Endomyocardial fibrosis: a form ofendemic restrictive cardiomyopath

Abstract

Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly childrenand adolescents, and is geographically restricted to some poor areas of Africa, Latin America andAsia. 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, noneare able to explain the occurrence of the disease worldwide.In endemic areas of Africa endomyocardial fibrosis is as common a cause of heart failure asrheumatic heart disease, accounting for up to 20% of cases of heart failure and imposes aconsiderable burden to the communities and the health systems. However, due to lack of resourcesfor research in these areas, the exact epidemiology, etiology and pathogenesis remain unknown, andthe natural history is incompletely understood.We here review the main aspects of epidemiology, natural history, clinical picture and management ofendomyocardial fibrosis, proposing new ways to increase research into this challenging andneglected cardiovascular disease.