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Transposition of the Great Arteries

Transposition of the great arteries is the most common cause of a “blue baby”, (ie. newborn congenital heart disease with low oxygen levels).

The first operations for this condition were described by Dr William Mustard from Canada and Dr Ake Senning from Sweden. These are life-saving operations, but as is the case with many operations for congenital heart disease, the heart is not completely normal after this surgery and there are important things that we need to watch out for. People who have had this surgery need life long cardiology follow-up.

The boxes below describe three of the most important problems that we see in adults with TGA after the Mustard and Senning operations

For further information about TGA and the Mustard and Senning operations click on the links at the bottom of the page

Right Ventricular Dysfunction

It means that you develop weakness of the right ventricle. After the Mustard and Senning operations this is the main pumping chamber of the heart, which pumps blood around your body.

Normally the right ventricle pumps blood to your lungs, but after the Mustard and Senning operations it pumps to your body. This means that it has to do a lot more work than it is designed for and often it gradually gets tired as a result of this.

If the right ventricle gets weak, it may not be able to pump enough blood around your body. It it gets worse, you may find that you get tired and breathless more easily and you may start to retain extra fluid.

We can use certain medications that may help to make the right ventricle deteriorate more slowly and other medications that help to shift any extra fluid that accumulates. There isn’t any surgery that can repair the right ventricle and some people have needed heart transplants if their right ventricles have become very weak.

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 let us know if you notice new symptoms as this can sometimes be a sign that your right ventricle is getting weaker.

Atrial Flutter

It’s an abnormal fast heart rhythm arising from the atriums (the top chambers) of the heart.

After the Mustard and Senning operations there is lots of scarring in the atriums because this is where the surgeon has to do the stitching. We know that when there is scarring it can be a set-up for short-circuits that lead to these abnormal rhythms.

It can be a dangerous heart rhythm because it can make your heart go too fast. This doesn’t always cause problems immediately, but if your heart stays fast for too long it will make your heart muscle get weak. Also it makes you more likely to develop blood clots in the atriums of the heart which can potentially break off and go to your brain, causing strokes.

To get you back into a normal heart rhythm we either use medications or cardioversion, which is an electrical shock to get your heart back into rhythm (you have a quick general anaesthetic for this). Click here to find out more about cardioversion.

To stop the atrial flutter happening again, first up we will often use heart rhythm medicines. Sometimes we will do 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 atrial flutter we will usually want to try to get you back into a normal heart rhythm as soon as possible.

Sinus Node Dysfunction

The part of your heart that generates the heart beat is not working as well as it should and the result is that your heart rate is slower than normal.

It happens because the part of the heart where the heart beat originates is very close to where the surgeon has to make their cuts at the time of the original operation. We know that there is often scarring of this area in people who have had the Mustard and Senning operations.

Your heart beat will be too slow and cannot speed up enough when you are more active. This means that you may not be able to be as active as usual. In some people the slow heart beat causes dizzy spells or even black-outs.

If your slow heart beat is causing problems we can put in a pacemaker. This is a small device that sits under the skin of your upper chest and connects to your heart through one of the big veins. The pacemaker detects if your heart goes too slow and gives a tiny electrical signal to your heart that makes it speed up.

Click on this link to find out more about pacemakers.

Probably not. This is something that is related to the original surgery rather than anything that has happened since.

If you do notice yourself slowing down or having dizzy spells or blackouts, get checked out by your doctor or let your cardiology team know so that we can check things out.