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Ebstein's Malformation
What is it?
Babies with Ebstein's malformation have a tricuspid valve that isn't developed right. This valve is one of the doors in the heart that allows blood to go between the chambers.
The tricuspid valve is the door to the right ventricle. It lets blood flow in one direction and stops leaks in the other direction. The tricuspid valve normally has three flaps or leaflets.
In Ebstein's malformation, one or two of the three flaps don't move normally. Because the tricuspid valve is not formed right, it doesn't work well and blood may leak backwards into the right atrium (upper chamber where the blood comes in) with each heartbeat. This will make the right atrium get too large.
Often times there's also a hole in the septum between the atria called an atrial septal defect. This allows blood without oxygen to be pumped out to the body and can sometimes cause cyanosis.
How is it treated?
If the condition isn't too bad, a child is watched for signs of the valve breaking down and surgery may not be needed for years.
Surgery is done to repair the tricuspid valve to reduce the amount of leaking. In some cases where the valve can't be repaired, it's replaced with a valve from a pig or a cow. During surgery, the atrial septal defect is also closed.