Congenital Heart Disease

Kriti Puri, MD,* Hugh D. Allen, MD,* Athar M. Qureshi, MD*†

*Department of Pediatrics, †CE Mullins Cardiac Catheterization Laboratories, The Lillie Frank Abercombie Section of Cardiology, Texas Children’s Hospital,
Baylor College of Medicine, Houston, TX

Education and Practice Gaps

Congenital heart disease (CHD) is present in about 9 of every 1,000 live-
born children. (1)(2)(3)(4)(5) Children with CHD are surviving longer, and
better understanding of the long-term complications of CHD is
continuously emerging. Hence, it is important to be comfortable with the
primary care requirements for these children, including physical
manifestations prior to surgery and interventional cardiac
catheterizations, as well as those concerning for potential need for
reintervention, the latest recommendations for endocarditis prophylaxis,
respiratory precautions and immunization considerations, and close
monitoring of development and behavior. In this article, we will discuss
the common types of cyanotic (“blue”) and acyanotic (“pink”) CHD
and the role of the primary care physician in the health care of
these children before and after surgery and interventional cardiac
catheterizations.

AUTHOR DISCLOSURE Drs Puri, Allen,

and Qureshi have disclosed no financial Objectives After completing this article, readers should be able to:
relationships relevant to this article. This
commentary does not contain a discussion 1. Describe newborn pulse oximetry screening for congenital heart
of an unapproved/investigative use of a
commercial product/device. disease (CHD) and clinical features of CHD during the newborn
period.
ABBREVIATIONS
ASD atrial septal defect 2. Describe the clinical presentations and briefly outline management
AVSD atrioventricular septal defect strategies of infants and children with different types of CHD.
CHD congenital heart disease
CHF congestive heart failure 3. Discuss single-ventricle palliation by using hypoplastic left heart
CoA coarctation of the aorta syndrome as the model lesion.
EKG electrocardiogram
HLHS hypoplastic left heart syndrome 4. Describe the evaluation and primary care management factors
IAA interrupted aortic arch (including endocarditis prophylaxis, immunizations, and exercise
IVC inferior vena cava restriction) in children with different types of CHD.
LV left ventricle
mBTT modified Blalock-Taussig-Thomas
PAPVD partial anomalous pulmonary
NEWBORN PRESENTATION OF CRITICAL CONGENITAL HEART DISEASE
venous drainage
PDA patent ductus arteriosus Newborns with critical congenital heart disease (CHD) may present with symp-
RV right ventricle
toms of cyanosis, congestive heart failure (CHF), poor pedal pulses, or a failed
SBE subacute bacterial endocarditis
TAPVD total anomalous pulmonary
newborn CHD pulse oximetry screen prior to discharge. CHD lesions that are
venous drainage dependent on a patent ductus arteriosus (PDA) to support flow either to the
VSD ventricular septal defect systemic circulation or to the pulmonary circulation will manifest with shock or

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 cyanosis, respectively, when the PDA starts to close. CHD lethargy and other signs of poor perfusion and normal
lesions that allow shunting of blood to the pulmonary saturations are concerning for critical obstructive lesions
circulation manifest with progressive symptoms of pulmo- at the level of the aortic valve or the aortic arch that
nary overcirculation as the pulmonary vascular resistance require rapid prostaglandin initiation. Sepsis and inborn
decreases. errors of metabolism are other situations that must be
It may be challenging to distinguish cardiac disease from evaluated and/or empirically treated in a sick newborn
pulmonary disease or sepsis in a newborn with cyanosis who has poor systemic output; however, cardiac evalu-
and/or respiratory distress. Chest radiography and assess- ation with an electrocardiogram (EKG), chest radio-
ment of response to oxygen and/or positive pressure ven- graph, and echocardiogram must be simultaneously
tilation as clinically indicated help to elucidate the cause. initiated.
Lack of response to 100% oxygen for at least 10 minutes The pulse oximetry screen for critical CHD in newborns
(hyperoxygenation) indicates that the etiologic origin for the was approved as a part of the Routine Universal Screening
clinical picture is likely cardiac, and further cardiac workup Program in 2011 and has been adopted in 46 states and the
is indicated. Hyperoxygenation will help infants with pul- District of Columbia. (5)(7)(8) The most common protocol
monary parenchymal etiologic origins and those with pul- used is shown in Fig 1 (variations exist, described else-
monary hypertension as causes of cyanosis. A cyanotic where [8]). This screen is intended to target detection of
newborn who is otherwise well appearing may also have types of critical CHD that will manifest in the newborn
a hemoglobinopathy, such as methemoglobinemia. How- period and require early intervention, including coarcta-
ever, chest radiography and echocardiography performed to tion of the aorta (CoA), interrupted aortic arch (IAA),
rule out cyanotic CHD are indicated in a cyanotic infant who tetralogy of Fallot, truncus arteriosus, tricuspid atresia,
is not responsive to hyperoxygenation. total anomalous pulmonary venous drainage, double-outlet
Features of CHF in a newborn include tachypnea, RV, Ebstein anomaly, pulmonary atresia, transposition
increased work of breathing, tachycardia, and hepatomeg- of the great arteries, hypoplastic left heart syndrome
aly. While tachypnea and increased work of breathing may (HLHS), and other single-ventricle lesions. A failed
also indicate pulmonary disease or an inborn error of newborn screen may also indicate other disease processes,
metabolism, hepatomegaly at examination suggests cardiac such as pulmonary hypertension, primary pulmonary pa-
involvement. It is useful to percuss the top of the liver, as renchymal or interstitial disease, or hemoglobinopathies.
well as palpate its edge to estimate the liver span (normal Regardless, a failed screening result is an indication for
newborn liver span is 4.5 to 5.0 cm). A chest radiograph prompt evaluation, including chest radiography, EKG, and
showing cardiomegaly and pulmonary vascular congestion echocardiography (supervised and evaluated by a pediatric
in an infant with respiratory distress and normal saturations cardiologist). If cardiac investigations or a pediatric cardi-
in all 4 extremities is indicative of a left-to-right shunting ologist are not easily available in such a situation, initia-
process. These physical examination and chest radiographic tion of prostaglandin infusion with close monitoring of
findings in a newborn prompt a differential diagnosis of the airway (due to risk of apnea with prostaglandin initia-
obligate shunts, such as arteriovenous malformations. In tion) and prompt transport to a higher-level center are
comparison, these examination and chest radiographic fea- recommended.
tures in a 2- to 8-week-old infant with decreased pulmonary
resistance suggest a dependent shunt, such as a large
ACYANOTIC HEART DISEASE
ventricular septal defect (VSD). N-terminal pro–B-type natri-
uretic peptide may be considered to help distinguish Atrial Septal Defects
between noncardiac and cardiac causes of respiratory dis- Atrial septal defects (ASDs) comprise 7% to 10% of CHD. (1)
tress. (6) Severe pulmonary hypertension may also cause (2)(3)(4) Based on their embryological origin, they can be
hepatomegaly and right ventricle (RV) dysfunction. Hyper- classified as secundum ASDs (the most common type, due
oxygenation will help reduce the pulmonary vascular resis- to a deficiency in the central part of the septum primum that
tance and ameliorate the pulmonary hypertension; however, develops from the middle of the atrial cavity and grows
in these instances, it will worsen shunting and pulmonary inferiorly to meet the endocardial cushion), primum ASDs
overcirculation in left-to-right shunt lesions. (due to deficient proliferation of the endocardial cushion),
Weaker femoral pulses in a child who has otherwise sinus venosus ASDs (defects near the superior vena cava
strong radial pulses is concerning for coarctation or inter- and right upper pulmonary vein, truly a form of partial
ruption of the aorta. Weak femoral pulses in the setting of anomalous pulmonary venous drainage), and other rarer

