A 14-year-old boy presents with exertional syncope. Examination reveals a harsh systolic murmur at the right upper sternal border radiating to the carotids and a delayed, soft carotid upstroke. Echocardiography shows a bicuspid aortic valve with a peak gradient of 75 mmHg across the valve. Which of the following is the most appropriate management?

• A Ross procedure with autograft transfer
• B Beta blocker therapy with annual echocardiographic review
• C Surgical aortic valve replacement with a mechanical prosthesis
• D Percutaneous balloon aortic valvuloplasty ✓

Explanation

Severe congenital aortic stenosis, defined by a peak gradient above 50 mmHg or symptoms such as syncope, requires relief of obstruction. In children and adolescents the valve leaflets are thin and noncalcified, so percutaneous balloon valvuloplasty is the first-line intervention. Valve replacement is reserved for failed valvotomy or calcified valves, and the Ross procedure is generally a later option because of autograft degeneration over time.

Reference: Nelson Textbook of Pediatrics, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.