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Seminars in Pediatric Surgery 22 (2013) 174178

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Seminars in Pediatric Surgery


journal homepage: www.elsevier.com/locate/sempedsurg

Neonatal cardiovascular physiology


Michael H. Hines, MD, FACS
Pediatric Cardiovascular Surgery, University of Texas Medical School at Houston, 6431 Fannin St, MSB 6.264, Houston, Texas

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

According to the World Health Organization, one in 33 infants


born has one or more congenital anomaly.1 Some of these children
will require surgical treatment in the neonatal period for defects of
the heart, neural tube, GI tract, etc., and other children may require
intervention in their rst month of life for acquired disorders
including necrotizing enterocolitis (NEC), reux, and feeding disorders. In order for the pediatric surgeon to properly care for
patients with these various problems in the neonatal period, he or
she must have some basic understanding of the normal physiology
of the neonatal heart and circulation, as well as the impact of
associated congenital heart defects, since many children are born
with defects involving multiple organs and organ systems. In this
article, we will rst review basic neonatal cardiac physiology,
focusing on the differences between older children and adults,
and the unique characteristics of the neonatal myocardium. Then
we will discuss the physiology of the major groups of cardiac
defects in neonates, including left-to-right shunts, right-to-left
shunts, obstructive lesions, and single-ventricle physiology. The
focus of each section will be to address how the neonatal cardiac
anatomy and physiology impacts the pediatric surgeon's perioperative care for neonates with non-cardiac lesions, defects, and
diseases.

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

E-mail address: michael.h.hines@uth.tmc.edu


1055-8586/$ - see front matter & 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1053/j.sempedsurg.2013.10.004

M.H. Hines / Seminars in Pediatric Surgery 22 (2013) 174178

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.

Transition to neonatal circulation


In what often appears to be a somewhat seamless transition
from the placenta to the native lungs for gas exchange, several
very signicant changes occur in the newborn circulation in the
rst few minutes of life, namely ination of the lungs, with
dramatic lowering of the pulmonary vascular resistance, and
separation from the placenta, with concurrent rise in systemic
vascular resistance. Since the direction of ow across the ductus
arteriosus is solely determined by these two forces, these changes
lead to the shift from fetal right-to-left ow to the newborn's leftto-right ow from the aorta into the pulmonary artery. Through a
combination of the change in direction of ow, an increase in pO2,
a drop in PGE synthesized by the placenta, along with chemical
changes such as in bradykinin, the unique contractile elements
within its walls, cause the ductus arteriosus to gradually close
physiologically over the rst 2448 h of life. As the pulmonary
resistance drops and the pulmonary blood ow increases, the
volume returned to the left atrium increases. The RV compliance
gradually increases as well, and the resultant change in the
differences in ventricular compliance allows the ap of the
foramen ovale to gradually close over in the rst few days to
weeks of life, minimizing mixing, so that the outputs of the left
and right ventricles become equivilent.2 When the foramen ovale
or ductus arteriosus does NOT close under the normal circumstances described, they are then referred to as patent, suggesting
an abnormal condition of patent ductus arteriosus (PDA) and
patent foramen ovale (PFO). In rare patients, the usual mechanisms do not occur, primarily related to failure of the PVR to drop,
and the patient continues to have signicant mixing through both
the PDA and PFO, leading to signicant hypoxemia. This has
classically been referred to as persistent fetal circulation and is
often seen as a result of persistent pulmonary hypertension of the
newborn (PPHN), sepsis, or diaphragmatic hernia (CDH). Under
these circumstances, the RV continues to contribute a large portion
of the cardiac output and the post-ductal saturations will be
signicantly lower than the pre-ductal saturations. Until the PVR
drops, the normal transition and changes in blood ow as well as
the normal progression of neonatal myocardial maturation and
hypertrophy described below are delayed. In premature infants,
the transition may be much slower, and at times, the ductus
arteriosus may not even close despite the usual changes in
systemic and pulmonary vascular resistance and the subsequent
reversal of ow. Whether this is due to lack of the appropriate
biochemical signals or inadequate maturation of the contractile
elements within the wall of the ductus is unknown. Pediatric
surgeons must recognize this possibility and the impact of a
potential large left-to-right shunt on organ perfusion (particularly
the gut), as well as uid status, urine output, etc. On occasion, the
PDA may need to be addressed prior to other procedures in order
to lessen the risk of other required surgery and anesthesia.
Normal neonatal myocardium
A great deal of biochemical, histologic, and physiologic details
are available regarding the changes the myocardium undergoes in

