Clinical and induced ventricular tachycardia in a patient with myotonic dystrophy

A 20 year old patient with myotonic dystrophy presented with hemodynamically significant ventricular tachycardia at a rate of 230 beats/min requiring cardioversion. Two days later, the identical tachycardia was reproducibly initiated and terminated in the electrophysiology laboratory using two extrastimuli in the right ventricle. Trials of procainamide and quinidine were not successful in controlling the induced rhythm and amiodarone was administered. On restudy with amiodarone, ventricular fibrillation was induced using a single extrastimulus. This case suggests that ventricular tachyarrhythmias may contribute to the known cardiac morbidity and mortality in myotonic dystrophy.