Tropical endomyocardial fibrosis: an overview

Abstract

Tropical endomyocardial fibrosis is the commonest form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas in the tropical and subtropical regions of the world such as Africa, Latin America and Asia including southern districts of India especially in the coastal belt of Kerala state. Sub-endocardial fibrosis affecting the apices and the inflow tracts of the right or left ventricle, or both; and varying degree of atrioventricular valve regurgitation defines the disease. Chronic systemic venous hypertension and severe pulmonary hypertension are characteristic features of right ventricular and Left ventricular endomyocardial fibrosis respectively. Due to lack of resources for research in the disease endemic areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. Various infections and toxic factors were postulated regarding its etiology. During the last few years, incidence of the disease has decreased considerably because of the significant improvement in the living standards of the people with the corresponding decline in the childhood malnutrition, infections, worm infestations and associated eosinophilia. It is a condition with high morbidity and mortality, for which no effective therapy is available. However, surgical management improves the natural history of this disease to some extent. We have conducted a systematic review of the most intriguing 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. We relied primarily on articles in the MEDLINE database with either ''endomyocardial fibrosis'' or ''endomyocardial sclerosis'' in the title.

Cardiac surgery in Africa: a thirty-five year experience on open heart surgery in Cote d’Ivoire

“…EMF is also discovered in inter-tropical countries in South and central America (Colombia, Brazil, Venezuela, Mexico), in India and in North America or in Asia (52,56,57). Several causes of EMF have been shown and none of them was validated; they are as follows (52)(53)(54)(55)(56)(57): eosinophilia, but endomyocardial biopsies made were negative; Infections such as toxoplasmosis, rheumatic fever, malaria, and helminthic parasites, toxic agents: cassava, serotonin; malnutrition; autoimmunity and ethnicity. Clinical patterns (52,54,58) include right ventricular failure symptoms like liver enlargement, ascites, peripheral edema, splenomegaly and tricuspid regurgitation; or left ventricular failure characterized by pulmonary hypertension and mitral regurgitation.…”

CSI position statement on management of heart failure in India

“…Idiopathic RCM is a rare condition that may present in both children and adults 452, 453. Both familial and sporadic cases have been described 454, 455.…”

Endomyocardial diseases cardiomyopathy: A review and pooled analysis of pathophysiology, diagnosis and clinical management

“…Aetiopathogenesis: The aetiopathogenesis of EMF remain unclear, warranting more systematic research and the use of contemporary technologies to test older classical hypothesis whose findings have varied across studies. The proposed causes or co-factors for the development of EMF include poverty, malnutrition, parasitic infestation, genetics and cluster ethnicity while proposed theories for the pathogenesis of EMF are eosinophilia, infectious disease and autoimmunity [9,[33][34][35][36][37]. Similarities in cardiac lesions with hypereosinophilic syndrome (HES) and the prevalence of parasites in the EMF endemic countries suggest eosinophilia toxicity and infections as potential primary aetiologic agents [9,19,20,[35][36][37][38][39].…”