Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study

Objectives: Studies indicate that other cardiovascular problems than aortic disease are a burden for patients with Marfan syndrome (MFS). The aim of the study was to assess the extent of this issue. Methods: A registry-based population study of patients with a Ghent II verified MFS diagnosis. Each patient was matched with up to 100 controls on age and sex. From the Danish healthcare system, we identified 407 MFS patients (from 1977 to 2014) and their cardiovascular events and compared them with those in 40,700 controls. Total follow-up time was 16,439 person years. Results: Mitral valve disease was significantly more common in MFS [HR: 58.9 (CI 38.1–91.1)] and happened earlier and more often in women than men with MFS [age at first registration: 22 vs. 38 years, HR: 2.1 (CI 1.0–4.4)]. Heart failure/cardiomyopathy was also more common in MFS [HR: 8.7 (CI 5.7–13.4)] and men were more affected than women, and at younger age [39 vs. 64 years, HR: 0.18 (CI 0.06–0.55)]. In all cases, atrioventricular block [HR: 4.9 (1.5–15.6)] was related to heart surgery. Supraventricular [HR: 9.7 (CI 7.5–12.7)] and ventricular tachycardia [HR: 7.7 (CI 4.2–14.3)] also occurred more often than in the control group. The risk of sudden cardiac death was increased [HR: 8.3 (CI 3.8–18.0)] but the etiology was unclear due to lack of autopsies. Conclusion: Non-aortic cardiovascular disease in patients with MFS is exceptionally prevalent and the range of diseases varies between women and men. Physicians caring for MFS patients must be aware of this large spectrum of cardiovascular diseases. Graphical abstract: [Figure not available: see fulltext.]