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CLINICAL STUDY: MARFAN SYNDROME

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

Anji T. Yetman, MD^*,*, Renee A. Bornemeier, MD, FACC^* and Brian W. McCrindle, MD, MPH, FACC

^* University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas, USA
The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

Manuscript received April 29, 2002; revised manuscript received September 11, 2002, accepted October 10, 2002.

^* Reprint requests and correspondence: Dr. Anji Yetman, Department of Cardiology, The Children’s Hospital, 1056 East 19th Avenue, B-100, Denver, Colorado 80218, USA.
Yetman.Angela{at}tchden.org

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.

Abbreviations and Acronyms   ECG   electrocardiogram   LV   left ventricle   LVED   left ventricular end-diastolic dimension   MR   mitral regurgitation   MVP   mitral valve prolapse

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