[The heart in myotonic dystrophy. Clinical and instrumental study in 17 patients (author's transl)].
17 patients with myotonic dystrophy have been studied. The skeletal muscle disease has been assessed by history, physical examination, electro-myography and muscle biopsy. Cardiac evaluation has been obtained by history, physical examination, chest X-ray, ecg at rest and after exercise, vectorcardiography and echocardiography. Only two patients presented symptoms and clinical findings of cardiac disease, otherwise conduction disturbances have been founded in 15 cases, pseudoinfarct pattern in 5 cases and loss of anterior vectors in 2 cases. Has to be remembered that in myotonic dystrophy sudden death has a very high incidence, which could be explained by the development of complete a-v block, because of the impairment of the conduction system. Therefore, in those patients with bifascicular blocks (3 cases), the electrophysiological study may be useful in order to evaluate the opportunity to put on a permanent pace-maker, to prevent sudden death.
D000293:Adolescent; D000328:Adult; D006332:Cardiomegaly / Q000209:etiology*; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications*; D014672:Vectorcardiography
Il cuore nella distrofia miotonica. Studio clinico e strumentale in 17 pazienti.