175

the transition from fetal to neonatal to pediatric structural and


functional states.3 However, practically speaking, there are two
major points signicant for pediatric surgeons operating on and
caring for neonates. First, the neonatal myocardium is immature
with less cellular (mitochondrial and DNA) and structural (myobril) organization, less contractile proteins (actin and myosin), and
less compliance (stiffer). This means that while it can respond to
volume on the FrankStarling curve, it does so to a much lesser
extent than mature myocardium. Thus, the neonatal heart
increases its cardiac output primarily through increases in heart
rate. While extremely fast rates of 200 or greater are sometimes
poorly tolerated because of very short lling times, one must be
very cautious about pharmacologically slowing the neonatal heart
rate when it appears to be too fast in the 170 or 180 range,
particularly in a neonate who is sick, infected, or otherwise
stressed and needs a higher cardiac output that the heart can
only achieve with a high rate. Similarly, the failing neonatal heart
that is severely volume loaded cannot respond to catecholamine
inotropic support with the usual increase in contractility, and it
often responds better to diuretics for uid removal and afterload
reduction with systemic vasodilators. The phosphodiesterase
inhibitor milrinone can be very useful in these circumstances as
it works as a systemic and pulmonary vasodilator as well as
provides some inotropic support without the increase in myocardial oxygen demand seen with catecholamines. Evaluation with
transthoracic echocardiography can easily evaluate the contractility and adequacy of preload for both the left and right ventricles,
as well as being able to often estimate the pulmonary artery
pressures if there is any visible tricuspid insufciency. This
information can often help tremendously in guiding therapy for
a very sick neonate. Then over the subsequent weeks to months of
life the neonatal myocardium undergoes rapid maturation, hypertrophy, and organization to be able to perform similar to mature
adult cardiac muscle.
The second important principle for pediatric surgeons relates to
calcium metabolism. Unlike mature cardiac muscle, the neonatal
myocardium has an underdeveloped sarcoplasmic reticulum and is
much more dependent on extracellular calcium sources. Signicant decreases in contractility and subsequent hypotension can be
observed when ionized calcium levels fall, and the drop can be
acute and dramatic. Careful attention to neonatal ionized calcium
levels is essential in the perioperative period, particularly when
neonates are not being fed enterally, are stressed or septic, are on
loop diuretics that promote calcium loss such as furosemide, or
have conditions associated with hypoparathyroidism or hypocalcemia such as DiGeorge syndrome, or the rst few days after
hypothermic cardiopulmonary bypass or deep hypothermic circulatory arrest.

Physiology of congenital heart defects


While the majority of patients treated by pediatric surgeons
have isolated congenital anomalies of the intestine, lung, airway,
biliary tree, etc., it is not at all unusual to treat a child with
multiple anomalies including structural cardiac defects. As congenital heart disease contributes to more neonatal deaths than any
other group of congenital anomalies, the presence of a structural
heart defect often has major implications in the timing and
procedure of choice for other congenital anomalies. It is also
common for the neonate with complex congenital heart disease
to require the care of the pediatric surgeon to either assist in the
patients recovery (gastrostomy tubes, peritoneal catheters, and
long-term intravenous access) or to manage direct complications
related to the cardiac disease, surgical repair, or palliation (malrotation, bowel perforations, and gastrointestinal bleeding).

