(Ebstein’s Malformation—Child; Anomaly, Ebstein’s—Child; Malformation, Ebstein’s—Child)
Ebstein’s anomaly is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.
This defect occurs when the tricuspid valve develops lower than normal in the right ventricle. Also, the valve does not open and close normally. This allows blood to leak in the wrong direction. Ebstein’s anomaly can be mild to severe.
Heart Chambers and Valves
Blood Flow Through the Heart
Ebstein's anomaly is present at birth. It is not known exactly why the heart does not develop normally.
Specific risk factors for Ebstein’s anomaly are not clear. Two possible risk factors include:
- Chromosomal abnormalities
- Environmental exposure
Symptoms vary depending on how severe the defect is. In some cases, there may not be any symptoms. In other cases, symptoms may include:
- Swelling in the abdomen and legs
- Blue or pale skin color
- Rapid heart beat or skipped heart beats
- Decreased energy
- Failure-to-thrive or gain weight as normal
- Shortness of breath
You will be asked about your child’s symptoms and medical history. A physical exam will be done. During the exam, the doctor may detect a heart murmur.
Images may be taken of your child's chest. This can be done with:
Other monitors and tests may be used to measure your baby's heart rhythm and function. This can be done with:
- A Holter or Event monitor
- Stress test
Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:
Medications may be prescribed to:
- Help restore normal heart rhythms
- Reduce fluid in the body
- Improve cardiac function
Depending on your child’s condition, the doctor may recommend:
- Surgery—Surgery may be needed to repair or replace the tricuspid valve. This will reduce leaking.
- Ablation procedure—This procedure may be done if your child is having abnormal heart rhythms. A catheter is threaded up to the heart. Abnormal tissue is destroyed to stop the abnormal rhythms.
There are no current guidelines to prevent Ebstein's anomaly because the cause is unknown. Getting appropriate prenatal care is always important.
Ebstein’s Anomaly Foundation
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Ebstein’s anomaly. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Ebsteins-Anomaly_UCM_307025_Article.jsp. Updated November 20, 2012. Accessed May 30, 2014.
Ebstein’s anomaly. Cincinnati Children’s Hospital website. Available at:http://www.cincinnatichildrens.org/health/e/ebstein. Updated December 2012. Accessed May 30, 2014.
About Ebsteins. Ebsteins Society website. Available at: http://www.ebsteins.org/about. Accessed May 30, 2014.
Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated August 9, 2013. Accessed May 30, 2014.
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_UCM_002029_Article.jsp. Updated February 15, 2013. Accessed May 30, 2014.
Last reviewed June 2015 by Kari Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.