Ebstein`s Anomaly

The treatment for Ebstein’s Anomaly will depend on how severe the condition is. Your child’s cardiologist will discus the treatment options appropriate for your child.

If your child has mild symptoms, he or she will need to be monitored by a paediatric cardiologist, and may never need treatment.


If your child has poor oxygen levels, meaning that there is not enough blood getting to the lungs, they will need open heart surgery.  This means that their heart will need to be stopped and opened in order to repair it. A machine will have to take over the job that the heart normally does – the heart bypass machine.  

The main aim of the surgery will be to get a good supply of blue blood to the lungs to collect oxygen. If the condition is not very severe, then the surgery will:

Tricuspid valve replacement

Sometimes the tricuspid valve is very deformed and needs to be replaced by a mechanical (artificial) valve. Artificial valves do not grow with the child, so further valve surgery may be needed as your child gets bigger.

Children with artificial valves will also need to take anticoagulants for the rest of their lives, which can have a number of implications for their health and lifestyle.
Fontan procedure

In some children with Ebstein’s Anomaly, the right ventricle is too small to function properly. In this case, the surgeons may connect the right atrium directly to the pulmonary artery (called the Fontan procedure). This means that the blue blood is pushed directly into the pulmonary artery without having to pass through the tricuspid valve and the right ventricle.

BT Shunt and Fontan procedure

Children born with a very severe form of Ebstein’s Anomaly may need to have a BT shunt to increase the amount of blood going to their lungs. This will be done soon after birth. The BT shunt is done by connecting a tube from an artery in the arm to the pulmonary arteries. This operation may be carried out through the side of the chest, while the heart is beating, or through the front using a heart lung bypass machine (open heart surgery).  

Further surgery (the Fontan procedure), can then be carried out two or three years later. Children may take some time to recover from these surgical procedures because they change the pressures in the circulation and the heart and lungs need time to adapt.

More about Ebstein´s Anomaly

Author(s): Children’s Heart Federation
Last updated: 2012-06-14