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Subsequently, it is important for the pediatric surgeon to have


some understanding of the basic categories of heart defects and
the implications of such defects on their care of general surgical
problems in these complex patients. Here, we will discuss the
basic physiology and impact of left-to-right shunts, right-to-left
shunts, obstructive lesions, and single-ventricle physiology on the
perioperative care of these patients by pediatric surgical teams.
Perhaps the most important feature for this group of patients is
assuring that the patient is cared for by an anesthesiology team
that thoroughly understands the patient's physiology, particularly
for neonates with uncorrected congenital defects or with palliated
single-ventricle physiology. Even the simple day-to-day common
application of 100% oxygen prior to induction of anesthesia and
intubation by an ill-prepared anesthesiologist can be lethal in a
neonate with the potential for a large left-to-right shunt, such as
truncus arteriosus, where a severe drop in pulmonary vascular
resistance steals essentially all the cardiac output, resulting in
extremely low diastolic blood pressure, coronary ischemia, and
ventricular brillation. While many infants with surgically corrected congenital defects such as D-transposition or total anomalous pulmonary venous connection may behave like any other
child without congenital heart disease, others may still have some
residual defects or physiology such as pulmonary hypertension
that still warrant extreme intraoperative care. Involving pediatric
cardiac anesthesia in the planning process and management can
often prevent intraoperative instability or even a life-threatening
crisis.

Left-to-right shunts (ASD, VSD, CAVC, and PDA)


In neonates, atrial septal defects, whether a common atrium or
a small patent foramen, are rarely hemodynamically signicant
other than their ability to provide a pop-off for a poorly functioning or hypoplastic ventricle. To understand why even very large
atrial septal defects do not cause problems, we must look at the
physiology of the defect. There is a commonly held concept that
the direction of ow across an ASD is determined by the difference
in pressures between the right and left atrium; however, this is
actually not correct. Were it accurate, then a very large ASD or
even a common atrium where the pressure is equal in the entire
chamber would then have no shunt, but we know that it is not the
case. In fact, the direction of ow is determined by the differences
in right and left ventricular compliance or stiffness. Consider the
ow of venous return into the heart into a common atrium. The
majority of ow occurs during diastole when the AV valves (mitral
and tricuspid) are open, so that effectively the blood has a choice
to enter either the LV or the RV. Because under normal circumstances, the RV is thinner, less muscular, and signicantly more
compliant than the left ventricle, there is a net left-to-right shunt.
Now imagine that the atrial septal defect slowly gets smaller and
reaches a point in which the amount of blood that would cross the
septum based on the physics of the ventricles (compliance) can no
longer cross because the smaller hole restricts the amount of
ow that can cross. At that point, the ASD is described as
restrictive and a pressure gradient is created across the hole.
So the pressure gradient is the result of ow against resistance,
rather than the driving force of the shunt. This important principle
applies not only to atrial defects but also to the ventricular and
great artery defects and connections and even to general arterial
owthe pressure is the result of the ow (cardiac output) against
the resistance (SVR). Because the neonatal right heart spent its
entire fetal life pumping the majority of the cardiac output against
systemic resistance, at birth it is thick and muscular, and therefore
less compliant than it will be a month or so later as it relaxes and
remodels, having to subsequently only pump against pulmonary

