Ebstein anomaly
Pronunciation: EB-stine
A congenital heart defect characterized by apical displacement of the tricuspid valve with atrialization of the right ventricle.
Full Definition
Ebstein anomaly is a rare congenital malformation involving abnormal development of the tricuspid valve and right ventricle. The key feature is apical displacement of the septal and posterior leaflets of the tricuspid valve, creating an 'atrialized' portion of the right ventricle that functions as part of the right atrium. The anterior leaflet is typically enlarged and sail-like. Severity varies from mild forms compatible with normal life to severe cases with cyanosis and heart failure. Associated abnormalities may include atrial septal defects and accessory conduction pathways. The diagnosis is confirmed by echocardiography showing the characteristic valve displacement.
Usage
Usage note: Named after Wilhelm Ebstein; note correct spelling without apostrophe in current usage.
In Context
- "The autopsy revealed classic features of Ebstein anomaly with severe apical displacement of the tricuspid valve." — congenital heart disease autopsy
- "Surgical repair of Ebstein anomaly required tricuspid valve reconstruction due to the dysplastic leaflets." — surgical pathology report