components. In addition, the tricuspid valve is imperforate. The
atrioventricular connections are concordant.
Fig. 8.29 The cartoon, drawn in surgical orientation, shows

the route of the axis of the atrioventricular conduction tissue,
illustrated in green, relative to the usual ventricular septal
defect in tricuspid atresia, and to a defect opening between the
apical trabecular component of the incomplete right ventricle
and the dominant ventricle. In the latter situation, the axis is
superior relative to the defect.
cavopulmonary connection. As with discordant ventriculoarterial connections. or with isomerism and biventricular but
tricuspid atresia, left-sided atresia can be Most clinicians refer to the combination mixed atrioventricular connections. In all
produced by an imperforate simply as transposition. This usage, these circumstances, it is the arrangements
atrioventricular valve rather than being due although widespread, is less than precise. at the atrioventricular junctions that
to the absence of the left atrioventricular In the past, disagreements were frequent as become the dominant features. There is,
connection. The imperforate valve can be to whether the term transposition should therefore, a need for a term that describes
found with biventricular (Figure 8.40) or be used only to describe hearts with only the combinations of concordant
double-inlet (Figure 8.41) atrioventricular discordant ventriculoarterial atrioventricular and discordant
connections. Irrespective of the specific connections26, or for description of any ventriculoarterial connections
morphology, the only surgical option will heart with an anterior aorta27. More (Figure 8.42). Previously, our preferred
be to create a functionally univentricular significantly, when transposition is used to term had been complete transposition. We
connection, the Fontan circulation being describe discordant ventriculoarterial now recognise that this term itself is less
constructed following one of the options connections, the term is not restricted to than perfect. Complete, as an adjective, was
discussed earlier. the combination with concordant first used to indicate that both arterial
atrioventricular connections. trunks were transposed across the
Transposition, if defined simply on the ventricular septum, thus distinguishing the
basis of discordant ventriculoarterial arrangement from the presumed partial
TRANSPOSITION arrangement, in which only the aorta was
connections, can also coexist with
A particularly important lesion is discordant atrioventricular connections, transposed across the septum. The latter
represented by the combination of with a double-inlet ventricle, with an anomaly is universally described now as a
concordant atrioventricular with absence of one atrioventricular connection, double-outlet right ventricle, although as
Sup.
Left Right
Inf.
Fig. 8.31 The right atrium in this specimen is viewed from above
and behind, in anatomical orientation, to show the atrial aspect of
an imperforate tricuspid valve. In this heart, the atrioventricular
Imperforate tricuspid valve connections were concordant (compare with Figure 8.26), and
Eustachian valve
there was a ventricular septal defect.
Imperforate Ebstein’s malformation
Right
atrium
Sup.
Left Right
Fig. 8.32 In this heart with an imperforate tricuspid valve and
Atrialised right
concordant atrioventricular connections, the tricuspid valve itself is
ventricular inlet
Inf. deformed by Ebstein’s malformation. The dotted green line shows
the location of the right atrioventricular junction.
we will discuss, problems also remain in transposition28. We suggest, therefore, that transposition. If discordant ventriculoarterial
defining this entity. This does not alter the the combination of discordant connections are found in other settings, we
fact that, in the strictest sense, all examples ventriculoarterial with concordant describe them as congenitally corrected
of discordant ventriculoarterial atrioventricular connections be considered transposition, or tricuspid atresia with
connections represent complete the default option for use of the term transposition, and so on.
Transverse aortic arch Left subclavian artery
Ascending aorta
Pulmonary
trunk
Sup.
Fig. 8.33 This specimen, viewed from the left side in anatomical
Ant. Post. orientation, shows the typical arrangement of the arterial trunks in
hypoplastic left heart syndrome due to combined aortic and mitral
Inf. Arterial duct atresia, but with the subclavian artery arising distally to the arterial
duct. Note the grossly hypoplastic transverse aortic arch.
Transverse aortic arch
Pulmonary
trunk Arterial duct
Sup. Fig. 8.34 This computed tomographic angiogram, viewed from

the left side in anatomical orientation (compare with Figure 8.33),
Left pulmonary artery
Ant. Post. shows the typical arrangement of the arterial trunks in hypoplastic
left heart syndrome, but with the subclavian artery arising in its
Inf. normal position proximal to the arterial duct. Note the major flow
pathway through the arterial duct to the descending aorta.
It is less than appropriate, however, to atrioventricular and discordant the majority of patients with the
use d-transposition as the same default ventriculoarterial connections. An combinations of mirror-imaged atrial
option. This latter term fails to account important subset of these patients can arrangement, concordant atrioventricular,
accurately for all patients having the usual have a left-sided aorta (Figure 8.43). Nor and discordant ventriculoarterial
atrial arrangement with concordant does d-transposition describe accurately connections (Figure 8.44). All these
Sup.
Right Left
Arterial duct
Inf.
Pulmonary
trunk
Good-sized
ascending aorta

the front, shows hypoplastic left heart syndrome with combined
Dominant right ventricle aortic and mitral atresia. In this heart, the aorta is of good size,
despite the gross hypoplasia of the left ventricle.
Pulmonary
trunk
Sup.
Post. Ant. Fig. 8.36 The computed tomographic angiogram from the patient
Good-sized shown in Figure 8.34, viewed from the right side, shows arterial
Transverse aortic arch ascending aorta pathways that are directly comparable to those seen in the heart
Inf.
dissected to produce Figure 8.35.
patients, nonetheless, can be described emphasised, can also occur in the setting dominate the anatomical and clinical
accurately as having transposition. of isomerism of the atrial appendages. considerations. We exclude these cases,
However, discordant ventriculoarterial The anomalies at the venoatrial and therefore, from the category of
connections, as we have already atrioventricular junctions then transposition as described here.
Sup. Arterial duct
Right Left
Inf.
Diminutive
ascending aorta Pulmonary
trunk
the front, shows the arrangement of the arterial trunks in
hypoplastic left heart syndrome due to combined aortic and mitral
atresia when there is a diminutive ascending aorta.
Diminutive
ascending aorta
Sup. Fig. 8.38 The computed tomographic angiogram shows an

Descending
aorta arrangement of the arterial pathways in hyoplastic left heart
Ant. Post. syndrome directly comparable to that in the specimen illustrated in
Figure 8.37 with a diminutive ascending aorta. Note the shelf-like
Aortic isthmus coarctation lesion (arrow) at the origin of the aortic isthmus. The
Inf.
shelf is composed of ductal tissue.
From the surgical standpoint, the and those with additional malformations anatomy is comparable within the whole
two major subgroups of hearts with sufficiently severe to complicate the group.
transposition are those without any clinical picture. The morphological Now, corrective surgical procedures are
major additional complicating lesions, aspects of the complicating lesions will almost always performed at the arterial
usually described as simple transposition, be dealt with in turn, but the atrial level. Operations designed to redirect
Incomplete right ventricle Sup.
Ant. Post.
Aorta
Inf.
Fig. 8.39 As shown by this specimen, in anatomical orientation

viewed from the left, mitral atresia is not always part of hypoplasia
of the left heart. In this example, there is absence of the left
atrioventricular connection, but the right atrium is connected to a
dominant ventricle with left ventricular trabeculations (not seen).
The incomplete right ventricle, supporting the aorta, is left-sided
Absent left atrioventricular connection Blind-ending left atrium and anterior. Had the left atrioventricular valve been formed, it
would almost certainly have been of tricuspid morphology.
Sup. Ventricular septal defect
Ant. Post.
Inf.
Fig. 8.40 In this specimen, seen in anatomical orientation and

photographed from the left side, there is an imperforate mitral
Imperforate
Hypoplastic left ventricle valve in the setting of concordant atrioventricular connections.
mitral valve
Note the hypoplastic left ventricle and ventricular septal defect.
venous blood at the atrial level should not probably the most important factor. The equally applicable in the setting of
be forgotten, however, particularly as these presence of discordant ventriculoarterial transposition. The entire terminal
manoeuvres are an integral part of double connections does not affect the position of groove should be avoided, as should the
switch procedures. When planning these the sinus node in any way, so the rules crest of the atrial appendage, because the
operations, known as the Mustard and enunciated in Chapter 2 for avoidance of node can extend in horseshoe fashion
Senning options, it is the disposition of the this node, and discussed earlier in the across the crest, although it is usually in a
cardiac nodes and their blood supply that is setting of the Fontan procedure, are lateral position. The nodal artery may
Imperforate LAVV Incomplete right ventricle
RAVV

Sup.
Fig. 8.41 In this specimen, seen in anatomical orientation with
the dominant left ventricle having been opened from the front,
Right Left
there is a double inlet to the dominant ventricle, with the
incomplete right ventricle in the left-sided position, but with an
Inf. imperforate left atrioventricular valve (LAVV). The right
atrioventricular valve (RAVV) is patent.
Pulm. Pulm..
trunk trunk
Morph.
Aorta Morph. Aorta
left atrium
left atrium
Morph. Morph.
right atrium right atrium
Morph.
Morph. Fig. 8.42 The cartoon shows the
left ventricle
left ventricle segmental combinations of
Morph. concordant atrioventricular and
Morph.
right ventricle discordant ventriculoarterial
right ventricle
connections that produce the
lesion usually described simply as
transposition. The arrangement
can exist in usual and mirror-
imaged variants. Morph.,
morphologically; Pulm.,
Usual atrial arrangement Mirror-imaged atrial arrangement
pulmonary.
enter the groove across the crest of the running across the superior border of the septostomy. A lateral course across the
appendage, or after it has taken a retrocaval oval fossa, usually described as being the atrial appendage is also significant
course. Of more importance is the course septum secundum (Figure 8.45). It is at (Figure 8.46).
taken by the nodal artery as is ascends the risk in this position when incising the Other considerations remain significant
interatrial groove. The artery frequently septum for either a Senning or Mustard when carrying out a venous switch
burrows into the atrial musculature, procedure, or during a Blalock–Hanlon procedure. Cannulation of the superior
Anterior intervent.
coronary artery
Aorta
Pulmonary Fig. 8.43 This surgical view, taken through a median sternotomy,
trunk shows a patient with transposition, defined on the basis of
Left concordant atrioventricular and discordant ventriculoarterial
connections, in which the aorta is anterior and leftward relative to
Sup. Inf. the pulmonary trunk. It would be a mistake to describe this
arrangement as d-transposition. Note the origin of the anterior
Right coronary artery Right interventricular (intervent.) artery from the right coronary artery,
with passage across the subaortic infundibulum.
Morph. right
Left-sided aorta atrial appendage
Sup. Fig. 8.44 In this heart with concordant atrioventricular and

discordant ventriculoarterial connections, shown in anatomical
Left orientation from the front, the aorta is left-sided relative to the
Right
pulmonary trunk. In this case, the heart is in the right chest, with
the apex pointing to the right, in the setting of mirror-imaged atrial
Morph. arrangement. Although exhibiting l-transposition, the
Inf. right ventricle arrangement is not congenitally corrected. Morph.,
morphologically.
Aorta
SCV
Oval fossa
Tricuspid
valve
Sup.
Fig. 8.45 This dissection of a specimen of transposition seen in
Post. Ant. anatomical orientation from the right side shows the relationship
of the artery to the sinus node, in this heart arising from the right
Inf. coronary artery, to the superior rim of the oval fossa. SCV, superior
caval vein.
Sup.
Post. Ant.
Inf.
Incision Fig. 8.46 This anatomical specimen with transposition is

photographed in anatomical orientation from the right side to
show how the artery to the sinus node, with the site of the node
shown by the blue textured oval, when arising laterally from the
Right coronary artery right coronary artery, has been transected by a standard incision in
the right atrial appendage.
caval vein should be performed a good suturing should be avoided in the area of pulmonary venous atrium in Mustard’s
distance from the cavoatrial junction, the superior border of the groove. If an operation, it can be made between the right
incising the right atrium well clear of the incision is required across the terminal pulmonary veins without fear of damaging
terminal groove. Traction, suction, or groove to widen the newly constructed the sinus node or its artery.
Sup.
Post. Ant.
SCV Inf.
Oval fossa
Fig. 8.47 This dissection is made in a normal heart, orientated

anatomically, by removing the endocardium from the inner surface
of the right atrium. It shows the non-uniform anisotropic
arrangement of the aggregated cardiomyocytes making up the
atrial walls. There is preferential conduction along the major axis of
muscle bundles such as the terminal crest, but no insulated tracts
Terminal crest Eustachian valve within the atrial musculature. The site of the atrioventricular node,
Coronary sinus
at the apex of the triangle of Koch, is shown by the star.
This discussion is also pertinent to the sinus should be carefully considered. It is transposition, can be just as variable as
genesis of arrhythmias after Mustard’s possible to perform this procedure without when found in isolation (Figure 8.48). The
operation or the Senning procedure. It had damaging the node and its zones of majority of defects open between the outlet
been suggested that the arrhythmias are transitional cells, but the incision will components of the ventricular mass, but
due to damage to purported specialised undoubtedly cross one of the preferential have their own peculiar characteristics. In
internodal pathways29. As we have routes of conduction. For this reason, it is such settings, typically with malalignment
explained in Chapter 2, there are no safer to place the inferior suture line so as to of the outlet septum, they can extend to
insulated tracts to be found within the atrial avoid the triangle of Koch completely. become perimembranous when there is
myocardium. Instead, the atrioventricular It is also important to place the fibrous continuity between the leaflets of
impulse is conducted from the sinus node interatrial baffles so as to minimise the risk the tricuspid and pulmonary valves
through the thicker muscles of the right of subsequent venous obstruction, either in (Figure 8.49), or have a muscular
atrial wall and septum, with the anisotropic the pulmonary or venous pathways. posteroinferior rim (Figure 8.50). The
aggregation of the cardiomyocytes Although it was suggested that venous distinguishing feature, as in tetralogy of
favouring preferential conduction obstruction was less likely to complicate the Fallot, is whether the posterocaudal limb of
(Figure 8.47). It is advantageous, therefore, Senning procedure, those who became the septomarginal trabeculation, or septal
to preserve at least one of these routes. If skilled in the Mustard procedure were able band, fuses with the ventriculoinfundibular
the terminal crest is to be divided to enlarge to achieve excellent results with a minimal fold. If there is fusion (Figure 8.50), the
the new pulmonary venous atrium, this is a incidence of late venous obstruction30. resultant muscle bar will buttress the
further reason to preserve the superior Almost certainly, therefore, it is the conduction axis, and there will be
border of the oval fossa. These procedures, surgical technique that determines the discontinuity between the leaflets of the
together with scrupulous avoidance of the likelihood of postoperative venous tricuspid and pulmonary valves. If there is
sinus node and its blood supply, mean that obstruction. no fusion of the muscular bars, the defect
arrhythmias can be reduced considerably, The major complicating lesion in will be perimembranous. The penetrating
if not totally avoided, after atrial redirection transposition is the presence of a atrioventricular bundle will thus be at risk
procedures. ventricular septal defect, with or without in this area of fibrous continuity
It is always important to avoid the obstruction of the left ventricular outflow (Figure 8.49).
atrioventricular node. The landmarks to tract. Any other lesion can coexist if These defects have other features of
this vital structure are the same as in the anatomically possible. The anatomy of the surgical significance. The tension
normal heart. Providing all surgery is additional lesions is then as described for apparatus of the tricuspid valve tends to
performed outside the triangle of Koch, the lesion found in isolation. course over the defect, attaching itself to
injury to the node will be avoided. The It follows, therefore, that ventricular the outlet septum or to the
technique of cutting back the coronary septal defects, when found in patients with ventriculoinfundibular fold. This makes
Doubly committed &

juxta-arterial
Aorta
Malalignment
Perimembranous
Fig. 8.48 The cartoon shows how the ventricular septal defect in
Muscular hearts with transposition, as in the normal heart, can be
perimembranous, muscular, or doubly committed and juxta-
arterial. The cartoon also emphasises the potential for
malalignment of the muscular outlet septum, a particular feature
of the ventricular septal defect in the setting of transposition.
Muscular
Aorta outlet septum
Tricuspid-
pulm. continuity
Fig. 8.49 This view of a specimen with transposition, seen in

Tricuspid anatomical orientation from the right ventricle, shows the right
Sup.
valve
ventricular aspect of a perimembranous defect (star). The defect
opens into the subaortic outlet with malalignment of the outlet
Right Left
septum, with overriding of the leaflets of the pulmonary valve.
Note the fibrous continuity posteroinferiorly between the leaflets
Inf. of the pulmonary (pulm.) and tricuspid valves. The conduction axis
is shown by the red dotted line.
closure of the defect difficult without circuit after an arterial switch. Of more ventriculoarterial connections, the anterior
producing damage to the tricuspid valve, significance is the divergence of the margin of a ventricular septal defect is
albeit that this is of less significance when margins of the parietal and anterior heart usually well circumscribed. In the setting of
the tricuspid valve is in the pulmonary walls. In hearts with concordant transposition, the muscular outlet septum
Aorta Malaligned muscular

Ventriculo- outlet septum
infundibular
fold
Caudal limb
of SMT
Fig. 8.50 This specimen, seen in anatomical orientation from the

Tricuspid valve Sup. right side, has a ventricular septal defect (star) opening into the
outlet of the right ventricle as in Figure 8.49, but with a muscular
posteroinferior rim produced by continuity between the caudal
Right Left
limb of the septomarginal trabeculation (SMT) and the
ventriculoinfundibular fold. Note the malalignment of the outlet
Inf. septum. The muscular rim protects the conduction axis, shown by
the red dotted line.
Aorta
Pulmonary
-mitral continuity
SMT
Sup.
Fig. 8.51 In this heart with transposition, seen in anatomical
Tricuspid valve orientation from the right side, a perimembranous defect opens
Right Left into the inlet of the right ventricle in the absence of malalignment
of the muscular outlet septum. The conduction axis, shown by the
Inf. red dotted line, is deviated posteroinferiorly, but still relates
directly to the rim of the defect. SMT, septomarginal trabeculation.
is frequently malaligned relative to the rest Defects can open into the inlet of the ventricular inlet, which are also
of the muscular ventricular septum as it right ventricle, and again be perimembranous, are the harbingers of
inserts into the anterocephalad wall of the perimembranous (Figure 8.51) or muscular straddling and overriding of the tricuspid
right ventricular outflow tract. The defect, (Figure 8.52). Some muscular defects open valve. This should always be suspected
therefore, is more difficult to close at this centrally, while others open apically, often when the right ventricle is hypoplastic.
anterior margin (Figures 8.49, 8.50). being multiple. Defects opening into the Straddling and overriding of the tricuspid
Sup.
Right Left
Inf.
Right atrium
Fig. 8.52 This heart, again with transposition, and photographed

Muscular ventricular septal defect through the right atrioventricular junction, has a muscular defect
opening into the inlet of the right ventricle. In this setting, the
conduction axis, shown by the red dotted line, courses
anterosuperior relative to the defect.
Sup.
Oval fossa
Right Left
Inf.
Fig. 8.53 In this heart with transposition, shown in anatomical

orientation, there is straddling and overriding of the tricuspid
valve. Because of the malalignment between the atrial and
ventricular septums, the atrioventricular (AV) node (red circle) is no
AV node displaced longer at the apex of the triangle of Koch (star), being deviated
Straddling and overriding
posteroinferiorly tricuspid valve posteroinferiorly, as shown by the superimposed course of the
conduction axis (red dotted line).
valve markedly increases the risks of and juxta-arterial defects can also be found, produced by any lesion which, in the
surgery, not least because of the abnormal but are the rarest variant (Figure 8.55). normal heart, would produce subaortic
disposition of the conduction tissues Subpulmonary obstruction in obstruction. Valvar stenosis often
(Figures 8.53, 8.54). Doubly committed transposition (Figure 8.56) can be accompanies the subvalvar abnormalities.
Malaligned
Overriding right AV junction ventricular
septum
Anomalous
AV node
Apex
Sup. Inf. Fig. 8.54 The cartoon, shown in surgical orientation, illustrates the
Regular AV node location of the conduction axis in hearts with malalignment between
(no contact)
the atrial and muscular ventricular septums, as found when there is
Base
straddling and overriding of the tricuspid valve. AV, atrioventricular.
Sup. Aortic-pulm.
Aorta continuity
Right Left
Inf.
SMT
Fig. 8.55 In this heart with transposition, seen in anatomical
orientation from the front, the ventricular septal defect (star) is
doubly committed and juxta-arterial, with fibrous continuity
between the leaflets of the aortic and pulmonary (pulm.) valves.
Tricuspid The defect also extends to become perimembranous. Note the
valve pulmonary-to-tricuspid fibrous continuity, which is reinforced with
Tricuspid-pulmonary continuity
a membranous flap. SMT, septomarginal trabeculation.
More rare lesions, such as anomalous bundle branch. The lesion also typically left coronary artery, or because they are
insertion of the tension apparatus of the extends on to the pulmonary leaflet of the formed by the pulmonary leaflet of the
atrioventricular valve, are probably beyond mitral valve, and can coexist with a mitral valve. The safest area for resection is
surgical repair. Aneurysm of the ventricular septal defect (Figure 8.58). The beneath the remnant of the left margin of
membranous septum, or similar fibrous difficulties are compounded when the the ventriculoinfundibular fold. When
tissue tags (Figure 8.57) should be readily fibrous obstruction is more extensive, subpulmonary obstruction coexists with a
amenable to removal, although a because it can form a subvalvar tunnel. The ventricular septal defect, there is frequently
subpulmonary fibrous shelf poses more other quadrants of the outflow tract are also posterocaudal deviation of the outlet
problems as it directly overlies the left vulnerable because of their proximity to the septum, which is inserted in the left
Deviated outlet septum Valvar
Septal bulge Tissue tags
Abnormal
tension apparatus
Fibrous shelf
Fig. 8.56 The cartoon shows the lesions that can produce subpulmonary
obstruction in the setting of transposition. Exactly the same lesions will
produce subaortic obstruction in hearts with concordant ventriculoarterial
connections.
Tissue tag Sup.

Pulmonary trunk
Ant. Post.
Inf.
Mitral valve
Fig. 8.57 This view of the left ventricle from a specimen with
transposition, seen in anatomical orientation from the left, shows a
Left ventricle tissue tag from the septal leaflet of the tricuspid valve herniating
through a perimembranous ventricular septal defect and
producing subpulmonary obstruction.
ventricle (Figure 8.59). This usually means outlet septum is also part of the REV and infundibular anatomy. These
that the aortic valve overrides the septum. procedure. Should the defect be situated variations do not alter the intracardiac
When the septal defect is substantial with other than between the ventricular outlets, anatomy, and are of relatively minor
this combination, the scene is set for an the chances of successfully transferring the surgical significance. For example, the
operative procedure such as the Rastelli or aortic infundibulum into the left ventricle aorta is usually anterior and to the right in
Nikaidoh operations. When placing the are considerably reduced. complete transposition (Figure 8.60) but as
aortic outlet into the left ventricle during Thus far, we have devoted attention already illustrated, the aorta may be
these procedures, it is possible to resect the exclusively to the segmental anatomy of anterior and to the left. This is the rule
outlet septum safely, which never harbours transposition. There is further variation to when the atrial chambers are mirror-
conduction tissue. Such resection of the be found in terms of arterial relationships imaged (Figure 8.44), but left-sided and
Sup. Pulmonary trunk
Ant. Post.
Inf.
Left ventricle Mitral

valve
Fig. 8.58 In this view of the left ventricular outflow tract of a
specimen with transposition, seen in anatomical orientation, there
is obstruction due to an extensive fibrous shelf that extends onto
the leaflet of the mitral valve (arrows).
VSD Deviated
outlet
septum
Left ventricle
Sup.
Ant. Post.
Fig. 8.59 This specimen of transposition, seen from the left
ventricle in anatomical orientation, has posterocaudal deviation of
Inf. Narrowed subpulm. outlet the outlet septum through a coexisting ventricular septal defect.
VSD, ventricular septal defect.
transposed aortas can also be encountered pulmonary trunk (Figure 8.61). These as a unifying terminology, as it makes little
when there is usual atrial arrangement different relationships do not alter the basic sense to use this term for description of the
(Figure 8.43). In even more rare cases, the anatomy. They do show why it is patient in whom the aorta is left-sided
aorta can be posterior and to the right of the inadvisable to use the term d-transposition when there is usual atrial arrangement.
Pulmonary
trunk
Sup.
Anterior Right Left Fig. 8.60 This heart is shown in anatomical orientation,
right-sided demonstrating the usual relationship of the aorta in the setting of
aorta transposition, namely anterior and to the right of the pulmonary
Inf. trunk. Note the parallel arrangement of the arterial trunks as they
extend into the mediastinum.
Right posterior aorta Anterior

spiralling
pulm. trunk
Fig. 8.61 In this specimen of transposition, shown in anatomical

