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Medical Therapy in Marfan Aortopathy — EECC MCQ

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ModerateAortic DiseaseMedical Therapy in Marfan AortopathyEECC

A 50-year-old man with Marfan syndrome on maximal losartan therapy has an aortic root diameter of 48 mm (stable over 3 years). He asks whether beta-blockers should be added. What is the evidence?

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Correct answer: C — Beta-blockers are recommended in Marfan syndrome to reduce the rate of aortic dilatation by decreasing aortic wall shear stress (dP/dt); they should be used in addition to, or as an alternative to, ARBs

Beta-blockers have been the mainstay of medical therapy for Marfan syndrome since Shores et al. (1994) demonstrated reduced aortic dilatation rate. They reduce aortic wall stress by lowering heart rate (reducing dP/dt — the rate of rise of aortic pressure) and blood pressure. ARBs (losartan, irbesartan) were hypothesised to provide additional benefit through TGF-beta pathway modulation, but the randomised COMPARE and Marfan Sartan trials showed that ARBs are non-inferior but not clearly superior to beta-blockers. The ESC Aortic Disease Guidelines recommend beta-blockers and/or ARBs for all Marfan patients with aortic dilatation. This patient's aortic root at 48 mm is below the surgical threshold (≥50 mm, or ≥45 mm with risk factors).

Reference: ESC (2024): Guidelines for Peripheral Arterial and Aortic Diseases