Ebstein’s Anomaly

The tricuspid valve is the valve between the right atrium and the right ventricle, and in Ebstein’s anomaly it is severely malformed and displaced. As a result, the tricuspid valve does not close well and there is leakage, known as insufficiency or regurgitation, of blood backwards from the right ventricle into the right atrium.

The right atrium becomes very enlarged, or dilated because of the regurgitation. The tricuspid regurgitation raises the pressure in the right atrium and, in most cases, there is a hole between the two upper chambers of the heart called an atrial septal defect, or ASD, which allows oxygen-poor blood to flow from the right atrium into the left atrium and the child appears blue or cyanotic.

Surgery can be performed to repair or replace the tricuspid valve in patients who have symptoms such as shortness of breath or exercise limitations. Patients with Ebstein’s anomaly are also prone to develop abnormal fast heart rhythms because the right atrium is so enlarged.

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