Endomyocardial fibrosis: past, present, and future

Abstract

Endomyocardial fibrosis (EMF) is a neglected idiopathic disorder, predominant in tropical and subtropical regions of the developing world. It is characterized by fibrotic thickening of the endocardium and myocardium of one or both ventricles. EMF was an important cause of heart failure which accounted for up to 20% of the cases in endemic areas of Africa (rural community in Mozambique), but during the last few years, incidents of the disease have decreased considerably. Although its pathogenesis and etiology are not fully understood, its pathology resembles conditions such as eosinophilic cardiomyopathy and hypereosinophilic syndrome. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling, and valvular insufficiency defines the disease. Confined to peculiar and limited geographical areas, the etiology remains blurred and it carries a grim prognosis. Medical care currently remains very challenging as one-third to half of patients with an advanced disease die within 2 years. Surgery in the correct setting can increase survival and especially in patients with advanced heart failure.

Keywords: Endomyocardial fibrosis; Heart failure; Restrictive cardiomyopathy.