vascular resistance. Therefore, there is rarely any signicant ow


across even a large ASD in the rst month or so of life, unless there
is some problem (obstruction or resistance to lling) with one of
the AV valves or owing to an underdeveloped ventricle.
At birth, isolated ventricular septal defects are also rarely of
hemodynamic signicance. The direction and volume of ow
across a VSD is determined by the difference between the systemic
and pulmonary vascular resistance as the ow occurs during
systole, when the semilunar valves are open and the AV valves
are closed. Because the pulmonary resistance is so high at birth
and drops steadily in the rst hours and days of life, a majority of
VSDs may not be diagnosed until the rst visit at the pediatricians
when the PVR has dropped and the ow across the VSD now
causes a murmur. VSDs are also said to be restrictive when their
size limits the amount of ow to less than the low PVR would
accept, thus creating a pressure gradient between the ventricles. In
general, this occurs when the VSD is smaller than the effective
orice of the aortic valve. In the presence of other defects such as
obstruction of the RV outow tract as in tetralogy of Fallot (TOF),
the VSD becomes very signicant and a murmur may be heard
very early in life. The physiology of this defect will be discussed
further in the next section.
A complete atrioventricular canal defect involves a primum
atrial defect just above the AV valves, and inlet-type VSD just
below the AV valves, and a common AV valve due to lack of normal
division into separate mitral and tricuspid components. The
physiology of ow is similar to each of the defects at the atrial
and ventricular levels, and the amount of left-to-right shunt
increases as the PVR drops signicantly, and the RV compliance
drops slightly. These patients may become symptomatic even in
the rst few weeks of life, depending on the rate of fall of the PVR
and the degree of insufciency of the common AV valve.
Finally, the patent ductus arteriosus connects the aorta and the
pulmonary artery at the level of the distal arch, well above and
beyond the semilunar valves. This means shunting (to the side of
lower resistance, usually into the lungs) occurs not only during
systole but also during diastole, causing overcirculation and early
symptoms, particularly in premature infants who also suffer from
underdeveloped lungs. This lesion can be very signicant in the
neonatal period. Smaller PDAs may not become symptomatic until
much later or may not ever cause symptoms depending on
their size.

Right-to-left shunts (TOF, D-TGA, and TAPVC)


The major factor of interest to pediatric surgeons in patients
with congenital heart disease with right-to-left shunts is cyanosis,
related to either inadequate pulmonary blood ow (TOF) or the
inability of the oxygenated blood to get out to the systemic
circulation (D-TGA and TAPVC). Most infants with cyanotic heart
disease can still maintain adequate cardiac output. So that even
with compromised pulmonary blood ow, the patient can maintain adequate oxygen delivery as long as the arterial saturation is
not too low and the hemoglobin remains adequate. The presence
of a metabolic acidosis and lactic acidosis is a sign that the oxygen
delivery is not adequate and some intervention is required to
improve one of those three factorscardiac output (volume),
oxygen carrying capacity (transfusion), or oxygen saturation of
the bloodwhich may involve improving mixing with a Rashkind
balloon atrial septostomy or placement of an aortopulmonary
shunt, for example. In the absence of a metabolic acidosis from
inadequate oxygen delivery, most stable neonates with cyanotic
heart disease have normal blood pressure, organ perfusion, urine
output, have normal wound healing, and can tolerate enteral feeds.
This is in contrast to the previously discussed patients with

M.H. Hines / Seminars in Pediatric Surgery 22 (2013) 174178

signicant left-to-right shunts and overcirculation heart failure,


who may demonstrate poor growth, tachypnea, feeding difculty,
and be in a catabolic state with poor organ perfusion and wound
healing, despite high oxygen saturations. It is important to
recognize this discrepancy between saturations alone and wellness of a neonate with complex heart disease, and as always,
thorough consultation with the team is helpful in perioperative
planning and care.

Obstructive lesions (AS, PS, and COA)


