Ebstein’s anomaly is a birth defect that involves the tricuspid valve. The tricuspid valve of the heart consists of three flaps. This valve separates the right ventricle (lower heart chamber) from the right atrium (upper heart chamber). When the heart relaxes, the flaps of the tricupsid valve open to let blood flow from the right atrium to the right ventricle. When the heart pumps, the flaps close to prevent blood flowing from the right ventricle back into the right atrium.
With Ebstein's anomaly, the flaps of the tricuspid valve are too far down in the right ventricle and may be larger or longer than normal. This may cause blood to flow the wrong way in the heart, which can cause a backup of blood leading to swelling of the heart. It may also cause fluid buildup in the lungs or liver. When not enough blood makes it from the heart to the lungs, this leads to oxygen-poor blood and can make a person with this condition appear blue.
Ebstein’s anomaly occurs in the womb. It is a rare condition and the exact cause is not known. Symptoms occur shortly after birth and may include blue lips and nails, which are a result of oxygen-poor blood.
Ebstein’s anomaly is usually associated with an atrial septal defect and can be associated with heart failure and rhythm problems to varying degrees.
People with Ebstein's anomaly, before and after treatment, are at risk for getting an infection within the heart valve (endocarditis). To help prevent this, they'll need to take antibiotics before certain dental and surgical procedures.