原发异常

Anomalía de Ebstein

这是什么??

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 房间隔缺损, or ASD (or a hole in the wall dividing the two upper chambers of the heart). The syndrome may be mild or severe.

症状是什么??

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, 成长失败, 乏力, rapid breathing or shortness of breath, 快速的心跳, 或者心脏节律问题.

If the valve abnormality is especially severe, the child may have decreased stamina, 乏力, 黄萎病, 有时液体潴留, which may lead to heart failure.

If your child has these symptoms, contact your doctor. If the symptoms persist, seek immediate attention. 

可以治疗吗??

Treatment will depend on the defect’s severity and specific symptoms. 治疗包括:

  • Medicines to help with heart failure
  • Oxygen or other breathing support
  • 矫正瓣膜的手术

What exams and 测试 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 测试 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 测试 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. 请参阅 心内膜炎 了解更多信息.

Congenital Heart Defect ID sheet

怀孕

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. 请参阅 怀孕 了解更多信息.