Document Detail


Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death?
MedLine Citation:
PMID:  12535830     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Anji T Yetman; Renee A Bornemeier; Brian W McCrindle
Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
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:
Created Date:  2003-01-21     Completed Date:  2003-03-04     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  329-32     Citation Subset:  AIM; IM    
Affiliation:
University of Arkansas for Medical Sciences, Arkansas Children's Hospital, Little Rock, USA. Yetman.Angela@tchden.org
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MeSH Terms
Descriptor/Qualifier:
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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