Outcome of septal myectomy in patients with Fabry's disease.

Fabry's disease has cardiac manifestations that may mimic hypertrophic cardiomyopathy, including left ventricular outflow tract (LVOT) obstruction. It is important to distinguish hypertrophic cardiomyopathy from Fabry's disease, which can be treated with enzyme replacement therapy (ERT). However, after adequate ERT, some patients have no resolution of cardiac symptoms; thus, septal myectomy might be considered. We present 2 cases of extended septal myectomy in patients with LVOT obstruction secondary to Fabry's disease.