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Late AV Valve Regurgitation Post-AVSD — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Late AV Valve Regurgitation Post-AVSDEECC

A 35-year-old man with surgically repaired complete AVSD (Down syndrome) is found to have a new 3/6 systolic murmur at the apex. Echocardiography shows moderate left AV valve regurgitation through a residual cleft. What is the most appropriate management?

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Correct answer: C — Surgical re-repair or replacement of the left AV valve if regurgitation is severe or progressing with LV dilatation — the cleft in the 'mitral' component of the common AV valve is the most common cause of late left AV valve regurgitation after AVSD repair

Late left AV valve regurgitation is the most common reason for reoperation after AVSD repair (10-15% of patients). The left AV valve in AVSD has a 'cleft' in the bridging leaflet tissue (not a true mitral valve — it has 3 leaflets unlike the normal mitral valve's 2). The cleft may be closed at initial repair, but can reopen or become insufficient over time. The 2020 ESC ACHD Guidelines recommend: (1) monitoring with serial echocardiography; (2) surgical intervention when regurgitation is severe with LV dilatation or symptoms; (3) re-repair is preferred over replacement when feasible; (4) Down syndrome is NOT a contraindication to re-operation (outcomes are comparable to non-Down AVSD repair, though anaesthetic/airway considerations and atlantoaxial instability must be addressed).

Reference: ESC (2020): ACHD Guidelines