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
Mitral valve disease and open heart surgery in osteogenesis imperfecta tarda.