Perhexiline and hypertrophic cardiomyopathy: a new horizon for metabolic modulation.

Despite the considerable morbidity and mortality associated 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