It is a leak of blood back through the tricuspid valve. Normally the tricuspid valve opens to allow blood to flow from the right atrium to the right ventricle (the right sided pumping chamber) and then closes when the right ventricle pumps blood out to the lungs. If the tricuspid valve does not close normally, blood leaks back through the valve into the right atrium.

In Ebstein’s anomaly, the three parts of the tricuspid valve (the valve leaflets) are not in their normal position and also have not formed normally. The septal leaflet in particular is often most abnormal. This can mean that the leaflets are unable to come together to form a tight seal when the valve closes, and as a result there is a leak of blood back through the valve.

The leaky tricuspid valve means that the heart cannot pump blood forward through the lungs as efficiently as normal, and also means that the right ventricle of the heart ends up doing extra work compared to normal and may gradually enlarge as a result. If the right ventricle becomes very stretched, the heart muscle may become weak and will eventually become permanently damaged.

If the tricuspid valve is very leaky and is causing problems with the right ventricle, we may recommend an operation, either to repair the tricuspid valve or sometimes to replace the valve.

Keeping active and trying not to get overweight will help to avoid extra trouble for your right ventricle. Not smoking and not drinking excessive alcohol is also important to keep your heart as strong as it can be.

It is important that you come to your appointments at the clinic so that we can keep an eye on your heart and pick up any changes before they become too severe. Make sure you let us know if you notice new symptoms as this can sometimes be a sign that your tricuspid valve and right ventricle are getting worse.

SVT stands for supra-ventricular tachycardia (it’s bit of a mouthful, so we usually use the abbreviation). It’s an abnormal fast heart rhythm arising from the upper parts of the heart.

In people with Ebstein’s anomaly there is often a condition called Wolff-Parkinson-White syndrome, where there is an extra electrical connection between the upper and lower heart chambers. This extra electrical connection causes a  short circuit which can result in the heart rate going much faster than normal.

SVT can be a problematic heart rhythm because it can make your heart go too fast. It doesn’t always cause major problems immediately, but some people can feel weak or dizzy as a result. If your heart stays fast for too long it will also make your heart muscle get weak.

To get you back into a normal heart rhythm we usually use heart rhythm medications, which sometimes need to be given intravenously if the fast rhythm is lasting for a long time. We may recommend taking oral medications to try to prevent the rhythm from happening again.

If the fast rhythms remain a problem, we will sometimes recommend a specialized heart catheter procedure to try to stop the abnormal rhythm from occurring. This is called a catheter ablation procedure. Click here to find out more about catheter ablation.

Not usually, although some people will notice that abnormal heart rhythms are made worse by things like excessive alcohol or caffeine.

If you notice a change in your heart rhythm it’s important that you get checked out straight away. If you do go into SVT and it does not settle down by itself, we can use medications to get you back into a normal heart rhythm.

Is is a hole between the top chambers of the heart (the atriums).

It is common for people with Ebstein’s anomaly to also have an atrial septal defect.

It can be a problem because blood with low oxygen levels can flow across the defect from the right atrium to the left atrium. This means that the amount of oxygen in the blood going to your body can be lower than normal.

In some cases we may recommend a procedure to close the atrial septal defect. This needs to be considered carefully, as the exact situation can be quite different from one person to the next.

Where possible, closure of atrial septal defects is done with a heart catheter technique, but occasionally a heart operation will be required.

No. This is something that is related to having Ebstein’s anomaly. Nevertheless, the amount of blood crossing the defect can change over time meaning that it can sometimes become more of a problem as you get older. Let us know if you notice yourself becoming more fatigued or if people start to notice your lips looking blue.