In order to reduce regional disparities in cardiological care for boys and female transmitters with dystrophinopathy, a working group (adult and child cardiologists, neuromuscular physicians and nurses, patient representatives), has published recommendations for follow-up and preventive care to be implemented at diagnosis and curative care once heart failure is detected. This consensus applies to children and adults with Duchenne muscular dystrophy as well as to symptomatic and non-symptomatic female transmitters (of whom an estimated 7-17% are at risk of developing cardiomyopathy).
A flow chart describes the approach to monitoring (regular ECG, echocardiography, cardiac MRI) and treatment of left ventricular dysfunction both before the appearance of an abnormality (monitoring every two years, ACE inhibitors from the age of 10 years onwards…) and in the presence of alterations (annual monitoring, ACE inhibitors or angiotensin II receptor antagonists, beta-blockers/ivabradine, mineralocorticoids…).
Therapeutic options for advanced cardiac dystrophinopathies (DMD-related heart disease) are also detailed according to the severity and typology of the cardiomyopathy.
