Chronologic study of signs of myocardiopathy in progressive muscular dystrophy
Barona Zamora P, Narbona García J, Alvarez Gómez MJ, Fidalgo Andrés ML, Sáenz de Buruaga J, Villa Elizaga I.
Departamento de Pediatria, Clínica Universitaria de Navarra, Facultad de Medicina, Pamplona.
In order to analyze the evolution of cardiomyopathy in progressive muscular dystrophies, thirty-three patients (17 with Duchenne type, 11 with Becker type and 5 with the autosomal recessive type dystrophy) were studied retrospectively. Cardiac and systemic follow-up every 3-6 months was made in 29 patients.
The electrocardiogram was the first test that became altered, followed by the echocardiogram and thoracic radiograph and finally heart failure manifestations. There was a direct correlation between age and the appearance of abnormal cardiac tests.
Electrocardiographic alterations, in patients who were less than 12.5 years of age, were significantly more frequent in the group with Duchenne dystrophy that in the no-Duchenne group. In regards to the appearance of the echocardiographic and radiographic abnormalities, there were no significant differences between the two groups.
However, we have noticed a trend towards a more frequent and earlier presentation of these abnormalities in the Duchenne's muscular dystrophy than in the no-Duchenne group.
CITATION An Esp Pediatr. 1993 Feb;38(2):173-7