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Treatment of the heart in Duchenne muscular dystrophy

Published online by Cambridge University Press:  17 February 2006

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Duchenne muscular dystrophy is the most common life-limiting muscle disorder of childhood. Survival has improved, largely due to more effective orthopaedic and respiratory management. However, abnormal cardiac muscle function (cardiomyopathy) develops in most patients by the age of 18 years and can cause heart failure, leading to 10–40% of deaths. This percentage may be increasing as management of the respiratory complications becomes more successful. As a result, in 2003 a European group recommended an ECG and echocardiogram at diagnosis, then every 2 years until the age of 10, and annually thereafter. If progressive abnormalities occurred they recommended treatment with angiotensin-converting enzymes (ACE) inhibitors and, if needed, beta-blockers. They commented that there was no evidence behind these recommendations and called for multicentre trials. At the end of 2005, the Cardiology section of the American Academy of Pediatrics published similar recommendations and commented on the minimal evidence base. However, as shown by the review article by English and Gibbs in this issue, not all cardiologists agree with these guidelines.

Type
Editorial
Copyright
2006 Mac Keith Press