Irsad Andi Arso

Overview

US: 1,000,000 adults with congenital

heart dz
20,000 more patients reach
adolescents yearly

*All figures from ACCSAP V unless otherwise noted

Adult Congenital Heart Disease

Atrial Septal Defect

Ventricular Septal Defect
Patent Ductus Arteriosus
Coarctation of Aorta
Tetralogy of Fallot
Ebstiens Anomaly

Hole between the two atria

Blood flows left to right
PFO Patent foramen ovale fails to
close
Right heart becomes dilated
Too much blood to the lungs

Three types

Primum ASD
Secundum ASD
Sinus venosus
AVSD Atrio ventricular septal
defect

Atrial Septal Defect

1/1500 live births

Secundum

most common ACHD (6-10%)

RAD

Primum

associated with other endocardial cushion defects (cleft

AV valves, inlet type VSD)
LAD

Sinus Venosus

large, associated with anomalous pulmonary venous

drainage (usually R superior PV)

Coronary sinus (rare)

associated with unroofed coronary sinus

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ASD- Anatomy/Prevalence
Secundum 75%
Primum 15%
Sinus Venosus 10%
Cor Sinus (rare)

Braunwaulds Heart Disease, 6th ed

Physiologic Consequences

Shunt Flow
Size of defect
Relative compliance of ventricles
Relative resistance of pulmonary/systemic circulation

LR shunting results in diastolic overload of RV and

increased pulmonary blood flow
RV dilatation/failure and rarely severe pulm HTN
(Eisenmengers) may ensue over time ~5%
With age, deterioration chiefly due to

decrease LV compliance, increased LR shunt

increase in atrial arrhythmias
pulm HTN develops, RV volume + pressure OL
1

Perloff, NEJM 1995

Clinical Symptoms

Often asymptomatic until 3-4th decade

for moderate-large ASD, may present
later in life for initially smaller ASD
Fatigue
DOE
Atrial arrhythmias
Paradoxical Embolus
Recurrent Pulmonary infections
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Physical Signs

S2 wide/fixed splitting
RV/PA palpable impulse (if lg defect)
systolic ejection murmur 2nd L ICS
mid-diastolic TV rumble

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Auscultation in ASD

Increased flow across the pulmonary

valve produces a systolic ejection murmur
and fixed splitting of the second heart sound.
Fixed splitting of S2 may in part be due to
delayed right bundle conduction.
Increased flow across the TV produces a
diastolic rumble at the mid to lower right
sternal border.

Older pt loses pulm ejection

murmur as shunt becomes
bidirectional
signs of pulm HTN/ CHF may
predominate

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ECG

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ECHO

Subcostal
view of
Intraatrial
Septum

Color
Flow/
Contrast

Good for
secundum
, primum
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Treatment

Medical : diuretics, ACEI, Aldactone

Repair
Consider when sxs, Qp:Qs>1.5
Interventional (Percutaneous Closure)
Only for secundum defects
94-96% success (Amplatzer)
adequate superior/inferior rim around ASD
no R-L shunting

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Treatment

Surgical Closure
Good prognosis:
closure age < 25, PA pressure <40
If >25 or PA>40, decreased survival due to
CHF, stroke, and afib

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Hole between the two ventricles

Left to right shunt majority
Dilated right heart too much blood
to lungs increase in pulmonary
pressure
Smaller defects can close
spontaneously
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Three types

Perimembranous VSD most common

Muscular VSD can be multiple
Apical VSD usually small
Variable in size

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Ventricular Septal Defect

May be anywhere in
intra-ventricular septumclinical course depends
on the shunt size and
involvement of
pulmonary vascular bed.
Approx of all VSDs are
small, and more than
close spontaneously.
Highest closure rates in the
first decade of life.

