,Q1 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
A patient with a large ventricular septal defect (VSD) and severe pulmonary
hypertension is being evaluated for operability. Which hemodynamic parameter
most reliably predicts a favorable postoperative outcome?
A. Pulmonary vascular resistance index (PVRI) less than 6 Wood units-m² CORRECT
B. Pulmonary-to-systemic blood flow ratio (Qp/Qs) greater than 2:1
C. Mean pulmonary artery pressure less than 50% of systemic mean
D. Transpulmonary pressure gradient less than 15 mm Hg
RATIONALE: PVRI below 6 Wood units-m², especially when responsive to pulmonary
vasodilators, indicates reversible pulmonary vascular disease and predicts good surgical
outcome. Qp/Qs >2:1 is typical of large VSD but does not assess operability. Mean PAP and
transpulmonary gradient are less specific and may be elevated in high-flow states without fixed
obstruction.
Q2 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
In a patient with complete transposition of the great arteries (D-TGA) and an intact
ventricular septum, which finding on echocardiography would contraindicate an
arterial switch operation (ASO) and instead favor a Mustard or Senning
procedure?
A. Left ventricular posterior wall thickness Z-score of -2.5 CORRECT
B. Mitral valve E/A ratio of 0.8
C. Right ventricular ejection fraction of 55%
D. Aortic valve annulus Z-score of -1.0
RATIONALE: In D-TGA with intact septum, the left ventricle (LV) is at low pressure and may
become deconditioned. An LV posterior wall thickness Z-score of -2.5 indicates significant LV
mass regression, increasing the risk of postoperative LV failure after ASO. This finding supports a
Mustard/Senning atrial switch, which maintains the RV as the systemic ventricle. E/A ratio, RVEF,
and aortic annulus size are not primary contraindications.
Page 2
,Q3 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
Which electrocardiographic finding is most consistent with arrhythmogenic right
ventricular cardiomyopathy (ARVC) in a pediatric patient?
A. Epsilon wave in leads V1-V3 CORRECT
B. Prolonged PR interval with delta waves
C. ST-segment elevation in leads II, III, aVF
D. Left bundle branch block with left axis deviation
RATIONALE: Epsilon waves, small deflections after the QRS in right precordial leads, are a
major diagnostic criterion for ARVC. Delta waves and prolonged PR suggest pre-excitation
(WPW). ST elevation in inferior leads may indicate ischemia or Brugada pattern. LBBB with left
axis deviation is seen in some cardiomyopathies but is not specific for ARVC.
Q4 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
A patient with a history of repaired tetralogy of Fallot presents with exertional
dyspnea. Echocardiogram shows severe pulmonary regurgitation and a right
ventricular end-diastolic volume index (RVEDVI) of 170 mL/m² by cardiac MRI.
Which intervention is most strongly indicated?
A. Pulmonary valve replacement (PVR) CORRECT
B. Beta-blocker therapy alone
C. Observation with annual echocardiograms
D. Catheter-based pulmonary artery angioplasty
RATIONALE: In repaired TOF with severe PR and RV dilation, pulmonary valve replacement is
indicated when RVEDVI >150 mL/m², RVESVI >80 mL/m², or there is symptomatic
arrhythmia/dysfunction. Beta-blockers do not address the volume load. Observation is
inappropriate given symptoms and RV size. Angioplasty is irrelevant without branch pulmonary
artery stenosis.
Page 3
, Q5 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
Which of the following best describes the mechanism of action of ranolazine in the
treatment of pediatric heart failure?
A. Inhibition of late sodium current, reducing intracellular calcium overload CORRECT
B. Blockade of beta-1 adrenergic receptors, decreasing heart rate
C. Inhibition of angiotensin-converting enzyme, reducing afterload
D. Antagonism of aldosterone receptors, preventing fibrosis
RATIONALE: Ranolazine inhibits the late sodium current (INa), which reduces sodium and
calcium overload in cardiomyocytes, thereby improving diastolic function and reducing
myocardial ischemia. Beta-blockers, ACE inhibitors, and aldosterone antagonists have different
mechanisms and are not the primary action of ranolazine.
Q6 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
In a neonate with ductal-dependent systemic circulation (e.g., hypoplastic left
heart syndrome) receiving prostaglandin E1, which adverse effect is most likely to
necessitate dose reduction?
A. Fever and apnea CORRECT
B. Hypotension and tachycardia
C. Seizures and intracranial hemorrhage
D. Necrotizing enterocolitis
RATIONALE: Prostaglandin E1 commonly causes fever and apnea, especially in neonates.
Apnea may require dose reduction or ventilatory support. Hypotension and tachycardia are less
common; seizures and intracranial hemorrhage are rare; NEC is not a direct effect of PGE1.