Sup. orientation from the front, the aorta is positioned posteriorly and
in a rightwards position relative to the pulmonary trunk, with
spiralling of the arterial trunks. This is the normal relationship of
Right Left
the arterial trunks, but still with discordant ventriculoarterial
connections. The arrows show that the right ventricle supports the
Inf. aorta, while the left ventricle gives rise to the pulmonary (pulm.)
trunk.
There are some clues to associated arrangement, any coexisting ventricular connection of the aorta to the left ventricle.
lesions to be drawn from these unexpected septal defect is frequently doubly When the aorta is posterior and the arterial
arterial relationships. When the aorta is committed and juxta-arterial. This trunks are normally related, there is usually
left-sided with the usual atrial arrangement is convenient for direct a subpulmonary infundibulum, with the
Sup.
Right Left
Pulmonary trunk
Inf.
Mitral
valve
Left ventricle
Fig. 8.62 This heart, photographed through the left ventricle in
anatomical orientation, shows a large subpulmonary infundibulum
(white double-headed arrow) separating the leaflets of the
pulmonary and mitral valves.
leaflets of the aortic valve in fibrous morphologically appropriate ventricles. arise from either of these sinuses, giving
continuity with the anterior leaflet of the The significant morphological feature is only eight potential patterns of sinusal
mitral valve through the roof of a that, thus far without reported exception, origin, including the arrangements in
perimembranous septal defect. This the coronary arteries arise from the aortic which both arteries arise from the same
unusual anatomy can create difficulty both sinuses that are adjacent to the pulmonary aortic sinus (Figure 8.66). Thus far, only
at initial diagnosis and at subsequent trunk (Figure 8.63). When performing the seven have been seen in the setting of
surgery. Variations in infundibular arterial switch, therefore, the surgeon is transposition34. Transfer of the arteries
morphology in themselves are unlikely to required to transfer the origins of the during the switch procedure can be
give problems. The expected subaortic coronary arteries across a relatively short compromised by an anomalous course of
muscular infundibulum in the right distance (Figure 8.64). This holds true the coronary arteries themselves in
ventricle is encountered most frequently irrespective of the relationship of the aorta relation to the vascular pedicle
along with fibrous continuity between the to the pulmonary trunk. (Figure 8.67), albeit that such variations
leaflets of the pulmonary and mitral valves The variable relationships between the can be suitably accommodated by
in the left ventricle. Rarely, there may be a aorta and the pulmonary trunk, however, appropriate surgical technique. An
complete muscular infundibulum in both produce problems in naming the aortic intramural course of the origin of the
ventricles (Figure 8.62). Even more rarely, sinuses and, hence, the origin of the coronary artery across a valvar
as described earlier, there may be a coronary arteries. Truly formidable commissure (Figure 8.66), or through the
subpulmonary infundibulum with conventions are created if attempts are aortic wall because of a high origin
continuity between the leaflets of the aortic made to catalogue each and every pattern31, (Figures 8.68, 8.69), creates greater
and mitral valves, particularly when the while use of simple alphabetical codes32 is problems, but these can also be resolved
discordantly connected aorta is in a self-evidently procrustean. As suggested now with appropriate surgical technique.
posterior position. by the group from Leiden33, it is best to The origin of the artery to the sinus node
The feature currently of greatest name the aortic sinuses as viewed from the can be very close to the origin of one
surgical importance in patients with stance of the observer standing in the non- coronary artery from an aortic sinus, or the
transposition is the morphology of the adjacent sinus of the aorta and looking sinus nodal artery can originate separately
coronary arteries. This is because of the towards the pulmonary trunk from the sinus. Also of note is mismatch
universal acceptance of the arterial switch (Figure 8.65). Thus, one sinus is always to between the commissures of the aortic and
procedure as the surgical treatment of the observer’s right-hand side. This is pulmonary valves (Figure 8.70)35. Despite
choice. This operation involves transecting designated as sinus no.1. The other sinus is all these anatomical variations, surgeons
the ascending aorta and pulmonary trunk, to the left-hand side, and is called sinus now find it possible to perform the arterial
and reconnecting them to their no.2. The three major coronary arteries can switch procedure with remarkable success.
Aorta
Pulm.
trunk
Mitral Tricuspid
valve valve
Sup.
Fig. 8.63 In this heart, the short axis is viewed from above in
anatomical orientation, the atrial myocardium and the arterial
Left Right
trunks having been removed. The ventriculoarterial connections
are discordant. The dissection shows how the coronary arteries
Inf. (arrows) arise from the two aortic sinuses that are adjacent to the
pulmonary (Pulm.) trunk.
Left Aortic root
Sup. Inf.
Right
Fig. 8.64 The picture, taken in the operating room, shows the
short distance required to transfer a button supporting one of the
Pulmonary root Right coronary arterial button
coronary arteries from the old aortic to the old pulmonary root
during the arterial switch procedure.
in this fashion, as with transposition itself although hearts with isomeric appendages
CONGENITALLY CORRECTED (Figure 8.42), the entity can exist with the can have left-hand ventricular topology in
TRANSPOSITION atrial chambers in their usual arrangement, association with discordant
We describe the combination of discordant or in mirror-imaged position ventriculoarterial connections.
atrioventricular and ventriculoarterial (Figures 8.72–8.74). Furthermore, like The complexities of congenitally
connections as congenitally corrected transposition, it cannot exist when there is corrected transposition reflect the fact that,
transposition (Figure 8.71). When defined isomerism of the atrial appendages, with the usual atrial arrangement, there is
Left-hand facing sinus Pulmonary trunk

“no. 2” Right-hand facing sinus
“no. 1”
Fig. 8.65 The cartoon shows how the two aortic sinuses
Sup. that face the pulmonary trunk can always be described
accurately as being to the surgeon’s left-hand or right-hand
side when standing in the non-adjacent sinus of the aorta
Post. Ant.
and looking towards the pulmonary trunk, irrespective of
the relationships of the arterial trunks. Conventionally, the
Non-adjacent sinus Inf. sinus to the right-hand side is described as being no. 1, while
that to the left-hand side is designated as being no. 2.
Left-hand facing sinus LCA Right-hand facing sinus

“no. 2” “no. 1”
RCA
Sup. Fig. 8.66 In this heart from a patient with transposition, the aortic
root has been opened and is viewed from the front. Both of the
Right Left coronary arteries arise from the left-hand facing sinus, or sinus no.
2. The main stem of the left coronary artery (LCA) was noted to take
an intramural course as it extended towards the left
Non-adjacent sinus Inf. atrioventricular and interventricular grooves. RCA, right coronary
artery.
malalignment between the inlet part of the (Figure 8.75). This abnormal feature the apex of the triangle of Koch, to
muscular ventricular septum and the atrial accounts for the most important surgical penetrate through the fibrous
septum. These two septal structures are in aspect of congenitally corrected atrioventricular junction and make contact
line at the crux but, when traced forwards, transposition, namely the unusual with the ventricular conduction tissues.
they diverge markedly. This produces a disposition of the atrioventricular Instead, an anomalous atrioventricular
gap, into which is wedged the conduction tissues36. Because of the septal node, found in an anterolateral position
subpulmonary outflow tract from the malalignment, it is not possible for the (Figure 8.76), as in the double-inlet left
morphologically left ventricle normal atrioventricular node, positioned at ventricle (see Figure 8.14), gives rise to the
Right coronary Solitary arterial stem Circumflex

artery from sinus no. 1 artery
Pulmonary
Aorta
trunk
Anterior Fig. 8.67 In this heart, photographed in anatomical orientation

interventricular from the front, the right and left coronary arteries arise from a
artery solitary vessel, with the right coronary and circumflex arteries then
passing in front of the arterial pedicle.
LCA Right-hand facing sinus

Left-hand facing sinus
“no. 1”
“no. 2”
RCA
Sup.
Fig. 8.68 In this heart, as in the specimen shown in Figure 8.66,
Left both coronary arteries arise within the left-hand facing sinus. In this
Right
heart, the orifice of the main stem of the left coronary artery (LCA)
is above the sinutubular junction. Having exited from the aortic
Non-adjacent sinus Inf. root, the artery courses between the arterial trunks in intramural
fashion (see Figure 8.69). RCA, right coronary artery.
penetrating atrioventricular bundle. The subpulmonary outflow tract, crossing the while the cord-like right branch of the
bundle penetrates the insulating plane of characteristic anterior recess of the bundle penetrates the septum to reach the
the atrioventricular junction lateral to the morphologically left ventricle, before left-sided morphologically right
area of fibrous continuity between the descending onto the muscular ventricular ventricle36. The discordantly connected
leaflets of the pulmonary and mitral valves. septum (Figure 8.77). Having branched, aorta arises from this morphologically right
A long non-branching bundle then runs the fan-like left branch is distributed in the ventricle, typically above a complete
around the anterior quadrants of the right-sided morphologically left ventricle, muscular infundibulum, and usually in a
Sup.
Right Left
Inf.
Aorta Pulmonary trunk
Intramural
course
Fig. 8.69 The heart shown in Figure 8.68 is photographed from

the left side, showing how the main stem of the left coronary artery
is embedded within the wall of the aorta as it runs towards the
Circumflex artery anterior surface of the heart, where it divides into its circumflex and
anterior interventricular branches.
Aorta
Pulmonary
trunk
Fig. 8.70 In this heart with transposition, in which the atrial

myocardium and the arterial trunks have been removed, and being
photographed from the atrial aspect, there is mismatch between
the zones of apposition of the leaflets of the aortic and pulmonary
valves (stars). Because of this, the distance for transfer of the right
coronary artery is increased (double-headed arrow).
left-sided position (Figure 8.78). When less than ideal to use d-transposition as normal. This situation is very much the
congenitally corrected transposition is an alternative term for regular exception. Usually, one or more of three
found in the mirror-imaged variant, transposition. associated lesions are found. These are a
however, it is the rule for the aorta to be When congenitally corrected ventricular septal defect, pulmonary
located in an anterior and right-sided transposition exists without any other stenosis, or anomalies of the left-sided
position (Figure 8.74). This is why it is anomaly, the circulation of the blood is atrioventricular valve37. It is these
Aorta Aorta
Morph. Morph.
left atrium left atrium
Pulm. Pulm.
trunk trunk
Morph. Morph.
right atrium right atrium
Morph. Morph. Fig. 8.71 The cartoon shows the

right ventricle right ventricle segmental combinations of
discordant atrioventricular and
Morph. ventriculoarterial connections
Morph.
left ventricle left ventricle that produce the lesion best
described as congenitally
corrected transposition. As
shown, the arrangement can be
found in usual and mirror-imaged
variants. Morph.,
morphologically; Pulm.,
Usual atrial arrangement Mirror-imaged atrial arrangement
pulmonary.
Aortic root
Right-sided
morph. LA
Morph. RCA
Sup. Fig. 8.72 The computed tomographic angiogram, viewed from

Right-sided the right side, shows a right-sided morphologically left atrium
Ant. morph. RV
Post (morph. LA) that is connected to a right-sided morphologically right
ventricle (RV), which then supports the aorta. This is congenitally
Inf. corrected transposition in the setting of mirror-imaged
arrangement of the organs. RCA, right coronary artery.
associated lesions that require surgical patient with concordant atrioventricular perimembranous type, and opens between
treatment. connections, it may be perimembranous, the ventricular inlets. Because of the
A ventricular septal defect is present in muscular, or doubly committed and juxta- wedged position of the pulmonary valve,
up to three-quarters of patients. As in the arterial (Figure 8.79). Usually it is of the the pulmonary trunk tends to override the
Sup.
Right Left
Right-sided
morph. LA Inf.
Left-sided
morph. RA
Right-sided
morph. RV
Left-sided Fig. 8.73 The image shows the four-chamber cut through the
morph. LV computed tomographic angiographic dataset shown in Figure 8.72.
The narrow neck of the right-sided atrial appendage (star) shows
that it has left morphology (morph. LA), while the other
appendage has pectinate muscles extending to the crux (white
arrow) confirming the left-sided location of the morphologically
right atrium (morph. RA). The nature of the apical trabeculations
confirm that the atrial chambers are connected to inappropriate
morphological ventricles (morph. LV, morph. RV).
Aorta
Pulmonary
trunk
Fig. 8.74 The short axis cut through the dataset created from the
Ant. computed tomographic angiograms shown in Figures 8.72 and 8.73
shows the right-sided and anterior location of the aorta relative to
Left Right the pulmonary trunk. This shows that d-transposition can also be
congenitally corrected. Note the normal position of the coronary
Right coronary artery arteries because of the right-handed ventricular topology that is a
Post.
feature of this segmental combination.
Plane of atrial septum Sup.
Right Right Left

atrium
Inf.
Fig. 8.75 This specimen with congenitally corrected

transposition, viewed in anatomical orientation from the
morphologically (Morph.) left ventricle, shows the malalignment
between the atrial (yellow double-headed arrow) and ventricular
Morph. left (red double-headed arrow) septal structures, with the
ventricle subpulmonary outflow tract wedged into this gap (white double-
headed arrow). The site of the atrioventricular conduction axis is
Plane of ventricular septum shown by the superimposed green loop. (Reproduced by kind
permission of Prof. Anton Becker, University of Amsterdam.)
Mitral valve Left

Anterolateral node
Sup. Inf.
Right
Aorta
Pulm.
trunk
Pulmonary valve Fig. 8.76 The cartoon shows the view of the morphologically
mitral valve in congenitally corrected transposition as seen by the
surgeon working through a right atriotomy. The locations of the
atrioventricular node and the conduction axis have been marked in
Ventricular septal defect Connecting node not
red. There is a perimembranous ventricular septal defect. Pulm.,
in triangle of Koch
pulmonary.
defect (Figure 8.80). When seen from the mitral valve (Figure 8.81). The abnormal closing the defect. The atrioventricular
right atrium, it is shielded by the superior position of the atrioventricular node and bundle arises from the anomalous
end of the zone of apposition between the the non-branching atrioventricular anterolateral node, penetrates the area of
leaflets of the right-sided morphologically bundle must be borne in mind when pulmonary-mitral valvar continuity, and
Left
Sup. Inf.
Right
Morph. left ventricle
Aorta
Fig. 8.77 In this cartoon, we show the view of a perimembranous

ventricular septal defect in congenitally corrected transposition, as
Pulm.
trunk would be seen by the surgeon working through a generous incision in
Pulmonary valve Ventricular septal defect the right-sided morphologically (Morph.) left ventricle. The course of
the conduction axis has been marked in green. Pulm., pulmonary.
Pulmonary trunk Sup.
Right Left
Inf.
Aorta
Morphologically
right appendage
Morphologically
right ventricle
Fig. 8.78 This specimen is photographed in anatomical

orientation from the front, showing the usual left-sided location of
the aorta relative to the pulmonary trunk in patients with
congenitally corrected transposition.
then runs around the subpulmonary mostly between the ventricular inlets, it can between the leaflets of the pulmonary
outflow tract to descend on the extend to become doubly committed and and mitral valves. It is still likely that the
anterosuperior margin of the defect juxta-arterial. It is then roofed by the conduction tissue remains in an
(Figure 8.77). conjoined leaflets of the aortic and anterosuperior position relative to the
The safest way of closing the defect pulmonary valves (Figure 8.83). When it is defect. A comparable case has been seen
without damaging the conduction system is perimembranous and juxta-arterial, the with both a perimembranous and a
to place sutures from the left-sided conduction tissue remains in an anterior muscular outlet defect. The conduction
morphologically right ventricular aspect position. In some patients, however, axis descended along the muscle bar
(Figure 8.82)38. Although the defect opens there can be a muscular inferior rim between them.
Valvar
continuity
Doubly committed Pulmonary

and juxta-arterial valve Tricuspid
valve
Pulm.
Perimembranous
trunk
Mitral
valve
Morphologically
left ventricle
Sup.
Muscular
Right Left
Ventricular
Fig. 8.79 The cartoon shows that, in congenitally corrected septal
transposition as in the otherwise normal heart, the ventricular defect Inf.
septal defect can be perimembranous, muscular, or doubly
committed and juxta-arterial. In congenitally corrected
transposition, however, the conduction axis, shown in yellow, Fig. 8.80 This view of a specimen with congenitally corrected
takes a grossly abnormal course relative to the defects. Pulm., transposition, shown in anatomical orientation from the front, illustrates the
pulmonary. relationships of a perimembranous ventricular septal defect opening into
the inlet of the morphologically left ventricle.
Left
Right-sided
mitral valve Inf.
Sup.
Right
Fig. 8.81 This view of a perimembranous inlet ventricular septal

Coronary sinus
defect in congenitally corrected transposition taken in the
operating room, as seen through the right-sided morphologically
mitral valve, shows how the defect and the subpulmonary outflow
tract are shielded behind the anteroseptal commissure of the valve.
Anomalous Regular The cross-hatched areas show the locations of the connecting and
anterior node node in triangle
of Koch non-connecting atrioventricular nodes. (Reproduced by kind
and bundle permission of Dr. Jan Quaegebeur, Columbia University, New
(no contact)
York.)
Left
Ventricular
septal defect
Sup. Inf.
Right
Fig. 8.82 This further view of the patient shown in Figure 8.81
illustrates how the axis of conduction tissue (cross-hatched area)
can be avoided by placing sutures through the defect on its
Needle on morphologically right ventricular aspect, which is left-sided when,
left side as in this patient, there was the usual atrial arrangement.
Coronary
of septum (Reproduced by kind permission of Dr. Jan Quaegebeur, Columbia
sinus
University, New York.)
Sup. Pulmonary Pulmonary-aortic

trunk continuity
Right Left
Inf.
Mitral
valve
Fig. 8.83 In this specimen, viewed in anatomical orientation, the

Pulmonary-tricuspid Morphologically perimembranous ventricular septal defect (star) extends to become
continuity left ventricle doubly committed and juxta-arterial, being roofed by a fibrous
continuity between the leaflets of the aortic and pulmonary valves.
As with transposition, any of the lesions Valvar stenosis in isolation is rare, but lesions (Figure 8.86), or muscular
that produces obstruction of the left frequently coexists with subvalvar obstruction, are also encountered39. The
ventricular outflow tract will produce obstructive lesions. Particularly significant overwhelming consideration in all of
pulmonary stenosis when the transposition in this respect are fibrous tissue tags these types of stenosis is the
is congenitally corrected (Figure 8.84). (Figure 8.85). Fibrous diaphragmatic presence of the non-branching bundle
Valvar stenosis
Pulmonary
trunk
Tissue tags
Fig. 8.84 The cartoon shows the lesions that can produce
subpulmonary obstruction in the setting of congenitally corrected
transposition. Note the anterior position of the conduction axis,
shown in yellow. As with transposition, the same lesions produce
Fibrous shelf subaortic obstruction in the setting of concordant
ventriculoarterial connections.
Sup.
Pulmonary
trunk
Right Left
Inf.
Mitral
valve
Morphologically Fig. 8.85 In this specimen, viewed in anatomical orientation

left ventricle through the morphologically left ventricle, a fibrous tissue tag
derived from the septal leaflet of the left-sided morphologically
tricuspid valve (star) herniates into the subpulmonary outflow
tract.
running around the anterior quadrants Ebstein’s malformation is the lesion that Ebstein’s malformation with concordant
of the outflow tract. Apart from tissue most frequently afflicts the left-sided atrioventricular connections. Should
tags, this relationship makes it very morphologically tricuspid valve, involving replacement of the valve become
difficult to resect these various lesions. downwards displacement of the septal and necessary, the location of the conduction
Placement of a conduit may be the safest mural leaflets (Figure 8.87). Only rarely is tissues in the right-sided atrioventricular
means of avoiding postoperative heart the inlet part of the ventricle dilated and junction takes them out of the area of
block. thinned, as occurs so frequently in danger.
Site of anterior Sup.

node
Right Left
Inf.
Mitral
valve
Fig. 8.86 In this specimen, seen in anatomical orientation

through the morphologically left ventricle, a fibrous shelf produces
subpulmonary obstruction in the presence of a perimembranous
Subpulmonary ventricular septal defect. The site of the axis of atrioventricular
Perimembranous
fibrous ring
ventricular septal defect conduction tissue has been superimposed in the cross-hatched
areas.
Morphologically left atrium Atrialised

ventricle
Morphologically
right ventricle
Sup. Fig. 8.87 This specimen with congenitally corrected

transposition, seen in anatomical orientation from the left side,
Left-sided
Ant. Post. morphologically shows Ebstein’s malformation of the left-sided morphologically
tricuspid tricuspid valve. There is no thinning of the musculature of the inlet
Inf. valve component of the morphologically right ventricle. The white
dotted line shows the location of the atrioventricular junction.
The other anomaly that affects the left tissues from an anterolateral node, but will who use biventricular correction have
valve, and that can also affect the right increase the risks of surgery markedly. observed disappointing results
valve40, is straddling of its tension Indeed, in the presence of straddling subsequent to simple correction of the
apparatus and overriding of the valvar atrioventricular valves, some may opt for associated lesions, because these
orifice (Figure 8.88). These anomalies do functionally univentricular rather than manoeuvres leave the morphologically
not alter the origin of the conduction biventricular surgical correction. Those right ventricle supporting the
Sup.
Ant. Post.
Left atrium
Inf.
Fig. 8.88 In this heart with congenitally corrected transposition,

Hypoplastic Straddling and overriding viewed in anatomical orientation from the left-sided
morphologically left-sided morphologically morphologically right ventricle, there is straddling and overriding
right ventricle tricuspid valve of the left-sided morphologically tricuspid valve. Note the
hypoplastic nature of the morphologically right ventricle.
systemic circulation. Because of this, possible to find a sling of conduction tissue setting can make enlargement of the defect
the trend now is to correct the defects running along the crest of the muscular a daunting procedure (Figure 8.90).
using the so-called double switch ventricular septum and joining anterior and Exceedingly rarely, it is possible to find
procedure, combining atrial redirection regular atrioventricular nodes36. This pulmonary atresia with an intact
with either an arterial switch41 or use of an possibility should be borne in mind for all ventricular septum and an imperforate
interventricular tunnel to the aorta, patients having discordant atrioventricular right-sided atrioventricular valve. When
placing a conduit from the connections and a double outlet from the seen from the right atrium, the
morphologically right ventricle to the morphologically right ventricle. arrangement can be indistinguishable from
pulmonary atrium42. This also makes it Some patients also have a single outlet in pulmonary atresia with an intact
possible to make corrections in association with discordant atrioventricular ventricular septum (compare Figure 8.91
patients with coexisting pulmonary connections, most frequently because of with Figure 8.31). The significant
atresia. pulmonary atresia (Figure 8.89). In this difference is that, unlike regular pulmonary
Some patients with discordant situation, there is typically a atresia with an intact ventricular septum, it
atrioventricular connections will have perimembranous ventricular septal defect. is the morphologically right ventricle that
ventriculoarterial connections other than If the defect is large, then, as we have must drive the systemic circulation when
discordant ones. We will discuss the discussed, it is possible to achieve the atrioventricular connections are
options for a double outlet from the biventricular repair by channelling the discordant (Figure 8.92). Aortic atresia, or
morphologically right ventricle in the next morphologically left ventricle to the aorta, common arterial trunk, can also rarely be
section, but our emphasis there will be on placing a conduit from the morphologically found with discordant atrioventricular
patients with concordant atrioventricular right ventricle to the pulmonary arteries, connections. A more important
connections. The variability to be and performing an atrial redirection combination is that found with
described can also be found when the procedure42. The defect, however, is not concordant ventriculoarterial connections.
atrioventricular connections are always large enough to permit channelling We will discuss this arrangement in
discordant, but then the atrioventricular of the morphologically left ventricle to the greater detail below. The significant
conduction axis will arise from an aorta (Figure 8.90). If restrictive, the surgical feature is that the combination
anterolateral atrioventricular node, as decision must be made as to whether the produces the ideal situation for
described for congenitally corrected defect can be enlarged, or whether the employment of an atrial redirection
transposition. In patients with discordant better option is to create the Fontan procedure, because this restores the
atrioventricular connections and a double- circulation. The anterocephalad location of morphologically left ventricle to pumping
outlet right ventricle, nonetheless, it is also the atrioventricular conduction axis in the the systemic circulation.
Left-sided anterior aorta Sup.
Post. Ant.
Inf.
Atretic pulmonary trunk Fig. 8.89 The image shows the base of the heart. The atretic
pulmonary trunk is posterior and right-sided relative to the aorta.
Morph. right atrial appendage The pulmonary arteries were fed through a persistently patent
arterial duct. Morph., morphologically.
Sup. Perimembranous VSD
Ant. Post. Aorta
Inf.
Fig. 8.90 The image shows the left-sided ventricle from the heart
illustrated in Figure 8.89. The atrioventricular connections are
discordant, and the aorta is supported by the left-sided
morphologically (morph.) right ventricle. The perimembranous
Tricuspid valve
ventricular septal defect (VSD) is restrictive. The dotted red line
shows the course of the atrioventricular conduction axis, which is
carried on the right side of the ventricular septum. Its
anterocephalad location relative to the defect would make
Left-sided morph. enlargement difficult, if not impossible, to achieve without
right ventricle
producing atrioventricular block if the decision were made to
attempt biventricular repair by channelling the morphologically
left ventricle to the aorta, and then performing an atrial redirection
procedure.
there is overriding of the aortic and from the ventricle that is also supporting
DOUBLE-OUTLET VENTRICLE pulmonary roots, respectively. It remains the other arterial root. Our approach to this
It is a well-known fact that spectrums of controversial as how best to describe the dilemma is to argue that, when the greater
malformation exist in the settings of both ends of these spectrums when the greater part of both arterial roots arises from the
tetralogy of Fallot and transposition when part of the overriding arterial root arises same ventricle, then the ventriculoarterial
SCV Imperforate mitral valve
Oval fossa
Sup. Fig. 8.91 The morphologically right atrium is photographed from

above and behind to reveal the presence of an imperforate
Right atrioventricular valve. When viewed in isolation, it is not possible to
Left
distinguish the morphological nature of the valve. As shown in
Figure 8.92, however, the atrioventricular connections were
Inf. Coronary sinus discordant, so the valvar membrane is morphologically mitral. SCV,
Sub-aortic infundibulum
Left-sided morph.
right ventricle
Tricuspid valve
Sup. Fig. 8.92 The image shows the left-sided ventricle in the heart
shown in Figure 8.91. The ventricle is coarsely trabeculated, and
Ant. Post. receives the morphologically (morph.) tricuspid valve. The heart
had pulmonary atresia with an intact ventricular septum, but in the
Inf. setting of discordant atrioventricular and ventriculoarterial
connections.
connection is that of a double outlet. On as both arterial trunks arise unequivocally supported exclusively from the right
this basis, we define a double-outlet from the one ventricle. An additional ventricle, but also that each arterial valve
ventricle whenever more than half of the problem arises in this situation, because it needed to have a completely muscular
circumference of both arterial valves takes was initially considered that, to justify the infundibulum, or conus. This approach,
its origin from the same ventricle43. In diagnosis of a double-outlet right ventricle, however, is inconsistent with the
many patients, this problem does not arise, not only did both arterial trunks need to be morphological method, which states that
one variable feature, in this instance the short axis. The 50% rule is simply a between the great arteries and the defect in
ventriculoarterial connection, should not pragmatic convention for distinguishing the ventricular septum that is important to
be defined on the basis of another feature between double-outlet and one-to-one the surgeon. Naming the hole between the
that is itself variable, namely the ventriculoarterial connections. When ventricles creates an additional difficulty. In
infundibular morphology. It is now based on such an approach, the topic of the patients having a ventricular septal defect in
accepted, therefore, that a double-outlet double-outlet ventricle becomes the setting of one-to-one ventriculoarterial
right ventricle can exist in the absence of straightforward, if not even simple. connections, the ventricular septal defect is
bilateral infundibulums44. As we will show, The double-outlet ventriculoarterial typically defined as the place of space limited
many hearts do indeed exist when the connection, defined as discussed previously, caudally by the crest of the muscular
arterial trunks arise from the right accounts only for an anatomical arrangement ventricular septum, and cranially by the
ventricle, but with fibrous continuity of the ventriculoarterial junctions. Hearts undersurface of the muscular outlet septum
between the leaflets of an arterial and an with this feature, therefore, can exist with (Figure 8.94, left-hand panel). When both
atrioventricular valve. Furthermore, even such a wide variety of configurations that the arterial trunks are supported within the right
when there is overriding of an arterial root, possible combinations seem almost limitless. ventricle, the muscular outlet septum is
there is usually little question as to the The cases to be illustrated in this section are exclusively a right ventricular structure
anatomical assignment of the abnormal those with both arteries arising from the (Figure 8.94, right-hand panel). The
valve, because rarely does it appear to be right ventricle in the setting of concordant analogous plane of space to the regular
equally committed to both chambers. In atrioventricular connections. The principles ventricular septal defect would be the locus
situations still creating difficulty, the to be established are equally applicable in the over which a patch is placed to tunnel one or
decision regarding commitment of the setting of the other atrioventricular the other arterial trunk into the left ventricle,
overriding root should be made according connections, remembering that when the this being the therapeutic option usually
to the connection of the leaflets as seen in atrioventricular connections are discordant, taken by the surgeon to restore biventricular
short axis, rather than on the basis of the or there is left-hand topology in the setting of circulations. This plane, however, is not the
alignment of the long axis of the ventricular isomerism, there is likely to be an hole that is commonly defined as the
septum (Figure 8.93). Making designations anomalously located atrioventricular node, ventricular septal defect in this setting.
on the basis of long-axis views can be or even dual atrioventricular nodes. Almost Instead, it is the exit from the left ventricle
misleading because of the motion of the always, there is a coexisting interventricular that is typically considered to represent the
heart during the cardiac cycle. This does communication in the presence of the double ventricular septal defect. Therefore, to avoid
not alter the arbitration as based on the outlet45. It is thus the anatomical relationship confusion, we prefer to name the hole as the
Artery
True override
Short axis section
is assessed relative
to chord of circle B A
Y
X
sh ta l
ta n
in rien epta
or tio
xis
o S
Y
Fig. 8.93 The cartoon shows how overriding
arterial valves are assigned to one or the other
ventricle on the basis of their connection as
judged in the short axis (left-hand panel), in
which it is possible to assess the tangent drawn
by the ventricular septum (X–Y) relative to the
A
B short axis of the overriding arterial trunk,
rather than the long axis of the ventricular
True override septum (A–B in the right-hand panel), which
is independent of can change depending on the motion of the
septal axis
heart.
Interventricular Fig. 8.94 The cartoon illustrates the

