Perhexiline and Hypertrophic Cardiomyopathy

D espite the considerable morbidity and mortality associ- ated with hypertrophic cardiomyopathy (HCM), proven therapeutic modalities for this disorder remain limited.The most feared complication, sudden death, usually due to ventricular tachyarrhythmias, can be averted by the use of implantable defibrillators, but patient selection for such procedures remains problematic.In recent years, evidence has accumulated that the presence of substantial outflow tract obstruction is associated with increased severity of symptoms and increased risk of sudden death, 1 and interventions based on amelioration of outflow tract obstruction, via surgical myectomy or alcoholic septal ablation, appear to be relatively effective in improving systematic status.2,3 Article see p 1562Less clear-cut is the efficacy of various forms of pharmacotherapy.Although there have been a number of reports of symptomatic improvement associated with therapy with ␤-adrenoceptor antagonists, calcium antagonists, disopyramide, and amiodarone, these studies have not been placebo controlled.Two further concerns are the lack of investigations of pharmacotherapy in patients with nonobstructive HCM, who constitute approximately two thirds of the total group, and the probability that the currently available forms of pharmacotherapy for HCM affect neither the risk of sudden cardiac death 4 nor the progression of left ventricular hypertrophy.