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Figure 1. Flowchart shows the protocol for newborn pulse oximetry screening for critical congenital heart disease. F¼foot, RH¼right hand. Image
obtained with permission from Kemper AR, Mahle WT, Martin GR, et al. Strategies for implementing screening for critical congenital heart disease.
Pediatrics. 2011;128:e1259.

forms of ASDs. Shunting of blood from left to right at the sternal border from excess flow across the tricuspid valve.
atrial level leads to increased diastolic blood volume in the There may be left precordial bulging due to the enlarged
RV, which causes right-sided chamber dilation. RV being present during cartilaginous rib development.
Clinical Manifestations and Diagnosis. ASDs are typi- Patients with ASDs are usually asymptomatic but may have
cally discovered when a murmur is heard at a regular 4- to 6- some fatigue. They are followed up with Doppler echocar-
month infant well-child visit. The murmur is loudest over diography to monitor enlargement of the right atrium and/
the pulmonic region and is associated with a fixed splitting or RV, which, if present, indicates hemodynamic signifi-
of the S2 during different phases of respiration and a loud S1. cance. (9) Infants with an ASD manifesting with features
The murmur heard is from increased blood flow across the of CHF (tachypnea, increased work of breathing, frequent
pulmonary valve because of the greater volume of blood in pauses in feeding, failure to thrive, profuse sweating while
the right side of the heart (relative pulmonic stenosis). An feeding, decreased activity levels) must be investigated for
additional diastolic murmur may be heard at the left lower an additional lesion such as a PDA, VSD with pulmonary

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 arterial stenosis, or a left-sided obstructive lesion (cor tria-
triatum, coarctation, aortic or mitral stenosis).
Management. Secundum ASDs can be repaired either
surgically by using a patch or a primary suture closure of the
defect or in the cardiac catheterization laboratory by using
an ASD closure device. (10)(11) ASDs that are too large or
do not have appropriate anatomy usually require surgical
repair. (12) There has been no significant difference shown
in studies comparing the procedures, and there is also no
difference observed clinically. Typically, in clinical practice,
patients with hemodynamically significant ASDs are
referred for intervention around 3 to 4 years of age. Rarely
do ASDs have to be closed earlier.

Ventricular Septal Defects Figure 2. Frontal chest radiograph in a patient with a ventricular septal
VSDs are the most common CHD lesion and are present in defect shows features of congestive heart failure, including
cardiomegaly and increased pulmonary vascular congestion, as well as
50% to 60% of all children with CHD. (1)(2)(3)(4) VSDs splaying of the bronchi, which is indicative of left atrial enlargement.
develop from defective formation of the interventricular
septum and are classified on the basis of their location in sometimes chlorothiazide, and/or spironolactone—and
the septum relative to the atrioventricular valves and the may require frequent evaluation of electrolyte levels.
right and left ventricular outflow tracts. Perimembranous Patients with failure to thrive may need up to 125 to 150
VSDs (deficiency in a fibrous part of the septum at the base kcal/kg per day of caloric intake through fortified formula.
of the heart) are the most common type, constituting about Close, reliable, and accurate monitoring of weight, with
80% of all VSDs, followed by doubly committed juxta-arterial attention to using the same weighing scale and reinforce-
(deficiency in the infundibular septum, between the pulmo- ment of education about proper mixing of formula, are
nary and aortic valves), muscular (in the trabecular part of the critical goals to be reviewed during the weight-check visits at
septum), and inlet (deficiency in the septum inferior to the the pediatrician’s office. With current surgical success in
perimembranous region and above the level of cordal treating these lesions, there is little need for prolonged
attachments of the atrioventricular valves). Size of the VSD is medical treatment. Surgical patch repair of the VSD is
defined relative to the area of the aortic valve: small (less than performed either in a symptomatic infant or in a toddler
one-third of the area), moderate (one-third to two-thirds of the who has enlargement of the left atrium or ventricle at echo-
area), and large (more than two-thirds of the area). cardiography. Sometimes perimembranous and doubly
Clinical Manifestations and Diagnosis. A newborn with a committed juxta-arterial VSDs can be associated with pro-
VSD may not initially have a murmur; however, as the lapse of the right or noncoronary cusp of the aortic valve
pulmonary resistance decreases with age, an S1-coincident through the defect, which may cause progressive aortic
pansystolic murmur can be heard the loudest over the left insufficiency. These VSDs are repaired even in asymptomatic
lower sternal border. A diastolic rumble at the apex may be patients. Some VSDs (eg, muscular VSDs) can be closed with
heard from excess flow across the mitral valve. A child with a devices in the cardiac catheterization laboratory. After VSD
hemodynamically significant VSD presents with features of repair, these patients need to have continued follow-up of
pulmonary overcirculation and CHF. A chest radiograph weight and nutrition to ensure regaining of growth curves.
shows cardiomegaly with pulmonary vascular congestion
(Fig 2), while EKG shows left or biventricular hypertrophy. Atrioventricular Septal Defects
As the child grows, some perimembranous VSDs can get Atrioventricular septal defects (AVSDs), (also known as
occluded by aneurysmal tissue, and muscular VSDs can atrioventricular canal defects), constitute 5% of CHD. (1)(2)
become smaller in size with muscular growth. One must be (3)(4) About 50% of patients with AVSDs have Down
wary of the perimembranous defect that becomes smaller syndrome. AVSDs arise from defects in the proliferation
owing to prolapse of the aortic valve. and fusion of the endocardial cushions and are classified on
Management. Children with VSDs are regularly followed the basis of the commitment of the common atrioven-
up by a pediatric cardiologist. Children with CHF symp- tricular valve to the ventricles, the presence or absence
toms are temporized with diuretics—usually furosemide, of separate orifices, and the anatomy of the subvalvar

