A 4-year-old girl has exertional dyspnoea. Examination shows a wide, fixed split S2 with a loud ejection systolic murmur and palpable thrill at the left upper sternal border, plus an early systolic click. Echo shows a domed pulmonary valve with a peak gradient of 75 mmHg across it. The BEST next step in management is:

• A Surgical pulmonary valvotomy
• B Balloon pulmonary valvuloplasty ✓
• C Prostaglandin E1 infusion
• D Annual follow-up with echocardiography

Explanation

Peak gradient above approximately 50 mmHg defines severe pulmonary stenosis and is an accepted indication for intervention. Percutaneous balloon valvuloplasty is the treatment of choice for valvar pulmonary stenosis with a favourable morphology, replacing surgical valvotomy. Prostaglandin is reserved for duct-dependent neonates, and observation is inappropriate at this gradient because untreated severe stenosis risks right ventricular failure and arrhythmia.

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

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Written and medically reviewed by the StethoPrep medical team.