| Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death? | |
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MedLine Citation:
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PMID: 12535830 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Anji T Yetman; Renee A Bornemeier; Brian W McCrindle |
Publication Detail:
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Type: Evaluation Studies; Journal Article |
Journal Detail:
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Title: Journal of the American College of Cardiology Volume: 41 ISSN: 0735-1097 ISO Abbreviation: J. Am. Coll. Cardiol. Publication Date: 2003 Jan |
Date Detail:
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Created Date: 2003-01-21 Completed Date: 2003-03-04 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8301365 Medline TA: J Am Coll Cardiol Country: United States |
Other Details:
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Languages: eng Pagination: 329-32 Citation Subset: AIM; IM |
Affiliation:
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University of Arkansas for Medical Sciences, Arkansas Children's Hospital, Little Rock, USA. Yetman.Angela@tchden.org |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Arrhythmias, Cardiac / epidemiology*, etiology, mortality Child Child, Preschool Death, Sudden, Cardiac / etiology* Echocardiography Electrocardiography, Ambulatory Female Follow-Up Studies Humans Infant Infant, Newborn Logistic Models Male Marfan Syndrome / complications*, mortality, ultrasonography Middle Aged Prevalence Prognosis Survival Analysis |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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