Ebstein's Anomaly

Our Children’s Heart Center team treats your child and offers you peace of mind, whether your child has a mild form of Ebstein’s anomaly or a more serious one. Our pediatric cardiology specialists are prepared to make an accurate diagnosis. And we offer the best possible treatment for your child. At all times throughout your child’s care, our doctors keep you in the know about what’s going on and what to expect.

About Ebstein’s Anomaly

Ebstein’s anomaly is a rare congenital heart condition (present at birth) that affects the right side of the heart. When a child has Ebstein’s anomaly, the tricuspid valve (located between the right upper and right lower chambers of the heart) is not able to do its job—managing the flow of blood from the heart’s right upper chamber (atrium) to the right lower chamber (ventricle). Ebstein’s anomaly occurs when the leaflets (thin pieces of tissue or flaps) of the tricuspid valve are not formed normally and are in the wrong position. The results cause leaking of blood backwards (regurgitation) from the right ventricle into the right atrium.

In addition to Ebstein’s anomaly, it is possible that your child’s heart may have other defects (such as an atrial septal defect) or an abnormal heart beat from an electrical problem in the heart. Our pediatric cardiology specialists will get the full picture during our diagnostic process and develop a treatment plan for your child.

Symptoms

The symptoms of Ebstein’s anomaly depend on the age of your child and the severity of the defect. Mild Ebstein’s anomaly may produce little or no symptoms. However, infants with severe defects can have dangerously low oxygen levels or poor circulation. This may make your child look pale or blue, or have trouble breathing.

In older children and adults, the symptoms of Ebstein’s anomaly may be:

  • Activity limitations
  • Heart murmur
  • Heart palpitations or irregular heartbeat
  • Low oxygen levels, particularly with exercise
  • Respiratory and circulatory issues, such as a shortness of breath and swelling

Diagnosis

Through ultrasound imaging during pregnancy, we may be able to diagnose Ebstein’s anomaly before your baby is born. We may also diagnose the condition after birth. In either case, we will carefully evaluate your child and help you decide next steps. Your child’s diagnostic work-up may include: 

  • Cardiac catheterization—a test that shows us how the heart is working by inserting a thin tube in through the blood vessels in the leg
  • Cardiac magnetic resonance imaging (MRI)—an imaging test that uses a powerful magnetic field, radio waves, and a computer to produce detailed pictures of the structures within the heart
  • Echocardiogram—a safe, noninvasive procedure that uses high frequency ultrasound to show the structure of your child’s heart, how blood flows through the heart, and how your child’s heart and circulatory system are working overall
  • Electrocardiogram (ECG or EKG)—a noninvasive procedure that shows electrical activity that involves placing stickers on your child’s chest
  • Exercise stress test—a test that involves taking an electrocardiogram (ECG or EKG)  while your child exercises on our treadmill or stationary bicycle to show how your child’s heart reacts to stress (exercise)
  • Heart rhythm monitoring—tests that keep track of the rhythm of your child’s heart, such as a Holter monitor or event monitor
  • Chest X-ray—an image that shows the position and size of your child’s heart

Treatments We Offer

In mild cases of Ebstein’s anomaly, no treatment may be necessary. In serious cases, we may use medication to relieve some symptoms. The only way to fix Ebstein’s anomaly is through one of the following surgical procedures: 

  • Cone procedure—reforms the malformed valve into a cone shape to improve its function
  • Surgical valve repair—a variety of techniques may be used to improve the tricuspid valve function
  • Tricuspid valve replacement—replaces the tricuspid valve with a valve made of biological tissue

Following treatment, we work with you to create a long-term care plan for your child’s lifetime. Lifelong care and follow-up will be necessary to monitor your child’s health. We will check how your child’s valve and heart function. 

Our Alliance with Children’s Hospital of Philadelphia

The Children’s Heart Center is an alliance between Mount Sinai and the Children’s Hospital of Philadelphia (CHOP). Thanks to this alliance, you can be assured that your child is getting outstanding care. We offer you the combination of our expertise and compassion with that of one of the nation’s leading children’s hospitals, according to U.S. News & World Report. 

Why Choose Children’s Heart Center

The Children’s Heart Center has doctors and nurses who are dedicated to providing the most personalized and expert care for your child with congenital heart disease, including Ebstein’s anomaly, at any age. We involve you, parents and family, as we use our expertise for diagnosis and treatment with the most effective minimally invasive techniques.

We are here for you and your child for life.