Ebstein Anomaly

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Ebstein Anomaly

Ebstein anomaly is a rare congenital (meaning it is present at birth) heart defect in which the heart’s tricuspid valve is abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium). The defect usually causes the valve to work poorly, often resulting in blood flowing back into the right atrium instead of to the right ventricle and out to the lungs. The backup of blood flow can lead to heart swelling and fluid buildup in the body. There may also be a narrowing of the valve that leads to the lungs (the pulmonary valve).

In many cases, patients also have a hole in the wall separating the heart’s two upper chambers, and blood flow through this hole may cause oxygen-poor blood to go to the body. This can result in a blueish tint to the skin, a condition called cyanosis. The exact cause is unknown, but the use of certain drugs such as lithium or benzodiazepines during pregnancy may play a role. It is more common among whites. Treatments vary but may include medication, oxygen and other breathing support, and surgery to correct the faulty valve.

The UAB Congenital Heart Disease Program offers the most advanced care for congenital (present at birth) heart disease, which often requires lifetime monitoring and care. Our multi-specialty team of pediatric and adult cardiologists, cardiovascular surgeons, cardiovascular anesthesiologists, and maternal-fetal medicine specialists have unique expertise in treating patients before birth and into adulthood.

UAB Medicine’s modern ultrasound equipment allows many heart defects to be diagnosed before a child is born. Screening exams performed at 18-20 weeks are recommended for expecting mothers or fathers known to have congenital heart disease. If a defect is discovered, our experts provide prenatal treatment and develop a plan for delivery and treatment after birth.

Thanks to advances in pediatric congenital care, the life expectancy for most patients now reaches far into adulthood. However, more than half of the people with congenital heart problems stop seeing a cardiologist once they turn 18. UAB’s Alabama Adult Congenital Heart Disease Program is designed to prevent that gap in care. As the only adult congenital heart disease program in the state and one of only a few in the country, our expertise greatly increases the chances that symptoms will be identified early. This helps ensure that less serious problems are addressed before they develop into larger, more life-threatening issues such as heart failure, arrhythmia, residual congenital heart defects, endocarditis, and stroke.