A total of 18 male patients with Duchenne muscular dystrophy (DMD), aged 8–29 years
(mean, 15.7 years), were prospectively studied to assess the cardiomyopathy associated
with DMD, using clinical parameters and noninvasive cardiovascular investigations:
electrocardiogram (ECG), Holter monitoring, and echocardiography. In addition, five clinical
tests of cardiovascular autonomic function were used to assess the role of the autonomic
nervous system in the pathogenesis of dysrhythmias. The majority of subjects were …

Duchenne muscular dystrophy (DMD) is a devastating progressive disease of striated
muscle deterioration. This fatal X-linked disorder results from the loss of the protein
dystrophin, which in turn causes striated muscle membrane instability. Cardiac dysfunction
is a growing problem in patients with DMD, but relatively little is known about the
pathophysiology of the dystrophic heart. At present, there is no effective treatment for DMD
and the current clinical approaches are primarily supportive in nature. This review will …