Ebstein’s Anomaly

What is Ebstein’s anomaly?

Ebstein’s anomaly is a very rare congenital heart defect. Babies with Ebstein’s anomaly have a tricuspid valve that did not form correctly.

The tricuspid valve is one of the valves that prevents blood from flowing backwards while it is being pumped into the lungs. In Ebstein’s anomaly, the tricuspid valve is usually unable to prevent blood from being pumped backwards. This is called “valve regurgitation.” This problem can also reduce the size of the chamber (right ventricle) that pumps the blue (oxygen-poor) blood to the lungs.

This condition is also called Ebstein’s malformation of the tricuspid valve. Doctors do not know what causes this heart defect.

  • Ebstein’s anomaly ranges in how serious it can be. For some newborns, it is a life-threatening problem. For others, it has very little effect.

    Ebstein’s anomaly includes problems such as: 

    • Having a blue complexion from getting too little oxygen in the blood (cyanosis)
    • Getting tired easily
    • The heart beating too fast (supraventricular tachycardia)

Ebstein’s Anomaly at Seattle Children’s

  • Seattle Children’s Heart Center is one of the best pediatric cardiology programs in the United States and is the top-ranked program in the Northwest, according to U.S. News & World Report. With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    Our heart team has treated many children with Ebstein’s anomaly. We have extensive experience with the treatment these patients may require, including medicines or surgery. Children with severe Ebstein’s anomaly receive comprehensive care through our Single Ventricle Program.

    We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who undergo heart surgery.

    Seattle Children’s has been treating children since 1907. Our team members are trained in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia (sedation) are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialistsknow how to help children understand their illnesses and treatments in ways that make sense for their age.

  • We provide accurate diagnosis, thoughtful counseling and pregnancy management support through our Prenatal Diagnosis and Treatment Program.

    The Adult Congenital Heart Disease Program shared by Seattle Children’s and the University of Washington can help with care throughout your child’s life.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s heart doctor (cardiologist), you are connected with newborn specialists (neonatologists), lung doctors (pulmonologists), nurses, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child’s health needs and help your family through this experience.

    Read more about the supportive care we offer.

Symptoms of Ebstein’s Anomaly

The symptoms of Ebstein’s anomaly range from life threatening to none at all.

Your child may have symptoms like these: 

  • Skin, lips or nails that look blue (cyanosis)
  • Fast breathing or trouble breathing
  • Fast, unregulated heartbeat pattern (supraventricular tachycardia)
  • Getting tired easily
  • Poor weight gain
  • Coughing
  • Swelling in the legs 

Symptoms of Ebstein’s anomaly may change as children get older. Some people with Ebstein’s anomaly have no symptoms until they are adults.

Diagnosing Ebstein’s Anomaly

To diagnose this condition, your doctor will examine your child, check their heartbeat and listen to their heart. In children with this anomaly, doctors can often hear a heart murmur.

The doctor will ask for details about your child’s symptoms, their health history and your family health history.

Your child will also need to have tests that provide more information about how their heart looks and works and how severe their condition is. These may include: 

For most people, the echocardiogram is the specific test that determines a diagnosis of Ebstein’s anomaly.

Treating Ebstein’s Anomaly

If their condition is mild, your child may only need to be watched for signs that their condition is getting worse. Your child may not need surgery for a while or may never need surgery at all.

Outside of the hospital, the most common medicines used in Ebstein’s anomaly are diuretics, which help the kidneys rid the body of extra salt and water. Children who also have supraventricular tachycardia may need medicines to control their heartbeat.

If tricuspid valve function is very bad, surgery may be done to improve it. In some cases, if the valve can’t be improved enough, it may need to be replaced with an artificial heart valve.

In the most severe cases, Ebstein’s anomaly affects the right ventricle so seriously that it will never be able to generate blood flow into the lungs. In these children, 2 or 3 surgeries will be needed to create a pathway for venous (blue) blood to flow into the lungs without passing through the right ventricle at all. These children receive complete care through our Single Ventricle Program.

Adult Congenital Heart Disease Program

Almost all children with Ebstein’s anomaly will need specialized care as adults. To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

Providers, see how to refer a patient.