Ebstein Anomaly

Ebstein’s anomaly is a rare congenital heart condition. Congenital means that something is present at birth. An anomaly is something that’s unexpected or unusual. Ebstein’s anomaly affects the function of the tricuspid valve, which is one of four valves in your heart.

A German physician named Wilhelm Ebstein first described the condition in 1866.

What happens to the tricuspid valve in Ebstein’s anomaly?

In people with Ebstein’s anomaly, this valve is:

• Malformed: The leaflets, or moving parts of the valve that open and close with each heartbeat, are abnormally shaped and can be stuck to the heart wall.
• Misplaced: The valve usually sits lower than normal in the heart.

As a result of these issues, blood leaks backward into the right atrium (regurgitation), or upper right chamber of the heart. The atrium can also become oversized. Over time, the enlarged right side of the heart can weaken, leading to heart failure.

Who does Ebstein’s anomaly affect?

People with Ebstein’s anomaly frequently have other heart conditions. Up to 94% have a hole in the wall that separates the two upper chambers of the heart (atrial septal defect or patent foramen ovale).

Defects in the other heart valves and the left chambers of the heart can also occur in people with Ebstein’s anomaly.

How common is Ebstein’s anomaly?

About one in every 200,000 babies are born with Ebstein’s anomaly. It accounts for less than 1% of all congenital heart defects.