Ebstein`s Anomaly

What is Ebstein’s Anomaly?

(© Competence Network for Congenital Heart Defects)

Ebstein was a doctor who first named this condition. Anomaly means something which is not usual.

The main features of Ebstein’s Anomaly are:

  • The tricuspid valve is in the wrong position. Instead of being between the right atrium and ventricle, it is low down in the right ventricle;
  • The right atrium is larger and the right ventricle smaller than in a normal heart. This means that less blue blood then normal is pumped into the lungs to receive oxygen and turn red;
  • There is often a hole between the atria – called an atrial septal defect – which lets blue blood travel from the right atrium to the left; and
  • The blood that is pumped from the left ventricle, into the aorta and round the body is too blue, meaning that it does not contain enough oxygen.

How your child may be affected

Ebstein’s Anomaly can range from very mild, with little symptoms, to very severe. How much your child is affected will depend on how much blood is able to get to the lungs and therefore how much oxygenated (red) blood is pumped around the body.

In the milder forms there may be no symptoms at all apart from a heart murmur. Some babies may have a bluish tint (cyanosis). In more severe cases, however, where there is very little blood getting to the lungs, your baby will be very ill at birth and need medical attention straight away.


Ebstein’s Anomaly can sometimes be picked up before birth during a pre-natal scan. After birth, if very little blood is getting to the lungs, your child will become very ill as soon as the foetal circulation system (ductus arteriosus) starts closing down. They will start to show signs of heart failure, be sweaty, tired and look blue (cyanosis).

If your baby has a milder form of the condition, there may not be any symptoms for some years apart from a heart murmur. The heart murmur is the sound of the blood leaking back through the abnormal tricuspid valve.

When a heart murmur is heard the tests used may include:

In older children, a fast heart rate (tachycardia), shortness of breath and chest pain may be the first symptoms of 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.

How it affects your child

Most children will be much better after treatment. They will be left with a scar down the middle of their chest, and there may be small scars where drain tubes were used. These fade very rapidly in most children, but they will not go altogether. Smaller scars on the hands and neck usually fade away to nothing.

Some of the following problems can occur after surgery or later in life:

  • It is common for the valves to leak a little, but if this becomes severe, they may need to be repaired or replaced with an artificial valve. If the valve is replaced, the new valve will need to be monitored to make sure it works effectively. Artificial valves may also need to be replaced as the child grows. Children with artificial valves will need to take anticoagulants for the rest of their lives, which can have a number of implications for their health and lifestyle.
  • Your child may develop a very fast heart beat (tachycardia). This may need to be treated with medicines – antiarrhythmic drugs.
Author(s): Children’s Heart Federation
Last updated: 2012-04-26