Ao Ventricular Ao PT communication
PT significant difference between the plane of
septal
Muscular defect space identified as the ventricular septal
outlet septum defect when there are concordant or
discordant ventriculoarterial connections
(one-to-one connections) as opposed to a
double outlet from the right ventricle. The
LV LV left-hand panel illustrates the situation with
RV
discordant ventriculoarterial connections, but
RV the concept is equally valid when the
connections are concordant. As shown in the
Crest of muscular right-hand panel, the hole usually identified as
ventricular septum
Patch for biventricular connection the ventricular septal defect is better
considered to be the interventricular
One-to-one
Double-outlet right ventricle communication. Ao, aorta; PT, pulmonary
ventriculoarterial connections
trunk; LV and RV, left and right ventricles.
Aorta
Pulm.
trunk
Morphologically
right ventricle
Sup.
Left
Fig. 8.95 This specimen, shown in anatomical orientation from
Right
the front, has a double outlet from the right ventricle with the
interventricular communication in subaortic position. The great
Inf. arterial trunks spiral in normal relationships as they leave the base
of the heart.
interventricular communication. patient will have had a double outlet. In outlet from the right ventricle. In many, the
Recognition of this fact also helps the contrast, if the surgeon considers that it was aorta and the pulmonary trunk are related
surgeon to determine whether a given possible simply to close the septal defect, more or less normally. The aortic valve,
patient has a double-outlet ventriculoarterial then the patient will have had concordant or therefore, is posterior and to the right of
connection. Should it be considered discordant ventriculoarterial connections. the pulmonary valve, and the pulmonary
necessary to place a patch so as to tunnel the The relationship of the arterial trunks is trunk spirals around the aorta as it extends
communication to an arterial root, then the another variable in patients with a double towards its bifurcation (Figure 8.95). In the
Pulmonary
trunk
Aorta
Sup.
Fig. 8.96 In this specimen, seen in anatomical orientation from

Right Left
the front and with a double outlet from the right ventricle, the
interventricular communication was in a subpulmonary position.
Inf. The arterial trunks rise in parallel fashion (black arrows) as they exit
from the base of the heart (compare with Figure 8.95).
PT
PT PT
Ao Ao Ao
Fig. 8.97 The cartoon, drawn in anatomical orientation, shows

the three typical orientations of the arterial trunks to be found in
hearts with a double-outlet right ventricle. The arrangements with
spiralling and parallel arterial trunks with the aorta to the right
have been shown in Figures 8.95 and 8.96. The rarest variant,
Spiralling great Great arteries Great arteries shown in the right-hand panel, is for the arterial trunks to rise in
arteries parallel parallel parallel fashion, but with the aorta in the left-sided position. Ao,
(‘normally related’) -aorta to right -aorta to left
aorta; PT, pulmonary trunk.
remainder, the arterial trunks arise from the communication is most usually subaortic In the light of these discussions, it is
base of the heart in parallel fashion, as when the arterial trunks are spiralling, and evident that the anatomy of the
anticipated when the ventriculoarterial subpulmonary when they extend into the interventricular communication needs to
connections are discordant. For a good mediastinum in parallel fashion, there is be considered in two ways. The first feature
proportion of these, the arterial valves are no direct correlation between these features. of significance is its proximity to the great
side-by-side (Figure 8.96); otherwise, the It is always necessary, therefore, to arteries45. The second feature concerns its
aortic valve is anterior. In a few cases, the determine precisely the location of the own intrinsic morphology. When
aorta is posteriorly located because the interventricular communication relative combining these two approaches, it must be
pulmonary trunk is enlarged. Almost to the arterial roots, and to establish remembered that, when there is a double
always, the aorta is to the right but, in a small whether it can be channelled safely to one outlet from the right ventricle, the
number of cases, the aorta can be left-sided or the other arterial root in an unobstructed muscular ventricular septum itself
relative to the pulmonary trunk fashion so as to achieve a biventricular separates only the inlet and apical
(Figure 8.97). Although the interventricular surgical repair. trabecular components of the ventricles. As
Sup.
Pulmonary trunk
Right Left
Aorta
Inf.
Muscular
outlet
septum Fig. 8.98 In this specimen, shown in anatomical orientation from
the front, the interventricular communication (star) is cradled
within the limbs of the septomarginal trabeculation (SMT). Note
that the posterocaudal limb of the trabeculation fuses with the
ventriculoinfundibular fold. The muscular buttress thus formed
protects the atrioventricular conduction axis. There is fibrous
continuity between the leaflets of the aortic and mitral valves in
Ventriculoinfundibular fold Caudal limb of SMT the roof of the interventricular communication, so that it is directly
adjacent to the aortic root.
we have emphasised, the outlet septum, or some distance away from the margins of the those having a double outlet from the right
its fibrous remnant, is found between the interventricular communication ventricle. The most frequent configuration
two outlets from the right ventricle, and (Figure 8.100). Even in the setting of is when the aortic valve is posterior and to
does not form the roof of the bilateral infundibulums, the the right of the pulmonary trunk
interventricular communication. interventricular communication can still be (Figure 8.95), usually with the
Except when non-committed, the perimembranous, as there can be interventricular communication in a
interventricular communication is cradled continuity between the leaflets of the mitral subaortic position (Figures 8.98–8.102).
between the limbs of the septomarginal and tricuspid valves (Figures 8.100, 8.101). This arterial relationship can also be
trabeculation, or septal band. Its other When the ventriculoinfundibular fold is found with a doubly committed and juxta-
borders vary depending on several features. attenuated, the leaflets of one of the arterial arterial defect (Figure 8.103). The
The first is whether the caudal limb of the valves are able to achieve fibrous continuity second group comprises hearts in which
septomarginal trabeculation fuses with the with an atrioventricular valve the aorta ascends parallel to the pulmonary
ventriculoinfundibular fold. If it does, (Figure 8.99). trunk (Figure 8.96), usually with its
there is a muscular inferior rim to the The final feature is the relationship of valve in either an anterior or side-by-side
defect. This protects the conduction tissue the muscular outlet septum to the other position, and to the right relative to the
axis as in tetralogy of Fallot or transposition structures. When attached to the anterior pulmonary valve, and with the septal
(Figure 8.98). If it does not, there is limb of the septomarginal trabeculation, the defect in a perimembranous or muscular
continuity between the leaflets of the defect is subaortic (Figures 8.98–8.101). but subpulmonary position
atrioventricular valves, and the defect is When, in contrast, the outlet septum is (Figure 8.102). This is the Taussig–Bing
perimembranous (Figure 8.99). The attached to the ventriculoinfundibular fold, variant46.
second feature is the extent of the or to the posterior limb of the The surgeon should be particularly
ventriculoinfundibular fold. It is the septomarginal trabeculation, the defect is wary when the interventricular
presence of well-formed folds bilaterally placed beneath the left-sided great artery, communication is non-committed47.
that produces bilateral muscular which is almost always the pulmonary Almost always, when it is subaortic,
infundibulums, or the bilateral conuses that trunk (Figure 8.102). When the outlet subpulmonary, or doubly committed, it
were initially considered to be part-and- septum is absent, the defect is doubly is possible to place a patch within the
parcel of the definition of the double-outlet committed and juxta-arterial right ventricle so as to channel the
right ventricle. The significant surgical (Figure 8.103). interventricular communication to one or
aspect of this feature is that the From this potential for variation, two the other outflow tract, thus achieving
infundibulums move the arterial valves groups of patients can be collected from biventricular circulations. This may not
Sup.
Pulmonary
trunk
Right Left
Inf.
Aorta
Fig. 8.99 In this specimen, shown in anatomical orientation, the

outlet septum is attached to the anterocephalad limb of the
Muscular septomarginal trabeculation (SMT) so that the defect (star) is
outlet
subaortic. There is extensive fibrous continuity between the leaflets
septum
of the mitral and tricuspid valves, making the defect
perimembranous, but there is also extensive continuity between
the leaflets of the aortic and tricuspid valves (black dotted line),
even though the aortic root is supported exclusively within the
right ventricle. The specimen shows that hearts can have a double
Ventriculoinfundibular fold Caudal limb of SMT outlet from the right ventricle without there being bilateral
infundibulums.
Muscular outlet septum Sup.
Right Left
Pulmonary
valve
Inf.
Aortic
valve
Fig. 8.100 In this specimen with a double-outlet right ventricle,

Morphologically seen in anatomical orientation, there is an extensive subaortic
right ventricle
infundibulum, along with a long subpulmonary infundibulum
(double-headed arrows), but the defect is perimembranous, with
fibrous continuity between the leaflets of the tricuspid and mitral
valves. The interventricular communication (star) is in the limbs of
the septomarginal trabeculation, and is adjacent to the subaortic
Tricuspid-mitral continuity outflow tract, but relatively distant from the aortic valve because of
the length of the subaortic infundibulum.
be possible if the defect is anatomically pathways are blocked by structures such as communication is between the ventricular
non-committed, or if the interventricular valvar tension apparatus (Figure 8.104). inlets (Figure 8.104), or is the ventricular
communication is adjacent to an outflow Anatomical non-commitment is most component of an atrioventricular septal
tract, but the potentially corrective usually found when the interventricular defect in patients with a common
Ventriculoinfundibular fold Pulmonary

Aorta
trunk
Fig. 8.101 In this specimen, seen in anatomical orientation, there

is extensive fibrous continuity between the leaflets of the tricuspid
and mitral valves, making the interventricular communication
Sup. perimembranous despite the presence of bilateral infundibulums
(black dotted line). Note that the subpulmonary infundibulum
(black brace) is narrowed by a squeeze between the outlet septum
Right Left
and hypertrophied septoparietal trabeculations. Thus, the heart
Tricuspid-mitral has the phenotypical features of tetralogy of Fallot in addition to a
fibrous continuity Inf. double-outlet connection and bilateral infundibulums. The star
shows the interventricular communication.
Sup.
Right Left
Pulmonary
Aorta trunk
Inf.

interventricular communication (star) is positioned in a
subpulmonary location. This is the so-called Taussig–Bing
malformation. Note the attachment of the muscular outlet septum
to the midpoint of the ventriculoinfundibular fold, along with
bilateral infundibulums (double-headed arrows). There is also
Muscular outlet septum tricuspid–mitral fibrous continuity, so the interventricular
Tricuspid-mitral continuity
communication is perimembranous.
atrioventricular junction (Figure 8.105). whether the defect is truly non-committed opens into the inlet of the right ventricle
The surgeon may be able to overcome (Figure 8.106). There is no question but (Figure 8.107).
these impediments and still achieve that the defect is non-committed both It is also important for the surgeon to
biventricular repair48. In this situation, anatomically and surgically when it is identify any other abnormalities in the
however, there may be arguments as to encased within the muscular septum and extremely heterogeneous group of
Aortic-pulmonary continuity
Pulmonary
valve
Aortic
valve
SMT
Sup. Fig. 8.103 In this specimen, shown in anatomical orientation, the

interventricular communication (star) is doubly committed and
juxta-arterial due to the failure of formation of the subpulmonary
Right Left
muscular infundibulum. There is fibrous continuity between the
leaflets of the aortic and pulmonary valves, and fibrous continuity
Ventriculoinfundibular fold Inf. between the aortic and tricuspid valves (red dotted line). SMT,
septomarginal trabeculation.
Aorta Outlet
septum
Pulm.
trunk
Sup.
interventricular communication opens between the ventricular
Right Left
inlets, and is shielded by the septal leaflet of the tricuspid valve.
This feature, coupled with the attachment of tendinous cords to
Inf. Perimembranous VSD the muscular outlet septum (arrows), makes the defect non-
committed. VSD, ventricular septal defect.
malformations linked together because of stenosis, valvar stenosis, coarctation, or producing the morphology of tetralogy of
a double-outlet ventriculoarterial interruption of the aortic arch. The Fallot, even in the setting of bilateral
connection. By far the most common set presence of subpulmonary infundibular infundibulums (Figure 8.101).
of anomalies relates to obstruction of the stenosis is a particularly frequent finding Coarctation and interrupted arch,
arterial outlets, such as infundibular when the septal defect is subaortic, often together with straddling of the mitral
Pulmonary
Aorta trunk
Outlet
septum
Sup. Fig. 8.105 In this specimen with a double-outlet right ventricle

and bilateral infundibulums (double-headed arrows), shown in
Right Left anatomical orientation, there is a common atrioventricular
junction guarded by a common atrioventricular valve. It is
Atrioventricular questionable whether the ventricular component of the
septal defect Inf. atrioventricular septal defect could be channelled to the
subpulmonary root. Anatomically, the defect is non-committed.
Pulmonary Sup.
trunk
Right Left
Aorta
Inf.
Fig. 8.106 In this specimen with a double-outlet right ventricle

(RV) and bilateral infundibulums (double-headed arrows), seen in
anatomical orientation, the interventricular communication is
again between the ventricular inlets (compare with Figure 8.105).
In this specimen, however, the inlet perimembranous defect could
Perimembranous defect almost certainly be committed surgically to the subpulmonary
opening to inlet of RV outlet. In this instance, therefore, the defect might well be
described as being subpulmonary rather than non-committed.
valve, are frequent accompaniments of the coronary arterial anatomy, including the situation, special consideration is needed
Taussig–Bing variant46. Common intramural arrangement, is found most when performing either a right
atrioventricular valves also occur in a frequently when the aorta is in an ventriculotomy or an arterial switch
significant number of cases. Unusual anterior or side-by-side position. In this procedure.
Aorta Pulmonary trunk
Muscular outlet septum
Fig. 8.107 In this specimen with a double-outlet right ventricle

and bilateral infundibulums (double-headed arrows), shown in
anatomical orientation, the interventricular communication is
Muscular inlet defect within the muscular septum and opens into the right ventricular
inlet. This defect is unequivocally non-committed.
Ventriculoinfundibular fold-
safe for conduction tissue but
resection goes outside heart Outlet septum-
safe
Anterocephalad
limb-beware septal
Conduction axis perforator
Fig. 8.108 The cartoon, illustrating a double-outlet right ventricle

with a subaortic interventricular communication, shows the major
Major danger with
arterial to tricuspid components of the outflow tracts, and illustrates the salient surgical
continuity features relating to each individual muscular component. The
conduction axis is shown in red, and the first septal perforating
artery as the red dotted line. The ventriculoinfundibular fold is
Posterocaudal limb–beware shown in green, the outlet septum in blue, and the septomarginal
conduction axis
trabeculation in yellow.
Surgical and anatomical considerations their relationships to each other, and to the into the ventriculoinfundibular fold, or into
for the potential corrective procedures interventricular communication. As the posterior part of the muscular
relate to the particular combination of described previously, in cases with ventricular septum. The outlet septum is
defects. It is possible, nonetheless, to subaortic defects the outlet septum usually always devoid of any vital structure, so it
establish some basic rules (Figure 8.108)49. inserts into the anterocephalad limb of the can be resected, or used as a secure site for
The outlet septum, almost always present, septomarginal trabeculation. With anchorage of sutures. In contrast, the
serves as a guide to the arterial valves and subpulmonary defects, it usually inserts ventriculoinfundibular fold is not a solid
bar of muscle. Care must be taken to avoid of the atrioventricular and arterial valvar is distinguished from the other types of
extensive dissection or resection of this leaflets, or is attenuated at some point to ventriculoarterial connection producing a
structure, as the right coronary artery lies permit fibrous continuity between them. single outlet from the heart, namely a
within its fold. Either arrangement can exist when the solitary aortic trunk with pulmonary
Knowledge of the type of ventriculoarterial connection is one of a atresia, a solitary pulmonary trunk with
interventricular communication, as in double outlet. aortic atresia, or a solitary arterial trunk52.
isolated ventricular septal defects, will give A double outlet from the left ventricle is The presence of the common truncal valve
accurate guidance to the disposition of the very much more rare than a double-outlet also distinguishes the entity from hearts
conduction tissue. Obstruction of the right ventricle. It can present with similar with a large aortopulmonary window,
subpulmonary outflow tract requires the variations in the position of the arterial which can effectively produce a common
same attention to detail as when dealing trunks, and their relationship to the intrapericardial arterial component.
with tetralogy of Fallot. Other cardiac interventricular communication, as we While the difference between a common
anomalies will have to be dealt with in the have described for the double-outlet right trunk and an aortopulmonary window is
context of the particular cardiac ventricle. As with the double-outlet right anatomical, the distinction between
configuration present. ventricle, the double-outlet left ventricle common and solitary arterial trunks is more
It may not have passed unnoticed that can be found with any atrioventricular semantic. We define an arterial trunk as
discussion thus far of the group of hearts connections. The rules for the disposition being solitary rather than common when it
unified because of their double-outlet of the conduction tissue will thus change is not possible to find any evidence of
ventriculoarterial connections has paid accordingly. intrapericardial pulmonary arteries52.
little attention to bilateral conuses. This is Description in this fashion resolves the
because, as explained in our introductory controversy as to whether the pulmonary
comments, we do not take this feature as an COMMON ARTERIAL TRUNK arteries, had they been present, would have
integral component of the group. To us, A common arterial trunk is one that arisen from the arterial trunk or from the
the term double outlet logically pictures a supplies the systemic, pulmonary, and heart (Figure 8.111). In patients with a
specific ventriculoarterial connection, and coronary arteries directly (Figure 8.109)50. solitary trunk as thus defined, it is the
that is what we have described. The The phenotypical feature of the lesion, arterial supply to the pulmonary circulation
presence or absence of bilateral however, is the commonality of the that is the key to surgical treatment. Such
infundibular musculature depends simply ventriculoarterial junction, which is patients are best considered along with
on whether the ventriculoinfundibular fold guarded by a common arterial valve tetralogy of Fallot with pulmonary atresia
interposes completely between the hinges (Figure 8.110)51. In this way, the anomaly (see page 236; Figure 7.159).
Systemic arteries
Pulmonary
arteries
Coronary arteries
Sup.
Right Left
Fig. 8.109 The specimen, shown in anatomical orientation,
illustrates the essence of a common arterial trunk. A solitary trunk
Inf. leaves the base of the heart and directly supplies the systemic,
pulmonary, and coronary arteries.
Sup.
Common arterial trunk
Right Left
Inf.
Fig. 8.110 The specimen, photographed in anatomical

Right ventricle orientation, shows how the phenotypical feature of the common
arterial trunk is the common ventriculoarterial junction, guarded in
this heart by a valve with three leaflets. The common trunk is
Common arterial valve supported predominantly by the right ventricle, with a potentially
restrictive interventricular communication (star).
Common Fig. 8.111 The cartoon illustrates the dilemma in defining the
Aorta arterial nature of an arterial trunk in the absence of the intrapericardial
trunk
pulmonary arteries (dotted channels). There is no way of knowing
Possible
Possible whether the pulmonary arteries, had they existed, would have
origin
origin originated directly from the heart (left-hand panel) or from the
from heart
from trunk
arterial trunk itself (right-hand panel). Had they originated from
the heart, the patent arterial trunk would have been an aorta. Had
the trunk itself given rise to atretic pulmonary arteries initially,
however, it initially would have been a common trunk. In this
situation, therefore, it is better to describe the arterial trunk simply
as a solitary structure.
The anatomical features that underscore is very rare to find other than concordant be restrictive. This problem does not occur
the options for, and success of, surgical ones. The trunk itself usually overrides the when the trunk is committed
treatment for a common arterial trunk are ventricular septum, being more or less predominantly or exclusively to the left
its connection to the ventricular mass, the equally committed to the right and left ventricle. Right ventricular origin,
state of the truncal valve, the morphology ventricles (Figure 8.112), although it can however, is frequently associated with the
of the interventricular communication, the take origin predominantly (Figure 8.110) presence of an atrioventricular septal defect
presence of associated anomalies, and the or exclusively (Figure 8.113) from the and a common atrioventricular junction
arrangement of the systemic and morphologically right ventricle. When the (Figure 8.114)53. This adds to the surgical
pulmonary arterial channels arising from trunk arises entirely from the right complexity, because not only is the septal
the common trunk. The lesion can exist ventricle, problems can be produced defect more distant from the arterial trunk,
with any atrioventricular connection, but it should the interventricular communication but the left ventricular component of the
Common
truncal valve
Left
ventricle
Right Fig. 8.112 The heart has been sectioned along its long axis, and is
ventricle
shown in anatomical orientation, demonstrating how the common
arterial trunk (black brace) overrides the crest of the muscular
ventricular septum (star), in this instance being equally committed
to both ventricles.
Common arterial trunk Sup.
Right Left
Inf.
Fig. 8.113 In this heart with a common arterial trunk, seen in

anatomical orientation, the trunk arises exclusively from the right
ventricle. There is a completely muscular subtruncal infundibulum
(black dotted line). The interventricular communication is
Restrictive interventricular communication restrictive. Should enlargement be attempted, the safe area for
resection is shown by the red dotted line.
common atrioventricular valve is trifoliate. The common ventriculoarterial tract54. Although the truncal valve can
The need to secure a competent left valve junction, which is the essence of the be supported by a complete
adds to the problems of surgical repair common trunk, exists because of failure infundibulum when it arises exclusively
significantly. of septation of the developing outflow from the right ventricle (Figure 8.113),
Common arterial trunk Atrioventricular septal defect
Bridging leaflets
Sup. Fig. 8.114 The heart, photographed from the front, the right
Right ventricle ventricle having been opened, has a common arterial trunk
Right Left supported exclusively by the right ventricle. The interventricular
communication, however, is an atrioventricular septal defect. The
bridging leaflets of the common atrioventricular valve are seen
Inf. extending into the left ventricle. The left atrioventricular valve was
trifoliate.
Sup. Origin of
pulmonary
arteries
Right Left
Inf.
Common
truncal
valve
Fig. 8.115 This heart, shown in anatomical orientation as seen

from the right ventricle, has a common arterial trunk overriding an
interventricular communication with a muscular posteroinferior
rim formed by fusion of the ventriculoinfundibular fold with the
posterocaudal limb of the septomarginal trabeculation (SMT). The
muscular rim protects the atrioventricular conduction axis. Note the
Ventriculoinfundibular fold Posterocaudal limb of SMT fibrous continuity between the leaflets of the truncal and mitral
valves in the roof of the left ventricle (black dotted line).
more usually the interventricular mitral valves in the roof of the left septomarginal trabeculation. In most
communication is directly juxta-arterial, ventricle (Figure 8.115). When viewed patients, the posterocaudal limb of the
and there is typically fibrous continuity from the right ventricle, the septal defect trabeculation fuses with the
between the leaflets of the truncal and is cradled between the limbs of the ventriculoinfundibular fold to produce a
Sup.
Common
truncal Right Left
valve
Inf.
Fig. 8.116 This heart with a common arterial trunk, viewed in