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attachments. Most commonly, patients have a primum type Management. Patients with AVSD with an additional
of ASD, as well as an inlet-type VSD component, and in diagnosis of Down syndrome may remain in the hospital
addition, they may have a lack of complete apposition or for a prolonged period of time as a neonate, owing to feeding
coaptation (so-called cleft) in the mitral valve. difficulties. All patients with AVSD are closely followed up,
Clinical Manifestations and Diagnosis. Patients with since they typically require surgical intervention around 4 to
AVSD typically have a systolic ejection or holosystolic mur- 6 months of age or earlier, if they have severe CHF or failure
mur from the VSD component. However, if the VSD is large to thrive. The diuretic and nutritional management and
and there is equalization of pressures in the ventricles (or if close monitoring for CHF is similar to that in patients with
pulmonary hypertension is present), a murmur may not be VSD. While the surgical mortality rate in the current sur-
heard. Additionally, these patients may have murmurs of gical era is less than 3%, the need for reintervention owing to
atrioventricular valve regurgitation, characterized as an S1- atrioventricular valve regurgitation or stenosis remains
coincident systolic murmur, heard the loudest over the apex about 10% to 15% at the 5-year postoperative mark. (13)(14)
in the case of left-sided regurgitation and the loudest over
the right lower sternal border in the case of right-sided Patent Ductus Arteriosus
regurgitation. A diastolic rumble may be heard, similar to An arterial duct is a normal fetal connection between the
that heard in patients with VSDs. An EKG in these children aorta and the pulmonary artery that is present in all new-
shows left, right, or biventricular hypertrophy and may have borns. It closes functionally within 24 hours and anatom-
a northwest axis (negative axes in leads I, II, III, and aVF), ically within 3 to 4 weeks in most patients. It may remain
which is suggestive of a diagnosis of AVSD [Fig 3]). These patent longer in some patients, especially infants born
children may also present with features of CHF and failure prematurely.
to thrive, with chest radiographic findings as previously Clinical Manifestations and Diagnosis. The examination
described (in the VSD section). Severe atrioventricular valve of patients with PDA varies with their age. In a newborn
regurgitation, creating more preload, will exacerbate earlier with higher pulmonary resistance and pressures, the PDA
CHF symptoms. may not shunt much blood and may not be audible. In an

Figure 3. Electrocardiogram of a patient with atrioventricular septal defect shows biventricular hypertrophy, as well as the northwest axis deviation, with
negative QRS axes in leads I and aVF.

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 older infant, however, one can detect a loud systolic or CHD. (1)(2)(3)(4) The degree of stenosis is categorized as
continuous murmur of blood shunting left to right during critical if the blood flow through the respective valve is
the entire cardiac cycle, which is loudest over the left insufficient and requires additional contribution from the
precordium. In an older patient with a hemodynamically PDA. These valves undergo aberrant tissue resorption dur-
significant PDA, one may be able to detect a diastolic rumble ing in utero development, which leads to thickened dys-
of transmitral flow into an enlarged left ventricle (LV). The plastic valves that do not open or close normally. Hence, they
left-to-right shunting caused by the PDA may also lead to create obstruction to blood flow and may also be regurgitant.
features of CHF. Chest radiographs illustrate features of Aortic stenosis may be associated with a bicuspid or even a
pulmonary overcirculation and cardiac enlargement, EKG unicuspid aortic valve, as well as other levels of obstruction
can show LV hypertrophy and ST-segment changes of in the left-sided circulation, including CoA, subaortic
ischemia (beware of this finding in a newborn, as it implies obstruction, and mitral stenosis. A bicuspid aortic valve has
a very large shunt), and Doppler echocardiograms can been found to be associated with aortic root dilation due to
demonstrate left atrium and LV enlargement, characteristics an aortopathy and requires close follow-up, even if there is
of flow, and anatomy of the PDA. no stenosis across it.
Management. Ibuprofen and indomethacin have been Clinical Manifestations and Diagnosis. Patients present
used to medically close PDAs in premature infants. Acet- with an ejection systolic murmur that is loudest over the
aminophen is also being studied as an alternative medical respective valvar region and may also have an ejection click
therapy. (15) A symptomatic or hemodynamically significant (if stenosis is moderate or mild). The aortic click is heard at
PDA is a class I indication for intervention, with either the apex and right upper sternal border and does not vary
device occlusion in the interventional catheterization labo- with respiration, whereas the pulmonary click heard at the
ratory or surgical ligation. (9) In a center with interventional left lower and upper sternal borders will be loudest when the
cardiac catheterization expertise, device occlusion is the patient exhales. The EKG may show ventricular hypertrophy
first-line approach for an older infant or child, with excellent of the RV in cases of pulmonic stenosis and of the LV in
results and extremely low complication rates comparable to cases of aortic stenosis. Doppler echocardiograms help
those of surgical ligation. (16) In babies with low birth estimate the velocity of blood flow across the valve. Based on
weight and those with extremely low birth weight, personnel the continuity equation, the narrower the valve area, the
at some centers lean toward surgical ligation, often at the faster the flow velocity. This flow velocity is then used to
bedside. However, successful PDA device occlusion has estimate the pressure gradient across the stenotic valve
been reported in small neonates, and the most recent series by using a modified Bernoulli principle (peak velocity
demonstrates successful catheter-based intervention in neo- in meters per second squared, times 4).
nates as small as 700 g. (17) Both procedures have similarly Management. Patients with critical aortic stenosis or
high success rates and a complication rate of less than 1%. pulmonic stenosis meet a class I indication to undergo a
Patients with left-to-right shunt lesions (eg, with a VSD, catheter-based balloon valvuloplasty procedure in the new-
AVSD, PDA, and, in very rare instances, an ASD) that born period to ameliorate ductal dependence. (9)(18) For
present late may have pulmonary hypertension. Pediatri- noncritical stenosis, the time of intervention is determined
cians may encounter these scenarios when examining by the presence of peak gradient of ‡50 mm Hg obtained
patients who have not had access to medical care (eg, across the aortic valve (‡40 mm Hg in the setting of ventricular
patients from some developing countries or areas of the dysfunction) or ‡40 mm Hg across the pulmonary valve at
United States that are medically underserved). The degree echocardiography or during cardiac catheterization, on the
of pulmonary hypertension due to pulmonary vascular basis of the American Heart Association guidelines (class I
obstructive disease may be prohibitive for repair. Some of indication [9][19]). Patients with aortic stenosis may undergo a
these patients will be treated with agents that lower pulmo- surgical valvuloplasty, depending on the valve anatomy, annu-
nary hypertension and may later in life go on to develop lus size, and experience and preference of the personnel at
Eisenmenger syndrome (when pulmonary vascular resis- the center. There has been no reported significant difference
tance exceeds systemic vascular resistance, resulting in a in survival or rate of aortic valve replacement between
reversal of the shunt) and its associated sequelae. patients undergoing balloon valvuloplasty and those
undergoing surgical valvuloplasty, although patients
Aortic and Pulmonic Valve Stenosis undergoing balloon valvuloplasty may have a greater fre-
Aortic stenosis constitutes 5% to 8% of all CHD, and quency of reintervention. (10)(20)(21) Patients undergoing
pulmonary stenosis constitutes about 8% to 10% of all balloon valvuloplasty of the aortic valve in the first year after

