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Diagnosis and management of ebstein anomaly of the tricuspid valve.
MedLine Citation:
PMID:  23001420     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. Since the original description in a 19-year-old laborer with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare condition has increased to the recognition that it is an abnormality not only of the TV, but also of the right ventricle (RV). EA is the result of failure of delamination of the TV leaflets from the interventricular septum, resulting in adherence of the leaflets to the underlying myocardium. This results in a wide variety of abnormalities, including apical and posterior displacement of the dilated TV annulus; dilation of the "atrialized" portion of the RV; and fenestrations, redundancy, and tethering of the anterior leaflet of the TV. The malformed TV is usually regurgitant, but may rarely be stenotic. The clinical manifestations of EA in the adult depend on several factors, including the extent of TV leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and presence of a right-to-left atrial level shunt. Over the past several decades, advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect.
Authors:
Eric V Krieger; Anne Marie Valente
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Current treatment options in cardiovascular medicine     Volume:  14     ISSN:  1534-3189     ISO Abbreviation:  Curr Treat Options Cardiovasc Med     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-11-27     Completed Date:  2012-11-28     Revised Date:  2013-07-25    
Medline Journal Info:
Nlm Unique ID:  9815942     Medline TA:  Curr Treat Options Cardiovasc Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  594-607     Citation Subset:  -    
Affiliation:
Seattle Adult Congenital Heart Service, University of Washington Medical Center, Seattle Children's Hospital, 1959 NE Pacific Street, Seattle, WA, 98195, USA, ekrieger@u.washington.edu.
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