Restrictive Cardiomyopathy in Childhood

Depending on the part of the world one lives in, restrictive cardiomyopathy is either one of the rarest forms of cardiomyopathy in childhood, with no cause usually identified, or it is secondary to a poorly understood disease, endomyocardial fibrosis, that is endemic in some populations. Regardless of the underlying cause, the outcome is poor once symptoms develop. This article reviews the definitions, epidemiology, etiologies, genetics, "overlap" phenotypes, clinical presentation, diagnostic evaluation, outcome, and management of pediatric patients with restrictive cardiomyopathy.