Ebstein Anomaly

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Ebstein anomaly is a rare heart defect that affects the tricuspid valve. The tricuspid valve separates the heart chamber that receives blood from the body (right atrium) from the chamber that pumps the blood on to the lungs (right ventricle).

Many children whose Ebstein anomaly is corrected by surgery can be as active as other kids.

As the leading providers of pediatric heart care in Louisville and Southern Indiana, cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are experienced with successfully repairing Ebstein anomaly.

Our specialists have the skill and experience to know when Ebstein anomaly needs surgery and when it can be treated without surgery.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

Leaky Tricuspid Valve

Normally, when the heart muscle relaxes, the tricuspid valve is open, allowing blood to flow into the right ventricle. When the right ventricle squeezes to pump blood out of the heart, the tricuspid valve closes to prevent backward leakage of blood from the right ventricle to the right atrium.

In Ebstein anomaly, the valve doesn’t work properly and often can’t close completely.

The result is a leaky tricuspid valve that allows blood to flow back into the right atrium. When a lot of blood leaks backward, the right atrium becomes enlarged and the right ventricle becomes smaller.

In a less common type of Ebstein anomaly, the tricuspid valve is tight, preventing normal forward blood flow. The result is a heart that doesn’t pump blood efficiently.

Ebstein Anomaly Symptoms

Ebstein anomaly symptoms can range from very mild to very serious. Kids with a milder form of the anomaly may not have any symptoms until they’re older. Two signs that an infant or child may have Ebstein anomaly are trouble breathing and cyanosis, a bluish coloring of the skin and nails.

Ebstein anomaly also can make a child:

In severe cases, a child also might have swelling (edema) in the legs or fluid in the belly (ascites).

A baby born with Ebstein anomaly often has other heart issues, such as an atrial septal defect or a patent foramen ovale. When either of these holes are open, oxygen-poor blood from the right side of the heart can leak into the left side of the heart and out to the body. This leads to a lower oxygen level in the bloodstream and the bluish color of the lips and nail beds.

In some children with Ebstein anomaly, another heart valve — the pulmonary valve —also may be tight or even sealed off. This condition also contributes to cyanosis.

Ebstein anomaly often affects the heart’s electrical system. Some kids will have an extra electrical pathway called Wolff-Parkinson-White syndrome (WPW), which can cause a very fast heartbeat (tachycardia) or an unsteady beat.

Doctors don’t know exactly why a baby’s heart develops Ebstein anomaly during pregnancy. But it isn’t caused by anything a mother does or doesn’t do during her pregnancy. Most cases of Ebstein anomaly are an accidental error of growth during pregnancy. Some genetic links have been found, but most cases don’t have a known genetic cause.

Ebstein Anomaly Diagnosis

Ebstein anomaly might be seen on ultrasound scans before birth. It may be recognized at birth because the baby’s skin looks blue or the baby’s heart makes unusual sounds.

The best test to confirm Ebstein anomaly is an echocardiogram (ultrasound of the heart).

Other tests may include:

  • Chest X-ray, which often shows a large heart
  • Electrocardiogram (ECG or EKG), a recording of the heart’s electrical activity. It may show abnormal heartbeats or signs that the heart’s right chambers are enlarged.
  • Exercise test (for older kids who can follow instructions)

Ebstein Anomaly Treatment

If the defect was found before birth, the delivery team will be ready to provide intensive care immediately in case the newborn is not doing well.

Most newborns with the anomaly don’t need immediate treatment. The specialists at Norton Children’s Heart Institute will monitor them closely to watch for changes and provide quick treatment if needed.

When treatment is needed, the most common treatments used initially are:

Babies born with Ebstein anomaly need continued follow-up care from a pediatric cardiologist (a doctor who specializes in treating heart conditions) because the heart’s ability to pump blood may change as the child continues to grow. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

Why Choose Norton Children’s Heart Institute

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

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