How Is Ebstein's Anomaly Treated?
In the beginning, medications may be given to try to reduce the pressure in the right ventricle so that less leakiness occurs. Sometimes, prostaglandin needs to be given to keep the ductus arteriosus (PDA) open until the pressure in the right ventricle gets lower. With a PDA, blood can still get to the lungs from the aorta if the right ventricle cannot pump blood to the lungs. Once the pressure in the lungs is lower, the right ventricle may be able to pump more blood to the lungs than leaks back across the tricuspid valve and the baby can survive. If this is not possible, heart surgery may be needed.
The options for a newborn infant are few. One is to try to repair the valve. Traditionally, this has not been very successful. Another option is to sew the valve shut so it doesn’t leak, and to perform a series of operations leading to a univentricular repair or Fontan operation. Sometimes there is a combination of approaches used. Another option is heart transplantation.
In an older child or adult, surgical operations to repair the valve have a higher success rate. There is also the possibility of replacing the valve with an artificial valve (not performed in babies because they will outgrow it too quickly).