a r t i c l e in f o
Keywords:
Neonatal
Cardiac physiology
Congenital heart disease
abstract
The pediatric surgeon deals with a large number and variety of congenital defects in neonates that
frequently involve early surgical intervention and care. Because the neonatal cardiac physiology is
unique, starting with the transition from fetal circulation and including differences in calcium
metabolism and myocardial microscopic structure and function, it serves the pediatric surgeon well to
have a sound understanding of these principles and how they directly and indirectly affect their plans
and treatments. In addition, many patients will have associated congenital heart disease that can also
dramatically inuence not only the surgical and anesthetic care but also the timing and planning of
procedures. Finally, the pediatric surgeon is often called upon to treat conditions and complications
associated with complex congenital heart disease such as feeding difculties, bowel perforations, and
malrotation in heterotaxy syndromes. In this article, we will review several unique aspects of neonatal
cardiac physiology along with the basic physiology of the major groups of congenital heart disease to
better prepare the training and practicing pediatric surgeon for care of these complex and often fragile
patients.
& 2013 Elsevier Inc. All rights reserved.
Introduction
Fetal circulation
Because the fetal lungs are not inated, and the fetus obtains
oxygen and carbon dioxide gas exchange via the placenta, there
must be some level of mixing and redirection of blood ow
involving the foramen ovale, the ductus arteriosus, the ductus
venosus, as well as the umbilical vessels and placenta. For the
purpose of this discussion of neonatal cardiac physiology, we will
address only the rst two lesions. The foramen ovale (FO) is
basically a hole in the central atrial septum formed by an overlapping of the septum primum inferiorly and leftward and the
septum secundum superiorly and rightward. Its structure is such
that it primarily directs well-oxygenated blood from the umbilical
vessels, via the inferior vena cava (IVC) and directly through the
foramen into the left atrium (LA). This oxygenated blood is then
ejected out the left ventricle (LV) through the aorta to the proximal
circulation including the coronaries and head vessels. At the same
time, the mostly deoxygenated blood from the superior vena cava
(SVC) tends to stream directly into and across the tricuspid valve
into the right ventricle (RV), where it is then ejected out the
pulmonary artery (PA). However, because the lungs are not yet
inated, the pulmonary vascular resistance remains very high, and
so the blood crosses the ductus arteriosus (DA) opposite the
pulmonary artery bifurcation, entering the proximal descending
aorta just past the left subclavian artery at the end of the aortic
arch. This streaming is advantageous in fetal life as it directs the
oxygen to where it is needed most, the heart and the brain.
Because of these two connections and preferential ow patterns,
the workload of the right and left ventricles are not equal, with the
RV providing about 65% of the fetal cardiac output and the LV
ejecting the remaining 35%. The uninated lungs only receive
about 10% or less of the cardiac output.
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Single-ventricle physiology
While this is the most complex mix of patients and cardiac
lesions, the physiology comes down to just a few basic principles
regarding adequacy of pulmonary blood ow and unobstructed
ow into and out of the systemic ventricle. Since our discussion
here centers around neonatal physiology, we will not discuss later
stages of palliation, such as the bidirectional Glenn or Fontan
operations, but rather will focus on neonatal palliation with
aortopulmonary shunts, pulmonary artery banding, atrial septectomies (or balloon septostomies), and complex outow reconstructions with the Norwood or DamusKayeStansel procedures.
As long as there is unobstructed ow out of the lungs and
inow into whichever ventricle is responsible for the systemic
blood ow, the primary concern for initial palliation of singleventricle patients is establishing enough, but not too much,
pulmonary blood ow. When ow is low and the patient is
dependent on keeping the PDA open for survival, the patient will
undergo placement of some form of aortopulmonary connection,
with ligation of the PDA. The size and length of the connection is
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selected based on the lesion and the size of the patient. When
there is unobstructed pulmonary blood ow, the goal must be to
protect the lungs from overcirculation, which can lead to the
development of elevated pulmonary vascular resistance that
would prevent future palliation with the Glenn and Fontan
procedures. In addition, this improves systemic perfusion by
limiting ow to the low-resistance pulmonary vascular bed. This
can be accomplished with the placement of a restrictive band
around the main pulmonary artery distal to the top of the
pulmonary valve but proximal to the bifurcation. The goal of both
these procedures is to create a balanced circulation with good
systemic cardiac output and sufcient, but not excessive, pulmonary blood ow.
When one ventricle is underdeveloped or hypoplastic, the
other ventricle must accomplish all the work of the circulation.
In order to do this however, particularly when the hypoplastic
ventricle is the result of an underdeveloped or atretic mitral or
tricuspid valve, there must be unobstructed ow across the atrial
septum into the opposite ventricle (e.g., right to left with tricuspid
atresia and left to right with hypoplastic left heart syndrome). This
can usually be accomplished with a balloon septostomy either at
bedside in the intensive care nursery with echocardiographic
guidance or in the catheterization suite with uoroscopy. Occasionally, this is not possible and a surgical septectomy is required
using a short period of cardiopulmonary bypass and cardioplegic
arrest.
While many single-ventricle patients can be palliated with
some combination of the above procedures, patients with variants
of hypoplastic left heart syndrome (HLHS) require a much more
complex early reconstruction such as the Norwood operation.
These patients compose the highest-risk group that congenital
heart surgeons encounter. Though there are some variations on
the theme, the basic premise of this operation is to construct an
adequate conduit for blood from the pumping ventricle (usually
the RV) from the proximal main pulmonary artery and valve along
with whatever exists of the ascending aorta (since this still
provides coronary blood ow) out to the body, and then providing
sufcient, but not excessive, pulmonary blood ow through a
small connection from the arterial tree in the form of a Gortex
shunt (or directly from the RV in the case of the Sano modication). This complex operation requires not only cardiopulmonary
bypass and cardioplegic arrest of the heart but also some period of
deep hypothermic circulatory arrest. And at the end of it all, they
remain palliated with a single right ventricle supporting their
circulation. This scenario is a setup for a number of potential
complications requiring the assistance of the pediatric surgeon
including long-term intravenous access, gastrostomy or jejunostomy tubes, peritoneal catheters for drainage of ascites or even for
peritoneal dialysis, as well as dealing with more severe complications such as necrotizing enterocolitis and bowel perforations.
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Other rare cardiac defects may also appear in the neonate and
provide special challenges to the pediatric surgeon, particularly if
the primary lesion has not yet been addressed. For example,
anomalous left coronary artery off the pulmonary artery (ALCAPA)
presents with poor function, and ischemia from loss of coronary
perfusion into the low-resistance pulmonary circulation, stealing
blood form the coronary bed. Unlike the vast majority of congenital heart defects, the goal here is to keep the pulmonary resistance
high to minimize the effect on the coronary circulation, so
inadvertent hyperventilation or hyperoxia can lead to ischemia,
ventricular brillation, and sudden death. This illustrates the need
for excellent communication, cooperation, and coordination
between the pediatric surgeons, pediatric cardiologists, pediatric
cardiac anesthesiologists, and congenital heart surgeons to assure
the patients get the appropriate care during their pediatric surgery
and perioperative course.
References
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