Endomyocardial fibrosis: A form of endemic restrictive cardiomyopathy

Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas of Africa, Latin America and Asia. It is a condition with high morbidity and mortality, for which no effective therapy is available. Although several hypotheses have been proposed as triggers or causal factors for the disease, none are able to explain the occurrence of the disease worldwide. In endemic areas of Africa endomyocardial fibrosis is as common a cause of heart failure as rheumatic heart disease, accounting for up to 20% of cases of heart failure and imposes a considerable burden to the communities and the health systems. However, due to lack of resources for research in these areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. We here review the main aspects of epidemiology, natural history, clinical picture and management of endomyocardial fibrosis, proposing new ways to increase research into this challenging and neglected cardiovascular disease.