Ebstein's Anomaly

Ebstein’s anomaly is the result of inferior displacement of the tricuspid valve into the right ventricle, which results in atrialization of the right ventricle. As a result, the right ventricle is very small and not infrequently hypocontractile. The posterior and septal leaflets of the tricuspid valve are often small and inadequate, and the anterior leaflet is very large and redundant, resembling a sail. About 25% of Ebstein’s anomaly patients have accessory pathways for AV conduction (Wolff-Parkinson-White syndrome), which often are multiple. About 50% of patients also have an ASD or a patent foramen ovale (PFO), and right-to-left shunting through these defects results in cyanosis.