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Ebstein's anomaly is a heart defect characterized by an abnormality in one of the heart's valves, which can lead to reduced blood flow. The defect causes the tricuspid valve — which separates the right ventricle from the right atrium — to leak blood into the right atrium from the wrong direction.
Ebstein's anomaly is a heart defect that is present at birth. The exact cause is unknown.
Symptoms can be mild, moderate, or severe. In serious cases, a backup of blood can result in swelling of the heart and a buildup of fluid in the lungs.
Complications include shortness of breath, reduced physical activity, heart enlargement, and even heart failure. Ebstein's anomaly treatments depend on the severity of the condition.
Ebstein's anomaly symptoms may be present at birth or they may not manifest until adulthood. In mild cases, there may be no symptoms and treatment is not necessary. Common Ebstein's anomaly symptoms include:
Babies born with the condition may have low oxygen levels or a bluish tint to their skin, which can result in an Ebstein's anomaly diagnosis. Your child's doctor may also hear abnormal heart sounds when they listen to the baby's chest with a stethoscope. In childhood and adult cases, tests that help doctors make a diagnosis include:
Ebstein's anomaly treatments depend on the extent of the defect. Some potential treatments include:
A variety of medications may help with treating Ebstein's anomaly or to manage side effects from the condition. For example:
A cone procedure, pioneered by Jose da Silva, MD, is the standard treatment for children with Ebstein’s anomaly.
It uses tissue from a child's own malfunctioning tricuspid valve to create a new, cone-shaped valve.
Because the reconstructed valve is from the child's own tissue, the body is more willing to accept it. And the valve grows with the child so surgeons will not need to replace it as the child gets older.
Dr. da Silva is one of the most experienced in performing the cone procedure in infants and young kids. He’s trained surgeons around the world in the technique.
Other, less common surgeries for Ebstein’s anomaly include:
The Da Silva Center for Ebstein's Anomaly at UPMC Children's Hospital of Pittsburgh provides world-class care for people with congenital heart defects. Our experts are among the most experienced in the world at treating children with Ebstein’s anomaly and other heart valve problems.
To make an appointment or consult on a patient, call the Heart Institute at 412-692-5540.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
UPMC Children’s Hospital of Pittsburgh
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
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Interested in giving to Children's Hospital? Visit Children's Hospital of Pittsburgh Foundation's website to make a donation online.