Ebstein’s Anomaly—Child
En Español (Spanish Version)

Definition
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

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Blood Flow Through the Heart

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Causes
This is a congenital defect. This means that the heart forms incorrectly when the baby is developing in the womb. The baby is born with the condition. It is not known why the heart develops this way in some babies.

Risk Factors
Specific risk factors for Ebstein’s anomaly are not clear. Two possible risk factors include:

  • Genetic abnormalities
  • Environmental exposure
Symptoms
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
  • Shortness of breath
Diagnosis
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:

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

Medication
Medications may be prescribed to:

  • Help restore normal heart rhythms
  • Reduce fluid in the body
  • Improve cardiac function
Procedures
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.
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
There is no way to prevent this condition. Getting prenatal care is always important.




RESOURCES:
Ebstein’s Anomaly Foundation

Ebsteins Society

CANADIAN RESOURCES:
Canadian Cardiovascular Society

Heart and Stroke Foundation of Canada

References:
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/heart-encyclopedia/anomalies/ebstein.htm. Updated December 2012. Accessed May 30, 2014.

About Ebsteins. Ebsteins Society website. Available at: http://www.ebsteins.org/?page_id=2. Accessed May 30, 2014.

Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at:  http://www.ebscohost.com/dynamed/what.php. 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 May 2014



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