This defect affects the tri cuspid valve. This valve guards the right atrium and ventricle. The valve is abnormally positioned since birth and there is severe leak of blood back into the right atrium leading to enlargement of right atrium and also part of the ventrical gets incorporated into right atrium (atrialization of right ventrical). People born with Ebstein anomaly will have associated defects like Atrial Septal defect, Pulmonary Artesia and Atrial arrhythmia (called W.P.W syndrome).

Mostly they present with right sided heart failure represented by leg swelling and liver congestion. Sometimes the presentation may be with life threatening arrhythmia.

Treatment

Repair of the right AV valve is the treatment of choice. But sometimes it fails and valve may have to be replaced or upper half of the blood to be redirected to the lungs to reduce blood flow to the right atrium to reduce leak.