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birth typically need reintervention in about 50% of the PDA open until the time of surgical repair. Some children
critical aortic stenosis cases and 33% of the noncritical aortic (particularly older children) are candidates for intervention
stenosis cases, necessitating long-term follow-up for reste- in the cardiac catheterization laboratory, with balloon angio-
nosis and/or regurgitation. (18)(19) Patients undergoing plasty and stent placement for the coarctation (Videos 1, 2).
balloon valvuloplasty of the pulmonary valve may not (22)(23) After repair, patients are followed up closely in the
require another procedure for several years or ever again; cardiology clinic to monitor for recurrence of CoA and
however, they do require regular cardiology re-evaluation persistent hypertension and continue to receive long-term
and Doppler echocardiography to assess the need for re- follow-up at 1–2-year intervals.
intervention due to restenosis and/or regurgitation. (9)

CYANOTIC CHD
CoA and Interruption of the Aorta
CoA is a narrowing at the isthmus of the arch of the aorta Tetralogy of Fallot
(with concomitant narrowing of the transverse arch in some Tetralogy of Fallot is the most common cyanotic CHD,
cases), constituting 5% to 8% of CHD. (1)(2)(3)(4) IAA is the accounting for 5% of all CHD. (1)(2)(3)(4) It develops from
most extreme end of this spectrum, with a discontinuation anterior malalignment of the interventricular septum,
of the arch and distal continuation through a PDA past the which leads to a VSD, as well as overriding of the VSD
point of interruption. IAA accounts for about 1.5% of CHD by the aorta. There is a narrowing of the pulmonary outflow
and is classified according to the site of the interruption: tract due to the septal deviation, and this causes RV outflow
type A is interrupted at the isthmus distal to the left sub- (infundibular) obstruction and consequent RV hypertrophy
clavian artery, type B is interrupted between the origins of (Videos 3, 4).
the left subclavian and left common carotid arteries (the Clinical Manifestations and Diagnosis. Patients with
most common type and frequently associated with chromo- tetralogy of Fallot have a harsh ejection systolic murmur
some 22 abnormalities), and type C is interrupted between heard over the pulmonic area, indicating pulmonic stenosis.
the origins of the innominate and left common carotid There may be a single second sound. Higher saturations
arteries (the most rare type). About 80% of patients with indicate less RV outflow obstruction. Patients who do not
IAA have an associated VSD. receive a diagnosis prenatally may present for the first time
Clinical Manifestations and Diagnosis. Severe and critical as children having a hypercyanotic spell in a period of agitation,
CoA and IAA may be detected at the time of newborn fever, or other concurrent illness. Agitation and crying
screening with a lower saturation level at the postductal site increase pulmonary vascular resistance, while also increas-
or via clinical suspicion on the basis of poor pedal or femoral ing the heart rate. Owing to a subsequently shorter diastolic
pulses at the first newborn visit to the pediatrician. These period, ventricular filling is less, which adds to the obstruc-
patients may present in extremis at 2 to 3 weeks of age, in tion to the RV outflow from its hypertrophic muscle bun-
shock, with feeble pulses, lethargy, poor feeding, decreased dles. “Tet spells” may become a vicious cycle and require a
urine output, and metabolic acidosis. While sepsis remains
the most common cause of this constellation of symptoms,
an echocardiogram should be performed for CoA and/or
IAA if the shock is unresponsive to fluid resuscitation. Mild
CoA may be detected on the basis of a gradient of more than
20 mm Hg between the upper- and lower-extremity blood
pressures (carefully performed with proper technique) dur-
ing infancy. A harsh systolic murmur that is loudest over the
back may be heard. CoA may also be found in young
adolescents undergoing workup for hypertension. There
may not be a clinically significant blood pressure gradient
in older patients with CoA because they develop collateral
vessels over time that supply blood distal to the narrowing.
In the case of collateralization, one can detect a continuous
murmur of the flow in these vessels over the chest wall.
Management. Newborns with severe or critical CoA and
Video 1. Aortogram in a teenager with coarctation of the aorta
IAA are dependent on prostaglandin infusion to keep the demonstrates narrowing of the descending aorta.

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 Management. The time of presentation and intervention
for tetralogy of Fallot is determined by the degree of RV
outf low obstruction and the limitation of pulmonary blood
flow. (24) These clinical indicators are the oxygen saturation
levels and the development of hypercyanotic spells. The
Doppler echocardiographic indicators are the pressure gra-
dients observed across the RV outflow tract. Children with
saturations less than 80% or those having hypercyanotic
spells are scheduled for surgery. Acute “Tet spells” are
managed by first helping to calm the patient (eg, handing
the child to the parent to hold, allowing the child to feed, or
administering sedation if needed), placing the child in a
knees-to-chest position (to increase systemic vascular resis-
tance; this may be achieved by having the parent hold the
baby in her arms and cradling the knees and chest together),
initiating oxygen (preferably through the least noxious route
for the child, for example, blow-by oxygen or use of a face
mask), administering a bolus of intravenous fluids, and,
Video 2. Aortogram obtained after angioplasty in a patient with
coarctation of the aorta who underwent stent placement in the finally, intravenous metoprolol (to slow the heart rate) and
cardiac catheterization laboratory. The narrowing is abolished with stent phenylephrine (to increase systemic vascular resistance).
placement.
Some patients will require anesthesia (on the way to the
operating room). Surgical management includes palliative
combination of heart rate control and vascular resistance procedures like a modified Blalock-Taussig-Thomas (mBTT)
manipulation to break. The murmur during a hypercyanotic shunt to help provide consistent pulmonary blood flow in
spell or “Tet spell” becomes softer because of decreased the setting of significant stenosis or atresia of the pulmonary
pulmonary flow. Iron deficiency anemia will accelerate the valve. Complete repair of the heart comprises closure of the
onset of these spells. VSD, as well as resection of the RV obstruction, resulting in

Video 3. Echocardiogram in a patient with tetralogy of Fallot. A 4-chamber view of the heart is shown, with the left side of the patient on the reader’s
right. The ventricular septal defect (VSD) and the right ventricle (RV) hypertrophy are demonstrated. The aorta arises from the left ventricle and overrides
the VSD. As the video plays, it sweeps through the heart anteriorly to show the pulmonary artery, which appears narrow. The hypertrophied muscle
bundles of the RV are also seen, especially in the subvalvar region, which come very close together in systole. This creates a setup for dynamic
obstruction to blood flow out of the RV.

