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Ebstein Anomaly

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What is Ebstein anomaly?

Ebstein anomaly is a heart condition in which the tricuspid valve doesn’t form properly before birth. The tricuspid valve normally separates the atria (right upper chamber of the heart) from the right ventricle (right lower chamber of the heart).

A typical tricuspid valve has three leaflets (flaps), which open and close to allow blood to flow from the atria to the right ventricle while limiting backwards flow. With Ebstein anomaly, the tricuspid valve does not close well, causing backward flow called regurgitation. The amount of backward flow depends on the severity of the anomaly. This condition can cause arrhythmias (irregular heartbeat) such as supraventricular tachycardia (SVT) and myopathy (abnormal function of the right ventricular heart muscle).

What causes Ebstein anomaly?

Ebstein anomaly occurs when one or two of the flaps of the tricuspid valve get “stuck” to the muscular wall between the heart’s left and right ventricles. As a result, it doesn’t close all the way and allows too much backward flow. Depending on the amount of the backward flow, your child may have an enlarged right atrial and poor function of the right ventricular muscle. This often causes an arrhythmia.

Who gets Ebstein anomaly?

Ebstein anomaly is a rare congenital (present at birth) heart disorder that affects about 1 in every 200,000 live births and accounts for less than 1% of all cases of congenital heart disease.

The exact cause of Ebstein anomaly is unclear, which means cases are random and hard to prevent. Few cases have clear genetic causes that are linked to family medical history. Experts think some cases might result from mothers being exposed to medications like lithium.

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