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PATHOPHYSIOLOGY

primarily depends on size&status of pulm. vascular bed rather

than location
Small communication (less than 0.5cm`) VSD is restrictive &
rt.ventricular pressure is normal does not cause significant
hemodynamic derangement(Qp:Qs=1.75:1.0)
Moderately restrictive VSD with a moderate shunt(Qp:Qs=1.52.5:1.0) &poses hemodynamic burden on LV
Large nonrestrictive VSDs(more than 1.0cm`) Rt&Lt
ventricular pressure are equalised(Qp:Qs is more than 2:1)
Large VSDs at birth ,PVR may remain higher than normal and
Lt to Rt shunt may intially limited involution of media of small
pulm.arterioles,PVR decreaseslarge Lt to Rt shunt ensues
In some infants large VSDs ,pulm. arteriolar thickness never
decreases pulm.obstructive disease develops .when
Qp:Qs=1:1 shunt becomes bidirectional,signs of heart failure
abate &pt. becomes cyanotic. (Eisenmenger syndrome)
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CLINICAL FEATURES

Race : no particular racial predilection

Sex :no particular sex preference
Age :
infants difficult in postnatal period,although ccf
during first 6mths is frequent,X-ray&ECG are
normal.
childrenafter first year variable clinical picture
emerges.
small VSD asymptomatic
large VSD:
-palpitation,dyspnoea on exertion,feeding
difficulties ,poor growth
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-frequent chest infections

PHYSICAL FINDINGS

Pulse pressure is relatively wide

Precordium is hyperkinetic with a systolic thrill at LSB

S1&S2 are masked by a PSM at Lt.sternal border

,max. intensity of the murmur is best heard at
3rd,4th&5th Lt interspace.Also well heard at the 2nd
space but not conducted beyond apex

Lt. 2nd space widely split &variable accentuated P2

Delayed diastolic murmur at the apex &S3

Presence of mid-diastolic ,low pitched rumble at the

apex is caused by increased flow across the mitral
valve

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INVESTIGATIONS

CHEST RADIOGRAPHY
- normal
- biventricular hypertrophy
- pulmonary plethora

ELECTROCARDIOGRAPHY
-smallVSD ~ normal tracing
-mod.VSD ~ broad,notched P wave characteristic of Lt. Atrial
overload as well as LV overload,namely,deep Q waves & tall
R waves in leads V5 and V6 and often AF
-large VSD ~RVH with rt. axis deviation. With further
progression biventricular hypertrophy;P waves may be
notched/peaked.

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INVESTIGATIONS .

ECHOCARDIOGRAPHY
two-dimensional &doppler colour flow

ANGIOGRAPHY
(cardiac catheterization and angiography)

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COMPLICATIONS

Congestive cardiac failure

Infective endocarditis on rt.ventricular side
Aortic insufficiency
Complete heart block
Delayed growth & development (FTT) in
infancy
Damage to electrical conduction system
during surgery(causing arrythmias)
Pulmonary hypertension
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INTERVENTION

3 MAJOR TYPES
SMALL (less than 3mm
diameter)
- hemodynamically
insignificant
- b/w 80-85% of all VSDs
- all close spontaneously
* 50% by 2yrs
* 90% by 6yrs
* 10% during school yrs

- muscular close sooner

than membranous
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MODERATE VSDs
* 3-5mm diameter
* least common group of children(3-5%)
* w/o evidence of ccf/ pulm.htn can be
followed until spontaneous closure
occurs.
LARGE VSDs WITH NORMAL PVR
* 6-10mm in diameter
* usually requires surgery Conservative
treatment

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DEFINITION
Patent ductus arteriosus
(PDA) is a heart problem
that is usually noted in
the first few weeks or
months after birth. It is
characterized by a
connection between the
aorta and the pulmonary
artery, which allows
oxygen-rich (red) blood
that should go to the
body to re-circulate
through the lungs

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IN DEPTH

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IN GROSS

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HEMODYNAMICS

LT TO RT SHUNT
OCCURS BOTH
DURING SYSTOLE
& DIASTOLE
LARGE AMT OF
BLOOD PASSES
THRU PULM ART-LT
ATRIUM MITRAL
VALVE

CONTINOUS
MURMUR

ACCENTUATED S1
MITRAL DELAYED
DIASTOLIC
MURMUR

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HEMODYNAMICS

LARGE FLOW THRU

LT VENT-DELAYED
CLOSURE OF
AORTIC VALVE
DILATATION OF
ASCENDING AORTA

LATE A2
PARADOXICALLY
SPLIT S2
AORTIC EJECTION
CLICK
AORTIC EJECTION
SYSTOLIC
MURMUR
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PRESENTATION

Frequent chest infections

fatigue
sweating
rapid breathing
heavy breathing
congested breathing
disinterest in feeding, or tiring while feeding
poor weight gain
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ON EXAMINATION

