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Cardiomyopathy of Duchenne muscular dystrophy

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Summary

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 asymptomatic, but four had abnormal physical findings. All had abnormal ECG, the commonest abnormality (in 16) being tall R waves or increased R/S ratios in the right precordial leads; 14 had abnormal findings on echocardiography, including three with poor left ventricular function and five with mitral valve prolapse (MVP). Labile abrupt sinus tachycardia was present in 11, and four had high-grade ventricular ectopy. None had definite clinical evidence of autonomic dysfunction.

The cardiomyopathy of DMD appears to be unrelated to disease severity. However, abnormal Q waves or Q/R ratios in ECG leads I, a VL, and V5–V6 are significantly related to young age (p<0.05), and high-grade ventricular ectopy occurred significantly more frequently (p<0.05) in older subjects (>15 years). Dysrhythmias were not related to the presence of MVP, poor left ventricular function, or autonomic dysfunction.

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Authors and Affiliations

  1. Princess Margaret Hospital for Children, Perth, Western Australia

    Luigi D'Orsogna

  2. Department of Cardiovascular Medicine, Sir Charles Gairdner Hospital, Perth, Western Australia

    John P. O'Shea

  3. Neuromuscular Research Institute, Queen Elizabeth II Medical Centre, Perth, Western Australia

    Geoffrey Miller

Authors
  1. Luigi D'Orsogna
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  2. John P. O'Shea
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  3. Geoffrey Miller
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D'Orsogna, L., O'Shea, J.P. & Miller, G. Cardiomyopathy of Duchenne muscular dystrophy. Pediatr Cardiol 9, 205–213 (1988). https://doi.org/10.1007/BF02078410

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