Restrictive cardiomyopathy.

BACKGROUND: Restrictive cardiomyopathy is characterized by ventricular diastolic dysfunction with a clinical course in childhood that is often progressive despite medical therapy.
METHODS: A review of the literature and clinical experience was used to summarize the natural history of this oftentimes devastating disease with a focus on diagnostic tools and therapeutic options.
RESULTS: The clinical presentation of restrictive cardiomyopathy can be highly variable, ranging from asymptomatic to overt signs of heart failure with pulmonary hypertension. Emerging noninvasive diagnostic tools are increasingly helpful yet there remains a role for invasive studies including cardiac catheterization with or without endomyocardial biopsy. A significant risk of sudden death exists and may not be limited to those with more pronounced symptoms.
CONCLUSIONS: Children with restrictive cardiomyopathy require comprehensive evaluation with careful surveillance. Early listing for cardiac transplantation should be considered.