Congenital Heart Disease

Ebstein Anomaly

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Ebstein anomaly is a rare defect in which the tricuspid valve, which prevents backflow of blood from the right ventricle into the right atrium, is deformed. The right side of the heart is where deoxygenated blood from the body is pumped into the lungs for oxygenation.

Ebstein anomaly usually consists of the valve being displaced downward into the ventricle. The three flaps, or leaflets, that make up the valve opening are malformed or absent. This results in blood leaking back into the atrium. In addition to the valve malformation, this condition also often includes:

• Enlarged right heart chambers
• Atrial septal defect, a hole between the atrial chambers, in about half of patients
• Irregular heart rhythm or arrhythmia

Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. Often, when Ebstein anomaly is first detected during adulthood, the condition is mild. More severe complications can result in heart failure and in cyanosis, in which the skin takes on a bluish tint due to a lack of oxygen. An adult may experience difficulty breathing, have problems with exercise, chest pain and fainting spells.

Reviewed by health care specialists at UCSF Medical Center.
Last updated May 8, 2007