Hypertrophic Cardiomyopathy in Children

Hypertrophic cardiomyopathy is a congenital disease that may be manifested in infancy, childhood, adolescence, or young adulthood. It is heterogeneous both genotypically and phenotypically, and as such, it requires an individualized approach to medical management. The symptomatic patient can have progression of symptoms but can be treated with appropriate medical or surgical therapy. The symptomatic infant usually has a grave prognosis and should be given early consideration for heart transplantation. The future holds promise because genotyping may recognize pedigrees with more "malignant" courses that can be treated more aggressively to prevent sudden death. The greatest challenge lies with the primary care physician who must take careful measures to identify those asymptomatic patients who may be at risk for sudden death during athletic activities.