Long-term outcome in patients with Marfan syndrome: Is aortic dissection the only cause of sudden death?

Anji T. Yetman, Renee A. Bornemeier, Brian W. McCrindle

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)329-332
Number of pages4
JournalJournal of the American College of Cardiology
Volume41
Issue number2
DOIs
StatePublished - Jan 15 2003

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ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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