Abstract 13024: Cardiovascular Disease Beyond the Aorta in Marfan Syndrome

Abstract

Introduction: Aortic disease is the major cardiovascular disease in Marfan syndrome (MFS), however numerous studies indicate that other heart conditions may also be a major disease burden for patients with MFS Hypothesis: We hypothesized that the burden of non-aortic cardiovascular disease in MFS is considerable and the aim of the study is therefore to assess the extent of this issue in Danish patient with MFS. Methods: A register-based population study of Danish patients with a Ghent II verified Marfan Syndrome diagnosis. All patients were alive January 1st 1994, at the beginning of the ICD-10 system. Each patient was age-matched with up to 100 controls with the same sex. Results: We identified 407 MFS patients from the Danish healthcare system (1977-2014) and compared our findings with 40,700 persons from the background population. We found the following hazard ratios and confidence intervals: Mitral valve disease HR: 60.8 [39.5-93.5], p<0.0001. Cardiomyopathy HR:12.7 [5.8-28.0], p<0.0001. Heart failure HR 8.2 [5.2-12.9], p <0.0001. Tachyarrhythmia HR: 9.7 [7.5-12.6], p <0.0001. AV-Block HR: 4.7 [1.5-15.1], p<0.01. Sudden cardiac death HR: 8.3 [3.8-18.0], p<0.0001. Discussion: The registration of mitral valve disease occurred earlier and more often in women with MFS compared to men with MFS (age at registration: 22 vs. 38 years, HR: 2.2 (1.1-4.6) Among patients with arrhythmia 20 % had ventricular ectopics or tachycardia. Forty percent of the cases with tachyarrhythmia were unrelated to heart surgery and 37 % of the total cohort had tachyarrhythmia without having any surgery done. AV-block was in all cases related to heart surgery. Twenty patients with MFS had a registration with heart failure, whereof 9 were diagnosed unrelated to heart surgery. Looking only at registrations unrelated to heart surgery the HR was 4.8 (2.5-9.5). There was no difference in registration of heart failure between men and women. The sudden cardiac death etiology was unclear due to lack of autopsies. Conclusion: The burden of non-aortic cardiovascular disease is massive in patients with MFS and this must be in focus in the caretaking of patients with MFS