Page 4
CONGENITAL HEART LESIONS
A patient with a large ventricular septal defect (VSD) and severe pulmonary
hypertension is being evaluated for operability. Which hemodynamic parameter
most reliably predicts a favorable postoperative outcome?
A. Pulmonary vascular resistance index (PVRI) less than 6 Wood units-m² CORRECT
B. Pulmonary-to-systemic blood flow ratio (Qp/Qs) greater than 2:1
C. Mean pulmonary artery pressure less than 50% of systemic mean
D. Transpulmonary pressure gradient less than 15 mm Hg
RATIONALE: PVRI below 6 Wood units-m², especially when responsive to pulmonary
vasodilators, indicates reversible pulmonary vascular disease and predicts good surgical
outcome. Qp/Qs >2:1 is typical of large VSD but does not assess operability. Mean PAP and
transpulmonary gradient are less specific and may be elevated in high-flow states without fixed
obstruction.
Q2 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
In a patient with complete transposition of the great arteries (D-TGA) and an intact
ventricular septum, which finding on echocardiography would contraindicate an
arterial switch operation (ASO) and instead favor a Mustard or Senning
procedure?
A. Left ventricular posterior wall thickness Z-score of -2.5 CORRECT
B. Mitral valve E/A ratio of 0.8
C. Right ventricular ejection fraction of 55%
D. Aortic valve annulus Z-score of -1.0
RATIONALE: In D-TGA with intact septum, the left ventricle (LV) is at low pressure and may
become deconditioned. An LV posterior wall thickness Z-score of -2.5 indicates significant LV
mass regression, increasing the risk of postoperative LV failure after ASO. This finding supports a
Mustard/Senning atrial switch, which maintains the RV as the systemic ventricle. E/A ratio, RVEF,
and aortic annulus size are not primary contraindications.
Page 2
,Q3 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
Which electrocardiographic finding is most consistent with arrhythmogenic right
ventricular cardiomyopathy (ARVC) in a pediatric patient?
A. Epsilon wave in leads V1-V3 CORRECT
B. Prolonged PR interval with delta waves
C. ST-segment elevation in leads II, III, aVF
D. Left bundle branch block with left axis deviation
RATIONALE: Epsilon waves, small deflections after the QRS in right precordial leads, are a
major diagnostic criterion for ARVC. Delta waves and prolonged PR suggest pre-excitation
(WPW). ST elevation in inferior leads may indicate ischemia or Brugada pattern. LBBB with left
axis deviation is seen in some cardiomyopathies but is not specific for ARVC.
Q4 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
A patient with a history of repaired tetralogy of Fallot presents with exertional
dyspnea. Echocardiogram shows severe pulmonary regurgitation and a right
ventricular end-diastolic volume index (RVEDVI) of 170 mL/m² by cardiac MRI.
Which intervention is most strongly indicated?
A. Pulmonary valve replacement (PVR) CORRECT
B. Beta-blocker therapy alone
C. Observation with annual echocardiograms
D. Catheter-based pulmonary artery angioplasty
RATIONALE: In repaired TOF with severe PR and RV dilation, pulmonary valve replacement is
indicated when RVEDVI >150 mL/m², RVESVI >80 mL/m², or there is symptomatic
arrhythmia/dysfunction. Beta-blockers do not address the volume load. Observation is
inappropriate given symptoms and RV size. Angioplasty is irrelevant without branch pulmonary
artery stenosis.
Page 3
, Q5 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
Which of the following best describes the mechanism of action of ranolazine in the
treatment of pediatric heart failure?
A. Inhibition of late sodium current, reducing intracellular calcium overload CORRECT
B. Blockade of beta-1 adrenergic receptors, decreasing heart rate
C. Inhibition of angiotensin-converting enzyme, reducing afterload
D. Antagonism of aldosterone receptors, preventing fibrosis
RATIONALE: Ranolazine inhibits the late sodium current (INa), which reduces sodium and
calcium overload in cardiomyocytes, thereby improving diastolic function and reducing
myocardial ischemia. Beta-blockers, ACE inhibitors, and aldosterone antagonists have different
mechanisms and are not the primary action of ranolazine.
Q6 DEMONSTRATE MASTERY OF HEMODYNAMIC PRINCIPLES AND THEIR APPLICATION TO
CONGENITAL HEART LESIONS
In a neonate with ductal-dependent systemic circulation (e.g., hypoplastic left
heart syndrome) receiving prostaglandin E1, which adverse effect is most likely to
necessitate dose reduction?
A. Fever and apnea CORRECT
B. Hypotension and tachycardia
C. Seizures and intracranial hemorrhage
D. Necrotizing enterocolitis
RATIONALE: Prostaglandin E1 commonly causes fever and apnea, especially in neonates.
Apnea may require dose reduction or ventilatory support. Hypotension and tachycardia are less
common; seizures and intracranial hemorrhage are rare; NEC is not a direct effect of PGE1.
Page 4
