Document Detail

Morphological aspects of Ebstein's anomaly in adults.
MedLine Citation:
PMID:  11005593     Owner:  NLM     Status:  MEDLINE    
The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.
C Frescura; A Angelini; L Daliento; G Thiene
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  The Thoracic and cardiovascular surgeon     Volume:  48     ISSN:  0171-6425     ISO Abbreviation:  Thorac Cardiovasc Surg     Publication Date:  2000 Aug 
Date Detail:
Created Date:  2001-01-03     Completed Date:  2001-01-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7903387     Medline TA:  Thorac Cardiovasc Surg     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  203-8     Citation Subset:  IM    
Department of Pathology, University of Padua Medical School, Italy.
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MeSH Terms
Age Distribution
Cause of Death
Child, Preschool
Coronary Angiography
Death, Sudden, Cardiac / epidemiology,  etiology
Ebstein Anomaly / classification,  complications,  embryology,  epidemiology,  pathology*,  physiopathology
Heart Failure / epidemiology,  etiology
Infant, Newborn
Italy / epidemiology
Middle Aged
Risk Factors

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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