The presence of volume loading to any chamber of the heart
leads to dilation, so that with mitral regurgitation the left atrium
dilates, and with aortic insufciency the left ventricle dilates, just
as it does with volume loading from a left-to-right shunt of a large
PDA. The cardiac muscle responds differently to the increase in
afterload from an obstructive lesion such as aortic stenosis, mitral
stenosis, or coarctation of the aorta. As it is required to generate
more force to overcome the afterload of the obstruction, the
ventricle gets hypertrophied, and this can be extensive even in a
neonate. If not addressed, the ventricle will eventually dilate and
demonstrate decrease in contractility. Once the obstruction is
relieved, the ventricular function will usually recover. In most
circumstances, the rst-line treatment for aortic and pulmonary
stenosis is balloon dilation in the cardiac catheterization suite, but
occasionally surgery is required. The presence of a severe coarctation of the aorta in the juxtaductal position in a newborn (critical
coarctation) can cause similar left ventricular dysfunction and
shock, and frequently requires resuscitation with inotropes, opening of the ductus with prostaglandin infusion to relax the
coarctation and allow the right ventricle to pump blood to body
distal to the coarctation (a return to fetal circulation) until the
narrowing is addressed surgically. Neonates with untreated
obstructions have ventricles that generate very high systolic
pressures, and because they cannot empty well, they also have
higher diastolic pressures. Since coronary ow occurs during
diastole, the ventricle can be signicantly ischemic, with a lower
threshold for brillation and other arrhythmias, so the pediatric
surgeon should take extreme care in the use of guidewires during
placement of central access in these patients. As these patients are
also dependent on adequate preload, dehydration from inadequate
intravenous uids perioperatively or acute vasodilation from
sedation or narcotics should also be avoided.

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

177

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.

Other special circumstances


As discussed, neonates with complex congenital heart disease
that have been palliated with procedures where the resultant
circulation provides optional pulmonary blood ow (that is ow
can go to either the systemic or pulmonary circulation depending
on the systemic and pulmonary vascular resistances) are at
increased risk for crisis and sudden death with even minor
episodes of aspiration from reux, feeding issues, particularly if
they also have paralyzed vocal cords from recurrent nerve injuries,
etc. They not only have less reserve than neonates with normal
hearts and circulation but also have more reactive pulmonary
vascular beds that can clamp down under stress or in a respiratory
crisis. Unlike a normal neonatal heart that will just force blood

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M.H. Hines / Seminars in Pediatric Surgery 22 (2013) 174178

through the lungs and tolerate elevated right-sided pressures, the


palliated heart simply pumps all the blood to the systemic
circulation with little to no pulmonary blood ow, leading to rapid
hypoxemia and arrest. In light of this fragility, many congenital
heart surgeons and pediatric cardiologists may encourage our
pediatric surgery colleagues to err well on the conservative side
by strongly considering the benet of a Nissen fundoplication
when placing a gastrostomy tube if the child shows any history or
suggestion of signicant reux. Similarly, patients with heterotaxy
syndrome and malrotation commonly have complex congenital
heart disease that usually requires palliation and a single-ventricle
pathway. While debate remains among pediatric surgeons on the
proper timing of surgery to treat malrotation, many congenital
programs favor proceeding with the Ladd procedure prior to
discharge from the initial hospitalization. Mortality remains very
high in neonates with palliated single ventricle who suffer from a
gastrointestinal complication such as a bowel perforation or segment of dead gut, primarily because these complex patients have
little to no reserve and cannot survive the signicant stress of this
type of severe event with shock, sepsis, and hypotension. As a
result, pediatric surgeons may nd themselves under some pressure to proceed with the Ladd procedure in these patients. Hopefully, appreciation for the physiology as described above, the lack
of physiologic reserve in these patients, and the rationale for
requesting this aggressive approach will allow the pediatric
surgeon to put the request in the proper perspective.

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
1. Congenital Anomalies. World Health Organization fact sheet N370, October 2012.
www.who.int/mediacentre/factsheets/fs370/en/index.html.
2. Sharma A, Ford S, Calvert J. Adaptation for life: a review of neonatal physiology.
Anesth Intensive Care Med. 2010;12(3):8590.
3. Price Jack F. Unique aspects of heart failure in the neonate. In: Shaddy RE, editor.
Heart Failure in Congenital Heart Disease: From Fetus to Adult. London, Dordrecht,
Heidelberg, New York: Springer-Verlag; 2011. p. 2142.