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normal saturations. Complete repair is replacing the palli- E1 until the time of surgery. Prostaglandin allows the PDA
ative approach in many centers. Even after complete tetral- to remain open, which shunts blood from the aorta into the
ogy of Fallot repair, patients require lifelong cardiology pulmonary circulation. This increases the amount of blood
follow-up, since their pulmonary valve may become more returning to the left atrium and mixing at the atrial level, as
regurgitant (depending on the surgical approach and the long as the foramen is open. Most patients undergo a
intervention on the valve annulus itself), which can lead to catheter-based procedure called balloon atrial septostomy
RV dilation. Patients may also develop arrhythmias because to help create or enlarge the ASD to allow more mixing
of the RV dilation and/or hypertrophy and scarring (as a while awaiting surgery. If cyanosis persists despite an open
result of the pulmonary valve regurgitation, as well as the PDA, a fluid bolus or inotropic support may help improve
primary surgery), with an increase in risk of sudden cardiac mixing and systemic arterial saturations. After repair, these
death in pediatric patients with QRS duration longer than patients require regular cardiology follow-up for life. The
170 ms on the EKG. (25)(26) Cardiac magnetic reso- first arterial switch procedure was performed in 1975, and
nance imaging is used to estimate the volume of the RV since the patients undergoing this procedure are followed
to determine the time for pulmonary valve replacement up for progressively longer periods of time, data about the
(catheter-based or surgical). While complete repair of tetral- long-term complications are emerging. Depending on the
ogy of Fallot ensures that these children have normal study, 5% to 30% of these patients have been reported to
saturations, most patients have a prolonged QRS duration require reintervention at 25-year follow-up for a variety of
and features of right bundle block on the EKG, owing to the reasons, most commonly regurgitation of the neoaortic
intervention on the interventricular septum. valve (more than 75%), supravalvar pulmonary stenosis
(more than 75%), and coronary artery disease (5% to 8%).
Transposition of the Great Arteries (28)(29)
Transposition of the great arteries is the second most
common cyanotic CHD, accounting for about 2% of all Truncus Arteriosus—Common Arterial Trunk
CHD. (1)(2)(3)(4) It is the most common cyanotic heart Truncus arteriosus accounts for 2% to 5% of CHD and
disease manifesting in the first week after birth. There is manifests early in the neonatal period. (1)(2)(3)(4) It
ventriculoarterial discordance, with the aorta arising from develops from lack of formation of the aorticopulmonary
the RV (usually anterior to the pulmonary artery) and the septum; hence, the common truncal outflow does not divide
pulmonary artery arising from the LV. Hence, the systemic into an aorta and a main pulmonary artery. There is always
and pulmonary circulations are in parallel, with systemic an associated VSD, and there is a common truncal valve,
venous (deoxygenated) blood returning to the right atrium, most commonly either tricuspid or quadricuspid. About
the RV, and going out the aorta again. There is mixing of one-third of the patients with truncus arteriosus have a
blood at the atrial level through a patent foramen ovale or an 22q11.2 deletion (DiGeorge or velocardiofacial syndrome).
ASD or at the ventricular level through a VSD (35% to 40% Clinical Manifestations and Diagnosis. Patients with
of transposition of the great arteries). truncus arteriosus present within the first 48 hours after
Clinical Manifestations and Diagnosis. Newborns with birth with symptoms of profound pulmonary overcircula-
transposition of the great arteries present with cyanosis tion. On examination, they have a systolic murmur heard
within the first 12 hours after birth and are not responsive the loudest over the left sternal border, as well as a loud S2.
to oxygen or mechanical ventilation. The presence of a VSD Multiple sounds may be heard that temporally coincide with
may delay presentation. Chest radiography may show a the timing of the S2. Their general physical examination
narrow mediastinal silhouette due to the orientation of the may also demonstrate features of 22q11.2 deletion, includ-
great vessels and thymic regression, and EKG results may be ing a small mouth (micrognathia), cleft lip and/or palate,
normal or show RV hypertrophy. There is usually no mur- and flat cheek bones (malar flattening). (30)
mur at examination; however, there may be a single S2 with a Management. Symptoms of pulmonary overcirculation
loud aortic component due to the orientation of the great may be managed with diuresis and fluid restriction. These
vessels. children undergo surgical repair in the first 2 weeks after
Management. Once the diagnosis is established, these birth. (31) The truncal outflow and the truncal valve are
patients require reparative surgery to switch the great vessels committed to the aorta, and the branch pulmonary arteries
to the appropriate ventricles, known as the arterial switch are committed to a conduit placed from the RV to the
procedure. (27) They may need respiratory support with pulmonary artery. These children should have normal satu-
oxygen, mechanical ventilation, and initiation of prostaglandin rations after repair. They continue to require close cardiology

