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Marfan Aortic Root Repair — RACP Adult Medicine MCQ

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HardCardiologyMarfan Aortic Root RepairRACP Adult Medicine

A 35-year-old man with Marfan syndrome (confirmed FBN1 mutation) has an ascending aortic aneurysm measuring 48 mm. His brother had an aortic dissection at age 30 (aortic diameter was 50 mm at the time). He is on losartan and bisoprolol. His aortic root has grown 4 mm in the past year. What is the most appropriate management?

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Correct answer: C — Prophylactic surgical aortic root replacement

Marfan syndrome with ascending aortic aneurysm 48 mm but with HIGH-RISK features: family history of aortic dissection at <50 mm AND rapid growth (4 mm/year, >3 mm threshold) = indication for prophylactic surgical repair at a lower threshold (≥45 mm with risk factors per ESC 2024). This patient meets criteria for prophylactic aortic root replacement (Bentall procedure or David valve-sparing root replacement). Pre-operative genetic counselling and family screening are essential.

Reference: ESC – 2024 – Aortic Disease; Ghent Nosology 2010; CSANZ 2024