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Endomyocardial fibrosis

Authors
Rebecca Cogswell, MD
Nelson B Schiller, MD
Harry Acquatella, MD, FACC, FAHA
Section Editor
Bernard J Gersh, MB, ChB, DPhil, FRCP, MACC
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

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

EMF refers to a specific syndrome with characteristic epidemiologic features. The epidemiology, pathophysiology, clinical manifestations, diagnosis, and treatment of EMF are reviewed here. Other cardiomyopathy syndromes with similar pathologies, including hypereosinophilia and/or fibrotic changes of the endocardium are addressed separately. (See "Carcinoid heart disease" and "Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis", section on 'Cardiac disease' and "Treatment and prognosis of myocarditis in adults", section on 'Eosinophilic myocarditis'.)

EPIDEMIOLOGY

Endomyocardial fibrosis (EMF) was first recognized in Uganda during the 1940s and accounts for as much as 20 percent of cardiac cases sent for echocardiography in that country in contemporary series [1,2]. Although accurate epidemiologic data are lacking [1], EMF is estimated to the most common form of restrictive cardiomyopathy worldwide.

Most studies of EMF occur in tropical regions where there is a high prevalence of disease such as Uganda [3], Nigeria [4], Ivory Coast, south India [5], and Brazil [6]. EMF also occurs in subtropical regions. An echocardiographic screening study in Mozambique found a population prevalence of 20 percent; however, this study included patients with early, subclinical disease [7]. Within endemic countries, there appears to be a regional variation [8,9].

EMF is primarily a disease of the young, occurring in children, adolescents, and young adults. In Uganda, a bimodal peak at ages 10 and 30 has been observed [10], and a similar pattern was found in Mozambique [7]. The differences between genders in the frequency of disease have been variable [7,10,11].

                

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Literature review current through: Nov 2016. | This topic last updated: Fri Sep 25 00:00:00 GMT+00:00 2015.
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