Dilated cardiomyopathy in a patient with Marfan syndrome accompanied by chronic type A aortic dissection and right atrial thrombus.
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.
Department of Cardiology, Goztepe Education and Research Hospital, Istanbul, Turkey.
Gökhan
G
Kahveci
Ayhan
A
Erkol
Fatih
F
Yilmaz
D000328:Adult; D000784:Aortic Dissection / Q000150:complications / Q000175:diagnosis*; D001014:Aortic Aneurysm / Q000150:complications / Q000175:diagnosis*; D002311:Cardiomyopathy, Dilated / Q000150:complications / Q000175:diagnosis*; D002908:Chronic Disease; D005260:Female; D006325:Heart Atria / Q000473:pathology*; D006331:Heart Diseases / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*; D013927:Thrombosis / Q000150:complications / Q000175:diagnosis*