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.
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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 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.
The goal of treatment is to reduce symptoms. Choices are:
- 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 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.
Ebstein’s Anomaly Foundation http://www.ebsteinsanomaly.org
National Organization of Rare Disorders https://rarediseases.org
Canadian Cardiovascular Society http://www.ccs.ca
Heart and Stroke Foundation of Canada http://www.heartandstroke.ca
Baumgartner H, Bonhoeffer P, et al; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC), Association for European Paediatric Cardiology (AEPC), ESC Committee for Practice Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010 Dec;31(23):2915-2957.
Ebstein anomaly. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/e/ebstein. Accessed November 4, 2020.
Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/ebstein-anomaly-of-the-tricuspid-valve. Accessed November 4, 2020.
Ebstein’s anomaly. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Ebsteins-Anomaly%5FUCM%5F307025%5FArticle.jsp. Accessed November 4, 2020.
Symptoms and diagnosis of congenital heart defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/SymptomsDiagnosisofCongenitalHeartDefects/Symptoms-Diagnosis-of-Congenital-Heart-Defects%5FUCM%5F002029%5FArticle.jsp. Accessed November 4, 2020.
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.
- Reviewer: EBSCO Medical Review Board Kari Kassir, MD
- Review Date: 09/2020
- Update Date: 05/07/2021