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 follow-up over the years as they outgrow their RV-to– due to backing up of blood flow prior to the site of the
pulmonary artery conduit (only 50% are free of RV-to– obstruction of pulmonary vein drainage and (b) a small
pulmonary artery conduit reintervention at 10 years), and heart on chest radiographs. The respiratory distress does
their truncal valve (now their committed “neoaortic” valve) not respond to oxygen and may potentially be worsened by
may become regurgitant (only about 23% are free of re- starting prostaglandin as more blood starts shunting to the
intervention for the truncal valve at 10 years). (31) Other pulmonary circulation at the ductal level, to the point of
than their cardiac concerns, they should also undergo pulmonary edema or lung bleeding.
22q11.2 deletion testing by way of chromosomal microarray PAPVD (which may or may not be associated with an
analysis and a genetics consultation, regardless of whether ASD) typically manifests later in life in childhood or ado-
the typical facies are present. (30) Serum electrolyte testing lescence with signs similar to those of an ASD, with an
for calcium levels should also be performed in all cases ejection systolic murmur that is loudest over the pulmonic
while awaiting chromosomal microarray analysis results to region owing to the increased blood returning to the right
rule out hypocalcemia that may be seen in patients with side of the heart. EKG shows right atrium and RV enlarge-
22q11.2 deletion. ment, and Doppler echocardiographic findings confirm the
diagnosis.
Total and Partial Anomalous Pulmonary Management. Obstructed TAPVD constitutes about one-
Venous Drainage third of the cases and is a surgical emergency. (30)(31)
Abnormal return of the pulmonary veins to the systemic Children with unobstructed TAPVD can be followed up
veins or the right atrium comprises about 1% of CHD. (1)(2) by a cardiologist for a brief time as outpatients with a definite
(3)(4) Anomalous pulmonary venous return is defined as (i) plan for early surgical repair, since obstruction can develop
partial anomalous pulmonary venous drainage (PAPVD) over time and manifests similarly with respiratory distress.
when at least 1 pulmonary vein returns to the left atrium After repair of TAPVD, patients continue to be closely
and (ii) total anomalous pulmonary venous drainage monitored as outpatients, since pulmonary venous obstruc-
(TAPVD) when none of the pulmonary veins return to tion can recur, with reported rates around 15%. (32)(33)
the left atrium. TAPVD can be further classified on the Evaluation of the anastomosis site at echocardiography is
basis of the site of return of the anomalous drainage to the especially important. Recurrent pulmonary venous obstruc-
systemic veins—supracardiac (into the superior vena caval tion manifests as respiratory distress, tachypnea, and
system), intracardiac (into the coronary sinus or right wheezing that is not responsive to bronchodilation. These
atrium), or infracardiac (into the inferior vena cava [IVC] patients require chest radiography and echocardiographic
or hepatic venous system). Blood returning from the pul- evaluation if they have not undergone a recent study to
monary veins to the right side of the heart causes right atrial assess their pulmonary venous drainage.
hypertension, with right-to-left shunting across a patent Stable patients with PAPVD are scheduled for elective
foramen ovale and/or an ASD. In TAPVD, all the venous surgical repair (if indicated) and are followed up as an
return to the heart returns to and mixes in the right atrium outpatient in the cardiology clinic every 1 to 2 years.
before shunting across the interatrial septum or coursing
across the tricuspid valve. Hence, the saturations in each of HLHS, Tricuspid Atresia, and Single-Ventricle Palliation
the cardiac chambers are the same. HLHS is the fourth most common form of cyanotic CHD,
Clinical Manifestations and Diagnosis. The number of comprising severe stenosis or atresia of the mitral and
veins returning anomalously and the degree of obstruction aortic valves. (1)(2)(3)(4) Owing to decreased flow from
determine the rate and severity of the manifestation. Phys- the left atrium into the LV, as well as from the LV into
iologically, TAPVD can be categorized as (i) obstructed, in the aorta, the LV is hypoplastic, and there is associated
which case the pulmonary venous return is impeded, which hypoplasia of the ascending aorta, as well (Video 5). This
can lead to pulmonary venous hypertension and pulmonary lesion is PDA dependent for systemic circulation, as the
edema, or (ii) unobstructed, in which case the anomalous descending aorta continues from the PDA insertion, and
drainage causes cyanosis due to mixing after return to the the coronary arteries, innominate arteries, left common
right side of the heart but does not cause respiratory distress carotid arteries, and left subclavian arteries are supplied
in the first days after birth. The newborn with obstructed primarily by retrograde flow from the PDA. Prostaglandin
TAPVD presents with respiratory distress and cyanosis infusion is required to maintain PDA patency. A correspond-
within 12 to 24 hours after birth, with a classic chest ing lesion on the right side of the heart is a hypoplastic RV in
radiographic finding of (a) “whiteout” of the lung fields the setting of severe tricuspid stenosis or atresia and severe

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 Video 4. Echocardiogram in a patient with tetralogy of Fallot. The doming, narrow pulmonary valve, and infundibular narrowing from muscle below
the pulmonary valve in the right ventricular outflow tract can be seen.

pulmonary stenosis or atresia. The absence of 2 normal-sized tract in some situations (severe stenosis or atresia of the
ventricles, the absence of a normal or repairable atrioventric- pulmonary or aortic valve), or any combination of these
ular connection (in the form of severe stenosis or atresia of the conditions indicates that patients should undergo single-
mitral or tricuspid valves), the absence of a repairable outflow ventricle palliation. (34) These lesions are dependent on

Video 5. Echocardiogram in a patient with hypoplastic left heart syndrome. A 4-chamber view of the heart is shown with the left side of the patient on
the reader’s right. The diminutive left-sided chambers can be seen, with the hypoplastic left atrium and the hypoplastic left ventricle, as well as
compensatory dilation and hypertrophy of the right ventricle.

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 a PDA maintained by prostaglandin to ensure cardiac out-
put into both systemic and pulmonary circulations.
The subtypes of these lesions have variable physiology
and are beyond the scope of this article. We will proceed to
discussing the stages of palliation by using HLHS as a
model.
The first stage is to help stabilize both cardiac outputs,
either by surgically constructing a “neoaorta” by using the
main pulmonary artery tissue to supplement the ascending
aorta (the Norwood procedure), creating an mBTT shunt (or
RV-to–pulmonary artery conduit) to supply blood flow to the
pulmonary arteries, and creating an ASD. This first stage
may also be performed in a “hybrid fashion” in the cardiac
catheterization laboratory and/or operating room (the
details are beyond the scope of this article).
The second stage, known as the Glenn procedure, involves
anastamosing the superior vena cava to the pulmonary
arterial system to direct part of the deoxygenated venous
blood directly to the lungs. This also allows take-down of the
mBTT shunt (or RV-to–pulmonary artery conduit), as there
is consistent pulmonary blood flow through the Glenn
connection.
The final step of this palliation (the Fontan procedure) is
Figure 4. Diagram shows the shunts and sites of anatsamoses for single-
to connect the IVC to the pulmonary arterial system as well, ventricle palliation by using hypoplastic left heart syndrome as a model.
to allow all the deoxygenated blood to go straight to the lungs 1a indicates the reconstructed neoaorta by using the hypoplastic
ascending aorta, as well as the normal main pulmonary artery, to ensure
without mixing in the heart first (Fig 4). The Fontan con- stable systemic outflow. 1b indicates the site of the modified Blalock-
nection may be fenestrated in some cases, creating a con- Taussig-Thomas shunt to direct blood flow from the right subclavian
artery into the pulmonary circulation. This is taken down at the time of
nection between the IVC–pulmonary arterial anastomosis the second stage of palliation—the Glenn anastomosis. This involves
and the pulmonary venous atrium. This may be especially the creation of an anastomosis between the superior vena cava and the
right pulmonary artery, 2. The final step in this palliation is the Fontan
useful as a “pop off” for blood passively returning from the anastomosis, 3. This disconnects the remaining deoxygenated blood
IVC to the pulmonary circulation in cases of high pulmo- coming in through the inferior vena cava and redirects it through the
Fontan conduit to the pulmonary circulation. This final step eliminates
nary vascular resistance. any mixing and completely separates the oxygenated and
Clinical Manifestations and Diagnosis. The presentation deoxygenated blood, ensuring normal saturations. LA¼left atrium,
LV¼left ventricle, RA¼right atrium, RV¼right ventricle. Printed with
of single-ventricle lesions depends on the adequacy of permission from Texas Children’s Hospital.
cardiac output. Systemic cardiac output is dependent on
the ductal blood flow. In patients with HLHS, when the duct murmur is heard with an RV-to–pulmonary artery conduit.
starts to close, the infant begins to have signs of poor As these children grow older and bigger, the relative size of
systemic perfusion with feeble pulses, poor urine output, their mBTT shunt (or RV-to–pulmonary artery conduit)
shock, and mottling, in addition to pulmonary overcircula- limits the amount of pulmonary blood flow for their body
tion, seen as tachypnea and respiratory distress. Chest surface area, and their saturations start to drift down into the
radiographs will show pulmonary vascular congestion, in 70% range. These patients are very closely followed up until
addition to possible cardiomegaly due to right ventriculo- they undergo the Glenn procedure. The period between the
megaly (as a result of the RV effectively handling systemic as Norwood and Glenn procedures (the “interstage period”) is
well as pulmonary cardiac output). a particularly vulnerable time. An intercurrent stress such as
Management. The first-stage surgical intervention is respiratory syncytial viral pneumonia can be devastating.
performed within the first 2 weeks after birth. Saturations The Glenn procedure is typically performed around 4 to 6
after the first-stage palliation can vary from 80% up to 95%, months of age. By this age, the pulmonary vascular resis-
depending on the amount of pulmonary blood flow. A tance decreases to the physiological minimum, and the
continuous or pansystolic murmur is easy to appreciate branch pulmonary arteries grow adequately, so that a stable
in a child with an mBTT shunt, and a systolic ejection and durable superior vena cava–pulmonary anastomosis

