Hypertrophic cardiomyopathy in children with end-stage renal disease and hypertension.

Cardiac function was evaluated by echocardiography (echo) in ten paediatric patients, 2.5 to 15 years of age, maintained on chronic dialysis. All had moderate to severe hypertension despite intensive antihypertensive medication. Four of ten patients showed, on echo, asymmetric septal hypertrophy (ASH) typical of hypertrophic cardiomyopathy (HCM). Two also had signs suggestive of outflow obstruction. Primary cardiomyopathy was excluded by a family survey of first-degree relatives. The incidence of HCM in our series is impressive (40%). The pathogenesis of this cardiac lesion in uraemia is unknown; long-standing severe hypertension may play a role. Early recognition of this form of cardiomyopathy has important clinical considerations. Echo should become a routine examination in the diagnosis, follow-up, and treatment of children with end-stage renal disease.