Abstract:
OBJECTIVES:We sought to assess outcomes in a series of young patients with Marfan syndrome and to define the prevalence of ventricular arrhythmias in this patient population. BACKGROUND:While sudden death is a well-recognized outcome in Marfan syndrome, ventricular arrhythmias are not well described. METHODS:Patients were followed with echocardiography, electrocardiography, and ambulatory electrocardiography. The prevalence and associated factors for ventricular dysrhythmias were defined. RESULTS:Seventy patients with Marfan syndrome diagnosed at birth to 52 years were followed for a period of up to 24 years. All patients had cardiovascular involvement and were started on medical therapy. No patient died from aortic dissection, while 4% died from arrhythmias. Ventricular arrhythmias were present in 21% and were associated with increased left ventricular size, mitral valve prolapse, and abnormalities of repolarization. CONCLUSIONS:Cardiac complications are rare in young patients with Marfan syndrome receiving medical therapy and close clinical follow-up. Sudden death still occurs, and appears more common in patients with a dilated left ventricle. Left ventricular dilation may predispose to alterations of repolarization and fatal ventricular arrhythmias.
journal_name
J Am Coll Cardioljournal_title
Journal of the American College of Cardiologyauthors
Yetman AT,Bornemeier RA,McCrindle BWdoi
10.1016/s0735-1097(02)02699-2subject
Has Abstractpub_date
2003-01-15 00:00:00pages
329-32issue
2eissn
0735-1097issn
1558-3597pii
S0735109702026992journal_volume
41pub_type
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