anatomical orientation, has an interventricular communication
(star) that is perimembranous, with fibrous continuity between the
leaflets of the truncal and tricuspid valves (black dotted line),
because the ventriculoinfundibular fold stops short of the caudal
limb of the septomarginal trabeculation (SMT). There is also fibrous
continuity between the leaflets of the tricuspid and mitral valves.
Ventriculoinfundibular fold Posterocaudal limb of SMT The atrioventricular conduction axis is at risk in the posteroinferior
rim of the defect.
muscular posteroinferior rim septum should the arterial trunk arise The possibilities initially described were
(Figures 8.110, 8.112, 8.115). As with exclusively from the right ventricle, and for the arteries to arise from a short
other defects of this type, the muscular there is spontaneous closure of a pre- confluent channel, to arise separately and
structure thus formed serves to buttress existing interventricular communication. directly from the left posterior aspect of the
the axis of atrioventricular conduction The latter cases are unlikely to come to trunk, or to arise separately from either side
tissue from potential surgical damage. surgical attention. of the trunk. The initial classification also
More rarely, there can be fibrous After surgical repair of a common trunk included a fourth category, but those fitting
continuity between the truncal and following the Rastelli technique, the in this grouping are recognised as
tricuspid valves. The defect is thus truncal valve effectively becomes the aortic representing examples of tetralogy of Fallot
perimembranous, and the conduction valve. Valvar incompetence or stenosis, if with pulmonary atresia, with pulmonary
axis is much more at risk during closure present, is therefore of considerable arterial supply via systemic-to-pulmonary
when connecting the common trunk to significance. This typically reflects collateral arteries. The second classification
the left ventricle (Figure 8.116). Should it dysplasia of the leaflets (Figure 8.117). The was proposed by Van Praagh and Van
be deemed necessary to enlarge a truncal valve itself usually has three leaflets Praagh57. They initially divided the cases
restrictive interventricular (Figure 8.118). Quadrifoliate valves are also into groups with or without an
communication, this can be achieved by relatively frequent, although the leaflets are interventricular communication. As we
resecting along its anterosuperior often of unequal size (Figure 8.119). In have discussed, it is exceedingly rare to find
margins (Figure 8.113). Sometimes, a such circumstances, the surgeon may patients having a common arterial trunk
second muscular defect coexists with the choose to convert the valve into a trifoliate and an intact ventricular septum; therefore,
juxta-arterial defect. If present, such pattern56. Valves with two leaflets are also it is their first group that is of most
defects must be identified and closed. found. significance. They also described four
Cases have also been described in which There are two classifications currently subtypes, and grouped together the first
the ventricular septum has been in use for the description of the variations two categorisations of Collett and Edwards,
considered intact. This was because the to be found in the arrangement of the retaining the third grouping as their second
leaflets of the truncal valve closed on the pulmonary and systemic channels taking type. The importance of their approach
septal crest during ventricular diastole55. origin from the common trunk. Both was that it highlighted cases with
The juxta-arterial defect in this setting, employ procrustean numerical obstruction of the systemic circulation,
however, is readily evident when the categorisations. The classical study of identified as a fourth grouping, and also
valvar leaflets open during diastole. It is Collett and Edwards50 was based on the emphasised the possibility for a separate
possible to have a truly intact ventricular mode of origin of the pulmonary arteries. origin of discontinuous pulmonary arteries,
Left coronary artery Sinusal origin of pulmonary arteries
Sup.
Post. Ant. Fig. 8.117 In this heart, the common arterial trunk has been
opened from the right side, and is shown in anatomical orientation.
The pulmonary arteries arise from within a truncal valvar sinus.
Inf. Dysplastic quadrifoliate valve Note the origin of the left coronary artery from within the same
sinus. The truncal valve is dysplastic, and has four leaflets.
Separate origin of pulmonary arteries
Left coronary
artery
Post.
Right Left
Fig. 8.118 The common arterial trunk has been windowed from
the front, showing its posterior wall, from which the right and left
Ant. Trifoliate truncal valve pulmonary arteries take separate origin. The truncal valve has three
leaflets. Note the high origin of the left coronary artery.
with one fed by a persistently patent arterial Praagh57, however, also pointed out that pulmonary dominance within the trunk
duct, which could then become the overall collection of patients with a itself. It is this notion of aortic as opposed to
ligamentous, these examples making up common arterial trunk could be divided pulmonary dominance that simplifies
their third group. Van Praagh and Van into those with aortic as opposed to categorisation and description51,
Artery to
sinus node
Post.
Fig. 8.119 In this example of a common arterial trunk, windowed
Right Left
and opened from the right side, the truncal valve has four leaflets,
but one is much smaller than the others (arrow). The artery to the
Ant.
sinus node takes origin from the right coronary artery (star). Note
the high origin of the left coronary artery.
Common arterial trunk Adjacent origin of pulmonary arteries
Fig. 8.120 In this example of a common arterial trunk, opened

through the right ventricle from the front, the pulmonary arteries
take origin in adjacent fashion from the leftwards and posterior
part of an aortic dominant trunk. The interventricular
communication (star) has a muscular posteroinferior rim (red
double-headed arrow).
recognising that on rare occasions it may be comparable dimensions. In most instances, the leftwards component of the trunk
possible to find patients in which the it is the aortic component of the trunk that (Figures 8.120, 8.121). If there is a
intrapericardial common trunk divides into is dominant. The pulmonary arteries confluent pulmonary component, it is
aortic and pulmonary components of usually arise adjacent to one another from usually very short (Figures 8.122, 8.123).
Post.
Right Left
Ant.
RPA
LPA
Fig. 8.121 The computed tomographic angiogram, viewed from

above, shows how, in the setting of common arterial trunks, the
pulmonary arteries usually arise adjacent to one another from the
leftwards and posterior component of the trunk (white double-
headed arrow). Note the crossing of the left pulmonary artery (LPA)
over the origin of the right pulmonary artery (RPA). This is
Common arterial trunk exaggerated when the pulmonary arteries take a more anterior
origin (see Figure 8.124).
Common arterial trunk Confluent origin of

pulmonary arteries
Left coronary
artery
Fig. 8.122 In this example of a common arterial trunk, the

pulmonary arteries take origin from a small confluent component
arising from the leftwards and posterior part of the common trunk,
which itself predominantly supplies the systemic circulation. The
interventricular communication (star) is perimembranous, as there
is fibrous continuity between the leaflets of the truncal and
tricuspid valves (black dotted line), reinforced by a membranous
flap (arrow).
The pulmonary arteries can arise more take separate origins from the posterior common pulmonary dominant trunk is
anteriorly, and are typically crossed at their component of the dominant aortic trunk found when the aortic component of the
origins (Figure 8.124). More rarely, the (Figure 8.118). All of these patterns trunk is hypoplastic, in association with
pulmonary arteries can arise within a facilitate connection to a right ventricular either coarctation or interruption of the
truncal valvar sinus (Figure 8.117)58, or can conduit during complete repair. The less aortic arch (Figures 8.125, 8.126). It is in
RPA
LPA
Post.
Fig. 8.123 The section comes from the computed tomographic
Right Left dataset prepared from the patient shown in Figure 8.121. There is a
very short confluent segment (black double-headed arrow) feeding
Ant. the origin of the right and left pulmonary arteries (RPA, LPA) from
the common trunk.
Right pulmonary artery
Aortic dominant trunk
Sup.
Right Left
Fig. 8.124 In this heart with a common arterial trunk, the
pulmonary arteries arise from the anterior part of the trunk, which
Inf. is aortic dominant. The left and right pulmonary arteries cross as
they extend towards the hilums of the lungs (red arrows).
this arrangement that the pulmonary pulmonary arteries can be crossed when cases, or when the pulmonary arteries
arteries arise from the sides of the trunk, there is pulmonary dominance are discontinuous, with one fed via an
which continues through a persistently (Figure 8.127). The cases with pulmonary arterial duct, it is rare to find persistence
patent arterial duct to feed the descending dominance produce greater problems for of the duct. It is in the instances where
aorta51. On occasion, however, the surgical correction. Apart from these there is persistence of the duct that the
LSCA
RSCA
Arterial duct LPA
RPA
LCCA
RCCA
Fig. 8.125 In this heart, the pulmonary component of the

common arterial trunk is dominant (red double-headed arrow). It
gives rise to the arterial duct, and to the right and left pulmonary
arteries (RPA, LPA), the latter arising from the sides of the
pulmonary component. The aortic component of the trunk is
grossly hypoplastic, supplying the right common carotid artery
(RCCA), but then interrupted beyond the origin of the left common
Hypoplastic aortic
carotid artery (LCCA). There is a retro-oesophageal origin of the
component
right subclavian artery (RSCA), which arises from the descending
aorta along with the left subclavian artery (LSCA), the descending
aorta itself being fed by the arterial duct.
Sup. Stented arterial duct
Right Left
Inf.
Hypoplastic aortic
component
Fig. 8.126 The computed tomographic angiogram, reconstructed

and seen from the front, shows another example of a pulmonary
Common arterial trunk dominant common arterial trunk. In this patient, the aortic arch
was interrupted at the isthmus. A stent has been placed in the
arterial duct, which supplies the descending aorta.
Stented arterial duct
Fig. 8.127 The dataset from the reconstruction of the computed

Sup.
tomographic angiogram shown in Figure 8.126 is viewed from
behind. It shows crossing of the pulmonary arteries as they arise
Left Right from the posterior aspect of the common trunk. Note the right and
left pulmonary arteries that have been banded (white arrows), and
Inf. Right pulmonary artery the stented arterial duct, which feeds the descending aorta in the
setting of the interrupted aortic arch.
systemic and pulmonary components are Concordant ventriculoarterial trunks arise from the morphologically
more balanced as they divide from the connections with parallel arterial appropriate ventricles, they extend towards
common trunk within the pericardial trunks the mediastinum in parallel rather than
cavity. spiralling fashion61,62. It is necessary,
Hearts with this combination do not
The surgeon should also take care to therefore, to appreciate that the direction of
necessarily have abnormal segmental
identify the origins of the coronary spiralling should be related to the topology
combinations, because if the
arteries59. When the truncal valve has four of the ventricular mass, and not the
atrioventricular connections are
sinuses, then the arteries typically arise position of the atrial chambers, the latter
concordant, the segmental junctions will be
from the right- and left-sided sinuses. being the approach taken by those who
normal despite the unexpected
There is less uniformity when, as is most produce terms such as isolated ventricular
relationships of the arterial trunks. It is also
frequently the case, the truncal root has inversion.
the case that patients with this arrangement
three sinuses. It is unusual, nonetheless, for Thus, in the normal heart, the
will be exceedingly rare, and it is possible
coronary arteries to arise from the anterior pulmonary trunk, as it emerges from the
that the unusual position of the aorta could
sinus60. Solitary coronary arteries are right ventricle, spirals in counter-clockwise
go unnoticed. The same arrangement at the
frequent59. When there are two coronary fashion around the aortic root as the
ventriculoarterial junctions, nonetheless,
arteries, the left coronary artery often takes pulmonary arteries extend into the hilums
can be found with all the other possibilities
an origin above the sinutubular junction, (Figure 8.129). In the mirror-imaged
for atrioventricular connections; thus it is
which on occasion can be very high, with arrangement, when there is left-hand
appropriate to consider the combinations in
transmural passage through the truncal ventricular topology, the pulmonary
a separate section of this chapter, the more
wall (Figure 8.128). The left coronary trunk spirals around the aortic root in
so as they have been remarkably difficult to
artery can also be closely related to the clockwise fashion (Figure 8.130). In the
understand. They are also currently
origin of the pulmonary arteries, arrangement of parallel trunks found with
described with the most arcane of
particularly when the pulmonary arteries concordant atrioventricular and
terminologies by some, including such
themselves take a truncal origin ventriculoarterial connections and the usual
spectacular phrases as isolated ventricular
(Figure 8.117). In all these settings, the atrial arrangement, the aorta arises from the
inversion, and anatomically corrected
coronary arteries are at risk if unrecognised ventricular mass in a left-sided and anterior
malposition. The essence of understanding
when the surgeon separates the pulmonary position (Figure 8.131). In most instances,
is to appreciate that, although the arterial
arteries from the common trunk. the aortic valve is also supported within the
Sup. Main stem of left coronary artery
Ant. Post.
Inf.
RPA
Common
LPA arterial
trunk
Fig. 8.128 The common arterial trunk is photographed from the

left side to show a very high origin of the left coronary artery, which
extends in intramural fashion as it exits the aortic dominant
Anterior interventricular artery Circumflex artery component of the trunk. Note the crossed origin of the right (RPA)
and left (LPA) pulmonary arteries (compare with Figure 8.124).
Pulmonary trunk
Aorta
Sup.
Right Left
Fig. 8.129 The base of the normal heart is photographed to show
the normal spiralling of the arterial trunks, with the pulmonary
Inf. Right ventricle trunk extending in counter-clockwise fashion around the aortic
root (black arrows).
left ventricle by a complete muscular aortic and the atrioventricular valves. It is ventricular inversion. We can illustrate the
infundibulum (Figure 8.132). It is not the insistence of the presence of bilateral simplicity of recognising parallel but
essential, however, for there to be infundibulums that creates the need to concordantly connected arterial trunks by
discontinuity between the leaflets of the invent the arcane terms like isolated reference to the arrangement as seen in the
Sup.
Right Left
Inf.
Aorta
Pulmonary trunk
Fig. 8.130 This heart is from a normal individual with mirror-

image arrangement of all the organs. The ventricular mass shows
left-hand topology, and the pulmonary trunk spirals in clockwise
Morphologically right ventricle
fashion relative to the aortic root as it extends into the
mediastinum (black arrows).
Pulmonary trunk Juxtaposed atrial appendages
Aorta

Sup.
Right Left
Fig. 8.131 This heart is from a patient with concordant
atrioventricular and ventriculoarterial connections. The aorta,
Inf. however, is left-sided and anterior relative to the pulmonary trunk.
Note the left-sided juxtaposition of the atrial appendages.
presence of a double-inlet left ventricle. trunk, arising from the incomplete right trunks, although rare, the incomplete right
In the most common variant of a double- ventricle, spirals around the aortic root in ventricle is usually left-sided. The
inlet left ventricle with concordant counter-clockwise fashion, as in the normal pulmonary trunk is thus also left-sided,
ventriculoarterial connections, the lesion heart (Figure 8.133). In the variant with but enters the mediastinum in parallel
known as the Holmes heart, the pulmonary concordant connections but parallel arterial fashion to the arterial trunk, rather than
Sup.
Aorta
Right Left
Inf.
Fig. 8.132 The left side of the heart demonstrated in Figure 8.131
is shown, opened from its left side. The morphologically left atrium
is connected to a left ventricle with right-hand ventricular
topology. The aorta arises from the left ventricle, its valve
supported by an extensive muscular infundibulum (double-headed
arrow). There is also a large perimembranous ventricular septal
defect. Because the atrioventricular connections are concordant,
Morphologically left ventricle Mitral valve the atrioventricular conduction axis is posteroinferiorly positioned
(yellow dotted line).
Pulmonary trunk
Aorta
Ventricular
septal defect
Sup.
Fig. 8.133 The heart shown has a double-inlet left ventricle with
a right-sided incomplete right ventricle and concordant
Right Left
ventriculoarterial connections, the lesion known as the Holmes
Incomplete morphologically
heart. The arterial trunks spiral in normal fashion, with the
right ventricle
Inf. pulmonary trunk extending in counter-clockwise fashion relative to
the aortic root (black arrows) (compare with Figure 8.129).
spiralling as would be expected were there left ventricle, are usually in fibrous incomplete right ventricle is right-sided, the
normal but mirror-imaged relations continuity with the leaflets of the pulmonary trunk then entering the
(Figure 8.134). Furthermore, the leaflets of atrioventricular valves (Figure 8.135). A mediastinum in a right-sided position
the aortic valve, arising from the dominant similar arrangement can be found when the parallel to the aorta. These patterns can also
Pulmonary trunk
Sup. Aorta
Ant. Post.
Inf.
Fig. 8.134 This heart exhibits a double-inlet left ventricle,

Ventricular incomplete left ventricle, and concordant ventriculoarterial
septal defect connections. In this specimen, however, the incomplete right
ventricle is left-sided, and the pulmonary trunk arises in parallel
Incomplete morphologically fashion, no longer spiralling around the aorta. This is an example of
right ventricle concordant ventriculoarterial connections with parallel arterial
trunks.
Aorta
LAVV
RAVV
Sup.
Fig. 8.135 The dominant left ventricle from the heart shown in
Figure 8.134 is opened. The aortic valve arises from the ventricle
Right Left
with its leaflets in fibrous continuity (black dotted lines) with both
of the atrioventricular valves (RAVV, LAVV). Note the ventricular
Inf. septal defect communicating with the left-sided incomplete right
ventricle (arrow).
be found when there is absence of either the with a double-inlet right ventricle, although parallel arterial trunks are those with
right- or left-sided atrioventricular we are not aware of any such descriptions. discordant atrioventricular connections.
connections and the left ventricle is Perhaps the most important subset of This arrangement is a close cousin to
dominant. They should also be anticipated patients with concordantly connected but congenitally corrected transposition, as
Sup. Perimembranous
Pulmonary trunk
VSD
Ant. Post.
Inf.
Fig. 8.136 The left-sided ventricle is photographed in a heart

Tricuspid with the usual atrial arrangement and discordant atrioventricular
valve connections. There is left-hand ventricular topology, but the
ventricle supports the pulmonary trunk above a complete muscular
Morphologically right ventricle infundibulum. The pulmonary trunk exits the heart parallel to the
aorta. Note the perimembranous ventricular septal defect (VSD).
Sup.
Post. Ant.
Aorta
Inf.
Fig. 8.137 The right-sided morphologically left ventricle is shown

from the heart illustrated in Figure 8.136. The atrioventricular
connections are discordant, but the aorta arises from the
morphologically left ventricle, with infundibular musculature
interposed between the leaflets of the aortic and mitral valves.
Because of the discordant atrioventricular connections, the
conduction axis is anterior to the arterial valve (red star), and
descends (red dotted line) cephalad relative to the
perimembranous ventricular septal defect (white star) . The
combination of discordant atrioventricular and concordant
ventriculoarterial connections produces the haemodynamics of
Mitral valve transposition, but correction with an atrial redirection procedure
will restore the morphologically left ventricle to a systemic role.
discussed earlier in this chapter. The form of transposition, the aorta is typically procedure, thus restoring the
combination of discordant connections at anterior and right-sided. It is important, morphologically left ventricle to its
the atrioventricular junction, but however, to recognise that there is left- appropriate systemic role. It is also
concordant ventriculoarterial connections, hand topology (Figure 8.136) in this important, of course, to recognise and deal
produces the haemodynamics of setting, as the optimal therapeutic approach with any associated malformations, such as
transposition. In addition, as in the usual is to perform an atrial redirection a ventricular septal defect (Figure 8.137).
Because the atrioventricular connections artery conduits for complex congenital 23. Rao PS, Jue KL, Isabel-Jones J, Ruttenberg
are discordant in this combination, the heart disease. Ann Surg 1980; 192: 382–389. HD. Ebstein’s malformation of the
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univentricular atrioventricular connection. Circulation 1979; 59: 403–411. in anatomical correction. Thorax 1978; 33:
J Thorac Cardiovasc Surg 1990; 99: 19. Essed CE, Ho SY, Hunter S, Anderson 418–424.
314–319. RH. Atrioventricular conduction system in 33. Gittenberger-de-Groot AC, Sauer U,
6. Anderson RH, Baker EJ, Redington AN. univentricular heart of right ventricular Oppenheimer-Dekker A, Quaegebeur J.
Can we describe structure as well as type with right-sided rudimentary Coronary arterial anatomy in transposition
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Abnormalities of the
9
great vessels
Abnormalities of the right (Figure 9.2). Such right-to-left shunting can

ANOMALOUS SYSTEMIC superior caval vein also be an iatrogenic phenomenon,
VENOUS DRAINAGE occasionally observed when low-lying atrial
These are extremely rare. The vein may be
Abnormal systemic venous connections septal defects are improperly closed (see
diminished in size. Alternatively, it may be
are usually of little surgical significance, Chapter 7). The more frequent anomaly
completely absent when the venous return
as their clinical consequences are limited. involving the inferior caval vein is
from the head, neck, and arms passes
The anomalies are apt to be encountered interruption of its terminal segment, with
through a persistent left superior caval vein
as the surgeon pursues a more complex return of the venous blood from the lower
to the right atrium by way of the coronary
associated intracardiac anomaly. They body through the azygos or hemiazygos
sinus (Figure 9.1) or, rarely, directly into
are of most significance in the setting of venous systems (Figure 9.3)2. The
the left atrium. Only this last situation
isomeric atrial appendages, which we malformation is seen most frequently with
requires surgical intervention. The other
discuss in Chapter 10, showing how so- isomerism of the left atrial appendages3. The
conditions, if encountered during an
called visceral heterotaxy is best hepatic veins usually drain independently
open-heart operation, would require some
considered in terms of right versus left into the right-sided or left-sided atrium,
adjustment from the usual technique used
isomerism. In this chapter, we consider although there can be a confluent
for cannulation. Although there is no
the features of the anomalous systemic suprahepatic channel, as is usually the case
definite evidence to this effect, we would
venous connections in their own right. when interruption and azygos continuation
not expect these abnormalities to affect the
They may be grouped into the categories is found with usual or mirror-imaged atrial
location of the sinus node.
of absence or abnormal drainage of the chambers (Figure 9.4).
right superior caval vein, anomalies of the
inferior caval vein, persistence or
Abnormalities of the inferior
abnormal drainage of the left superior
caval vein Persistence of the left superior
caval vein, and abnormal hepatic venous caval vein
The inferior caval vein has been described to
connections. Abnormalities of the connect directly to the morphologically left Persistence of the left superior caval vein is
coronary sinus usually fall into one of atrium1, producing right-to-left shunting, the most common malformation involving
these groups, although unroofing, although we have never seen such a lesion. the caval veins (Figure 9.1). In about
which produces an interatrial When the inferior caval vein does drain to three-fifths of reported cases4, the persisting
communication through the right atrial the left atrium, this is usually because a left vein is joined to the right superior caval
orifice of the sinus, has been discussed persistent Eustachian valve directs flow from vein by a brachiocephalic vein. When
in Chapter 7. the right atrium through the oval fossa arranged in this fashion, the left caval vein

Left atrial
appendage
Pulmonary
trunk
Left
Sup. Inf.
Fig. 9.1 This operative view through a median sternotomy shows

Right
a left superior caval vein entering the pericardial cavity between
the left atrial appendage and the pulmonary trunk.
Fig. 9.2 The cartoon shows the most frequent mechanism by

Persisting Eustachian valve
which the venous return through the inferior caval vein drains to
the left atrium. It is deflected across a defect within the oval fossa
(red arrow) due to persistence of the Eustachian valve.
can simply be clamped or ligated, so as to connects with the coronary sinus, traversing veins are normally connected to the
avoid flooding the field when the heart is the inferior left atrioventricular groove to morphologically left atrium, the pattern of
opened. If connections with the right vein are empty into the right atrium through a larger venous return is comparable to the
not apparent, a trial period of occlusion will than normal orifice (Figure 9.6). In such ‘snowman’ types of anomalous pulmonary
usually indicate whether venous hearts, the Thebesian valve is often venous connection (see later). A variant of
hypertension will give problems. A left attenuated or absent. There are also reports the levoatrial cardinal vein is found when
superior caval vein is found not infrequently of the persistent vein obstructing flow from there is atresia of the right atrial orifice of the
in patients with cyanotic heart disease, being the pulmonary veins towards the atrial coronary sinus (Figure 9.9). A vertical vein
reported in up to one-fifth of patients with vestibule6. It is certainly the case that the can drain the coronary venous blood to the
tetralogy of Fallot, and one-twelfth of venous channel could bulge into the cavity of brachiocephalic vein and thence to the right
patients with Eisenmenger’s syndrome5. The the left atrium. atrium via the superior caval vein
venous channel can be the route of partially (Figure 9.10).
or totally anomalous pulmonary venous
Levoatrial cardinal vein
connection. It can also empty directly into the
left atrium, as in the constellation described Another anomaly that, although rare, ANOMALOUS PULMONARY
as unroofing of the coronary sinus (see warrants consideration is the levoatrial VENOUS CONNECTION
Chapter 7). Much more frequently, the vein cardinal vein7,8. This channel connects the Very rarely, the pulmonary veins may be
persists as an isolated venous channel, which left atrium to the systemic venous system. It obstructed or totally atretic at their atrial
usually receives the hemiazygos vein before is found with associated lesions such as mitral junction9. This is unlikely to be a surgically
penetrating the pericardium and passing atresia, where it functions as the only route remedial situation. Much more commonly,
between the left atrial appendage and the left for pulmonary venous return (Figures 9.7, the pulmonary venous system, either
pulmonary veins (Figure 9.5). It then 9.8). In this case, although the pulmonary totally or in part, has an anomalous
Sup. Right bronchus
Left Right
Inf.
Aorta
Fig. 9.3 The heart is photographed from behind, showing the

venous return from the abdomen returned to the heart through
the azygos system of veins (arrows). The inferior caval vein is
Hepatic veins to right atrium interrupted below the diaphragm, and returns only the blood
received through the hepatic veins.
Azygos vein
Superior caval vein
Morph. right
appendage
Fig. 9.4 This heart, photographed from the right side in

Sup.
anatomical orientation, shows the termination of the azygos vein,
which is returning the blood from below the diaphragm to the
Post. Ant. superior caval vein in the setting of interruption of the inferior
caval vein (see Figure 9.3). Note the structure of the
Inf. Hepatic veins morphologically (Morph.) right appendage, indicating the
presence of the usual atrial arrangement.
Left pulmonary Left superior caval vein

veins
Left appendage
Fig. 9.5 The heart is photographed from above in anatomical

orientation, the domes of both atrial chambers having been
Sup. removed. A persistent left superior caval vein enters the left
atrioventricular groove between the left appendage and the left
Post. Ant. pulmonary veins, with its course through the groove marked by the
red dotted lines. It enters the right atrium through the enlarged
coronary sinus (see Figure 9.6.) Note that, as it passes through the
Inf. Coronary sinus left atrioventricular groove, it potentially obstructs pulmonary
venous flow to the mitral valvar vestibule.
SCV
Oval fossa
ICV
Sup.
Fig. 9.6 This photograph in anatomical orientation shows the

Post. Ant.
enlarged orifice of the coronary sinus within the right atrium of a
Enlarged mouth of heart with a persistent left superior caval vein. This is not the same
coronary sinus Inf. heart as is shown in Figure 9.5. SCV, (right) superior caval vein; ICV,
inferior caval vein.
Azygos vein
Anomalous vein SCV
Left atrium
Morph. right
appendage
Fig. 9.7 This specimen, from a patient with hypoplasia of the left
Sup. heart, photographed in anatomical orientation, shows the course
of the so-called levoatrial cardinal vein. The pulmonary veins
Post. Ant. connect in normal fashion to the left atrium, but the anomalous
vein drains the atrial contents to the superior caval vein (SCV). Thus,
Pulmonary veins Inf. there are normal venous connections, but anomalous drainage
(arrows).
Sup.
BCV
Left Right
Inf.
Anomalous vein
Left atrium
Fig. 9.8 The reconstructed dataset prepared from a computed

tomographic angiogram shows a levoatrial cardinal vein extending
from the right lower pulmonary vein and taking a tortuous course
before draining into the brachiocephalic vein (BCV). The patient
Pulmonary veins
had coexisting mitral atresia and an intact atrial septum.
connection. There is a plethora of morphologically left atrium. Consequently, morphologically left atrium, there is a
possibilities for abnormal pulmonary if all the veins from one lung connect to a totally anomalous pulmonary venous
venous connections10. The arrangements site other than the left atrium, the connection. This, of necessity, implies a
should be described as specifically and as arrangement can be described as a bilateral totally anomalous connection.
unambiguously as possible (Figure 9.11). unilateral totally anomalous pulmonary Any combination can readily be
The pulmonary veins connect anomalously venous connection. If all the veins from described by treating each lung as an entity.
when they are not joined to the both lungs connect to sites other than the The unifying feature in all of these
Sup.
Post. Ant.
Inf.
Tricuspid
valve
Oval
fossa
Fig. 9.9 In this heart, there is atresia of the mouth of the

