Ebstein Anomaly
Pronunciation: EB-stine
A congenital heart defect characterized by abnormal development and displacement of the tricuspid valve leaflets into the right ventricle.
Full Definition
Ebstein anomaly is a rare congenital heart defect involving malformation of the tricuspid valve, where the septal and posterior leaflets are displaced toward the apex of the right ventricle. This displacement creates an 'atrialized' portion of the right ventricle that functions as part of the right atrium. The condition results in tricuspid regurgitation and reduced functional right ventricular size. Clinical presentation ranges from asymptomatic to severe cyanosis and arrhythmias, depending on the degree of valve displacement and associated anomalies such as atrial septal defects or accessory conduction pathways. Treatment options include valve repair, replacement, or in severe cases, conversion to single ventricle physiology.
Usage
Usage note: Named after Wilhelm Ebstein. Ensure proper capitalization as it is an eponym.
In Context
- "Severe Ebstein anomaly presented with marked cardiomegaly and profound tricuspid regurgitation." — Echocardiography report
- "The patient with Ebstein anomaly developed supraventricular tachycardia requiring ablation therapy." — Electrophysiology consultation note