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 were noted in 15 cases, a pseudoinfarct pattern in 5 cases and loss of anterior vectors in 2 cases. It must 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 insert a permanent pacemaker, to prevent sudden death.
|Translated title of the contribution||The heart in myotonic dystrophy. Clinical and instrumental study in 17 patients|
|Number of pages||11|
|Journal||Giornale Italiano di Cardiologia|
|Publication status||Published - 1980|
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine