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Endomyocardial fibrosis

INTRODUCTION

Endomyocardial fibrosis (EMF) is a disease that is characterized by fibrosis of the apical right ventricle (RV), left ventricle (LV), or both ventricles. The clinical manifestations are largely related to the consequences of impaired ventricular filling, including left and right sided heart failure.

The pathogenesis, presentation, diagnosis and treatment of EMF will be reviewed here. EMF refers to a specific syndrome with characteristic epidemiologic and clinical features. Other cardiomyopathy syndromes with similar pathologies, including hypereosinophilia and/or fibrotic changes of the endocardium are addressed separately (see "Carcinoid heart disease" and "Clinical manifestations, pathophysiology, and diagnosis of the hypereosinophilic syndromes", section on 'Cardiac disease' and "Natural history and therapy of myocarditis in adults", section on 'Eosinophilic myocarditis'.

EPIDEMIOLOGY

EMF was first recognized in Uganda during the 1940s and, in contemporary series, accounts for as much as 20 percent of cardiac cases sent for echocardiography in that country [1,2]. The majority of reported cases have come from West and Central Africa. Additional reports of similar conditions have come from other wet regions near the equator, including parts of South America, Kerala State in India, and sporadic cases from other parts of the world. '

Regional variation in the frequency of EMF has been reported in endemic countries. Echocardiographic screening of a random sample of the population of a rural area of Mozambique revealed a prevalence of 20 percent [3]. Only 23 percent of subjects with echocardiographic evidence of EMF were symptomatic.

PATHOGENESIS

The characteristic clinical features of EMF result from the long-term effects of apical fibrosis, which can include:
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