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can be created. After the Glenn procedure, the saturations defect require subacute bacterial endocarditis (SBE) pro-
are in the 80% to 90% range, and these patients generally do phylaxis for 6 months after the procedure and indefinitely if
not have any murmurs. In some patients, an S1-coincident residual defects are present around a patch and/or a device.
systolic murmur of atrioventricular valve regurgitation may All children with unrepaired cyanotic CHD require SBE
be heard. prophylaxis. Children who have prosthetic or bioprosthetic
After the Glenn procedure, as the children grow older, valves or conduits implanted percutaneously or surgically
their oxygen saturations trend downward, as the proportion should receive lifelong SBE prophylaxis. Children with a
of their cardiac output from the lower part of their body that prior history of endocarditis or who have valvular heart
returns through the IVC increases relative to the proportion disease in the context of heart transplantation also require
from the upper body and the head that returns through the SBE prophylaxis. (38)
superior vena cava. These patients are followed up as After cardiac surgery via median thoracotomy, patients
outpatients on a semiannual basis until their final Fontan observe sternal precautions for 6 to 8 weeks. (39)(40) The
palliation at around 2 to 4 years of age, depending on trends precautions vary from center to center, but the general
of saturation and the prevalent clinical practice at each principles include (i) no prone position or “tummy time”
institution. Hence, accurate documentation of saturations unless they can roll and be prone voluntarily, (ii) close
at the interim pediatric office visits is extremely helpful in supervision when playing with other children and avoiding
case a downward trend is noted. After the Fontan palliation, crowded places, and (iii) avoiding picking up or lifting the
the patients are expected to be fully saturated in the case of a patient by the arms or armpits and instead trying to “scoop
nonfenestrated Fontan procedure or around 90% to 95% up” the patient. The patient can continue to be in an age- and
saturated in the case of a fenestrated Fontan procedure. weight-appropriate car seat after cardiac surgery. After the
These children are still at risk of developing right-sided period of sternal restrictions, the patient is allowed to
heart failure or systemic ventricular heart failure because exercise as tolerated. After a cardiac catheterization, most
they have a single ventricle driving their entire circulation. children should be ambulating within 2 to 6 hours and
Hence, accurate assessment and documentation of history should be back to full activities within 3 to 5 days.
of CHF symptoms, such as tachypnea, poor exercise toler- Children with CHD are at risk for developmental delay
ance, decreasing appetite, diarrhea, chronic cough, or pedal and disability, with greater complexity of CHD lending a
edema, are important for these patients during their pedi- higher risk. (41) The American Heart Association guidelines
atric clinic visits, especially if the parents have a new for neurodevelopmental screening for these patients help
concern. stratify these patients into low- and high-risk groups. Neo-
nates or infants that require open-heart surgery (cyanotic
and acyanotic CHD), children with cyanotic CHD (regard-
GENERAL PRINCIPLES OF IMMUNIZATION,
less of open-heart surgery during the neonatal or infant
SUBACUTE BACTERIAL ENDOCARDITIS PROPHYLAXIS,
period), and patients with CHD accompanied by certain
AND POSTOPERATIVE AND CARDIAC
comorbidities (such as prematurity, known genetic syn-
CATHETERIZATION CARE
drome, prolonged hospitalization, or cardiopulmonary
All patients undergoing cardiopulmonary bypass should not resuscitation) are at increased risk. These children should
receive live vaccines for 2 weeks before or 6 weeks after be directly referred to a developmental pediatrician, while all
cardiopulmonary bypass. Cytokine and immunologic other patients with CHD should still continue regular
markers return to normal by 2 months after surgery, and the assessment and surveillance of neurodevelopmental status
vaccination schedule can be resumed. (35)(36) Most children with their pediatrician.
should receive the influenza vaccine. Children younger than Finally, the transition of care to an adult CHD specialist is
12 months with unrepaired cyanotic CHD or those receiving an important issue for children with CHD as they enter
diuretic therapy for CHF in acyanotic CHD should receive young adulthood. (42) Adult CHD is a growing field of
respiratory syncytial viral prophylaxis with palivizumab. (37) practice and expertise; however, many pediatric cardiolo-
Further, for children who meet the criteria for respiratory gists continue to follow up their patients during adulthood
syncytial viral prophylaxis after a palliative surgery involv- as well, especially if there is a constraint of adult providers
ing cardiopulmonary bypass, a postoperative dose of 15 comfortable with complex CHD. From the pediatrician’s
mg/kg palivizumab should be considered. perspective, these children should continue to be followed
Children undergoing cardiac surgery or interventional up in the pediatrician’s office until the typical age when the
catheterization procedures to repair a lesion or occlude a office transitions care to adult providers. The process of

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 transition to adult CHD specialists is performed by the
cardiologist, along with detailed communication of the 5. On the basis of clinical evidence, as well as clinical consensus,
CHD-related diagnoses and past procedures. Owing to although most children with corrected CHD have a normal
limitations in the familiarity of adult cardiac surgeons with quality of life, lifelong cardiology follow-up is needed for all of
these patients to monitor the development of any long-term
CHD, as well as adult interventional cardiologists, most of
complications and/or sequelae. (13)(14)(20)(24)(27)(30)(39)
the long-term procedures for these patients are performed
by pediatric CHD specialists.

