What is Ebstein's Anomaly?

D. Jeffress
D. Jeffress
A deformed tricuspid valve cannot pump blood as it should.
A deformed tricuspid valve cannot pump blood as it should.

Ebstein's anomaly is a type of congenital heart defect that impairs the functioning of the right atrium and ventricle. The tricuspid valve between the two chambers is unusually low, and the openings in the valve may be attached to the wall of the atrium. A deformed tricuspid valve cannot efficiently pump blood to the lungs, which can lead to shortness of breath, fatigue, and heart palpitations. In serious cases, blood may begin to flow backward through the heart and present life-threatening complications. Doctors can usually treat a mild Ebstein's anomaly with medications and careful monitoring, but severe defects require emergency surgery shortly after birth.

Tricuspid valve defects are very rare, and medical researchers have not been able to identify an underlying cause. Some studies suggest that the use of lithium, benzodiazepines, and some other anxiety-reducing medications during pregnancy may increase the risk of Ebstein's anomaly. It is clear that Ebstein's anomaly arises early in embryonic development, and it is often accompanied by other heart defects. Depending on how low the tricuspid valve is situated in the heart and how misshapen it is, symptoms can range from barely noticeable to life-threatening.

A person who is born with a mild variety of Ebstein's anomaly may not experience problems until adolescence or adulthood. Symptoms tend to develop gradually as blood flow through the heart progressively worsens. An individual may have shortness of breath and fatigue after physical activity, swelling in the extremities, and occasional heart palpitations. Over time, poor blood circulation to the lungs can increase fatigue and cause the skin and lips to turn blue.

Treatment is not always necessary for patients with relatively minor heart defects. Many people simply need to schedule regular checkups with their doctors to make sure blood pressure and blood oxygen levels stay within normal ranges. If symptoms start to develop, daily medications are usually effective at stabilizing the heart rate. Surgery may be considered in adolescence or adulthood if tests show that heart failure is likely to occur.

A severe Ebstein's anomaly causes potentially fatal complications at birth. An infant may be blue and limp, and have a very fast or slow heartbeat. A baby is placed on an artificial respirator to stabilize breathing while blood tests and x-rays are performed. Specialists carefully inspect the heart, lungs, and other organs to check for additional defects. Surgery to repair or replace the tricuspid valve can usually eliminate the need for a heart transplant.

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    • A deformed tricuspid valve cannot pump blood as it should.
      A deformed tricuspid valve cannot pump blood as it should.