[The electrotherapy of the arrhythmias in 3 cases of myotonic dystrophy (Steinert's disease)].Cardiologia. 1996 Dec; 41(12):1209-14.C
Myotonic dystrophy is a progressive multisystem disease with autosomal dominant inheritance. Cardiac involvement is an integral part of the disorder, the most prominent manifestations being various conduction defects and rhythm disturbances sometimes related to syncope or sudden cardiac death. We describe 3 cases admitted to our Centres. Two patients received a permanent pacemaker for atrioventricular block of different degree and in the third case an implantable cardioverter defibrillator with antibradycardia pacing was inserted for malignant ventricular tachyarrhythmias and advanced atrioventricular block during atrial flutter.