Ebstein's Anomaly of the Tricuspid Valve

Ebstein’s anomaly of the tricuspid valve is a condition where the position of the tricuspid valve is not correct.

The tricuspid valve separates the right atrium and the right ventricle of the heart. The valve has three leaflets and opens and closes to allow blood to flow from one chamber to the next. When the tricuspid valve sits very low, more in the space of the right ventricle, it is called Ebstein’s anomaly. This causes the blood to leak backwards into the right atrium.The low position can cause the right ventricle to be too small to function as the pump to the lungs. These issues cause enlargement of the atrium.

In some children with mild valve problems, treatment is not necessary. Others may need a surgical valve replacement. In the most severe cases, the ventricle is underdeveloped in addition to the tricuspid valve abnormality. The child will need to be treated as a single ventricle patient with a series of surgeries for staged palliation.