Atretic mouth of
coronary sinus coronary sinus. The coronary venous blood returns through a
vertical vein, as shown in Figure 9.10.
Vertical vein
Sup.
Fig. 9.10 The heart shown in Figure 9.9 is photographed from
behind to show the vertical vein that drains the coronary veins
Ant. Post. (arrows), themselves connected to the obstructed coronary sinus
(see Figure 9.9), to the left brachiocephalic vein, and thence to the
Coronary sinus superior caval vein. This situation is analogous to the levoatrial
Inf.
cardinal vein providing pulmonary venous egress in the setting of
an obstructed left atrium.
combinations is the connection of one or drainage be identified. Historically, this has be subdivided into supracardiac and
more pulmonary veins to the systemic been described as supradiaphragmatic or cardiac patterns. Supracardiac connection
venous system or to the right atrium. It is infradiaphragmatic (Figure 9.12). may be to a right or left superior caval vein,
equally important, therefore, that the site of Supradiaphragmatic drainage can further the brachiocephalic or innominate vein, or
Partially anomalous
unilateral drainage
Fig. 9.11 This cartoon, in anatomical

Totally anomalous orientation, shows how an anomalous
bilateral drainage connection of the pulmonary veins can be well
accounted for by describing the connection of
the veins from each lung as separate entities,
and then describing partially as opposed to
totally anomalous drainage on each side. The
site of drainage needs to be specified
Totally anomalous unilateral drainage
separately (see Figure 9.12).
Supracardiac connection
• to superior caval vein
• to azygos vein
Cardiac connection
• to LSCV & coronary sinus
• direct to right atrium
Infradiaphragmatic &
infracardiac connection Fig. 9.12 This cartoon, in anatomical orientation, shows the
• to portal venous system potential sites for an anomalous connection of the pulmonary
• to inferior caval vein
veins. LSCV, left superior caval vein.
even the azygos vein. Connection to the obstructed when the vertical vein crosses in beneath the heart, ascending in the right
brachiocephalic vein is most frequent. The front of the left bronchus, but obstruction paravertebral gutter and receiving the right
combination of drainage via a left-sided is more usually produced when the venous pulmonary veins, before draining to the
vertical vein, and thence via the channel is caught in a vice between the left superior caval vein via the azygos vein
brachiocephalic vein to the superior caval pulmonary artery and the left bronchus (Figure 9.16). More bizarre patterns of
vein (Figures 9.13, 9.14), produces the (Figure 9.15). When the route of drainage drainage can occur when the right-sided
typical ‘snowman’ configuration as seen on involves the azygos vein, the left veins cross to the left, join with the
chest radiography. The drainage can be pulmonary veins tend to cross vertically left-sided veins, and make their connection
SCV
Ascending vein
Ventricular mass
Fig. 9.13 This heart, photographed from the front in anatomical

orientation, has a totally anomalous pulmonary venous connection
to the superior caval vein (SCV). With this arrangement, the
heart forms the body of the ‘snowman’ seen in the chest
radiograph, with the anomalous vein forming the head of the
snowman as it courses to drain to the superior caval vein (arrow).
Sup.
Left Right
Inf.
Ascending vein
Pulmonary venous confluence Fig. 9.14 The reconstructed dataset prepared from a computed
tomographic angiogram shows a supracardiac totally anomalous
Ventricular mass pulmonary venous drainage as viewed from behind. The
brachiocephalic vein drains to the superior caval vein.
Sup. Left pulmonary artery
Right Left
Inf.
Fig. 9.15 In this specimen, from a patient with a totally

anomalous pulmonary venous connection, the mediastinum
is photographed from the front, the heart having been
reflected from its pericardial cradle. The draining channel from
Left bronchus the pulmonary venous confluence is obstructed in a
Pulmonary venous confluence
bronchopulmonary vice.
Morph. right appendage (left-sided)
LSCV
Fig. 9.16 This heart is from a patient with a mirror-imaged

arrangement of all the organs, including the heart. Note the
left-sided position of the morphologically (Morph.) right atrial
appendage. The heart has been reflected from its pericardial
Sup.
cradle, and the photograph taken from the front. The pulmonary
veins drain anomalously to the left-sided superior caval vein (LSCV),
Right Left with the collecting channel passing below the heart and
Pulmonary venous confluence ascending within the left-sided paravertebral gutter. The stars
Inf. show the right pulmonary vein, while the arrow shows the left
upper pulmonary vein.
to the azygos vein adjacent to the been seen only in the setting of isomerism of connection is usually total except when there
diaphragm11. the right atrial appendages (Figure 9.18). is a partially right-sided connection to the
Drainage directly to the right atrium is The pulmonary veins, even when they inferior caval vein. This latter arrangement
typically through the coronary sinus return to the heart, are always connected in is the so-called scimitar syndrome, a name
(Figure 9.17). In our experience, a totally anatomically anomalous fashion when both taken from its likeness to a Turkish sword
anomalous connection to the right atrium of the atrial appendages are of right when seen in the chest radiograph12. In the
other than through the coronary sinus has morphology. An infradiaphragmatic usual infradiaphragmatic connection, the
Sup. Left pulmonary veins
Ant. Post.
Inf.
Left atrium
Fig. 9.17 This specimen, viewed from the left side in anatomical
Mouth of coronary sinus Right pulmonary veins orientation, has an anomalous connection of all four pulmonary
veins to the coronary sinus, which drains to the right atrium.
Sup. Morphologically right appendage
Post. Ant.
Inf.
Fig. 9.18 In this heart, all four pulmonary veins (arrows) are
connected directly to the right atrium, the lowermost vein through
two tributaries. There was isomerism of the right atrial
Absence of coronary sinus
appendages. Note the absence of the coronary sinus.
common channel connecting both lungs subsequent to closure of the venous duct. connection can also drain to a mixed site. In
lies outside the pericardium posterior to the Occasionally, the channel breaks up into a the image shown in Figure 9.23, the upper
left atrium (Figure 9.19). A vertical vein series of branches that connect to the pulmonary veins drain in supracardiac
drains through the diaphragm as a single gastric veins (Figure 9.22). It is very rare to fashion to the superior caval vein, while the
channel to enter the portal vein or the find an infradiaphragmatic connection inferior pulmonary veins drain to the
venous duct (Figures 9.20, 9.21). The return directly to the inferior caval vein. The coronary sinus. Any combination should be
from the lungs becomes obstructed totally anomalous pulmonary venous anticipated.
Body of left atrium
Pulmonary venous
confluence
Sup.
Ant. Post. Fig. 9.19 This specimen, viewed from the left side in
anatomical orientation, has an anomalous connection of all
Inf. pulmonary veins to a descending channel (arrow) that passes
through the diaphragm to join the portal venous circulation.
Pulmonary venous
confluence
Sup.
Left Right Fig. 9.20 In this specimen, from a patient with a totally
Hepatic portal anomalous pulmonary venous connection, the mediastinum is
vein viewed from behind. The descending pulmonary venous channel
Inf.
passes through the diaphragm and empties into the hepatic
portal vein.
The salient anatomical features the anomalous connection, and the mouth of the superior caval vein, the
relating to surgical repair of anomalous proximity of the anomalous veins to the so-called sinus venosus defect, is
pulmonary connections include the left atrium. As pointed out in Chapter 7, always accompanied by an anomalous
type of abnormal connection, the site of an interatrial communication in the connection of the right pulmonary
Pulmonary veins
Vertical vein
Sup.
Left Right
Inf.

tomographic angiogram shows infracardiac and
infradiaphragmatic totally anomalous pulmonary venous
Hepatic portal vein drainage as viewed from behind. The descending vertical vein
drains to the hepatic portal venous system.
Confluence Sup.
Right Left
Inf.
Descending
vein
Fig. 9.22 In this specimen, from a patient with a totally

Leash of anomalous pulmonary venous connection, the heart has been
draining vessels reflected from its pericardial cradle. The descending channel passes
through the diaphragm and fragments into a leash of small veins
that join the gastric veins.
veins to the superior caval vein. The Other sites of connection, or potential venous obstruction. The appropriate
need to safeguard the sinus node and sites of anastomosis, often require landmarks must be borne in mind. Two
its blood supply has already been construction of an extensive atrial junction types of anomalous connection pose
emphasised. to guard postoperatively against pulmonary particular problems. The first is when the
Brachiocephalic vein Sup.
Left Right
Inf.
Left superior pulmonary vein

tomographic angiogram shows mixed totally anomalous
pulmonary venous drainage as viewed from behind. The left
superior pulmonary vein drains to the brachiocephalic vein, and
Left inferior and right
Coronary sinus pulmonary veins thence to the superior caval vein, while the left inferior and the
right pulmonary veins drain to the coronary sinus.
anomalous venous return is by way of the pulmonary venous trunk is further from aorta, but is anatomically independent of
coronary sinus (Figures 9.17, 9.23). We the posterior left atrial wall than might the hypoplasia. It can coexist with other
initially thought that the myocardium be expected (Figure 9.19). Because of this, lesions that diminish left-sided flow, but
between the coronary sinus and the left the anastomosis constructed between the coarctation can, and does, occur
atrium was common to both structures. We trunk and left atrium is vulnerable to independently. Tubular hypoplasia, when
now know that the sinus has its own obstruction, particularly at its lateral involving the isthmic segment of the aortic
myocardial wall, separate from that of the extreme. This may become evident only arch, can then be considered as part of a
left atrium13. It is possible, nonetheless, to when bypass is discontinued and the heart spectrum leading to atresia or interruption
incise both walls so as to unroof the sinus. fills with blood. of the aortic arch.
At the same time, the orifice of the coronary Much has been made of the role of
sinus can be made confluent with the atrial ductal tissue in the aetiology of coarctation.
septal defect, which is typically within the ANOMALIES OF THE AORTA Although there was a time when some
oval fossa. It is important to remove enough doubted its significance, the evidence
of the walls to ensure that the patch placed The congenital anomalies of the thoracic supporting its inclusion in the coarctation
across the orifice of the coronary sinus and aorta that are of interest to the surgeon shelf (Figure 9.24) is now unequivocal14–16.
oval fossa does not produce obstruction at include coarctation, the spectrum of partial The surgeon, therefore, needs to be aware
the site of the incised sinus septum. Ideally, to complete interruption of the aorta, and of the importance of removing all the
the incised walls of the atrium and coronary vascular rings and slings. ductal tissue during repair. The
sinus should also be repaired, because the significance of the duct itself devolves upon
incisions create a route to the inferior its patency. If the duct is patent, typically
Aortic coarctation
atrioventricular groove. Thus far, however, there is an associated congenital anomaly
we are unaware of any reports of Coarctation is a congenitally derived that promotes increased flow of blood
tamponade following such procedures. discrete shelf-like (Figure 9.24) or waist- through the pulmonary trunk, and diverts
The surgeon should be aware, nonetheless, like (Figure 9.25) lesion within the aorta, blood away from the proximal aorta. In
that a potential opening is created to the which causes obstruction to the flow of these circumstances, the associated
extracardiac spaces. When incising the blood. It is most often found adjacent to the anomaly tends to dominate the picture, and
coronary sinus, it is also important to avoid opening of the persistently patent arterial will determine the most appropriate
the atrioventricular node at the apex of the duct (Figure 9.26), or the arterial ligament surgical therapy. On the other hand,
triangle of Koch. if the duct has closed, but can occur coarctation with a closed duct is very likely
The second problematic type of proximally or more distally, and even in the to be an isolated lesion (Figure 9.25),
connection is the infradiaphragmatic abdominal aorta. It is usually accompanied except for the occasional association with
variant. The extrapericardial common by some degree of tubular hypoplasia of the a bifoliate aortic valve17.
Isthmal hypoplasia
Arterial duct
Coarctation shelf is
ductal tissue
Sup.
Ant. Post.
Fig. 9.24 This specimen, viewed in anatomical orientation, shows
Inf. the ductal tissue that forms the shelf lesion of aortic coarctation.
Descending aorta
Note the coexisting hypoplasia of the aortic isthmus.
Transverse aortic arch Left subclavian

artery
Sup.
Ant. Post. Fig. 9.25 The reconstructed dataset prepared from a computed
Descending aorta
tomographic angiogram shows a waist-like constriction (arrows)
Inf. between the aortic isthmus and the descending aorta. In this
patient, the arterial duct has closed.
The chief concerns of the surgeon relate aorta during repair. If the collateral arteries induce a cerebrovascular accident
to the specific anatomy of the coarctation. are less well developed (Figure 9.28), secondary to rupture of a berry aneurysm.
The nature of the collateral circulation is of however, clamping the aorta may have There may be distal aortic hypotension,
particular significance. With well- several deleterious consequences. These at pressures of less than 50 mmHg, which
developed collateral arteries (Figure 9.27), include strain on the left ventricle due to endangers the splanchnic and spinal
there is little danger of cross-clamping the proximal hypertension, which may also vascular beds. Irrespective of the nature
Sup. Left subclavian

artery
Ant. Post.
Inf. Aortic
isthmus

tomographic angiogram shows a coarctation lesion (arrow) in a
juxtaductal position with a persistently patent arterial duct. In this
Arterial duct patient there is considerable length to the aortic isthmus (compare
with Figure 9.25).
Left subclavian
Recurrent laryngeal nerve
artery
Coarctation at isthmus
Ant.
Inf. Sup.
Enlarged intercostal arteries

Fig. 9.27 This operative view, through a left thoracotomy,
Post. shows aortic coarctation with enlarged intercostal arteries, part
of a well-developed collateral circulation.
of the collateral arteries, the spinal occlusion of the intercostal vessels adjacent Other noteworthy features include the
circulation is best preserved by to the operative field seems to be a position of the thoracic duct (Figure 9.29),
interrupting none, or as few as possible, reasonable compromise in this difficult and the occasional presence of an anomalous
of the intercostal vessels. Temporary situation. artery (Figure 9.30), sometimes referred to
Arterial ligament Aortic arch

Ant.
Inf. Sup.
Post.
Coarcted segment
Fig. 9.28 In this operative view through a left thoracotomy, from

Normally sized intercostal artery Left subclavian artery a patient with aortic coarctation (compare with Figure 9.27), the
collateral circulation is not well developed.

Aorta
Ant.
Inf. Sup.
Fig. 9.29 This view through a left lateral thoracotomy shows the
Post. Thoracic duct thoracic duct as it passes from the area of coarctation behind the
left subclavian artery.
as Abbott’s artery. When present, this artery anomalous vessel that, if not properly as absence, atresia, or interruption of the
arises from the posteromedial aspect of the managed, can lead to substantial bleeding arch. Included in this group are those cases
proximal descending aorta. Whether it is an during the repair19. with a fibrous cord or bridge of tissue
enlarged bronchial artery, or persistence of (Figure 9.31), as well as those with a gap, or
the evanescent fifth arch, as initially absolute discontinuity, at some point in the
Interruption of the aortic arch
suggested by Hamilton and Abbott18, is arch (Figures 9.32, 9.33). Interruption can
irrelevant. What is important is that the Discontinuity of the aorta, as opposed to occur at one of three positions: at the
surgeon is aware of the existence of this coarctation, has been variously referred to isthmus, between the left subclavian and
Descending aorta Aortic isthmus Ant.
Inf. Sup.
Post.
Abbott’s artery
Fig. 9.30 As seen through a left thoracotomy, the proximal
descending aorta and distal arch have been rotated anteriorly. The
Intercostal arteries
large collateral vessel referred to as Abbott’s artery is seen arising
proximally to the area of coarctation.
Left subclavian artery Atretic cord
Aorta
Sup.
Right Left Fig. 9.31 This specimen, photographed in anatomical

orientation, has atresia of the isthmus, with a thin fibrous cord
Duct to descending running from the site of effective interruption at the left subclavian
Inf. aorta artery to the distal descending aorta, which is fed through the
arterial duct.
left common carotid arteries, and between rarely, a right arch can be affected cardiovascular malformations are of
the left common carotid and similarly20. As patients with discontinuity critical importance, for they affect the
brachiocephalic arteries (Figure 9.34). of the aorta are unlikely to survive without operative outcome as much as does an
These lesions typically are found in the surgical treatment, they are a particular interrupted arch itself. Almost always,
setting of a unilateral left aortic arch but, challenge to the clinician. The associated there is a patent connection to the distal
RSCA
LCCA
LSCA
RCCA
Duct
Pulmonary
Sup. Aorta trunk
Fig. 9.32 In this specimen, shown in anatomical orientation as
Right Left photographed from the front, the aortic arch is interrupted (star) at
the isthmus, with a retro-oesophageal origin of the right subclavian
artery (arrow). The descending aorta is fed from the pulmonary
Inf. trunk via the arterial duct. LCCA and RCCA, left and right common
carotid arteries; LSCA and RSCA, left and right subclavian arteries.
Sup. Ascending aorta

Pulmonary trunk
Left Right
Inf.
Arterial duct
BCA
LCCA
Descending Fig. 9.33 The reconstructed dataset prepared from a computed

aorta tomographic angiogram shows interruption of the aortic arch
(white dotted double-headed arrow) in between the left common
carotid artery (LCCA) and the left subclavian artery (LSCA). The
LSCA
hypoplastic ascending aorta also supplies the brachiocephalic
artery (BCA).
aorta through the duct (Figure 9.33). aortopulmonary window is found. The resulting in subaortic stenosis
A proximal septal defect is also the rule. ventricular septal defect typically is (Figure 9.35). Any type of defect,
This is most frequently a ventricular associated with posterior and leftward nonetheless, can be found. Abnormal
septal defect but, occasionally, an displacement of the outlet septum, ventriculoarterial connections are not
RCCA RCCA
RCCA
LCCA LCCA
LSCA LCCA
LSCA
LSCA
Fig. 9.34 This cartoon, in anatomical

orientation, shows the different sites of
interruption of the aortic arch. The ascending
aorta, and the vessels it feeds, are shown in red.
The pulmonary trunk, and the vessels fed via
the arterial duct, are shown in blue. The right
subclavian artery usually takes origin from
the brachiocephalic trunk, but can have a
Interruption Interruption Interruption retro-oesophageal origin from the descending
between between at isthmus aorta (see Figure 9.32). LCCA and RCCA, left
carotid arteries LCCA and LSCA (‘Type A’) and right common carotid arteries; LSCA, left
(‘Type C’) (‘Type B’)
subclavian artery.
VSD with muscular post-inf. rim
Fig. 9.35 This view of a specimen sectioned to replicate the

parasternal echocardiographic cut, in the setting of interruption of
the aortic arch, seen in anatomical orientation, shows posterior
Deviated outlet septum Narrowed subaortic outlet deviation of the outlet septum into the left ventricle, with
obstruction of the subaortic outflow tract. The ventricular septal
defect (VSD) has a muscular posteroinferior (post-inf.) rim.
unusual, and present their own particular emphasises the importance of defining development of the aortic arches is a
problems for operative reconstruction21. clearly the nature and effect of associated genuine aid to understanding the
A less lethal but fairly frequently abnormalities. Successful anatomical morphology of these anomalies. Initially,
associated arterial abnormality is the correction is as dependent on appropriate the arterial system is bilaterally
aberrant origin of a retro-oesophageal right management of these accompanying lesions symmetrical, with five sets of arches
subclavian artery from the distal aorta as it is on establishing aortic continuity. encircling the gut, and on occasion the
(Figure 9.32). Because the arch is remnants of a fifth set being evident for
interrupted, symptoms of a vascular ring short periods (Figure 9.36). Of these, only
Vascular rings
are unlikely, unless a ring is created while the third, fourth, and sixth arches remain
reconstructing the arch. Conversely, if the Aortic rings are malformations associated recognisable in the postnatal heart.
aberrant artery is brought forward, it can be with abnormal regression of part of a Remnants of the third arch supply the
useful in repairing the aorta. This double aortic arch. Knowledge of the head. The left fourth arch becomes part of
1–3 1–3
4 4 4 4
6 6 Ao PT
Ao PT
Arterial duct
Left aortic arch
Fig. 9.36 This cartoon, in anatomical orientation, shows the

patterns of formation of the aortic arches believed to exist during Fig. 9.37 This cartoon shows how the definitive system of aortic
embryological development. The numbers refer to the arteries arches is derived from the embryological primordiums depicted in
supplying the pharyngeal arches of the developing embryo. The Figure 9.36. Note that the subclavian arteries (arrows), derived from
transient fifth aortic arches are shown by the red dotted lines. The the seventh segmental arteries, have migrated proximal to the
arrows show the seventh segmental arteries, which will become the origin of the ducts, and that the right aortic arch has atrophied
subclavian arteries. Initially they are distal to the insertion of the sixth (yellow and red dotted channels). The left sixth arch persists as the
aortic arch. Ao, aorta; PT, pulmonary trunk. arterial duct.
R
L
Oesophagus
SCA
SCA
Trachea
CCA CCA
Duct
Duct
Fig. 9.38 The cartoon shows the perfect hypothetical double arch, with right (R) and left
(L) components, and with a subclavian artery (SCA), common carotid artery (CCA), and arterial
Ao
PT duct arising from each arch. The descending aorta (arrow) is in the midline, or in a ‘neutral’
position. Ao, aorta; PT, pulmonary trunk.
the definite aortic arch. The left sixth arch which then forms a ring around the trachea each fourth arch in the hypothetical
becomes the arterial duct (Figure 9.37). and the oesophagus. The so-called system, while an arterial duct connects each
The subclavian arteries, which are the hypothetical double aortic arch23 is a arch to its companion pulmonary artery
seventh cervical intersegmental arteries, concept that explains malformations (Figure 9.38). Persistent patency of the
come to take origin from the fourth arch encircling the tracheo-oesophageal pedicle entire double arch obviously will cause
between the third and sixth arches when on the basis of incomplete regression. The problems (Figures 9.39, 9.40), while
the heart migrates inferiorly22. Thus, hypothetical pattern comprises a midline inappropriate interruption or atresia at any
normally during early development, most anterior aorta, continued anteriorly on each part of the ring can produce multiple
of the right-sided structures regress, side by the fourth arches to bilateral variations of abnormal anatomy24. Such
leaving a left arch and a left-sided aorta posterior aortic segments, which join to situations are illustrated in Figures 9.41
(Figure 9.37). It is failure of this normal form a midline descending aorta. Common and 9.42, which show a left aortic arch with
regression that leaves the double arch, carotid and subclavian arteries arise from an aberrant right subclavian artery. Stewart
Tracheo-oesophageal
pedicle
Right-sided arch
Left-sided arch
RSCA
RCCA
LCCA
LSCA
Post.
Fig. 9.39 In this heart, there is persistence of both the right- and
left-sided aortic arches, with the origin of the common carotid (RCCA,
Left-sided Right Left
LCCA) and subclavian (RSCA, LSCA) arteries in symmetrical fashion
arterial
from the superior surface of the arches. There is a ligamentous
ligament
Ant. left-sided arterial duct. The arrangement produces obvious
compression of the tracheo-oesophageal pedicle.
Tracheo-oesophageal
pedicle
RCCA
LCCA
Ant.
RSCA
Left Right Fig. 9.40 The reconstructed dataset shows persistence of both the
right- and left-sided aortic arches, again with the origin of the
LSCA
Post. common carotid (RCCA, LCCA) and subclavian (RSCA, LSCA) arteries
in symmetrical fashion from the superior surface of the arches.
and colleagues23 provided an exhaustive list analysis rather than alphanumeric and may not necessarily cause compression
of the potential malformations, which they notation24. It should be noted that, as in the of the trachea or oesophagus. In addition,
catalogued in complex alphanumeric latter example of focal atresia, the atretic the size of the component parts of the ring
fashion. Our preference is for descriptive segment may not persist as a fibrous cord, is at least as important in producing
Diverticulum
of Kommerell
RSCA
Fig. 9.41 This cartoon shows how an aberrant origin of the right
subclavian artery (RSCA) is explained on the basis of the
hypothetical double arch. The proximal portion of the right arch is
known as the diverticulum of Kommerell, and the initial segment
of the right arch is interrupted between the right common
carotid and right subclavian arteries (star). The duct, shown in the
left-sided position (black curved line), has become ligamentous in
this example.
Ant.
RCCA Left Right
Post.
Trachea
LCCA
Fig. 9.42 The computed tomographic dataset shows the origin

of a retro-oesophageal right subclavian artery (RSCA) from a
left-sided aortic arch. The trachea, but not the oesophagus, is seen
in the reconstruction. The potentially double arch is divided
between the right subclavian and right common carotid (RCCA)
LSCA
arteries. The left common carotid (LCCA) and left subclavian (LSCA)
RSCA
arteries arise from the left-sided aortic arch. The arterial duct is not
seen in this reconstruction, but was left-sided. Therefore, this
patient does not have a complete vascular ring.
symptoms as the particular anatomical considerable tracheo-oesophageal It may then connect to the descending aorta
arrangement. This accounts for the high compression is not obvious until the duct is on either the right or left side of the
incidence of symptoms associated with a divided, and its ends are allowed to spring vertebral column. Such a right-sided aortic
double aortic arch. When only a fibrous apart (Figure 9.45). arch is considered abnormal with the usual
cord is found, compression is not always A right-sided aortic arch can exist aside atrial arrangement, but is normal in
apparent. Figures 9.43 and 9.44 show a from its association with vascular rings. patients with the mirror-imaged
duct taking origin from the left subclavian The arch is right-sided when it crosses over arrangement. In either case, it may or may
artery, which arises from a right arch. The the main stem of the right-sided bronchus. not produce clinical problems. Its chief
Ant.
Left Right
Post.
Aorta
Pulmonary Trachea
trunk
LCCA
Fig. 9.43 This computed tomographic dataset is the mirror-image

