Document Detail

The spectrum of cardiac defects in the Ehlers-Danlos syndrome, types I and III.
MedLine Citation:
PMID:  7352721     Owner:  NLM     Status:  MEDLINE    
Nineteen patients with types I and III Ehlers-Danlos syndrome were hospitalized at our institution between 1973 and 1978. Chest roentgenogram, electrocardiogram, and echocardiogram were done; 11 patients underwent cardiac catheterization. Thirty-five cardiac or great vessel abnormalities were detected. Fifteen patients had nitral valve prolapse; six also had tricuspid valve prolapse. Dilatation of the aortic root or extasia of the sinuses of Valsalva, or both, occurred in six patients. Dilatation of the pulmonary artery and annulus caused pulmonary regurgitation in one patient. Congenital heart defects included bicuspid aortic valve (two), pulmonary valvular stenosis (one), ventricular septal defect (two), and an atrial septal defect (one). The apparent high prevalence of cardiovascular abnormalities in hospitalized patients with types I and III Ehlers-Danlos syndrome necessitates a careful cardiovascular evaluation. Conversely, Ehlers-Danlos syndrome types I and III should be excluded in patients with mitral or tricuspid valve prolapse, great vessel dilatation, and congenital heart defects.
C V Leier; T D Call; P K Fulkerson; C F Wooley
Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of internal medicine     Volume:  92     ISSN:  0003-4819     ISO Abbreviation:  Ann. Intern. Med.     Publication Date:  1980 Feb 
Date Detail:
Created Date:  1980-03-27     Completed Date:  1980-03-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372351     Medline TA:  Ann Intern Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  171-8     Citation Subset:  AIM; IM    
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MeSH Terms
Ehlers-Danlos Syndrome / complications*
Heart / radiography
Heart Catheterization
Heart Defects, Congenital / complications*,  diagnosis
Heart Diseases / complications
Middle Aged

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