Cardiac Center

Ebstein’s Anomaly of the Tricuspid Valve

Before you begin reading about this heart defect, please read the explanation of how the normal heart works for a basic understanding of its structure and function.

• What is Ebstein’s anomaly of the tricuspid valve?
• What are the symptoms?
• How is it diagnosed?
• What are the treatment options?
• What kind of follow-up care is required?

What is Ebstein’s anomaly of the tricuspid valve?

The tricuspid valve separates the right upper chamber (the right atrium) and the right lower chamber (the right ventricle) of the heart. A normal tricuspid valve has three leaflets, and opens and closes to allow blood to flow from the upper to the lower chamber without going backwards.

“Anomaly” means a difference or irregularity. In a child born with Ebstein’s anomaly, the tricuspid valve formed irregularly during fetal development. One or two of its three leaflets may be “stuck” to the walls of the right ventricle or the septum (the muscular wall between the heart’s left and right ventricles). The valve may be severely malformed, and placed lower than in a normal heart; as a result the right atrium may be larger and the right ventricle smaller than normal.

In a normal heart, the tricuspid valve closes completely when the right ventricle squeezes to push blood to the lungs, to prevent blood from going backwards. With Ebstein’s anomaly of the tricuspid valve, some blood “leaks” back into the right atrium because the valve doesn’t close completely. The atrium may become enlarged because of the extra volume of blood; in severe cases, heart failure can result.

Some children with this condition also have an atrial septal defect (ASD), a hole in the wall between the two upper chambers of the heart, or other heart defects.

This condition can also be associated with problems in the built-in electrical system that controls the heart’s pumping. Some children with Ebstein’s anomaly have Wolff-Parkinson-White syndrome, which means there is an extra electrical connection between the upper and the lower chambers of the heart. This can lead to an arrhythmia, or an abnormal heartbeat.

Ebstein’s anomaly is named after Wilhelm Ebstein, the German physician who was the first to describe it.

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What are the symptoms?

Symptoms may include some or all of the following:

• blue or purple tint to lips, skin and nails (cyanosis)
• heart murmur and clicks– extra heart sounds heard when a doctor listens with a stethoscope
• racing heart
• shortness of breath
• fatigue

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How is it diagnosed?

In severe cases, the newborn will exhibit cyanosis (blue tint to the skin) and will have difficulty breathing. Those children will be transported from their birth hospitals and admitted to the Tabas Cardiac Intensive Care Unit.

In many cases of Ebstein’s anomaly, symptoms are not severe. A pediatrician might notice a heart murmur when the child is older and refer him or her to a pediatric cardiologist. Sometimes this condition isn’t diagnosed until adulthood.

Diagnosis will require:

• chest X-ray
• echocardiogram – sound waves create an image of the heart
• electrocardiogram (ECG) – a record of the electrical activity of the heart
• exercise test

More rarely, cardiac catheterization will be required: a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus (“belly button”).

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What are the treatment options?

Open-heart surgery to fix the valve is required in some cases. The surgeon may be able to repair the tricuspid valve so that it functions more normally. Or, the surgeon will replace the tricuspid valve with a valve from a donated organ or from an animal. If the child has a septal defect, the surgeon will sew it closed or place a patch over it.

In the most severe cases, when the right ventricle (the right lower chamber) is much smaller than normal, Ebstein’s anomaly is treated as a single-ventricle heart defect.

A series of surgeries (at least three) will redirect the flow of blood. The Cardiac Center team will explain this to the family in detail.

Children who have Wolff-Parkinson-White syndrome may require ablation, a catheterization technique to get rid of the extra electrical connection.

In many cases of Ebstein’s anomaly, treatment is not necessary. Instead, the Cardiac Center team will monitor the child during follow-up visits, once a year or more, to ensure complications aren’t developing.

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What kind of follow-up care is required?

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Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physician. Patients will need to carefully follow doctors’ advice, including staying on any medications prescribed and, in some cases, limiting exercise.

Children who were critically ill as newborns and required open-heart surgery as infants may have a longer road to recovery.

Sometimes children with this condition experience heart problems later in life, including irregular heartbeat and leaky valves. Medicine, additional surgery and/or cardiac catheterization may be required.

Into adulthood

We will help patients transition care to an adult congenital heart disease specialist.

The Philadelphia Adult Congenital Heart Center, a joint program of The Children’s Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.

Because of enormous strides in medicine and technology, today most children with heart conditions go on to lead productive lives as adults.

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Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.

Reviewed by: Jeffrey R. Boris, MD
Date: August 2009