Document Detail

Ventricular dysrhythmias in children with Marfan's syndrome.
MedLine Citation:
PMID:  3976608     Owner:  NLM     Status:  MEDLINE    
A teenager with Marfan's syndrome required resuscitation and was found to have multiform premature ventricular contractions and ventricular tachycardia. Of 24 children with Marfan's syndrome, eight (33.3%) were found to have ventricular dysrhythmias, including three with ventricular tachycardia. Six of these eight patients had mitral valve prolapse, and five had prolonged QT or QTU intervals corrected for heart rate. However, only two patients had severe mitral regurgitation, five had only mild heart disease, and one had no detectable heart lesion. The role of mitral valve prolapse and/or delayed repolarization in the development of ventricular dysrhythmia was explored. Delayed repolarization, especially when combined with mitral valve prolapse, is associated with occurrence of ventricular dysrhythmia. Serious ventricular dysrhythmia can occur in children with Marfan's syndrome with or without substantial valve disease, and the dysrhythmia appears to progress with age.
S Chen; L F Fagan; S Nouri; J L Donahoe
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of diseases of children (1960)     Volume:  139     ISSN:  0002-922X     ISO Abbreviation:  Am. J. Dis. Child.     Publication Date:  1985 Mar 
Date Detail:
Created Date:  1985-04-08     Completed Date:  1985-04-08     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0370471     Medline TA:  Am J Dis Child     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  273-6     Citation Subset:  AIM; IM    
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MeSH Terms
Age Factors
Arrhythmias, Cardiac / complications*,  physiopathology
Child, Preschool
Heart Conduction System / physiopathology
Heart Ventricles
Marfan Syndrome / complications*,  physiopathology
Mitral Valve Prolapse / complications,  physiopathology
Tachycardia / complications,  physiopathology

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