INSPECTION
CAROTID
PULSATIONS
HYPERKINETIC & LT
VENTRICULAR TYPE
OF APICAL IMPULSE

PALPATION
SYSTOLIC OR
CONTINOUS THRILL
AT 2ND LT
INTERSPACE

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AUSCULTATION

ACCENTUATED S1
NARROWLY OR PARADOXICALLY SPLIT
S2
LOUD P2
CONTINOUS/GIBSONS/TRAIN-INTUNNEL MURMUR BEST HEARD IN
INFRACLAVICULAR REGION
MITRAL DELAYED DIASTOLIC MURMUR
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CXR FINDINGS

CARDIOMEGALY
LA ENLARGEMENT
LV ENLARGEMENT
PROMINENT
AORTIC KNUCKLE
PULM PLETHORA

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ECG CHANGES

NORMAL AXIS WITH LT

VENTRICULAR HYPERTROPHY

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ECHO PICTURES

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MANAGEMENT

MEDICAL
INDOMETHACIN 0.1
mg/kg/dose,orally,bd for three doses
Digoxin for increasing working capacity
of heart
Diuretics to reduce preload on heart

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SURGICAL REPAIR :DEVICES

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Coarctation of the Aorta

Males twice as frequently as females.

98% of all coarctations at segment of
aorta adjacent to ductus arteriosus.
Produced by both an external narrowing
and an intraluminal membrane.
Blood flow to the lower body maintained
through collateral vessels.

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Narrowing in
proximal descending
aorta
May be long/tubular
but most commonly
discrete ridge
Natural hx: poor
prognosis if
unrepaired
Aortic
Aneurysm/dissection
CHF
Premature CADz

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CLINICAL

Absent or weak femoral pulses.

Systolic pressure higher in upper
extremities than in lower
extremities; diastolic pressures are
similar.
Harsh systolic murmur heard in the
back.
Rib notching on CXR pathognomonic
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Rib notching

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Treatment

Despite surgery, patients still have significant

morbidity/mortality with average age 38
Up to 70% of repaired patients still go on to
develop HTN, pathology not well understood
Recurrence in 8-54% of repairs, can undergo
repeat surgery or balloon angioplasty
Aortic Aneurysm/ruputure may occur despite
successful repair and correction of HTN (freq
around anastomosis site on patch repair
30% in one study)
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Tetralogy of Fallot

4 features
Malalignment VSD
Overriding Aorta
Pulmonic Stenosis
RVH

Variability correlates
with degree of RVOT
obstruction and
size/anatomy of PA
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ToF: Surgical Treatment

Systemic Pulmonary Shunt

Blalock-Taussig
Waterston (RPA)
Potts (LPA)

Complete Repair

takedown of prior shunt

patch VSD
resection of subpulmonic obstruction
transannular patch around pulm valve annulus
(usually leads to severe PI)
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Ebsteins Anomaly

Atrialization of RV, sail-like

TV, TR
50% ASD/PFO
50% ECG evidence of
WPW
Age at presentation varies
from childhoodadulthood
and depends on factors
such as severity of TR,
Pulm Vascular resistance in
newborn, and associated
abnormalities such as ASD

www.ucch.org

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Massive cardiomegaly,
mainly due to RAE

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Ebsteins: Clinical Presentation

Pediatric
murmur

Adult (unrepaired with ASD)

atrial arrhythmias
murmur
cyanosis

RL shunt NOT due to PulmHTN but TR jet directed

across ASD

exercise intolerance

Surgery in pts with significant TR/sxs

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Eisenmengers Syndrome

Final common pathway for all

significant LR shunting in which
unrestricted pulmonary blood flow
leads to pulmonary vaso-occlusive
disease (PVOD); RL
shunting/cyanosis devleops
Generally need Qp:Qs >2:1

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Eisenmenger Complications

Coagulopathy/platelet consumption
Brain abcesses
Cerebral microemboli
Airway hemorrhage
especially moving from lowerhigher
altitudes (air travel, mountains)

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Eisenmenger: Treatment

Sxs +polycythemia phlebotomy

Careful if microcytosis, strongest predictor of
cerebrovascular events

RULE OUT CORRECTABLE DISEASE

Once diagnosis established, avoid
aggressive testing as many patients die
during cardiovascular procedures

Diuretics prn, oxygen

Definitive: Heart Lung transplant

Prostacyclin therapy may delay, expensive
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