Ebstein anomaly is a birth defect that affects the tricuspid valve, which connects the heart’s right chambers and helps control how blood moves through the heart.

Normally, blood flows through the tricuspid valve on its way from the heart’s upper right chamber to the lower right chamber. The valve opens and closes to keep blood moving in the correct direction.

But in Ebstein anomaly, the tricuspid valve isn’t formed correctly, so the flaps that make it open and close don’t function properly, and part of the valve is in the wrong place.

Some babies with Ebstein anomaly are born with other heart problems, like an abnormal right ventricle, an atrial septal defect (ASD), pulmonary stenosis or pulmonary atresia. Often, Ebstein anomaly affects the heart’s electrical system too, causing an irregular heartbeat.

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What Are the Signs and Symptoms of Ebstein Anomaly?

This condition can range from mild to serious. In mild cases, Ebstein anomaly might not cause any symptoms at all, and can go undetected for many years.

More serious cases of Ebstein anomaly can cause the following symptoms.

  • Bluish or greyish skin, lips and nails
  • Heart murmur
  • Heart beat that’s too fast, too slow or irregular
  • Trouble breathing, especially with activity
  • Coughing
  • Poor appetite
  • Poor growth
  • Low energy
  • Swelling in the legs
  • Swelling in the belly

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born using a fetal echocardiogram, or echo. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow. 
 

What Causes Ebstein Anomaly?

Ebstein anomaly occurs before birth, when the fetal heart doesn’t develop properly during early pregnancy. It’s possible that in some cases, it’s passed down genetically from a parent. But most of the time, there’s no known cause.

How is Ebstein Anomaly Treated?

  • Medical management of heart rhythm problems or heart failure
  • Catheter ablation: Procedure using a long, thin tube (catheter) to stop the path of electrical signals that cause arrhythmia

If needed, Connecticut Children’s cardiac surgeons specialize in cutting-edge techniques to treat Ebstein anomaly. Our team is known for performing complex procedures on even the tiniest babies, with outstanding surgical outcomes.

  • Tricuspid valve repair: (based on the patient’s anatomy, ex. Cone procedure) to try to use the patient’s own valve and reconstruct it
  • Tricuspid valve replacement: Replaces the valve
  • Maze procedure: Creates incisions in the heart’s upper chambers to stop the path of electrical signals that cause arrhythmia
  • In severe cases, heart transplant

Most patients with Ebstein anomaly need to follow up with their heart doctor throughout life. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides important tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.