pediatrics
Ebstein Anomaly
Introduction
Ebstein anomaly is a rare congenital cardiac defect that consists of variable apical displacement from the annulus and dysplasia of the septal and inferior leaflets of the tricuspid valve. The anterior leaflet always has a normal annular insertion, but is often dysplastic with abnormal distal attachments. This anomaly results in a portion of the right ventricle functioning as an atrium, with resulting decrease in the functional right ventricular volume. A wide variety of adverse hemodynamic sequelae may occur related to tricuspid valve dysfunction, right ventricular failure, cyanosis due to atrial shunting, and arrhythmias. There is a wide spectrum of manifestations depending on the age at presentation. Diagnosis is with ECG and imaging. Treatment is with medications and surgery, and sometimes with interventional catheterization. (See also Overview of Congenital Cardiovascular Anomalies .) 