[Sustained monomorphic ventricular tachycardia in myotonic dystrophy]. / Taquicardia ventricular monomórfica sostenida en la distrofia miotónica.

Myotonic dystrophy is an hereditary multisystemic disease, characterized by slowly progressive myotonic atrophy of skeletic muscles. The heart is frequently affected with occurrence of arrhythmias and His-Purkinje system dysfunction and, less frequently, myocardial dysfunction. The surface ECG is the most sensible indicator of heart disease, and the most common electrophysiological finding is the prolongation of the H-V interval. Patients usually have few cardiovascular symptoms, but when present, the most frequent are: syncope, arrhythmias, atrioventricular block, congestive heart failure and sudden death. We present two patients with sustained monomorphic ventricular tachycardia as initial presentation of cardiac disease.