Ebstein's anomaly

Synonym: Ebstein's malformation

Ebstein's anomaly is a rare congenital heart defect, where the tricuspid valve is deformed and transposed into the the right ventricle.

Classification

Depending from the grade of transposition Ebstein's anomaly is classified as light or severe.

Findings

• leaking tricuspid valve
• dilatation of right atrium
• small right ventricle, often with low output
• In some cases atrial septal defect or ventricular septal defect with shunts.

Symptoms

• Central cyanosis
• dyspnea
• atypical angina
• palpitations
• supraventricular arrhythmias.

Reference
W. Ebstein, Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238-254. Translation in American Journal of Cardiology, New York, 1968, 22: 867-872.