of the arrangement shown in Figure 9.42, with a retro-oesophageal
origin of the left subclavian artery (LSCA) from a right-sided aortic
Diverticulum arch, with the dorsal part of the right arch persisting as the
of Kommerell diverticulum of Kommerell. In this instance, however, the left
subclavian artery continues as the left-sided arterial duct, creating a
Arterial duct vascular ring. The trachea, but not the oesophagus, is seen in the
Aberrant LSCA reconstruction. The potentially double arch is divided between the
left subclavian and left common carotid (LCCA) arteries.
Double ligation of duct Vagus nerve
Aberrant LSCA
Ant. Fig. 9.44 This operative view, taken through a left thoracotomy,
shows, as already seen in Figure 9.43, an arterial duct that arises
from an aberrant left subclavian artery (LSCA), itself coursing in a
Inf. Sup.
retro-oesophageal position from a right aortic arch. The
arrangement produces a vascular ring, as shown in Figure 9.43. The
Recurrent laryngeal nerve Post. duct has been doubly ligated prior to its division. Note the size and
location of the vagus nerve and its recurrent laryngeal branch.
surgical significance lies in its association Patients with a right aortic arch usually construction of a subclavian-to-pulmonary
with about one-quarter of cases of tetralogy have a mirror-imaged arrangement of the arterial shunt. With mirror-imaged
of Fallot, and approximately half of all brachiocephalic arteries. This fact can be branching, the first branch of the aorta
patients with common arterial trunks. significant when contemplating beyond the coronary arteries is the
Retracted ends of divided duct
Oesophagus
Ant.
Inf. Sup.
Fig. 9.45 After division of the duct in the patient shown in
Figure 9.44, the ends of the arch have sprung apart to free the
Post. oesophagus. The appearance subsequent to the surgical procedure
shows the initial effect of the vascular ring.
brachiocephalic trunk, which subsequently left common carotid and left subclavian Less frequently, the anomalous
gives rise to the left subclavian and arteries, with the isolated right subclavian pulmonary artery, usually the right, can
common carotid arteries. Under such artery arising via a right-sided arterial duct arise directly from the ascending aorta
circumstances, the surgeon may elect to from the right pulmonary artery. (Figure 9.49). This is sometimes termed a
perform a left-sided anastomosis so as to hemitruncus, but we do not recommend
avoid kinking of the right subclavian artery use of this term, because almost always there
as it is turned down into the mediastinum. PULMONARY ARTERIAL are two normally formed arterial valves.
The duct, however, usually remains a ANOMALIES Rarely, the right pulmonary artery may take
left-sided structure, connecting the By far the most common malformation of an unusually high origin from the right side
brachiocephalic trunk to the left pulmonary the pulmonary arteries, excluding atresia or of the ascending portion of a common arterial
artery. When arising from a retro- stenosis, is for either the right or left trunk. Even in this setting, the descriptive
oesophageal left subclavian artery, the pulmonary artery to have an aortic origin. title of common trunk with anomalous origin
presence of the left-sided duct will produce Most frequently the anomalous artery arises of the right pulmonary artery is preferable to
a vascular ring (Figures 9.42, 9.43, 9.46)24. via the arterial duct. Although seen in the hemitruncus.
The hypothetical double arch, as presence of pulmonary atresia, usually When the anomalously connected
proposed by Stewart and his colleagues23, with the other lung supplied by major pulmonary artery arises directly from the
also accounts for isolation of the systemic-to-pulmonary collateral arteries aorta, surgical repair consists simply of
brachiocephalic arteries. In these (Figure 9.48), it can be found with the other detachment from the aorta and
anomalies, which can involve either the pulmonary artery connected to the patent reattachment to the pulmonary trunk. This
brachiocephalic artery itself, or the pulmonary trunk. In this arrangement, the is somewhat more difficult in the presence
common carotid or subclavian arteries, and duct will often close with time. There will of a common trunk, but the same basic
which can be associated with either the then be apparent unilateral absence of that principle is followed for repair. When the
usual left-sided aortic arch or a right aortic pulmonary artery that was initially fed anomalous pulmonary artery is fed through
arch, the isolated artery arises from a through the duct. This is common in a duct, or connected by a ligament, it will
pulmonary artery via a persistently patent tetralogy of Fallot or a double-outlet right arise from the aortic arch or a
arterial duct. In the example shown in ventricle, but about half of the patients thus brachiocephalic artery. Care must be taken
Figure 9.47, there is persistence of both the afflicted have otherwise normal hearts. In during surgical reconstruction to ensure
right-sided and left-sided arterial ducts. our experience, it is rare to find true absence that ductal tissue is not incorporated with
The aortic arch is interrupted between the of the hilar pulmonary artery. the anastomosis, as it may subsequently
Trachea Oesophagus
RSCA
LSCA
Right arch
RCCA
Arterial
duct
LCCA Fig. 9.46 The specimen shows a potential double aortic arch
that is interrupted between the left common carotid artery (LCCA)
Sup. and the left subclavian artery (LSCA), the latter arising in a
retro-oesophageal fashion from the right-sided aortic arch. Despite
the incomplete nature of the double arch, the presence of the
Right Left arterial duct, running from the left subclavian artery to the left
pulmonary artery, produces a vascular ring around the tracheo-
Inf. Pulmonary trunk oesophageal pedicle. The right common carotid (RCCA) and right
subclavian (RSCA) arteries arise from the right aortic arch.
Right pulmonary RSCA

artery
LSCA
LCCA
RCCA
Pulmonary
trunk
Fig. 9.47 In this heart, there is interruption of the aortic arch

between the left common carotid (LCCA) and left subclavian (LSCA)
Sup.
arteries. The ascending aorta, however, gives rise to the two
common carotid arteries only, the right subclavian artery (RSCA)
Right Left being isolated, and arising from the right pulmonary artery via
Ascending the right-sided arterial duct (white star). The descending aorta
aorta Inf. and the left subclavian artery are fed by the left-sided arterial duct
(red star).
constrict and produce stenosis at the site of of its being disproportionately small and left pulmonary artery originates within
repair. In cases with unilateral absence of a having to receive the entire right the right chest (Figures 9.50, 9.51),
pulmonary artery, it is usual to find ventricular output25. and passes between the trachea and
pulmonary hypertension and pulmonary A much more rare anomaly is when oesophagus to the left pulmonary
hypertensive vascular disease in the the left pulmonary artery arises from the hilum, creating the vascular sling
normally connected lung, probably because right pulmonary artery. The abnormal (Figs. 9.52)26. This may result in repeated
Arterial ligament Sup.
Left Right
Inf.
Fig. 9.48 In this specimen, photographed from behind, the left

pulmonary artery was initially fed from a left-sided duct, which has
Collateral arteries to right lung become ligamentous. The right lung is fed through systemic-to-
pulmonary collateral arteries.

Pulmonary trunk
Aorta Left
Fig. 9.49 In this operative view, taken through a median
Sup. Inf. sternotomy, the right pulmonary artery is seen arising directly from
the aorta. This should not be described as hemitruncus, as there are
Right pulmonary artery Right separate aortic and pulmonary pathways, the pulmonary trunk
continuing as the left pulmonary artery.
respiratory problems, requiring division from the aorta or brachiocephalic been reported. The reason for this is
and transplantation of the offending artery, but an abnormal right pulmonary not clear.
artery. As discussed earlier, the right vessel arising from the left pulmonary In the rare occurrence of agenesis of
pulmonary artery may arise anomalously artery has not, to our knowledge, the right lung, displacement of the heart
Descending aorta Arterial duct Trachea
Right pulmonary
artery
Sup.
Left Right Fig. 9.50 This specimen is photographed from behind. The left
pulmonary artery arises from the right pulmonary artery. It then
Inf. Left pulmonary artery extends between the trachea and the oesophagus, which has been
removed, producing the pulmonary arterial sling (see Figure 9.52).

Right pulmonary
artery
Aorta
Post.
Right Left
Trachea
Fig. 9.51 This computed tomographic dataset shows the origin of
the left pulmonary artery from the right pulmonary artery. It
Ant. extends between the trachea and the oesophagus, producing the
pulmonary arterial sling (see Figure 9.52).
into the right chest may create a ring-like bronchus against the spinal column.
disposition that can obstruct the left Division of the ligament, and grafting PERSISTENCE OF THE ARTERIAL
bronchus. The displaced heart, pulling of the aorta, may be necessary to allow DUCT AND AORTOPULMONARY
on the left pulmonary artery, draws the the pulmonary trunk and aorta to WINDOW
ligament and descending aorta across spring apart and unroof the constricting Persistence of the arterial duct
the left bronchus, compressing the circle27–29. (Figure 9.53) occupies a special place in
Right
pulmonary
artery
Oe
Left
pulmonary
artery
T
Fig. 9.52 The cartoon shows the arrangement known as the

pulmonary arterial sling, illustrated in Figures 9.50 and 9.51. Oe,
oesophagus; T, trachea.
Sup.
Ant. Post.
Inf.
Aorta
Pulmonary
trunk
Fig. 9.53 The reconstructed computed tomographic

Left pulmonary artery angiogram shows a persistently patent arterial duct (white
double-headed arrow).
the study of cardiovascular disease, as it safely by techniques of interventional always predictable, and the operative
was the first congenital malformation to catheterisation31,32, its interruption results uniformly excellent.
be cured by operative intervention30. remains a paradigm of the best of Because the arterial system develops
Although now the duct can be closed surgical science. The anatomy is almost with bilateral symmetry, a persistent duct
Patent arterial duct
Pulmonary trunk
Left Aorta
Sup. Inf.
Right shows the origin of a persistently patent arterial duct from the
distal extent of the pulmonary trunk.
may be either right- or left-sided, although confused with the aortic isthmus, attached by another more fibrous fold to
the latter is overwhelmingly more particularly in the infant, and it is possible the bronchus. It is this firm fibrous fold that
common. Because the duct can persist on to identify the left pulmonary artery as a prevents easy circumscription of the duct
either side, or bilaterally, then as discussed patent duct. Even under the best with a right-angled clamp. To minimise the
earlier, it may be important as part of a conditions, these other structures may be risk of tearing the wall of the duct, this
vascular ring, or in providing the origin of mistakenly ligated in place of the duct. The tissue must be divided by sharp dissection.
an isolated brachiocephalic artery caveats of this procedure have been This can be done through a superior
(Figure 9.47). Persistent patency may also elegantly reviewed33. Approached laterally, approach over the aortic end of the duct, or
play an important physiological role when the best anatomical guide to the duct is the by freeing the aorta and retracting it
it accompanies other complex congenital vagus nerve and its recurrent laryngeal medially. In the latter instance, small but
cardiovascular anomalies, such as branch. The vagus nerve passes along the potentially troublesome bronchial vessels
interruption of the aortic arch, aortic or subclavian artery, crossing over the aortic may be encountered, arising from the
pulmonary valvar atresia, or discordant arch before heading in a posterior direction posterior wall of the aorta. Another
ventriculoarterial connections. In this to disappear behind the hilum. Just at the anatomical note of caution involves the
section, we confine our discussion to the level of the duct, it gives off the recurrent thoracic duct and its tributaries in the area
primary congenital condition of an isolated nerve (Figure 9.55), which then curves of the origin of the subclavian artery
left-sided persistently patent arterial duct. beneath the inferomedial wall of the duct (Figure 9.29). Division of any of these
Although it is possible to approach the before ascending along the posteromedial major lymph vessels is liable to lead to
patent duct anteriorly, and sometimes aspect of the aorta into the groove between chylothorax and its attendant difficulties.
necessary to use this route (Figure 9.54), the trachea and oesophagus. Should the lymphatic trunks be
the normal operative approach is through a Access to the duct may be achieved by inadvertently divided, they must be ligated
left lateral thoracotomy (Figure 9.55). The incising the mediastinal parietal pleura, to avoid chylothorax. The duct in an infant
duct arises from the posterosuperior aspect either between the phrenic and vagus or small child can measure from one to 15
of the junction of the pulmonary trunk and nerves, or more posteriorly over the aorta millimetres in length and diameter. Rarely,
left pulmonary artery. It courses itself. With either approach, the recurrent it can be aneurysmally dilated
posteriorly and slightly leftwards to join the nerve must be visualised to guard against (Figure 9.56)34. These may be aneurysms
junction of the aortic arch and descending direct trauma or injury by traction. The of a truly patent duct, or may simply be a
aorta just distal to and opposite the origin of fold of pericardium extending over the dilated ductal diverticulum with a closed
the left subclavian artery. Its pulmonary pulmonary end of the duct may be lifted pulmonary end. In general, the short and
end is covered by a fold of pericardium, and away by sharp dissection. The fat duct should be cross-clamped, divided,
its aortic end by parietal pleura. It may be posteromedial wall of the duct is firmly and oversewn to minimise the chances of
Ant. Recurrent laryngeal nerve Pericardial reflection
Inf. Sup.
Post.
Vagus nerve
Fig. 9.55 In this operative view, a persistently patent arterial duct

is seen through a left thoracotomy (compare with Figure 9.54).
Patent arterial duct Thoracic aorta Note the location of the vagus nerve and its recurrent laryngeal
branch.
Aneurysmal duct
Ascending aorta
Descending aorta
Sup.
Pulmonary trunk Right Left

Fig. 9.56 The heart is photographed in anatomical orientation
Inf. from the front. The arterial duct is patent, elongated, and
aneurysmal.
incomplete ligation or tearing of the vessel The clinical presentation of an ventriculoarterial connection. Where, in a
wall. For the longer thin duct, a triple aortopulmonary window is often similar to common trunk, there is a solitary arterial
ligation technique has proved to be safe and that of a common arterial trunk. The valve, an aortopulmonary window is always
effective35. anatomical distinction lies in the mode of associated with separate aortic and
Aortopulmonary Descending
window aorta
Aortic
valve
Sup.
Right Left
Fig. 9.57 This specimen, shown in anatomical orientation, the
aorta having been opened, shows an aortopulmonary window.
Inf. Pulmonary trunk Note the presence of the separate arterial trunks and valves
proximal to the defect (see also Figure 9.46).
Right pulmonary artery Duct
Left
pulmonary
artery
Aorta
Fig. 9.58 This specimen has an aortopulmonary window in the

Sup.
setting of the origin of the right pulmonary artery from the aorta
and interruption of the aortic arch. The heart is sectioned to
Post. Ant.
replicate the left anterior oblique subcostal echocardiographic
Pulmonary valve section. In this heart, the window (open double-headed arrow) has
Inf. some length. Note also the separate walls of the arterial trunks
proximal to the window (closed arrow).
pulmonary valves (Figure 9.57). Although The defect itself is located usually in the from the aorta (Figure 9.58). This means
the ventricular septum is usually intact, this right lateral wall of the pulmonary trunk that its opening into the left side of the
defect frequently is associated with anterior and opposite to the origin of the ascending aorta is just distal to the
additional congenital cardiovascular right pulmonary artery, which frequently sinutubular junction. The window can also
anomalies36. overrides the window, or arises directly be associated with the origin of one of the
coronary arteries from the pulmonary discrepancy between presumed anatomical markedly delayed, some cases not
trunk. The defect itself can take the form of and revealed clinical significance, it now presenting until childhood or even
a well-demarcated short tubular channel, or seems preferable to account for these adolescence. The clinical problems are
present as an extensive fenestration. malformations on a descriptive basis. produced by the extent of ischaemia of the
Surgical repair is best accomplished Within such a descriptive categorisation, left ventricular myocardium, which can be
through an incision in the ascending aorta the anomalies can be grouped into those extreme. It is important to reattach the
using standard cardiopulmonary bypass with an anomalous origin of the coronary artery to the aorta as soon as the diagnosis is
techniques. As a general rule, closed arteries from the arterial roots, those with made. In most instances, the artery arises
methods are not advisable, not even for the an anomalous course of the epicardial from the left sinus of the pulmonary trunk,
more tubular defects. When the window coronary arteries, those with anomalous to the right-hand side of the observer
supplies a vital part of the circulation, it communications between the coronary standing, figuratively speaking, in the
cannot be closed without providing an arteries and other structures within the non-adjacent sinus39. It is relatively easy to
alternate source of blood for the dependent heart, or combinations of such findings. transfer the arterial origin, together with a
segment of the circulation. button of pulmonary sinus, back to the
left-hand adjacent sinus of the aorta. It is
Anomalous origin of the
rare now to create an aortopulmonary
ANOMALIES OF THE CORONARY coronary arteries
tunnel in order to reconnect the artery to the
ARTERIES The coronary arteries can arise anomalously aorta40. Either of these procedures,
Congenital malformations of the coronary either from the pulmonary trunk or, rarely, nonetheless, is usually preferable to simple
arteries in otherwise normally structured from the right or left pulmonary artery. ligation of the coronary artery. The pattern
hearts have, traditionally, been categorised They can also take an anomalous origin that may create problems in transfer is when
as major or minor. This approach was from the aorta itself. It is the anomalous the artery arises from a branch of the
rooted in the belief that the so-called major origin from the pulmonary trunk that is of pulmonary trunk rather than from the trunk
anomalies were those that produced most clinical significance. Often described itself. Other patterns, such as the anomalous
symptomatology, whereas the minor as the Bland–White–Garland syndrome, origin of the right coronary artery from
lesions were thought to be of no clinical the anomalous origin of the left coronary the pulmonary trunk, do not produce the
relevance37. The potential danger of such a artery from the pulmonary trunk same degree of symptomatology, and often
classification became evident when it was (Figures 9.59–9.61) usually presents in do not come to the attention of the surgeon.
realised that some lesions categorised as infancy with ischaemia of the left ventricle If encountered, an anomalous right
minor within this concept could be a cause (Figure 9.62). If there is a well-developed coronary artery can be transferred back to
of sudden cardiac death38. Because of this collateral circulation, presentation can be the aortic root readily.
Left coronary artery Pulmonary trunk
Ant.
Inf. Sup.
Fig. 9.59 This view through a left lateral thoracotomy shows a silk
Post. Left atrial appendage ligature encircling a left coronary artery that is arising from the
pulmonary trunk.
Sup.
Ant. Post.
Inf. Pulmonary trunk
Circumflex artery

orientation, the main stem of the left coronary artery takes origin
from the pulmonary trunk, arising from one of the sinuses adjacent
Anterior interventricular artery to the aorta. It then divides into the circumflex and anterior
interventricular arteries.
Left coronary artery Circumflex artery
Aorta
Pulmonary
trunk
Post.
Right Left Fig. 9.61 The reconstructed computed tomographic angiogram

shows an anomalous origin of the left coronary artery from the
Anterior interventricular artery pulmonary trunk. Note the tortuous collateral circulation (arrow)
Ant.
from the right coronary artery.
We have already discussed the arteries usually arise from the two aortic facing sinus, while the right coronary artery
significance of an anomalous origin of one sinuses that are adjacent to the pulmonary arises from the right-hand facing sinus.
coronary artery from the aorta itself in trunk. In the normal heart, the left Accessory orifices supplying either the
Chapter 4. As we showed, the coronary coronary artery arises from the left-hand infundibular artery, the artery to the sinus
Dilated left ventricle Sup.
Ant. Post.
Inf.
Fig. 9.62 In this anatomical specimen, photographed in

anatomical orientation, the left ventricle is dilated secondary to an
Ischaemic myocardium anomalous origin of the left coronary artery from the pulmonary
trunk, while the ventricular myocardium is obviously ischaemic.
node, or an artery supplying the walls of the guarding the two aortic coronary sinuses. artery can then course between the aortic
arterial trunks, are by no means This is described as an intramural root and the subpulmonary infundibulum
uncommon. These accessory branches arrangement (Figure 9.63). Surgical to reach the right atrioventricular groove
should not be interpreted as representing unroofing is not difficult, but whether such (Figure 9.65). Yet another pattern is for
anomalies. The circumflex and anterior relief is always necessary has still to be the right coronary artery to take its normal
descending branches of the left coronary determined. origin from the right-hand facing sinus,
artery can also rarely take separate origins The finding of a single coronary artery but then to continue beyond the crux as
from the left-hand facing sinus in an also represents an anomalous origin from the circumflex artery, and to terminate at
otherwise normal heart. Usually the the aortic root. This can be found in two the obtuse margin as the anterior
arteries arise within the coronary aortic patterns. In the first, all three coronary interventricular artery (see Chapter 4).
sinuses, but origin at the sinutubular arteries arise from the same aortic sinus, This pattern probably has no clinical
junction, or just above it, should not be with one, two, or three orifices within the significance. In addition to an anomalous
considered abnormal. High origin, sinus. Usually it is the right-hand sinus origin, congenital malformations can also
however, or an oblique course of a coronary that gives origin to the coronary arteries. afflict normally attached arteries,
artery through the wall, particularly when There is then an associated anomalous producing the effect of an anomalous
crossing the peripheral end of a zone of epicardial course of the branches of the left origin. Particularly important in this
apposition between the valvar leaflets, coronary artery as they reach the respect is congenital atresia of the main
should be considered anomalous, as should anticipated locations. As discussed in stem of the left coronary artery
the origin of either coronary artery from an Chapter 4, this can involve the anterior (Figure 9.66). This can be another
inappropriate sinus or, as occurs very interventricular branch tracking between substrate for sudden death in an adolescent
rarely, from the non-facing aortic sinus41. the aorta and the pulmonary trunk, with or young adult43. Unfortunately, this
The origin from an inappropriate sinus is of the potential for its constriction. anomaly is unlikely to be diagnosed prior
greatest importance, as this is now well Alternatively, the main stem of the left to the event, when it could readily be
established to be a harbinger of sudden coronary artery can run across the treated by surgical manoeuvres.
cardiac death42. As we showed in subpulmonary infundibulum, tracking
Chapter 4, either coronary artery can arise towards the left atrial appendage before
Anomalous epicardial course
from an inappropriate sinus. The artery dividing into the circumflex and anterior
of the coronary arteries
then courses between the arterial trunks, interventricular arteries (Figure 9.64). The
typically crossing the peripheral end of the solitary coronary artery can also arise from Although an anomalous epicardial course
zone of apposition between the leaflets the left aortic sinus, and the right coronary can be found in otherwise normally
Sup.
Fig. 9.63 The tubular aorta has been removed from the aortic
Left Right root, showing the origin of the right coronary artery from the left
coronary aortic sinus, with intramural coursing across the
Inf. Right aortic sinus peripheral attachment of the valvar leaflets at the sinutubular
junction (arrow).
Solitary coronary artery Left main stem

Left sternotomy, shows a heart in which all the coronary arteries arise
from a solitary coronary artery that takes origin from the right-
Sup. Inf. hand adjacent aortic sinus. The main stem, which divides beneath
the left appendage to become the circumflex and anterior
Right coronary artery Right interventricular arteries, crosses the origin of the pulmonary trunk
from the right ventricle.
structured hearts, it is seen more chance observations at autopsy, such as passage between the aorta and the
frequently in hearts that are themselves the origin of the circumflex coronary pulmonary trunk as discussed in
malformed, such as those with discordant artery from the right coronary artery, with Chapter 4. In this respect, muscular
ventriculoarterial connections or a passage through the transverse sinus to bridging of the epicardial coronary
common arterial trunk. The clinical reach the left atrioventricular groove arteries (Figure 9.68) may also be of
significance of these anomalies has yet to (Figure 9.67). Others may be of relevance, although the significance of this
be fully determined. Some are found as significance for sudden death, such as the finding has still to be established.
Anterior interventricular artery Right coronary artery
Infundibulum
Aorta
Ant. Fig. 9.65 This computed tomographic dataset shows the origin of
the right coronary artery from the anterior interventricular artery,
Left Right with subsequent coursing between the subpulmonary
infundibulum and the aortic root. There was a solitary coronary
Circumflex artery Post. artery arising from the left coronary aortic sinus. Note the
narrowed profile of the right coronary artery.
Non-coronary aortic sinus Atretic left coronary artery
Sup.
Fig. 9.66 This view of the opened aortic root,
photographed in anatomical orientation,
Right Left
shows atresia of the main stem of the left
coronary artery. Note the enlarged orifice of
Enlarged right coronary artery Inf. the right coronary artery. The specimen came
from an adult who died suddenly.
Anomalous communications ventricular septum, notably in pulmonary coronary artery is most frequently
of the coronary arteries atresia with an intact ventricular septum involved, and it may connect to the right
(see Chapter 7, page 235 and Figure 7.157). atrium (Figures 9.69, 9.70), the superior
Fistulous communications between the
Such communications can also be found caval vein, the coronary sinus, the right
coronary arteries and the ventricles are seen
in otherwise normally structured hearts, ventricle, the pulmonary trunk, a
most frequently when atresia of an outflow
and the anomalous artery can be joined to pulmonary vein, or the left ventricle.
tract is seen in the setting of an intact
any other cardiac structure. The right The fistulas can also take origin from the
Retro-aortic
circumflex
artery
Aorta
Right
coronary
artery
Sup.
Post. Ant.
Fig. 9.67 This infant heart is photographed in anatomical
Artery to
orientation. The circumflex coronary artery arises from the right
sinus node Inf.
coronary artery, and passes through the transverse sinus, behind
the aorta, to reach the left atrioventricular groove.
Myocardial
bridging
Sup.
Right Left
Fig. 9.68 In this heart, photographed from the front in

Inf. anatomical orientation, there is extensive myocardial bridging
across the anterior interventricular artery.
Normal right coronary artery Left
Sup. Inf.
Right

sternotomy, with the right atrial appendage retracted, shows a
fistula running from the right coronary artery to the base of the
Clamp on appendage appendage. The white loop is placed around the normal distal
Fistula segment of the right coronary artery, while the red loop encircles
the fistula itself.
Normal coronary artery
Left
Sup. Inf.
Ends of divided fistula Right

Fig. 9.70 The broad fistula shown in Figure 9.69 has been divided.
left coronary artery (Figure 9.71). The myocardium from which the steal has is to bypass the hinge of an aortic valvar
communication itself can be a large solitary occurred. leaflet (Figure 9.72). Typically, the
orifice, or a complex worm-like aneurysmal tunnels extend between the aorta and
cavity. When the communication is simple the left ventricle, although aorto-right
Aortoventricular tunnels
and large, shunting across it can be ventricular tunnels do exist
considerable and the artery itself can be These tunnels are rare lesions. The (Figure 9.73). It is the ones
markedly dilated. Steals can also result, anatomical arrangement is difficult to communicating with the left ventricle
with consequent ischaemia in the area of understand, as the essence of the lesions that are most frequent44. The anomalous
Post.
Right Left
Ant.
Aorta
Fistula
Fig. 9.71 The reconstructed computed tomographic angiogram

