Ebstein's Anomaly -- Child

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Ebstein’s Anomaly—Child

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

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

Blood Flow Through the Heart

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
Palpitations or feeling of skipped beats
Decreased energy
Failure-to-thrive or gain weight
Shortness of breath

During the exam, the doctor may detect a heart murmur .

These symptoms may be due to other conditions. If your child has any of these, talk to the doctor right away.

Diagnosis

The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:

Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply
Chest x-ray —an imaging test that uses low amounts of radiation to create an image of the chest
MRI scan —a test that uses magnetic waves to make pictures of structures inside the chest
CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
Electrocardiogram —a test that measures the electrical activity of the heart
Other monitors (Halter or Event monitor) and tests (eg, stress test ) to measure the heart’s rhythm and function

Treatment

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

Medication

The doctor may prescribe medicines to:

Help restore normal heart rhythms
Reduce fluid in the body

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 appropriate prenatal care is always important.

RESOURCES

Ebstein’s Anomaly Foundation http://www.ebsteinsanomaly.org/

Ebstein’s Society http://www.ebsteins.org/

CANADIAN RESOURCES

Canadian Cardiovascular Society http://www.ccs.ca/

Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

References

American Heart Association. Ebstein’s anomaly. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=11075 . Accessed July 1, 2010.

American Heart Association. How your cardiologist diagnoses heart defects. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=152 . Updated June 2010. Accessed July 5, 2010.

Cincinnati Children’s Hospital. Ebstein’s anomaly. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/ebstein.htm . Updated July 2009. Accessed July 1, 2010.

DynaMed Editorial Team. Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated February 11, 2010. Accessed July 1, 2010.

Ebstein’s Society. Ebstein’s anomaly. Ebstein’s Society website. Available at: http://www.ebsteins.org/?page%5Fid=2 . Accessed July 1, 2010.

Mayo Clinic. Atrioventricular canal defect. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745/DSECTION=risk-factors . Updated June 3, 2010. Accessed July 7, 2010.

Revision Information

Reviewer: Michael Woods, MD
Review Date: 09/2012 -
Update Date: 00/93/2012 -

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.

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