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Ebstein Anomaly—Child

  • Cynthia M. Johnson, MA
Publication Type:


Ebstein Anomaly—Child

(Ebstein Malformation—Child; Anomaly, Ebstein—Child; Malformation, Ebstein—Child)


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.

Heart Chambers and Valves.

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Blood Flow Through the Heart.

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

Risk Factors

Risk factors for Ebstein anomaly are not clear. Some possible ones are:

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


Problems can be mild to severe. Some children may not have symptoms. Those who do may have:

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


The doctor will ask about your child’s symptoms and health history. A physical exam will be done.

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.


Surgery may be done right away to:

  • Fix or replace the tricuspid valve to reduce leaking
  • Destroy abnormal tissue to stop abnormal heart rhythms

Medicine may be given to:

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

Lifelong monitoring by a heart specialist will be needed.


There are no known guidelines to prevent Ebstein anomaly.





  • Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at:  https://www.dynamed.com/condition/ebstein-anomaly-of-the-tricuspid-valve.
  • Ebstein's anomaly. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/e/ebstein.
  • Congenital heart defects. American Heart Association website. Available at: https://www.heart.org/en/health-topics/congenital-heart-defects.
  • 7/14/2017 DynaMed Literature Surveillance Update https://www.dynamed.com/condition/ebstein-anomaly-of-the-tricuspid-valve: Patorno E, Huybrechts KF, et al. Lithium use in pregnancy and the risk of cardiac malformations. N Engl J Med. 2017 Jun 8;376(23):2245-2254.


  • Kari Kuenn, MD
Last Updated:

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.