Ebstein’s Anomaly

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What is Ebstein’s anomaly?

Diagram of a healthy heart This is a healthy heart. The tricuspid valve lets blood flow from the right atrium into the right ventricle to get pumped to the lungs. The valve keeps blood from leaking back into the atrium.

Ebstein’s anomaly is a very rare congenital heart defect. In children with Ebstein’s anomaly, the tricuspid valve did not form the way it should.

Normally, the right ventricle of the heart pumps blue (oxygen-poor) blood to the lungs. The tricuspid valve works like a door, opening to let blood flow into this ventricle and closing to keep blood from leaking backwards. In Ebstein’s anomaly, the tricuspid valve cannot stop blood from leaking backwards. This is called tricuspid valve regurgitation.

In some people, the leak is mild. In others, it is so severe that it leads to heart failure, and too little blood goes to the lungs. Sometimes, the location of the abnormal valve makes the right ventricle smaller than normal.

Most of the time, doctors do not know what caused Ebstein’s anomaly. It can be linked with other heart problems, like a hole in the heart, a fast heartbeat or heart muscle disease.

This condition is also called Ebstein’s malformation of the tricuspid valve.

Heart Center at Seattle Children's

Our pediatric cardiologists and cardiac surgeons have more advanced training and experience operating and performing procedures on babies, children and teens than at any other children's hospital in the Pacific Northwest.

What are the symptoms of Ebstein’s anomaly?

For some people, Ebstein’s anomaly has very little effect in childhood, and it may cause no symptoms. For others, it is life threatening from birth.

Your child may have symptoms like these:

Skin may look blue or purple tinged, mottled (different shades or colors), grayish or paler than usual; the lips, mouth, gums, fingernails or toenails may look bluish (cyanosis)
Fast breathing, trouble breathing or feeling short of breath
Fast heartbeat (supraventricular tachycardia)
Skipped heartbeats (palpitations)
Getting tired easily
Swelling in the legs

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

How is Ebstein’s anomaly diagnosed?

Fetal diagnosis

Usually, doctors can diagnose Ebstein’s anomaly when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing baby’s heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease.

Your obstetrician may refer you for a fetal echo if your family has a history of congenital heart disease or if a routine prenatal ultrasound shows a problem.

Seattle Children’s Fetal Care and Treatment Center team can care for you when you are pregnant if your developing baby has a known or suspected problem.
Diagnosis after birth
To diagnose this condition, your child’s doctor will examine your child, check their heartbeat and listen to their heart. In children with this problem, 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 tests to get more information about how their heart looks and works and how severe their condition is. These may include:
- Chest X-rays
- Echocardiogram
- ECG or EKG (electrocardiogram)
- MRI (magnetic resonance imaging) of the heart
- Cardiac catheterization
- Electrophysiology study

How is Ebstein’s anomaly treated?

Your child’s treatment plan will depend on how well their tricuspid valve and right ventricle work.

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.

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

If their tricuspid valve function works very poorly, your child may need surgery to improve it. In some cases, if surgeons are not able to improve the valve enough, a child may need their tricuspid valve 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 pump blood to the lungs. These children need 2 or 3 surgeries to make a path for blue (oxygen-poor) blood to flow into their lungs without passing through their right ventricle at all. These children receive complete care through our Single Ventricle Program. Those who have a Fontan procedure get coordinated, ongoing, team-based care through our Fontan Clinic.

Ebstein’s Anomaly at Seattle Children’s

The experts you need are here
- The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many children with Ebstein’s anomaly.
- Our doctors and surgeons are experts in the treatments your child may need, including surgery to repair or replace their tricuspid valve or make a new path for blood to flow to their lungs.
- Children with severe Ebstein’s anomaly receive comprehensive care through our Single Ventricle Program.
- We also have a pediatric cardiac anesthesia team and Cardiac Intensive Care Unit for children who need them. General anesthesia is a medicine we give to people before surgery so they are fully asleep during the procedure.
- Your child’s team includes other experts from Seattle Children’s based on their needs, like neonatologists and pulmonologists.
Care from birth through young adulthood
- If your developing baby is diagnosed with Ebstein’s anomaly before birth, our Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.
- Your child’s treatment plan is custom-made. We plan and carry out their treatment based on the specific details of their heart condition. We closely check your child’s needs to make sure they get the care that is right for them at every age.
- We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
Support for your whole family
- We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
- Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
- Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
- Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make your experience as smooth as possible.
- Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
- Read more about the supportive care we offer.