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  • Title: [Sustained monomorphic ventricular tachycardia in myotonic dystrophy].
    Author: Kuzmicic Calderón B, Valls Arara V, Brugada Terradellas J, Navarro Michel X, Navarro López F.
    Journal: Rev Esp Cardiol; 1994 Dec; 47(12):843-6. PubMed ID: 7855381.
    Abstract:
    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.
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