What is Ebstein’s Anomaly?

A rare heart defect, Ebstein’s anomaly is where the tricuspid valve is abnormal in parts. This valve separates the right ventricle from the right atrium. It is a congenital defect. The defect has the valve working poorly and allowing the blood to go the wrong way. It can lead to fluid buildup or heart swelling. The causes are unknown and it is a rare defect. It is more predominant in Caucasians.


Right after birth, signs can be bluish nails and lips. If it is severe, they can have breathing difficulties and be very sick. Older children show signs like fast heartbeats, shortness of breath, fatigue, failure to grow, coughing, and rapid breathing.


Upon a physical exam, the doctor may hear a murmur or other abnormal heart sounds. They may order a chest x-ray, an MRI of the heart, an EKG or monitor the electrical activity of the heart, or do an ultrasound of the heart.


Depending on the severity of the symptoms and defect, treatment options will include medications, breathing support, oxygen therapy, and surgery. The earlier that one starts to get symptoms, the worse the condition gets. Some have no symptoms or very mild symptoms. Some of the complications that may arise include brain abscess, blood clots, abnormally fast heart rhythms and abnormally slow heart rhythms.

This condition isn’t too common and may be detected as soon as birth or may arise later in the child’s life. Some people can live well with the condition while others will be plagued with health issues.

Source: A.D.A.M.

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