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Answer 4 of 50

Acyanotic congenital heart disease

A 9-month-old girl with a large 8 mm perimembranous ventricular septal defect has taken furosemide, spironolactone, captopril and digoxin for 5 months. She still sweats and tires with feeds; weight 5.8 kg (below the 3rd percentile), respiratory rate 62/min, liver 3 cm down, accentuated P2. Echo shows a left-to-right shunt with right ventricular systolic pressure 60 mmHg.

What is the most appropriate management?

Correct answer: A — Cardiac catheterization to measure pulmonary vascular resistance, followed by surgical closure of the ventricular septal defect

A large ventricular septal defect with failure to thrive, heart failure and pulmonary hypertension needs catheterization to confirm operability, then surgical closure.

Five months of furosemide, spironolactone, captopril and digoxin have failed: medical therapy in infancy is a bridge to closure, not an alternative to it.
An accentuated P2 with an estimated right ventricular systolic pressure of 60 mmHg means the pulmonary circulation is already under threat.
Catheterization measures the pulmonary vascular resistance and the Qp:Qs and confirms the shunt is still reversible, which is what decides operability.
Closure must happen within the first year of life, before pulmonary vascular disease becomes irreversible (Eisenmenger syndrome).

★ Clinical pearl

A large ventricular septal defect with failure to thrive and pulmonary hypertension that does not respond to medical therapy must be closed within the first year of life; when the pulmonary pressure is high, catheterization first proves the resistance is still reversible.

Exam note

Recalls disagree here and there is no published answer key, so the stem decides.
Most recalls answer catheterisation then surgical closure, which is correct when the stem carries pulmonary hypertension such as an accentuated or palpable second sound or a right ventricular systolic pressure of 60 mmHg: the resistance must be shown to be still reversible before the defect is closed.
A minority answer treat the heart failure and refer for surgery with no catheterisation, which is right only when the haemodynamics are already documented and there is no pulmonary hypertension, for example a known Qp:Qs above 2 to 1 with a normal second sound.
Signs that the operable window is closing: a shrinking pansystolic murmur with a louder single S2, loss of the mid-diastolic rumble, falling saturations and a smaller heart on chest film.

Go deeper — why the other options fail, and what candidates recalled

Option	Why not
B	Nutritional support and diuretics are reasonable adjuncts but they cannot fix a large shunt. Another 3 months of failure to thrive with pulmonary hypertension risks irreversible pulmonary vascular change and a worse surgical outcome.
C	The pulmonary hypertension here is caused by the shunt itself and is expected to regress after closure. Pulmonary vasodilators are for pulmonary vascular disease that persists after closure, or that makes closure impossible, not for an operable left-to-right shunt.
D	A large perimembranous defect in a 5.8 kg infant is not device territory. The rim sits close to the aortic valve and to the conduction tissue, so device closure at this size carries a real risk of aortic regurgitation and complete heart block; surgical patch closure is the standard at this age and weight.

What candidates actually recalled: Catheterization and surgery (found in 4 recall sets)

Consistent with 2018 AHA/ACC adult congenital heart disease guideline; Nelson Textbook of Pediatrics

Case Complete