Ebstein Anomaly

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

No other congenital heart surgery program in Kentucky, Ohio or Southern Indiana is rated higher by the Society of Thoracic Surgeons than the Norton Children’s Heart Institute Pediatric Cardiothoracic Surgery Program.

  • 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.
  • Our board-certified and fellowship-trained pediatric cardiovascular surgeons are leaders in the field as clinicians and researchers.
  • More than 5,000 children a year visit Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, for advanced heart care.
  • Norton Children’s Heart Institute successfully performs more than 17,500 procedures a year.
  • The Society of Thoracic Surgeons rated Norton Children’s Heart Institute among the best in the region after studying years of our patients’ outcomes and our ability to treat a range of pediatric heart conditions, including the most severe.
  • Norton Children’s Heart Institute has satellite outpatient offices in Ashland, Bowling Green, Campbellsville, Elizabethtown, Frankfort, London, Madisonville, Murray, Owensboro, Paducah and Shepherdsville in Kentucky; as well as Corydon, Jasper, Madison and Scottsburg in Indiana; 28 tele-echocardiography locations in Kentucky and Southern Indiana; and six fetal echocardiography locations across Kentucky.
  • The American Board of Thoracic Surgery has awarded the cardiothoracic surgeons at Norton Children’s Hospital with subspecialty certification in congenital heart surgery.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
  • Our multidisciplinary approach to pediatric heart surgery brings together our specialists in cardiothoracic surgery, cardiology, anesthesiology, cardiac critical care and other areas to create a complete care plan tailored for your child.
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Heart team helps firefighter ‘feel 100%’ after surgery for Ebstein anomaly

Luke Coward was born with Ebstein anomaly of the tricuspid valve, an extremely rare congenital heart condition that accounts for less than 1% of all congenital heart conditions. From the time he was diagnosed at […]

Read Full Story

Collaborative heart care helps Indiana boy’s Ebstein anomaly

Audrey Sims’ first clue that her twins’ birth would be complicated came at 14 weeks of pregnancy, when a routine ultrasound found that one of her sons, Aiden, had a blocked lymph node, which can […]

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We take a lot of deep breaths

I found out when I was 23 weeks pregnant that Joslyn had Ebstein’s anomaly, a congenital heart defect that I also have. We were under the impression that it wasn’t hereditary — but evidently that’s […]

Read Full Story
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Heart team helps firefighter ‘feel 100%’ after surgery for Ebstein anomaly

Luke Coward was born with Ebstein anomaly of the tricuspid valve, an extremely rare congenital heart condition that accounts for less than 1% of all congenital heart conditions. From the time he was diagnosed at […]

Read Full Story

Collaborative heart care helps Indiana boy’s Ebstein anomaly

Audrey Sims’ first clue that her twins’ birth would be complicated came at 14 weeks of pregnancy, when a routine ultrasound found that one of her sons, Aiden, had a blocked lymph node, which can […]

Read Full Story

We take a lot of deep breaths

I found out when I was 23 weeks pregnant that Joslyn had Ebstein’s anomaly, a congenital heart defect that I also have. We were under the impression that it wasn’t hereditary — but evidently that’s […]

Read Full Story

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