An asymptomatic 6-year-old girl is found on school screening to have a harsh ejection systolic murmur at the upper left sternal border with a palpable thrill, a widely split second heart sound, and an early systolic click. She is acyanotic with normal peripheral pulses. Chest X-ray shows a normal sized heart with prominence of the main pulmonary artery. What is the most likely diagnosis?

• A Ostium secundum atrial septal defect
• B Small restrictive ventricular septal defect
• C Valvular pulmonary stenosis ✓
• D Patent ductus arteriosus

Explanation

Valvular pulmonary stenosis produces an ejection systolic murmur at the upper left sternal border with a thrill, an early systolic ejection click, and a wide split S2 because right ventricular ejection is prolonged. Poststenotic dilation produces the prominent main pulmonary artery on X-ray. An ASD gives a softer murmur with FIXED splitting of S2, and the fixed nature is the feature that kills that distractor here.

Reference: Park's Pediatric Cardiology for Practitioners, 7th ed.

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Written and medically reviewed by the StethoPrep medical team.