Ebstein’s Anomaly | Diagnosis and Treatment

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How is Ebstein’s anomaly diagnosed?

If your newborn baby is born with a bluish tint to the skin, or your child’s pediatrician suspects a heart condition, you will be referred to a pediatric cardiologist for a physical exam. The doctor will listen to your baby’s heart and lungs, measure the oxygen level in his or her blood (non-invasively) and make other observations to help determine the diagnosis.

If the doctor hears a heart murmur, the sound and character of the murmur and its location in the chest will give the cardiologist an idea of the kind of heart problem your baby may have.

In some cases, Ebstein’s anomaly is found before birth during a fetal ultrasound.

The doctor may order one or more of the following tests to help with the diagnosis:

What are the treatment options for Ebstein’s anomaly?

Non-surgical

Treatment depends on the severity of the child’s anomaly. Children with a mild anomaly may be able to use medication alone to control symptoms, including:

Children who have arrhythmias and heartbeat irregularities that cannot be controlled by medication may need a procedure, called radiofrequency catheter ablation, to treat these heart arrhythmias.

Surgical

There are several surgical options for repairing your child's tricuspid valve and repairing other defects that are associated with Ebstein's anomaly. These techniques include:

  • Cone procedure: in this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is surgically reshaped into a cone that opens and closes.
  • Surgical valve repair: surgeons reposition the existing valve and reconfigure the shape of the tricuspid valve leaflets.
  • Tricuspid valve replacement: if a child’s tricuspid valve is too malformed to be repaired, the existing valve will need to be replaced with a mechanical valve or a bioprosthetic valve, made of human or animal tissue.
  • Bidirectional Glenn procedure: When the right ventricle is not adequate in size sometimes the bidirectional Glenn procedure is needed as well.

In children who can’t be treated with a catheter, arrhythmia are addressed at the time of surgery with surgical ablation.

What is the long-term outlook for children with Ebstein’s anomaly?

Treatments for Ebstein's anomaly are continually being refined, and the long-term outlook is continually improving. Most children who've had surgery recover and grow normally.

Even so, your child will need periodic monitoring — and possibly medication —since he or she could be at some risk for abnormal heart rhythms (arrhythmias) and heart failure. In a few cases, children who have had surgery for Ebstein's anomaly may need additional surgery.

Your child's cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and adulthood, where they are followed by the Adult Congenital Heart Program. Most people who have had congenital heart disease repair have an ongoing relationship with their cardiologist.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital
300 Longwood Avenue, Boston, MA 02115
For Patients: 617-355-6000
For Referring Providers: 844-BCH-PEDS | 844-224-7337

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