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Question

Please can I have some information about Ebstein's anomaly?

Answer

Ebstein's anomaly describes a condition in which there is a malformation of the tricuspid valve in the heart. The tricuspid valve controls blood flow in the right ventricle, or chamber, of the heart. In Ebstein's anomaly, the tricuspid valve has an extra portion, which divides the right ventricle into two parts, when usually there is only one chamber. The valve also allows blood to flow the wrong way, and the right ventricle becomes much larger than it should as a result of all this.

This is one of the so-called cyanotic congenital (present at birth) heart diseases, because the end result is that the baby's blood is unable to pick up enough oxygen when it passes through the lungs. This is one of a number of causes of a "blue baby".

In very mild cases, there will be almost no symptoms, the baby growing normally and, as a child and adult, complaining of tiredness and occasional palpitations and abnormal heart beat.

However, many babies with Ebstein's anomaly are born with problems. They are blue, indicating lack of oxygen in the blood, have a huge heart and may go into heart failure.

The prognosis will vary in each case, depending on the severity of the anomaly, which will be determined by how severe the child's symptoms are and also by "looking" at the heart using techniques such as echocardiography.

Treatment also varies according to severity. In serious cases, the infant will have surgery to the heart when very young to allow better oxygenation of the blood. There are then often further repairs later in life. Details of what type of surgery would be required would have to be discussed with the specialist dealing with the case, since it is not always the same each time.

In mild cases, children are treated with medication to control any abnormal heart rhythm, and surgery can be delayed until adolescence or young adulthood.

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