Highlights the importance of close cardiovascular follow-up in patients with Marfan syndrome due to the rare risk of dilated cardiomyopathy.
May indicate cardiomyopathy risk in Marfan syndrome independent of valvular dysfunction; leaves open whether enhanced screening protocols are warranted.
Marfan's syndrome (MFS) is an autosomal dominant disorder of connective tissue involving musculoskeletal, cardiovascular and ocular systems. Aortic disease is the leading cause of mortality in MFS. Among all, dilated cardiomyopathy in the absence of severe valvular dysfunction is a very rare cardiovascular feature of MFS. We report a case of biventricular heart failure in a patient with MFS, complicated by chronic type A aortic dissection and right atrial thrombus. This report clearly highlights the importance of close cardiovascular follow-up in patients with MFS.
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Kahvecı et al. (2010) studied this question.
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