Ebstein's Anomaly

Ebstein's 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.

Why UAB

The UAB Congenital Heart Disease Program offers the most advanced care for structural heart disease, which often requires lifetime monitoring and care. Our multidisciplinary team of pediatric and adult cardiologists, cardiovascular surgeons, cardiovascular anesthesiologists, and maternal/fetal medicine specialists are uniquely qualified to treat patients before birth and into adulthood. In addition to seeing patients at the UAB Women & Infants Center and The Kirklin Clinic of UAB Hospital, UAB Medicine’s congenital heart disease specialists also see patients at North Alabama Children's Specialists in Huntsville and Physicians to Children/Central Alabama Children's Specialists in Montgomery.

Our 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 expectant mothers (or fathers) known to have congenital heart disease. If a defect is discovered, prenatal treatment and planning for delivery and postnatal treatment can be undertaken.

Thanks to advances in pediatric congenital care, the life expectancy for most patients now reaches far into adulthood, but more than 60% 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 handful in the country, our expertise greatly increases the chances that symptoms will be identified early. This helps ensure that smaller problems are addressed before they develop into larger, more life-threatening issues such as heart failure, arrhythmia, residual congenital heart defects, endocarditis, and stroke.

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Related Conditions

Listed below are some conditions that may be related to or share some similarities with the condition addressed on this page.

Clinical Trials

UAB is an active participant in research and clinical trials for the diagnosis and treatment of Epstein's Anomaly. We encourage you to speak to your physician about research and clinical trial options and browse the link below for more information.

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