Why is this necessary?

The heart is a muscle and dystrophin is needed to make it work properly. People with Duchenne lack dystrophin. Duchenne can affect the heart in one of two ways. Firstly, the conduction tissue may be damaged, resulting in abnormal heart rhythms (the heart beating too fast or too slow). These can lead to symptoms such as dizzy spells, palpitations or blackouts.

Fast rhythms can be treated and prevented with drug therapy. Slow rhythms are usually caused by a heart block and treatment may require insertion of a pacemaker (a minor operation carried out under local anaesthetic). Heart rates can be checked by electrocardiogram (ECG).

Secondly, there may be a problem with the pumping action of the heart, called cardiomyopathy, symptoms such breathlessness may occur. Drug treatment can be given to improve the heart’s pumping action.

Often heart problems can occur without any symptoms so it is very important to have frequent heart checks so that treatment can start as early as possible. Heart function should be checked at least once every two years from the age of diagnosis until the age of ten and yearly after that, or as soon as symptoms arise.

Heart function can be checked by ECG, echocardiogram or cardiac MRI.

Female carriers who show no signs of (or very slight) muscle weakness may have some cardiomyopathy and should also have regular assessments.

There are a number of drugs already available that can help with heart problems (see link) and research is ongoing to find better treatments to keep the heart stronger for longer.

What drugs are currently used to protect the heart?

Please find out more on our Treatment for the Heart page here.

For further information we recommend this comprehensive guide for families, published by Treat NMD, PPMD, UPPMD & MDA.