Familial Dilated Cardiomyopathy and Spontaneous Ventricular Arrhythmias

Two members of a family with (autosomal dominant) dilated cardiomyopathy and symptomatic short-lasting ventricular tachyarrhythmias were each treated with an ICD in the course of their disease. One patient had an episode of torsades de pointes induced by amiodarone, and the ICD failed to recognize some events. Cardiac arrest recurred in this setting. Treatment with bisoprolol was helpful in maintaining an acceptable functional status and in preventing multiple shocks until transplantation became mandatory. Bisoprolol was not tolerated by the second patient, who had several episodes of syncope because of nonsustained ventricular tachycardia. His functional course went downhill fast, and he received a heart transplantation 16 months after implantation of an ICD, which had not delivered any shocks, in spite of one symptomatic short ventricular tachycardia. (Am J Cardiol 1996;78(suppl 5A):102–104)