High-Yield Verified Practice Questions & Rationales
INTRODUCTION
Mastering pediatric cardiology requires a profound command of shifting
neonatal physiology, complex congenital structural pathways, and delicate
cellular pharmacology. This comprehensive question bank provides an
elite-level assessment tool designed to simulate the rigorous case-based
reasoning demanded by the American Board of Pediatrics (ABP)
certification exam.
Question 1
A 4-day-old infant presents to the emergency department with profound
cyanosis, tachypnea, and poor feeding. Physical examination reveals a
quiet precordium and a single, loud second heart sound (\(S_{2}\)) with
no prominent murmur. Prostaglandin \(E_{1}\) (\(PGE_{1}\)) infusion is
initiated, which improves systemic oxygenation. An urgent
echocardiogram confirms d-Transposition of the Great Arteries (d-TGA)
with an intact ventricular septum and a restrictive patent foramen ovale
(PFO). What is the most appropriate immediate next step in management?
A) Emergent arterial switch operation (ASO)
B) Administration of high-dose furosemide
,C) Balloon atrial septostomy (Rashkind procedure)
D) Surgical creation of a Blalock-Taussig-Thomas shunt
Answer: C) Balloon atrial septostomy (Rashkind procedure)
Explanation: In neonates with d-TGA and an intact ventricular
septum, mixing of systemic and pulmonary blood relies completely
on a patent ductus arteriosus and an atrial communication. If the
PFO is restrictive, severe cyanosis and metabolic acidosis occur
rapidly despite PGE1 administration. A balloon atrial septostomy
improves interatrial mixing immediately, stabilizing the infant until
definitive surgery (the arterial switch operation) can be performed
within the first few weeks of life.
Question 2
A 14-year-old competitive basketball player undergoes a routine sports
screening evaluation. The electrocardiogram (ECG) demonstrates left
ventricular hypertrophy and deep, narrow Q waves (> 3 mm) in the
inferior leads (II, III, aVF) and lateral leads (\(V_{5}\)-\(V_{6}\)). An
echocardiogram reveals asymmetric septal hypertrophy with a maximal
thick measurement of 22 mm and systolic anterior motion (SAM) of the
mitral valve. Which of the following findings is the strongest independent
risk factor for sudden cardiac death (SCD) in this patient?
A) Maximal left ventricular wall thickness of 22 mm
B) Non-sustained ventricular tachycardia (NSVT) on ambulatory Holter
,monitoring
C) Left atrial diameter of 41 mm
D) Mild resting left ventricular outflow tract (LVOT) gradient of 25 mmHg
Answer: B) Non-sustained ventricular tachycardia (NSVT) on
ambulatory Holter monitoring
Explanation: Risk stratification for sudden cardiac death in
Hypertrophic Cardiomyopathy (HCM) is critical for determining ICD
placement. Independent risk factors include a history of aborted
SCD, unexplained syncope, a family history of early HCM-related
death, maximal LV wall thickness ≥ 30 mm, an abnormal blood
pressure response to exercise, and documented non-sustained
ventricular tachycardia (NSVT). A wall thickness of 22 mm is
abnormal but does not meet the high-risk threshold of ≥ 30 mm on
its own.
Question 3
A 6-month-old infant with a known large, unoperated ventricular septal
defect (VSD) presents with poor weight gain, diaphoresis during feedings,
and tachypnea. On physical exam, the infant is thriving poorly, has a
prominent precordial heave, a loud holosystolic murmur at the lower left
sternal border, and a mid-diastolic rumble at the apex. The pulmonic
component of the second heart sound (\(P_{2}\)) is markedly
, accentuated. Which pathophysiologic mechanism best explains the mid-
diastolic rumble heard at the apex?
A) Mitral valve regurgitation caused by left ventricular dilation
B) Increased blood flow across the mitral valve during diastole
C) Concomitant congenital mitral stenosis
D) High-velocity turbulent jets crossing the VSD into the right ventricle
Answer: B) Increased blood flow across the mitral valve during
diastole
Explanation: In large left-to-right shunts (such as a large VSD or
PDA), pulmonary blood flow is vastly increased. This excess volume
returns to the left atrium and flows through the mitral valve into the
left ventricle during early diastole. This massive increase in forward
flow creates a relative mitral stenosis effect, producing a low-
pitched mid-diastolic rumble at the apex. It serves as a clinical
indicator of a large, hemodynamically significant left-to-right shunt.
Question 4
A 4-year-old child presents with a 6-day history of high fever, bilateral
non-purulent conjunctivitis, changes in the oral mucosa (including
erythema and dry, cracked lips), polymorphous rash, and unilateral
cervical lymphadenopathy. Laboratory evaluations indicate elevated
acute-phase reactants (ESR and CRP). Intravenous immunoglobulin (IVIG)