Definition

Ebstein anomaly is a rare heart defect. It happens when the tricuspid valve forms lower than normal in the right ventricle. Also, the valve does not open and close as it should. This lets blood leak in the wrong direction. Ebstein anomaly can be mild to severe.

Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
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Causes

This problem is present at birth. It is not known exactly why the heart does not form the right way.

Risk Factors

Specific risk factors are not clear. Some possible ones are:

  • Chromosomal abnormalities
  • Environmental exposure
  • Certain medications taken by a pregnant mother, such as lithium

Symptoms

Symptoms vary based on how severe the defect is. In some children, there may not be any. In other children, they are:

  • Blue or pale skin color
  • Rapid heart beat or skipped heart beats
  • Low energy
  • Shortness of breath
  • Chest pain

Diagnosis

You will be asked about your child’s symptoms and health history. A physical exam will be done. During the exam, the doctor may find aheart murmur.

Pictures may be taken of your child's chest. This can be done with:

An electrocardiogram may also be done to measure the electrical activity of the heart.

Treatment

Talk with the doctor about the best plan for your child. Often, surgery is needed right away. The options are:

Medication

Medications may be given to:

  • Help restore normal heart rhythms
  • Reduce fluid in the body
  • Improve the way the heart works

Procedures

Depending on your child’s health, the doctor may advise:

  • Surgery—Surgery may be needed to fix or replace the tricuspid valve. This will reduce leaking.
  • Ablation procedure—This may be done if your child is having abnormal heart rhythms. A tube is threaded up to the heart. Abnormal tissue is destroyed to stop the abnormal rhythms.

Lifelong Monitoring

Your child will have regular exams from a heart specialist. In some cases, your child may need antibiotics before some dental or medical procedures. This is to prevent infections.

Prevention

Ebstein anomaly can’t be prevented because the cause is unknown.

Revision Information

  • Reviewer: Kari Kassir, MD
  • Review Date: 06/2018 -
  • Update Date: 06/28/2018 -