Ebstein's Anomaly
What is it?
Ebstein’s anomaly is a malformed heart valve between the right upper and lower heart chambers that does not properly close to keep the blood flow moving in the right direction. Blood may leak back from the lower to upper chambers on the right side of the heart. This may result in significant enlargement of the right heart chamber. In more extreme cases the size of the right ventricle (larger, lower chamber) is too small to allow for enough blood to go to the lungs.
This syndrome also is commonly seen with an atrial septal defect, or ASD (or a hole in the wall dividing the two upper chambers of the heart). The syndrome may be mild or severe.
What are the symptoms?
Symptoms can vary from mild to very severe. Some people may not have any symptoms, or symptoms may develop later in life.
The symptoms that may appear shortly after birth include bluish-colored lips and nails due to low blood oxygen levels. If severe, the baby may have trouble breathing and feeding. Symptoms in older children include cough, failure to grow, fatigue, rapid breathing or shortness of breath, fast heartbeat, or heart rhythm problems.
If the valve abnormality is especially severe, the child may have decreased stamina, fatigue, cyanosis, and sometimes fluid retention, which may lead to heart failure.
If your child has these symptoms, contact your doctor. If the symptoms persist, seek immediate attention.
Can it be treated?
Treatment will depend on the defect’s severity and specific symptoms. Treatment may include:
- Medicines to help with heart failure
- Oxygen or other breathing support
- Surgery to correct the valve
What exams and tests will be done?
Children with Ebstein's anomaly should receive continued care from a pediatric cardiologist (and an adult cardiologist as they move into adulthood). Besides getting information from routine exams, the cardiologist may use tests such as electrocardiograms (EKG), chest X-ray, MRI of the heart and echocardiograms.
What activities will my child be able to do?
If valve leakage is mild and tests show no abnormal heart rhythms, your child can usually participate in most sports. Exercise will help strengthen the heart and improve blood flow. Your child’s cardiologist may recommend avoiding certain intense competitive sports. Ask which activities are appropriate. When the child becomes an adult, continue to consult a cardiologist about appropriate exercise activities.
What about preventing endocarditis?
People with an unrepaired or repaired valve are at risk for endocarditis. Ask your pediatric cardiologist about your child's need to take antibiotics before certain dental procedures to help prevent endocarditis. This is a lifelong consideration. See the section on Endocarditis for more information.
Congenital Heart Defect ID sheet
Pregnancy
For women, pregnancy puts many increased demands on the heart. In most cases women with mild Ebstein's anomaly will be able to safely deliver a baby. Still, each woman should be evaluated individually. Consult with your cardiologist about the safety of pregnancy. See the section on Pregnancy for more information.