Termination in
left ventricle shows a fistula taking origin from the left coronary artery (arrow)
and terminating in the left ventricle.
Sup.
Right coronary leaflet Ant. Post.
Aorta Inf.
Fig. 9.72 The aortic root has been opened and photographed
from the front. The red cord is passing through an aorto-left
Left ventricle ventricular tunnel, which bypasses the hinge of the right coronary
aortic valvar leaflet.
channel, coursing from the supports, extends through the tissue is supported by the ventricular
left ventricle between the base of the plane that separates the aortic and myocardium, while its roof is made up of
afflicted aortic sinus and the leaflet it pulmonary roots. The base of the tunnel arterial wall.
High origin of both coronary arteries Aortoventricular tunnel
Sup.
Left Right Fig. 9.73 The aortic root has been opened and is photographed
from behind. The aortic end of a tunnel is seen, which courses
Inf. Right aortic leaflet through the supraventricular crest and opens into the right
ventricular infundibulum.
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Positional anomalies
10
of the heart
The surgical problems posed by cardiac cardiac position. We emphasise the need to cardiac position. In the past, formidable
malformations may be considerably segregate the syndromes, preferably into the conventions were constructed, using
increased when the heart itself is in an subsets of right versus left isomerism, as the terms such as dextrocardia,
abnormal position. This is, in part, due to prognosis is markedly different for the two dextroposition, dextrorotation, or arcane
the unusual anatomical perspective variants. variants such as pivotal dextrocardia1. In
presented to the surgeon because of the practice, the only requirement is for a
malposition, and also to the abnormal system that accounts for the two major
THE ABNORMALLY POSITIONED
locations of the cardiac chambers, which features in independent fashion, namely
HEART
may necessitate approaches other than those the position of the cardiac mass relative to
already discussed. Cardiac malposition Account should be taken of not only an the silhouette of the chest, and the
itself, nonetheless, does not constitute a abnormal position of the heart within the direction of the cardiac apex. In the past,
diagnosis. Any normal or abnormal chest, but also the direction of its apex. we have defined dextrocardia as the
segmental combination can be found in a These are independent features. In the situation in which the heart is in the right
heart that, itself, is abnormally located. The normal individual, the heart is positioned chest, with the apex pointing to the right
heart may be normal, despite its abnormal with its apex to the left, and with two- (Figure 10.1). But what happens on the
location, but extremely complex anomalies thirds of its bulk to the left of the midline. rare occasion when the heart is in the right
are frequently present. Consequently, the A mirror-imaged atrial arrangement is chest, but its apex is pointing to the left?
very presence of an abnormal cardiac usually accompanied by a mirror-imaged This situation is readily, and
position emphasises the need for a full and cardiac arrangement. The expected unambiguously, described as the heart
detailed segmental analysis of the heart. All arrangement is for the cardiac apex to located in the right chest, with its apex
the rules enunciated in Chapter 6 apply point to the right, with the greater part of pointing to the left. Those who wish to
should the heart not be in its anticipated the cardiac mass in the right hemithorax. give nominative definitions to the
position. In this chapter, we confine In the setting of isomerism of the atrial arrangement are free to do so, but the
ourselves to a description of abnormally appendages, there is no norm. Thus, for descriptive approach is entirely adequate,
positioned hearts, giving a more detailed all patients with isomerism, and equally and much less liable to misconstruction.
discussion for specific types of malposition. for those with the usual or mirror-imaged Thus, the heart can be described as being
We conclude with a review of the surgical atrial arrangements and abnormally mostly in the left chest, mostly in the right
significance of isomerism of the atrial positioned hearts, it is necessary to have a chest, or symmetrical. The apical
appendages, which is generally agreed to be system that permits a simple and straight- orientation can be to the left, to the right,
one of the major harbingers of abnormal forward description of the abnormal or to the middle.
Morphologically
right atrial appendage Left
Sup. Inf.
Right
Aorta
Apex to right
Pulmonary trunk Fig. 10.1 This operative view through a median sternotomy
shows a patient with a mirror-imaged arrangement of the organs.
Morphologically right ventricle The heart is mostly in the right chest, with the apex pointing to the
right (arrow).
variants, arguing that all cases can be heart and various abdominal organs are
EXTERIORISATION OF THE HEART described as thoracic or thoracoabdominal. displaced into an omphalocele. A
The description given earlier does not In the thoracic type, which is the most variant of this abnormality is seen in
account for the most severe cardiac common, there is a sternal defect and patients with Cantrell’s syndrome4. These
malposition, namely exteriorisation of the absence of the parietal pericardium. The patients have a cleft of the lower sternum.
heart, or ectopia cordis. It has been said that heart protrudes directly from the thorax3. The heart is beneath the skin in the upper
the heart can protrude from the thoracic There is usually an associated omphalocele, epigastrium, and is associated with an
cavity in cervical, thoracic, and the thoracic cavity is small. omphalocele (Figure 10.2). When the skin
thoracoabdominal, or abdominal positions. In the thoracoabdominal type, the sternal was opened in the patient shown in
A previous review2 questioned the defect is usually confluent with deficiencies Figure 10.2, the heart was seen to occupy a
existence of the cervical and abdominal of the abdominal wall and diaphragm, so the position in the midline (Figure 10.3),
Collapsed umbilical hernia
Left
Fig. 10.2 This picture, taken from the right side of the
Sup. Inf. epigastrium with the patient supine, shows the heart bulging
through the skin (star) because of a cleft in the lower sternum and
Right diastasis of the rectus muscles. An associated abdominal hernia is
collapsed.
Pulmonary trunk
Left
Sup. Inf.
Right
Aorta
Fig. 10.3 The skin and upper sternum have been opened in the
patient shown in Figure 10.2, revealing the heart to be positioned
Morphologically right ventricle in the midline, with the apex pointing to the middle of the chest
(arrow).
extending towards the omphalocele abnormally positioned hearts, therefore, it because, in congenitally corrected
through a diaphragmatic defect. is essential that the surgeon establish the transposition, the anterior interventricular
These patients frequently have locations of the chambers within the coronary artery arises from the right-sided
complex intracardiac defects, often abnormally located organ so that the coronary artery. Thus, in a heart that at
including a diverticulum of the left operation can be planned appropriately. first sight appears to represent simple
ventricle. transposition, finding the anterior
interventricular artery arising from the
CRISS-CROSS AND right-sided coronary artery should alert the
RIGHT-SIDED HEART SUPEROINFERIOR VENTRICLES surgeon to the possible diagnosis of
Our descriptive system provides no With the sophistication of modern discordant atrioventricular connections
information as to whether the abnormal diagnostic techniques, it is unlikely that the and congenitally corrected transposition.
location of the heart is the result of surgeon will be presented with a patient The criss-cross arrangement, perhaps
congenital malformation. The heart may be having an abnormally located heart without better described as twisted atrioventricular
in the right chest secondary to a pulmonary a full preoperative diagnosis. Particular connections7, can also be found in the
defect, or because of gross enlargement of arrangements of the cardiac chambers can setting of concordant atrioventricular
its right-sided chambers. In either case, the still give major difficulties in diagnosis, connections, when the morphologically
problems of surgical access are similar. notably the arrangements known as criss- right ventricle will be left-sided
Indeed, there is no reason why, in a patient cross hearts, or superoinferior ventricles. (Figures 10.5, 10.6). When the
with a lesion such as congenitally corrected In these anomalies, the ventricular atrioventricular connections are twisted so
transposition, the heart should not be relationships, or more rarely the ventricular as to produce the criss-crossing
positioned in the right chest because of topology, when topology is defined as arrangement, usually there is an additional
pulmonary problems or right-sided proposed in Chapter 6, are not as superior–inferior relationship of the
hypertrophy. anticipated for the given atrioventricular ventricles. This is a consequence of the
There are certain lesions, nonetheless, connections5. In a patient with congenitally cardiac mass being tilted as well as rotated
that spring to mind when the heart is corrected transposition and the criss-cross (Figure 10.7), so that both criss-crossed
located in the right chest with the usual arrangement, the morphologically right and superoinferior arrangements are
atrial arrangement, or when the heart is ventricle will be predominantly right-sided present. Again, the interventricular
left-sided in patients with mirror-imaged rather than in its anticipated left-sided coronary arteries are useful because they
atrial chambers. The most notable of these position6. This is a consequence of rotation indicate the plane of the ventricular
is, indeed, congenitally corrected of the ventricular mass in its long axis septum, serving as excellent guides to the
transposition. In this lesion, of course, not (Figure 10.4). The distribution of the position of the ventricular cavities. The
all the cardiac chambers are in their coronary arteries is of considerable help in distribution of the conduction tissue in
expected positions. In all patients with determining the position of the ventricles these bizarre hearts, however, is governed

orientation, illustrates the rotational abnormality
around the long axis (inset) that produces the so-
called criss-cross abnormality. The left-hand panel
shows a heart with congenitally corrected
transposition in the absence of rotation around
the long axis. The morphologically left ventricle is
right-sided. Subsequent to rotation (right-hand
panel), the morphologically right ventricle
occupies a right-sided position, although still
connected discordantly to the morphologically left
atrium. The arrows in the right-hand panel show
the crossing atrioventricular valves, the dotted line
Anterior depicting the posterior connection to the
Right-sided Left-sided morphologically left ventricle, which has rotated
left ventricle right ventricle right ventricle
to a left-sided position.
Fontan
conduit
Morph.
left atrium
Fig. 10.5 The reconstructed three-dimensional magnetic

Morph. RV
Morph. LV resonance image, shown in cross-section, illustrates the twisted
atrioventricular connections (arrows) that produce the criss-cross
Post. heart. In this patient, there are concordant atrioventricular
connections, so the twisting of the ventricular mass places the
Right Left morphologically right ventricle (Morph. RV) in a left-sided position
relative to the morphologically left ventricle (Morph. LV). As shown
in Figure 10.6, both arterial trunks arose from the morphologically
Ant.
right ventricle.
Oversewn
pulmonary root
Aorta
Fig. 10.6 The reconstructed three-dimensional magnetic

Morph. RV
resonance image is from the patient shown in Figure 10.5, but
viewed from the right side. The morphologically right ventricle
(Morph. RV) is superiorly located relative to the morphologically
Morph. LV Sup. left ventricle (Morph. LV), but as shown by the black arrows, it
retains its right-hand topology despite being left-sided. Both
Post. Ant. arterial trunks arise from the right ventricle, with the aorta in a left-
sided position. The pulmonary trunk has been divided and
Fontan conduit
oversewn, and a conduit placed from the inferior caval vein to the
Inf.
pulmonary arteries so as to create the Fontan circulation.
by the connections between the chambers, atrioventricular valves. These abnormal double-outlet right ventricle and left-sided
and not by their position. These hearts are relationships can be found with any aorta being present in the heart shown in
often further complicated by the presence combination of atrioventricular and Figure 10.6. Even in these rare situations,
of straddling and overriding ventriculoarterial connections, with a the topological arrangement of the

orientation, shows the tilting along the long
axis of the ventricular mass in a heart with
congenitally corrected transposition, which
usually has side-by-side ventricles (central
panel). The tilting produces a superoinferior
relationship of the ventricles, almost always in
association with twisting of the
atrioventricular connections (see Figures 10.4
to 10.6). Depending on the direction of tilting,
either the morphologically (Morph.) left
ventricle (left-hand panel) or the
Morph. left ventricle Usual side-by-side Morph. right ventricle
uppermost uppermost morphologically right ventricle (right-hand
ventricles
panel) can be positioned uppermost. Most
usually it is the right ventricle that assumes the
superior location. As shown in Figure 10.6,
superoinferior ventricular relationships can
also be found when the atrioventricular
connections are concordant.
Aorta
Sup.
Pulm.
trunk
Right Left
Inf.
Tricuspid
valve
Fig. 10.8 In the heart shown in Figure 10.6, the right ventricle
with a double outlet showed right-hand topology despite the
twisted atrioventricular connections. In this image, the
morphologically right ventricle exhibits left-hand topology (black
arrows), again with a double outlet from the ventricle. The
Morphologically right ventricle atrioventricular valve, however, is morphologically tricuspid, and
connects the right ventricle to the usually positioned right atrium.
Despite the left-hand topology, the atrioventricular connections in
this heart are concordant. This is a very rare example of segmental
disharmony5. Pulm., pulmonary.
ventricular mass is as anticipated for the circumstances, however, the ventricles can handed (Figure 10.6). On occasion,
atrioventricular connections. Thus, when not only occupy unusual positions, but the nonetheless, the right atrium can be joined
there is rotation of the ventricular mass topology is not as expected for the existing to a morphologically right ventricle that
sufficient to produce crossing of the atrioventricular connections. Hence, when shows left-hand ventricular topology, with
atrioventricular connections, this does not the usual atrial arrangement coexists with the right ventricle being additionally left-
disturb the intrinsic relationships of the concordant atrioventricular connections, sided (Figure 10.8). This situation is one of
two ventricles7. In exceedingly rare the ventricular topology is usually right- the rare occasions when it is necessary to
Morphologically left atrial appendage
Left
Sup. Inf.
sternotomy, shows the different morphology of the
Morphologically right atrial appendage Right morphologically right as opposed to the morphologically left atrial
appendages in a patient with the usual atrial arrangement.
describe ventricular topology in addition to patient has one spleen, multiple spleens, or isomeric right appendages (Figures 10.16–
accounting for the atrioventricular no spleen at all. Determining the 10.18), it is the rule to find complex
connections5. morphology of the appendages brings intracardiac anomalies, usually with the
attention directly to the heart, enabling the absence of the spleen. The pulmonary
surgeon working in the operating room to veins will be connected in a totally
HEARTS WITH ISOMERIC ATRIAL
make the diagnosis of isomerism anomalous fashion even when they are
APPENDAGES
immediately, even if this had not been joined to the heart (Figure 10.19). Most
The problems occurring in hearts with predicted by the preoperative studies. frequently, there are major anomalies of
isomeric atrial appendages have been The surgeon can readily distinguish the systemic venous drainage. A common
mentioned already. Hearts with this appendages as being either atrioventricular valve is usually present
arrangement are not only found in unusual morphologically right or left (Figure 10.9). (Figures 10.16 – 10.18), often with a
positions, but are almost always associated The surgeon should always confirm double-inlet ventricle (Figure 10.19).
with an abnormal arrangement of the whether the patient possesses lateralised Pulmonary stenosis or atresia is frequently
thoracoabdominal organs; hence the atrial appendages. The finding of isomeric found, and there are bilateral sinus
popular rubric of visceral heterotaxy. Many left (Figure 10.10) or right (Figure 10.11) nodes11–13. Increasing operative
will continue to classify these patients in appendages should immediately alert the experience now shows that even the most
terms of ‘asplenia’ and ‘polysplenia’8. It is surgeon to potential problems over and complex combinations can be treated
of greater value for the surgeon to base any above those anticipated in the patient with surgically.
system of categorisation on the morphology the usual or a mirror-imaged atrial Although isomerism of the
of the atrial appendages. Splenic arrangement. It is now also possible to morphologically right appendages is almost
morphology does not always correspond to distinguish directly the presence of always accompanied by severe intracardiac
the atrial anatomy, itself based on the isomeric atrial appendages using computed malformations, this is not necessarily the
extent of the pectinate muscles relative to tomography (Figures 10.12–10.15), while case when there are isomeric left
the atrial vestibules. There is a greater use of other three-dimensional techniques appendages (Figures 10.20–10.22). Thus,
correspondence between the anatomy of such as magnetic resonance imaging will the surgeon is more likely to be confronted
the appendages as thus determined and readily reveal the arrangement of the with an undiagnosed case in the setting of
what is expected of the ‘splenic syndromes’ bronchial tree and the abdominal organs. left isomerism. Therefore, it becomes
than between these syndromes and splenic There should be no problem, therefore, in important to know that the sinus node is in
morphology9,10. More important, it is of distinguishing those patients having an anomalous position. It is usually
little consequence to the cardiac surgeon at isomeric right as opposed to isomeric left hypoplastic. If it can be found, it will be
the time of operation whether his or her appendages. In hearts from patients with located close to the atrioventricular
Left Morphologically
left atrial
appendage
Sup. Inf.
Right
Aorta

Right
superior sternotomy, shows that the appendage on the right side of the
caval heart has left morphology. In this patient, the left-sided appendage
vein was also morphologically left, so the patient had isomerism of the
morphologically left atrial appendages.
Morphologically right atrial appendage
Left
Fig. 10.11 In this patient, the left-sided atrial appendage is
Sup. Inf. broad-based, with a wide junction to the body of the atrium. Note
that the left-sided superior caval vein joins to the atrial roof, and
Left superior
that there is a left-sided terminal groove. The right-sided
caval vein + Right
terminal groove appendage was also of right morphology. The patient has
isomerism of the right atrial appendages.
Morph. right atrial appendage
Morph. right ventricle

Sup.
Post. Ant. Fig. 10.12 The reconstructed computed tomographic angiogram

shows a patient with a right-sided morphologically (Morph.) right
atrial appendage, the right-sided atrium joining to a
Inf.
morphologically right ventricle.
Sup.
Morph. right atrial appendage
Ant. Post.
Inf.
Aorta
Fig. 10.13 The image shows the left side of the heart in
Figure 10.12 reconstructed. The left-sided atrial appendage also
has a broad base, and is morphologically (morph.) right. The
patient has isomerism of the morphologically right appendage.
Morph. right ventricle Note the incomplete left ventricle in the posteroinferior position
(star). The aorta arises from the dominant right ventricle.
Ant.
Aorta Right Left
Post.
Fig. 10.14 The cross-sectional image is from the same dataset as

shown in Figures 10.12 and 10.13. Both appendages (stars) are
broad-based (black double-headed arrows), with pectinate muscles
extending to the crux (white arrows; see also Figure 10.18). There is
obvious isomerism of the right atrial appendages (compare with
Figure 10.15).
Sup.
Right Left
Aorta
Inf.
Common atrium
Fig. 10.15 The computed tomogram in this patient, seen from

the front, shows the presence of two narrow appendages (stars)
with narrow necks (black double-headed arrows). This patient has
isomerism of the morphologically left atrial appendages (compare
with Figure 10.14).

Sup.
Post. Ant.
Inf.
Septal strand
Fig. 10.16 The heart has been opened through the right
atrioventricular junction close to the crux, and the parietal wall
spread upwards. The pectinate muscles encircle the right-sided
vestibule, indicating that the right-sided appendage is
Pectinate muscles to crux morphologically right. Note the common atrioventricular valve
(star), and absence of the coronary sinus.

Sup.
Ant. Post.
Inf.
Fig. 10.17 The left side of the heart shown in Figure 10.16 has
been opened through an incision close to the crux. The pectinate
muscles also encircle the left-sided vestibule, showing that there
Septal strand Pectinate muscles to crux are morphologically right atrial appendages bilaterally. The star
shows the common atrioventricular valve.
Common atrioventricular junction

Sup.
Left Right
Inf.
Fig. 10.18 In this heart, the atrial chambers have been

disconnected from the atrioventricular junctions inferiorly, and
tilted superiorly. The pectinate muscles encircle the entirety of the
common atrioventricular junction, indicating the presence of
isomeric right atrial appendages. The common junction is guarded
Pectinate muscles to crux by a valve with separate right and left atrioventricular valvar
orifices.
Pulmonary veins to atrial roof

Sup.
Left Right
Inf.
Fig. 10.19 In this heart from a patient with isomeric right atrial
appendages, the pulmonary veins drain in anatomically anomalous
fashion to the atrial roof, even though returning directly to the
heart. Note that the common atrioventricular valve is connected
exclusively to a dominant left ventricle, indicating the presence of a
Pectinate muscles to crux
double-inlet connection.
junction11–13. Interruption of the inferior affected cases, there may be a common atrioventricular connection, but there is
caval vein, with return through the azygos atrioventricular valve14. Pulmonary frequently aortic coarctation.
venous system, is a frequent stenosis or atresia is not usually a feature, Whatever the types of isomerism, when
accompaniment. In the more severely nor is the presence of univentricular there is a common atrioventricular valve
Morphologically
Sup. left atrial
appendage
Post. Ant.
Inf.
Fig. 10.20 The right-sided atrium in this heart has a

morphologically left appendage.
Morphologically
Sup. left atrial
appendage
appendage
Ant. Post.
Inf.
Fig. 10.21 The image shows the opposite side of the heart
illustrated in Figure 10.20. The left-sided appendage is also of left
morphology.
and each atrium is connected to its own diagnosed as having congenitally corrected conduction tissue joining dual
ventricle, it is frequent to find a left-hand transposition. The presence of isomeric atrioventricular nodes (Figure 10.22, right-
pattern of ventricular topology. In this appendages, however, makes it highly hand panel)11. This places the entire edge
setting, the patient may well have been likely that there will be a sling of ventricular of the ventricular septum at risk should
Left bundle Anterior

branch in connection
left-sided
ventricle
orientation, shows the disposition of the axis of
Sling atrioventricular conduction tissue in hearts
with isomerism of the atrial appendages,
biventricular atrioventricular connections, and
a common atrioventricular valve. The
arrangement varies depending on whether
there is right-hand ventricular topology (left-
hand panel), when there is only a posterior
connection of the ventricular conduction
tissues, or left-hand topology (right-hand
panel), when the ventricular conduction tissues
are connected by both posterior and anterior
atrioventricular nodes, producing a sling of
Posterior Posterior
connection conduction tissue along the crest of the
connection
muscular ventricular septum.
surgical correction be attempted. When 4. Cantrell JR, Haller JA, Ravitch MM. A patients with visceral heterotaxy. Ann
isomerism of the appendages is found with syndrome of congenital defects involving Thorac Surg 1995; 60: 561–569.
biventricular atrioventricular connections the abdominal wall, sternum, diaphragm, 10. Uemura H, Ho SY, Devine WA, Anderson
and a right-hand pattern of ventricular pericardium and heart. Surg Gynecol Obstet RH. Analysis of visceral heterotaxy
1958; 107: 602–614. according to splenic status, appendage
topology, the atrioventricular conduction
5. Anderson RH, Smith A, Wilkinson JL. morphology, or both. Am J Cardiol 1995;
axis should be expected in its usual
Disharmony between atrioventricular 76: 846–849.
posterior position (Figure 10.22, left-hand connections and segmental combinations – 11. Smith A, Ho SY, Anderson RH, et al. The
panel). This entire discussion emphasises unusual variants of “criss-cross” hearts. J diverse cardiac morphology seen in hearts
the significance of full sequential segmental Am Coll Cardiol 1987; 10: 1274–1277. with isomerism of the atrial appendages
analysis of any patient presented for cardiac 6. Symons JC, Shinebourne EA, Joseph MC, with reference to the disposition of the
surgery. et al. Criss-cross heart with congenitally specialized conduction system. Cardiol
corrected transposition: report of a case Young 2006; 16: 437–454.
References with d-transposed aorta and ventricular 12. Dickinson DF, Wilkinson JL, Anderson
preexcitation. Eur J Cardiol 1977; 5: KR, et al. The cardiac conduction system
1. Wilkinson JL, Acerete F. Terminological 493–505. in situs ambiguous. Circulation 1979; 59:
pitfalls in congenital heart disease. Reappraisal 7. Seo J-W, Yoo S-J, Ho SY, Lee HJ, 879–885.
of some confusing terms, with an account of a Anderson RH. Further morphological 13. Ho SY, Seo J-W, Brown NA, et al.
simplified system of basic nomenclature. Br observations on hearts with twisted Morphology of the sinus node in human
Heart J 1973; 35: 1166–1177. atrioventricular connections (criss-cross and mouse hearts with isomerism of the
2. Van Praagh R, Weinberg PM, Matsuoka R, hearts). Cardiovasc Pathol 1992; 1: atrial appendages. Br Heart J 1995; 74:
Van Praagh S. Malpositions of the heart. 211–217. 437–442.
Edited by Adams FH, Emmanouilides GC. 8. Stanger P, Rudolph AM, Edwards JE. 14. Uemura H, Anderson RH, Ho SY, et al.
In: Moss’ Heart Disease in Infants, Children Cardiac malpositions: an overview based on Left ventricular structures in
and Adolescents. Baltimore, MD: Williams & study of sixty-five necropsy specimens. atrioventricular septal defect associated
Wilkins, 1983; pp 422–458. Circulation 1977; 56: 159–172. with isomerism of the atrial appendages
3. Byron F. Ectopia cordis. Report of a case 9. Uemura H, Ho SY, Devine WA, Kilpatrick compared with similar features with usual
with attempted operative correction. LL, Anderson RH. Atrial appendages and atrial arrangement. J Thorac Cardiovasc
J Thorac Surg 1948; 17: 717–722. venoatrial connections in hearts with Surg 1995; 110: 445–452.
Index
Numbers in italics refer only to illustrations
bicuspid 210–12, 213–14 isomerism 19, 129–32, 133, 143, 369–76

