Ebstein anomaly
Pronunciation: /EB-styne/
A congenital heart defect characterized by abnormal formation and displacement of the tricuspid valve.
Full Definition
Ebstein anomaly is a congenital malformation of the tricuspid valve characterized by apical displacement of the septal and posterior leaflets into the right ventricle, resulting in 'atrialization' of part of the right ventricle. The anterior leaflet is typically enlarged and sail-like. The severity ranges from mild tricuspid regurgitation to severe cyanosis with right heart failure. Associated defects may include atrial septal defects and accessory conduction pathways (Wolff-Parkinson-White syndrome). Treatment options range from medical management to tricuspid valve repair or replacement, depending on symptom severity and functional status.
Usage
Usage note: Often associated with Wolff-Parkinson-White syndrome; specify severity in documentation.
In Context
- "The echocardiogram revealed severe Ebstein anomaly with significant tricuspid regurgitation." — Diagnostic report
- "Cone reconstruction was performed for symptomatic Ebstein anomaly in the adolescent patient." — Surgical note