To view teaching slides that accompany this article,

visit http://pedsinreview.aappublications.org/content/
38/10/471.supplemental.
Summary
1. On the basis of clinical consensus and research observation,
newborns with ductal-dependent congenital heart disease
(CHD) may present with cyanosis or shock as the patent ductus
arteriosus starts to close, indicating a need for prostaglandin to
maintain output to the pulmonary or systemic circulation. (5)(7)(8)
2. On the basis of research evidence, as well as clinical consensus,
children with acyanotic shunting lesions can present with features
of congestive heart failure and require nutritional and diuretic
optimization until the time of early surgical correction. (1)(2)(9)
3. On the basis of research evidence, as well as clinical consensus,
children with cyanotic heart disease may undergo early
corrective surgery or, in some instances, undergo palliation with
a shunt (modified Blalock-Taussig-Thomas shunt) to supply
pulmonary blood flow prior to their corrective surgery. (22)(32)
4. On the basis of strong clinical evidence, children who undergo
single-ventricle palliation are at risk for developing
complications at any stage of the palliation. (31) References and Suggested Readings for this article are at http://
pedsinreview.aappublications.org/content/38/10/471.

Additional Resources for Pediatricians

AAP Textbook of Pediatric Care, 2nd Edition
• Chapter 234: Congenital and Acquired Heart Disease - https://pediatriccare.solutions.aap.org/chapter.aspx?
sectionid=135605095&bookid=1626
Point-of-Care Quick Reference
• Congenital and Acquired Heart Disease - https://pediatriccare.solutions.aap.org/content.aspx?gbosid=165499

Parent Resources from the AAP at HealthyChildren.org

• Challenges Faced by Parents of Children with Congenital Heart Disease: https://www.healthychildren.org/English/health-issues/
conditions/heart/Pages/Challenges-Faced-by-Parents-of-Children-with-Congenital-Heart-Disease.aspx
• Pulse Oximetry Screening to Detect Newborn Critical Congenital Heart Disease: https://www.healthychildren.org/English/ages-stages/
baby/Pages/Newborn-Pulse-Oximetry-Screening-to-Detect-Critical-Congenital-Heart-Disease.aspx
For a comprehensive library of AAP parent handouts, please go to the Pediatric Patient Education site at http://patiented.aap.org.

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PIR Quiz
There are two ways to access the journal CME quizzes:
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1. A 15-day-old male neonate presents with tachypnea, tachycardia, and hepatomegaly but REQUIREMENTS: Learners
no heart murmur. The neonate originally passed the newborn pulse oximetry heart screen. can take Pediatrics in Review
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C. Large ventricular septal defect. articles for AMA PRA
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E. Truncus arteriosus. must demonstrate a minimum
2. A female newborn with Down syndrome receives a diagnosis of atrioventricular septal performance level of 60% or
defect. She was evaluated by the genetics service, and her chromosome study findings are higher on this assessment. If
pending. Her newborn screening result is normal, with no evidence of hypothyroidism. you score less than 60% on the
Physical examination findings are significant for typical Down syndrome features. Heart assessment, you will be given
examination demonstrates a holosystolic murmur. Which of the following is the most additional opportunities to
appropriate next step in the care of this patient? answer questions until an
A. Monitor the patient for possible feeding difficulties prior to discharge. overall 60% or greater score
B. Restrict caloric intake to 75 cal/kg per day. is achieved.
C. Restrict total fluid intake to three-fourths maintenance levels. This journal-based CME
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3. A male full-term neonate born via spontaneous vaginal delivery is admitted to the nursery. which the learner completes
The initial newborn examination performed in the first hours after birth yielded the quiz.
unremarkable findings, and the baby was allowed to room in with mom. At 24 hours after
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findings were significant for poor lower-extremity pulses when compared to the upper
extremities and a harsh systolic murmur that was heard loudest over the back. Newborn
oximetry screening showed a saturation of 89% in the lower extremities, compared to 94%
in the upper extremities. Four extremity blood pressures showed a difference of 20 mm Hg 2017 Pediatrics in Review now
between the upper and lower extremities. In addition to starting intravenous fluids and is approved for a total of 30
prostaglandin infusion, which of the following is the next best step in management of this Maintenance of Certification
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C. Echocardiography. Portfolio Program. Complete
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4. You are treating a female neonate in the well-baby nursery who is cyanotic 24 hours after
score on each, and start
birth. At physical examination she has micrognathia, malar flattening, and a harsh systolic
claiming MOC credits as early
murmur, with maximal intensity at the left sternal border. Which of the following is the
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most appropriate next step in the care of this baby?
to claim MOC points, go to:
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C. Immediate pulmonary balloon dilation.
D. Stat administration of digoxin.
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 5. A male newborn was delivered via emergency cesarean delivery due to fetal heart
decelerations. Apgar scores were 5 and 6 at 1 and 5 minutes, respectively. The baby was
placed on 100% oxygen at birth. Eight hours after birth, he was noted to have tachypnea
and respiratory distress, as well as poor systemic perfusion with feeble pulse, poor urine
output, shock, and mottling. Chest radiographs showed pulmonary vascular congestion.
Echocardiography showed cardiomegaly, hypoplastic left heart syndrome, tricuspid
atresia, and a single ventricle. Which of the following is the most appropriate next step in
the care of this patient?
A. Balloon atrial septostomy, followed by reparative surgery to switch the great
vessels.
B. Fontan procedure, followed by a Glenn procedure and the modified Blalock-
Taussig-Thomas (mBTT) shunt.
C. mBTT shunt at 4 to 6 months of age.
D. Normal saline bolus of 20 mL/kg.
E. Prostaglandin infusion until staged repair is performed.

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 Congenital Heart Disease
Kriti Puri, Hugh D. Allen and Athar M. Qureshi
Pediatrics in Review 2017;38;471
DOI: 10.1542/pir.2017-0032

Updated Information & including high resolution figures, can be found at:
Services http://pedsinreview.aappublications.org/content/38/10/471
References This article cites 40 articles, 17 of which you can access for free at:
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 Congenital Heart Disease
Kriti Puri, Hugh D. Allen and Athar M. Qureshi
Pediatrics in Review 2017;38;471
DOI: 10.1542/pir.2017-0032

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/38/10/471

Data Supplement at:

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