A tricuspid 213, 214 anomalous pulmonary venous connection
unicuspid 210, 212 132, 330, 369
Abbott’s artery 337, 338 aortic valve juxtaposition 17
absent pulmonary valve syndrome 239 anatomy 45, 46, 53, 55–60, 73–81, 210 left see left atrium, appendage
accessory pathways 114–19, 114 aortic–mitral fibrous curtain 45, 56, 58, 79 right see right atrium, appendage
left-sided 116, 117 conduction axis and 79, 82, 85, 112, 218 situs inversus 129
paraseptal 117–19, 118 in double-outlet ventricle 295–6, 297 atrial fibrillation 122–3, 126
right-sided 117, 118 endocarditis 218 atrial flutter 120–1
aneurysms imperforate 145 atrial maze operations 124–6
membranous septum 274 insufficiency 210, 218, 221–2 atrial morphology
patent arterial duct 350 leaflets 45, 46, 73–5, 75–7, 78–9 left 34–5
ansa subclavia 12 left coronary 79, 80 in malformed hearts 129–32
anterior septum 119 non-adjacent 79 right 19–34
anterolateral muscle bundle hypertrophy 216–17 right coronary 79 atrial septal defects 155–62
aorta overriding 145, 197, 198 oval fossa 22, 156, 157–8
anatomy 45–7 prolapse 195, 197, 198, 218, 223 sinus venosus 156–9, 160–2, 332–3
arch 45–6, 48 sinutubular junction 45, 47, 52, 75, 77 unroofing of the coronary sinus 159–62, 163–4,
development 340–1 stenosis see aortic stenosis 334
double 341–3 ventriculoarterial junction 52, 54, 77, 148, 149 atrial septum 22–5, 27, 151, 152
interrupted 300–1, 337–40 zones of apposition 55, 79, 211 atrio–Hisian tracts 114
right-sided 343–4 see also aortic atresia atriopulmonary connection 250–2
ascending 45, 47 aortic–mitral fibrous curtain 45, 56, 58, 79 atrioventricular bundle (bundle of His) 59, 112,
coarctation 211, 300–1, 334–7 aortopulmonary collateral arteries 224, 232–4 114–19, 189
intercostal arteries 46–7, 336 aortopulmonary window 351–3 in congenitally corrected transposition 280,
common arterial trunk 308–9, 312 aortoventricular tunnels 359–61 285–6
congenitally corrected transposition 281–6, 286 arrhythmias in double-inlet ventricle 252
coronary arteries, anomalous origins 91–6, 313, postoperative 124–6, 260, 270 valvar anatomy and 79, 85
354–5 ventricular tachycardia 123 in ventricular septal defects 187–91
descending 46–7 see also supraventricular tachycardia atrioventricular conduction axis 25–31, 112–13
double-outlet ventricle 295–6, 297, 300–1 arterial duct 9 in atrial appendage isomerism 130–1, 375–6
hypoplasia 334 in aortic coarctation 334, 336 in atrioventricular septal defects 167–70, 181–2,
parallel arterial trunks 313–19 in common arterial trunk 311 184–5
pulmonary arteries arising from 345–6, 347 embryology 341 in common arterial trunk 307
spiral arrangement of arterial trunks 313, 314 pulmonary arteries arising from 232, 233, 234, in congenitally corrected transposition 280–1,
mirror-image 315 345, 347 285–6
tetralogy of Fallot 231–2 surgical closure 348–51 when closing a ventricular septal defect 285–6
transposition 275–8 in tetralogy of Fallot 232, 233, 234 in double-inlet ventricle
vascular rings 340–5 arterial ligament 49, 347 left dominant 252
see also common arterial trunk arterial switch procedure 278, 279–82 right dominant 254
aortic atresia arterial trunk(s) 145 septation procedure 252–3
in congenitally corrected transposition 291 common see common arterial trunk ventricular septal defect enlargement 247–8
with mitral atresia 260, 263–6 parallel 313–17 in double-outlet ventricle 291, 294
aortic root 16, 52, 59, 91, 159 discordant atrioventricular connections Fontan procedure and 121, 124–5
aortic sinuses 78, 91–4, 124, 211–12 317–19 in parallel arterial trunk 319
in aortic stenosis 218 relationship to arterial valves 148 in tetralogy of Fallot 226, 228–9
Leiden nomenclature 93 solitary 145, 303, 304 in transposition 270
in transposition 278, 280 spiral arrangement 313, 314 tricuspid valve 85
aortic stenosis mirror-image arrangement 315 atresia (Fontan procedure) 125, 259–60, 261
subvalvar 210, 213–18 arterial valves 52–5, 73–8, 148, 210 straddling 200, 201
dynamic 218 attachments/support 55–60 valvar relationships 68, 79, 84–5, 86, 218
fixed 214–17 malformations 145, 210–41 ventricular pre-excitation 113–19
supravalvar 218, 219–21 see also aortic valve; pulmonary valve in ventricular septal defects 187–91
valvar 210–13 atrial appendages 16–17, 129, 130, 369 see also sinus node
378 Index
atrioventricular groove 27, 118, 230 brachiocephalic artery 12, 46 surface anatomy 16, 17, 18, 19
atrioventricular junction morphology in congenital mirror-imaged 344–5 see also specific conditions
malformations 132–9 brachiocephalic vein 2, 5, 109 congenitally corrected transposition 144, 279–91,
biventricular and mixed atrioventricular 135, pulmonary venous drainage via 328, 329 366
143 bronchial arteries 46, 47, 48, 350 complicating lesions 282–91
concordant 134, 143 bronchial obstruction 348 conoventricular perimembranous defects 191
discordant 134, 143 bundle of His see atrioventricular bundle coronary arteries 91–105
uniatrial biventricular 136, 138, 143, 254, 258 bundles of Kent 115 anomalies
univentricular 136–9, 145 classification 353
valves 140–3 epicardial course 94–6, 355–6, 358
ventricular morphology 132–3, 134, 143 C fistulous communications 357–9
ventricular relationships 143–5 intramural course 355, 356
see also double-inlet ventricle Cantrell’s syndrome 365–6 origins 91–6, 211–12, 313, 353–5
atrioventricular nodal tachycardia 119 cardiac position/malposition see under heart in common arterial trunk 310, 314
atrioventricular node 25–6, 112, 113, 118 cardiac veins 105–7, 161 in congenitally corrected transposition 366
aortic valve and 79, 82, 85 great 86, 87, 106, 107 in double-outlet ventricle 301
artery 28, 31, 70, 89, 105, 118 middle 106 interventricular
in atrioventricular septal defects 168, 169, 185 small 106 anterior 88, 96, 100–1, 103, 366
in congenitally corrected transposition 280 see also coronary sinuses inferior 89, 97–8, 100, 101, 105
in double-inlet ventricle 252 carotid artery, left 46 left 19, 87–8, 99–101
mitral valve and 68, 69, 85 Carpentier’s classification of mural leaflet scallops anomalies 92, 94, 353, 354–5, 357, 360
in transposition repair 270 66, 68 atresia 355, 357
atrioventricular septal defects 155–6, 166–85, 209 caval veins circumflex 70, 71, 88–9, 96, 101–2, 105
bridging leaflets inferior common arterial trunk 313, 314
morphology 170–6 abnormalities 322, 323, 324 dominance 70, 101, 105
relationship to septal structures 172–8 atrial appendage isomerism 132, 374 intermediate 100, 103
in common arterial trunk 306 oval fossa and 156, 159 main 99
complete 172 scimitar syndrome 330 myocardial bridging 102, 106
conduction pathways 167–70, 181–2, 184–5 sinus venosus and 156, 160 right 19, 39, 97–9
Gerbode defects 34, 164–5, 165–7 persistent left superior 8, 17, 18, 322–3, 325 anomalies 92, 93, 94, 95, 353, 357, 359
hypoplasia of the ventricular septum 179–81 unroofing of the coronary sinus 159–60, dominance 97–8, 101
intermediate 172, 177 161–2, 164 in tetralogy of Fallot 231
left ventricular dominance 184–5 superior valves and 72, 89
left ventricular outflow tract is narrow 182–3 abnormalities 322 in transposition 278, 279–82
mural leaflets 171 proximity of phrenic nerve 3, 7 valves and 87–9
partial 172, 178 sinus venosus and 156–9, 161 mitral 70, 71, 88–9
right ventricular dominance 183–4 in venous switch procedures 267 tricuspid 72, 75, 89, 97, 98, 100
terminology 162–4 total cavopulmonary connection 248–50, 252, 259 coronary sinuses 106–7, 322
ventricular shunting only 175–6, 178–80 cavoatrial junction 159 atresia 325, 327
atrioventricular septum central fibrous body 31–3, 45, 57–9 in atrioventricular septal defects 168–70
membranous see membranous septum ventricular septal defects 186 dilation 325
muscular 27, 151, 153 circumflex coronary artery 71, 96, 101–2, 105 pulmonary venous drainage via 330, 331, 334
atrioventricular valves 52, 53–4, 55–6, 61–6 valves and 70, 88–9 unroofing 159–62, 163–4, 334
common 141, 170–6, 174 coarctation of the aorta 211, 300–1, 334–7 valvar relationships 70, 86–7, 87–88
atrial appendage isomerism 373–4 intercostal arteries 46–7, 336 coronary veins 105–7, 161
common arterial trunk 306 commissural cords 63, 64 see also coronary sinuses
double-inlet ventricle 246, 255 commissures 55, 63 Cox maze III procedure 126
double-outlet ventricle 301 common arterial trunk 145, 148, 303–13 criss-cross heart 145, 366–9
in congenitally corrected transposition arterial anatomy 307–13 cysterna chyli 107
289–91 definition 303, 304
malformations 199–208 interventricular communications 306–7
surgical repair 304–5
morphology in malformed hearts 140–3 D
see also mitral valve; tricuspid valve truncal valve 303, 307, 308–9
azygous vein 11, 12 conduction axis see atrioventricular conduction axis d-transposition 263, 276
with inferior caval vein abnormalities 324 congenital malformations Damus–Kaye–Stansel procedure 247
pulmonary venous drainage via 328–30 cataloguing 148–9 dead-end tract 112
morphological analysis 129 dextrocardia 364
atrial arrangement 129–32 see also right-sided heart
atrioventricular connections 132–9 diverticulum of Kommerell 343, 344
B
cardiac position 148 double aortic arch 341–3
Bachmann’s bundle 22 valves 140–3, 145, 148 double-inlet ventricle 136, 245–54
basal cords 63, 65, 68, 74 ventricular relationships 143–5 conduction axis 247–8, 252–3, 254
bicuspid valve see mitral valve ventricular topology 132–3, 134, 143 left dominant 136, 245, 247–53
Bland–White–Garland syndrome 353, 354–5 ventriculoarterial junctions 145–8 Fontan procedure 247, 248–52
Index 379
Holmes heart 248, 315, 316 hemitruncus 345 left ventricular outflow tract (subaortic outflow
parallel arterial trunks 314–17 hepatic veins 322 tract) 45
septation procedure 252–3 Holmes heart 248, 315, 316 obstruction/stenosis 80, 83, 210, 213–18
right dominant 246, 254 Horner’s syndrome 12 in atrioventricular septal defects 182–3
solitary/indeterminate ventricle 136, 139, 140, hypoplastic left heart syndrome 140, 260, in transposition 274, 276
254, 256 263–6 Leiden convention (aortic sinus nomenclature)
double-outlet ventricle 145, 146, 147, 225, 261, 91, 93
292–303 levoatrial cardinal vein 323, 326
definition 292–4 I ligament of Marshall 49
discordant atrioventricular connections 291, 294 linea alba 2
left 303 indeterminate ventricle 140, 254, 256 lung
nature of the interventricular communication inferior caval vein see caval veins, inferior agenesis of the right lung 347–8
294–9 infundibular artery 93, 99 arterial supply in tetralogy of Fallot 224, 232–4
surgical repair 297, 302–3 infundibulum 38, 39, 41, 83, 151 lymphatic system 6, 10, 107–10, 337, 350
double switch procedure 291 absent in ventricular septal defect 196, 197
ductus arteriosus see arterial duct atretic 235–6, 237
in double-outlet ventricle 297, 298–9, 303
M
morphological analysis 145–8
stenosis 300 macro-reentrant atrial tachycardia 125
E
in transposition 278 Mahaim connections 114, 119
Ebstein’s malformation 117, 202–4, 262 interatrial groove see Waterston’s groove median sternotomy 2–3, 5, 16
in congenitally corrected transposition 289, 290 intercostal arteries 46–7, 336 mediastinum 2, 3, 3
ectopia cordis 365–6 intercostal spaces membranous septum 32–4, 45, 79, 151
Eisenmenger’s syndrome 323 fourth 3, 12 aneurysm 274
endocarditis of the aortic valve 218 fifth 6 ventricular septal defect 186
epicardium 14–16 sixth 3, 8 mitral atresia 137, 141, 199, 254, 258, 260–1, 323
Eustachian ridge 23, 29 intercostal veins 6, 8, 9–10 mitral valve
Eustachian valve 156, 260, 323 interrupted aortic arch 300–1, 337–40 anatomy 53, 61–70, 177
exteriorisation of the heart 365–6 interventricular arteries clefts 206, 208
anterior 88, 96, 100–1, 103, 366 conduction axis and 68, 69, 85
inferior 89, 97–8, 100, 101, 105 coronary arteries and 70, 71, 88–9
F coronary sinuses and 70, 86–7
dilation 201
Fallot’s tetralogy see tetralogy of Fallot
K fibrous support 45, 56, 58, 59, 61
fibrous tissue imperforate 140–1, 261, 266
aortic valve 79, 81 Koch’s triangle 25–8, 29–30, 72, 112, 119, 152 leaflets 44, 61–2, 63, 66–7, 66, 171, 173
aortic–mitral fibrous curtain 45, 56, 58, 79 Konno procedure 80, 83 aortic 56, 64, 65, 66
central fibrous body 31–3, 45, 57–9, 186 mural 62, 65, 66–7, 68
fibrous trigones 33, 56, 57 offset with respect to tricuspid valve 26, 30
Fontan procedure L overriding 142–3
combined mitral and aortic atresia 260–1 papillary muscles 44, 68, 69, 200
conduction axis and 121, 124–5 laryngeal nerve see recurrent laryngeal nerve
lateral thoracotomy parachute deformity 207–8, 209
double-inlet ventricle prolapse 64, 66, 205, 206–8
left dominant 247, 248–52 left 3–6
right 6–12 straddling 142, 200
right dominant 254 tendinous cords 62, 63–4, 67–8
tricuspid atresia 125, 257–60, 261 latissimus dorsi 3
left atrium 34–5 zone of apposition 56, 66–7
foramen ovale see oval fossa see also atrioventricular valves; mitral atresia
access 15, 19, 34
appendage 17, 34, 36, 129, 130, 131 moderator band 41, 147, 148, 153, 155
isomerism 19, 129–30, 131, 132, 133, 369–74 in tetralogy of Fallot 231, 232
G muscle of Lancisi (medial papillary muscle) 40,
vestibule 35, 37
gastric veins 331, 333 left bundle branch 45, 85, 112, 115 70, 73
Gerbode defects 34, 164–7 in congenitally corrected transposition 281 Mustard procedure 266, 269–70
Glenn procedure 162, 250 in double-inlet ventricle 252
left ventricle 43–5
double-outlet 303 N
H incomplete 254, 255 Nikaidoh procedure 275
in univentricular atrioventricular connections nodal arteries
heart 137–8, 145
malposition 364 atrioventricular 28, 31, 70, 89, 105, 118
ventriculoarterial junction 52, 54 sinus see sinus node, artery
criss-cross 145, 366–9 left ventricular dominance 136, 184–5, 247–53
exteriorisation 365–6 nodule of Arantius 74
Fontan procedure 247, 248–52 non-branching bundle 181, 252, 280, 285–6
right-sided 347–8, 364, 366 Holmes heart 248, 315, 316
position 2, 148 non-coronary sinus 78, 92
parallel arterial trunks 314–17 Norwood procedure 247, 260
surface anatomy 14, 16–19 septation procedure 252–3
380 Index
trunk 45, 47–9, 145 venous sinus 19, 20, 26

O aortopulmonary window 351–3 vestibule 34
oblique ligament 16 atriopulmonary connection 250–2 right bundle branch 85, 112, 182, 191
oblique sinus 15–16 coronary artery arising from 353, 354–5 in congenitally corrected transposition 281
omphalocele 365–6 dilated, in pulmonary valve insufficiency right ventricle 19, 35–43
ostium primum defects 155–6, 166–85, 209 239–41 double-outlet see double-outlet ventricle
ostium secundum defects see oval fossa, defects in double-outlet ventricle 295–6 incomplete
outflow tract tachycardias 123 parallel arterial trunks 313–19 double-inlet ventricle 247, 248, 248, 252
oval fossa 23, 27–28, 151, 152 solitary 145, 303, 304 tricuspid atresia 257, 259
defects 156 see also common arterial trunk in univentricular atrioventricular connections
absent 158 pulmonary atresia 137–8, 145
deficient 22, 157 in congenitally corrected transposition 291, 292 ventriculoarterial junction 52, 54
perforated 157 with intact ventricular septum 235–8, 260, 261 right ventricular dominance 183–4, 254
in tetralogy of Fallot (with ventricular septal right ventricular outflow tract
defect) 224, 232–4 obstruction/stenosis 210, 218
pulmonary hypertension 346 in double-outlet ventricle 300
P pulmonary stenosis 224–6 in transposition 273, 275
papillary muscles in congenitally corrected transposition 288–9 in tetralogy of Fallot 227–31
mitral valve 44, 68, 69, 200 subvalvar 210, 273 see also infundibulum
tricuspid valve supravalvar 221–4 right-sided aortic arch 343–4
anterior 41, 231 in tetralogy of Fallot 232 right-sided heart 347–8, 364, 366
medial 40, 70, 73 in transposition 273 Ross procedure 84
parachute deformity valvar 219–21
mitral valve 207–8, 209 pulmonary valve
tricuspid valve 208 absent pulmonary valve syndrome 239 S
parallel arterial trunks (with concordant anatomy 55, 57, 73–8, 81–4
ventriculoarterial connections) 313–17 dysplasia 218, 224 scimitar syndrome 330
and discordant atrioventricular connections imperforate 145, 236–7, 238 secondary atrial septum (septum secundum) 151
317–19 insufficiency 238–41 secundum defects see oval fossa, defects
patent arterial duct 9 leaflets 38, 39, 73–5, 82–3 semilunar valves see aortic valve; pulmonary
aortic coarctation 334, 336 left adjacent 82 valve
common arterial trunk 311 non-adjacent 82, 83 Senning procedure 266, 270
surgical closure 348–51 right adjacent 82, 83 septal band see septomarginal trabeculations
tetralogy of Fallot 232, 233 overriding 145 septal perforating arteries 42, 100, 104, 105
pectinate muscles 19, 22 ventriculoarterial junction 52, 54, 148, 149 septation procedure 252–3
atrial arrangements 129, 131–132 see also pulmonary atresia; pulmonary stenosis septomarginal trabeculations (septal band) 40–1,
penetrating atrioventricular bundle see pulmonary veins 7 42, 147–8, 153, 226
atrioventricular bundle (bundle of His) anomalous connection 157, 163, 323–34, 369, in tetralogy of Fallot 226, 231, 232
pericardial cavity 14–16 374 in ventricular septal defect 194–5
oblique sinus 15–16 atrial fibrillation and 122 double-outlet ventricle 297
transverse sinus 14–15, 45, 59, 68, 99 pulmonary venous sinus 35 septoparietal trabeculation 41, 42
pericardial fold 15 hypertrophy in tetralogy of Fallot 225, 229,
pericardium 14–16 230–1
see also moderator band
phrenic nerve 2–3, 4, 5 R
left 6, 8, 46 serratus anterior 3
right 6, 7, 7 Rastelli classification 171–2, 174 Shone’s syndrome 208
portal vein 331, 332–3 Type A 174 sinus node 19, 21
primary atrial septum (septum Type B 175 artery
primum) 151 Type C 175 course 19, 20–2, 23–6, 36, 99, 101,
pulmonary arteries Rastelli procedure 275 102–3
aortic origin 345–6, 347 recurrent laryngeal nerve oval fossa and 156, 158
common arterial trunk 307–13 closure of patent arterial duct 350 preservation 34, 159, 251, 259–60,
left 49 left 4–6, 9, 46 266–7
arising from right 346–7, 348–9 right 11, 12 variants 21, 23, 102, 103–4, 106, 269
in pulmonary atresia with intact ventricular right atrium 19–34 horseshoe 20, 21
septum 238 access 19 isomerism of the atrial appendages
in pulmonary valve insufficiency 239–41 appendage 17, 19, 129, 130, 131 130–1, 369
right 12, 49–50, 347 anomalous pulmonary venous connection sinus venosus defects 158–9, 163
spiral arrangement of arterial trunks 313, 314 132, 330, 369 transposition 266
mirror-image 315 isomerism 129, 130, 132, 133, 143, 369, sinus venosus defects 156–9, 160–2, 332–3
in tetralogy of Fallot 224, 232–4 370–2 sinutubular junction 45, 47, 52, 75, 77
total cavopulmonary connection 248–50, 252, pulmonary venous drainage to 330, 331 pulmonary stenosis 219, 221
259 septal surface 22–5, 27 supravalvar aortic stenosis 218, 219–21
Index 381
situs inversus 129 arterial supply 2

situs solitus 129 venous drainage 2, 5 U
solitary arterial trunk 145, 303, 304 thyroid artery, inferior 2 univentricular atrioventricular connections 136–9,
solitary origin of the coronary arteries 94–6, 97, tissue tags 145
313, 355, 356–357 aortic stenosis 217 unroofing of the coronary sinus 159–62, 163–4, 334
solitary ventricle 136, 139, 140, 254, 256 subpulmonary obstruction
Sondergaard’s groove see Waterston’s groove in congenitally corrected transposition
spleen 129, 369 288, 289
strut cords 63, 65, 68 in transposition 275
V
subaortic outflow tract see left ventricular outflow total cavopulmonary connection 248–50, 252, 259 vagus nerve 2, 4, 5, 46
tract trabeculations closure of patent arterial duct 350
subclavian arteries 6, 10, 11, 46 comparison of left and right ventricles 43, valvar annulus, in echocardiography 55,
development 341 132, 134 60, 77
interrupted aortic arch 339, 340 left ventricle 44–5 valve of Vieussens 107
isolated 345, 346 right ventricle 37–43 vascular pedicle 47
subclavian sympathetic loop (ansa subclavia) 12 see also moderator band; septomarginal vascular rings 340–5
subpulmonary obstruction/stenosis 210, 218 trabeculations; septoparietal trabeculation vascular sling 346–7, 348–9
in double-outlet ventricle 300 tracheo-oesophageal compression 342 venae cavae see caval veins
in tetralogy of Fallot 227–31 transposition 261–78 venous duct 331
in transposition 273, 275 aorta 275–8 venous sinus
sudden cardiac death 94, 355, 356 with complicating lesions 270–5 pulmonary 35
sulcus terminalis (terminal groove) 19, 20 coronary arteries and the arterial switch systemic 19, 20, 26
superior caval vein see caval veins, superior procedure 278, 279–82 venous switch procedures 266–70
superoinferior ventricles 366–9 similarities in parallel arterial trunk 318 ventricle(s) 17–19
supraventricular crest 38–9, 40, 83, 152, terminology 261–4, 276 margins 18–19
155, 226 venous switch procedures 266–70 morphological analysis 132–3, 134, 143
supraventricular tachycardia see also congenitally corrected transposition univentricular atrioventricular connections
atrial fibrillation 122–3, 126 transverse sinus 14–15, 45, 59, 68, 99 136–9, 145
atrial flutter 120–1 triangle of Koch 25–8, 29–30, 72, 112, 119, 152 ventricular relationships 143–5
atrioventricular nodal tachycardia 119 tricuspid atresia 137, 141, 199, 203, 251, solitary 136, 139, 140, 254, 256
postoperative 124–6, 260, 270 254–60 superoinferior 366–9
Wolff–Parkinson–White syndrome tricuspid valve see also left ventricle; right ventricle
114–19 anatomy 34, 61–6, 70–2 ventricular pre-excitation 113–19
surgical approaches 19 conduction axis and 85 ventricular septal defects 186–99
left-sided thoracotomy 3–6 Fontan procedure 125, 259–60, 261 categorisation 186, 188
median sternotomy 2–3, 5, 16 straddling valve 200, 201 in common arterial trunk 306–7
right-sided thoracotomy 6–12 coronary arteries and 72, 75, 89, 97, 98, 100 conduction axis 187–91
‘swiss-cheese’ ventricular septal defect 193 dilation 201 in congenitally corrected transposition 283–8,
dysplasia 205, 206 291
Ebstein’s malformation 117, 202–4, 262 in double-inlet ventricle 247–8
T in congenitally corrected transposition large defects not to be confused with 247, 254,
289, 290 257
tachycardia see supraventricular tachycardia; fibrous annulus rare/absent 59, 62, 72 in double-outlet ventricle 294–9
ventricular tachycardia imperforate 140–1, 142, 260, 261–2 doubly committed and directly juxta-arterial
Taussig–Bing malformation 200, 297, 299, 300–1 leaflets 36–7, 38–9, 61–2, 63, 70–1 187, 195–8, 300
tendon of Todaro 25, 32, 59, 63, 112, 113 anteriosuperior 70 congenitally corrected transposition
terminal crest 19, 22 inferior 71 286, 288
terminal groove 19, 20 septal 70, 73, 190 tetralogy of Fallot 226, 230
tetralogy of Fallot 146–147, 225–34 offset with respect to mitral valve 26, 30 transposition 273, 274, 277
absent pulmonary valve syndrome 239 papillary muscles in interrupted aortic arch 339, 340
aortic connection 231–2 anterior 41, 231 muscular 186, 191–5
conduction axis 226, 228–9 medial 40, 70, 73 tetralogy of Fallot 226, 228
left superior caval vein 323 parachute deformity 208 transposition 270, 272, 272, 273
pulmonary atresia 224, 232–4 straddling/overriding 142–3, 189, 199, 200, perimembranous 186–91, 199
subpulmonary obstruction 227–31 201–3 congenitally corrected transposition 287
ventricular septal defects 226, 227, 228, 230 in congenitally corrected transposition 290–1 and doubly committed 198
Thebesian valve 106–7, 108, 323 in transposition 272–3 tetralogy of Fallot 226, 227
thoracic artery, internal 2, 3, 7 tendinous cords 62, 63–4, 70, 71, 74 transposition 270, 271, 272
thoracic duct 6, 10, 107–10, 337, 350 in ventricular septal defect 189, 190, 199 in tetralogy of Fallot 226, 227, 228, 230
thoracotomy transposition 270–1, 272–3 in transposition 270–3, 277
left-sided 3–6 zones of apposition 37 tricuspid valve and
median sternotomy 2–3, 5, 16 see also atrioventricular valves; tricuspid atresia atresia 260
right-sided 6–12 truncal valve 303, 307, 308–9 cleft 190
thymus gland 2, 3, 4, 45 truncus arteriosus see common arterial trunk
382 Index
ventricular septal defects (cont.) valvar/truncal relationships 148, 149

overriding/straddling 189, 199, 270–1, valves 145 W
272–3 ventriculoinfundibular fold 83, 147, Warden operation 159
ventricular septal hypoplasia 179–81 152, 155 Waterston’s groove (interatrial groove) 19, 22, 26,
ventricular septum 151–5 double-outlet ventricle 297 28, 34, 35, 251
ventricular tachycardia 123 ventricular septal defect Wolff–Parkinson–White syndrome 114–19
ventriculoarterial junctions 52–3, 77 194–5
common see common arterial trunk vestibule
morphological analysis 145–8 of mitral orifice 35, 37
connections 145 of tricuspid orifice 34
X
infundibulum 145–8 visceral heterotaxy 129, 369 xiphoid process 2

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Wilcox's Surgical Anatomy of The Heart 4th Ed

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Wilcox’s Surgical Anatomy

Robert H. Anderson, BSc, MD, FRCPath

Diane E. Spicer, BS, PA(ASCP)

Andrew C. Cook, BSc, PhD

Cambridge University Press is part of the University of Cambridge.

Preface page vii

Surgical approaches to the heart 1

Anatomy of the cardiac chambers 13

Surgical anatomy of the valves of the heart 51

Surgical anatomy of the coronary circulation 90

Surgical anatomy of the conduction system 111

Analytical description of congenitally malformed hearts 128

Lesions with normal segmental connections 150

Lesions in hearts with abnormal segmental connections 244

Abnormalities of the great vessels 321

Positional anomalies of the heart 363

Long axis of body

Long axis of heart

Fig. 1.1 The computed tomogram, with the

Thymic veins Brachiocephalic vein Sup.

Fig. 1.2 This view, taken at autopsy,

Left phrenic nerve

Left vagus & recurrent laryngeal nerve

Left internal thoracic artery

Fig. 1.6 As shown in this cartoon of a median

Left pericardiophrenic vein

Left phrenic nerve

Fig. 1.7 This operative view, taken through a

Right atrial Left

Right phrenic nerve

Fig. 1.9 This operative view, taken through a

Right phrenic nerve

Left phrenic nerve

Bloodless triangle Fig. 1.12 The cartoon shows the location of

Left vagus nerve

Left phrenic nerve

Aortic isthmus Left subclavian artery

Thoracic Inf. Sup.

Fig. 1.17 This anatomical image, taken at

Sup. Inf. Fig. 1.18 This operative view, taken through

Left appendage Left

Pulmonary trunk Sup. Inf.

Right Clamp in transverse

Fig. 2.4 Operative view through a median

Pulmonary trunk Oblique ligament Left pulmonary veins

Coronary sinus Sup.

Left pulmonary veins

Left atrial appendage Left

Fig. 2.8 Operative view through a median sternotomy showing

Left atrial appendage

Fig. 2.9 The operative view through a median sternotomy shows

Left superior caval vein

Systemic venous sinus Inf.

Right Right atrial

Crest of appendage Left

Sinus node Terminal groove

Left Roof of left atrium

Fig. 2.15 Operative view through a median sternotomy showing

Superior caval vein Waterston’s groove

Inferior caval vein

Terminal crest Deficient oval fossa

Roof of left atrium

Artery to sinus node