Research Article: Endomyocardial Fibrosis: Still a Mystery after 60 Years

Date Published: February 27, 2008

Publisher: Public Library of Science

Author(s): Gene Bukhman, John Ziegler, Eldryd Parry, John Gyapong Owusu

Abstract: The pathologist Jack N. P. Davies identified endomyocardial fibrosis in Uganda in 1947. Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America. In Kampala, the disease accounts for 20% of heart disease patients referred for echocardiography. We conducted a systematic review of research on the epidemiology and etiology of endomyocardial fibrosis. We relied primarily on articles in the MEDLINE database with either “endomyocardial fibrosis” or “endomyocardial sclerosis” in the title. The volume of publications on endomyocardial fibrosis has declined since the 1980s. Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.

Partial Text: September 2007 will mark the 60th anniversary of the description of endomyocardial fibrosis (EMF) in Uganda by the pathologist Jack N. P. Davies [1]. Observed by Arthur Williams as early as 1938, Davies and his colleagues at Makerere University delineated the clinico-pathologic features of this new restrictive cardiomyopathy, still called Davies disease by some [2],[3],[4]. Although virtually unknown outside of the tropics, cases of EMF continue to surface from parts of equatorial Asia and South America where the disease afflicts impoverished children and young adults [5]. The highest prevalence of this condition likely remains, however, in regions of sub-Saharan Africa. As a rough estimate, the burden of EMF may well compare in scope to Chagas cardiomyopathy [6].

The clinical manifestations of EMF of either ventricle overlap with other conditions that cause heart failure or ascites. For this reason, a conclusive diagnosis of EMF depends on imaging or surgical visualization of the heart during life, or on autopsy after death [26],[27],[28].

Theories about the etiology of EMF have tried to explain the condition’s unusual geography and pathology. The apparent concentration of EMF in the tropics has led to a search for infectious or nutritional causes. In particular, the similarity of EMF lesions to those in Löffler endocarditis and carcinoid heart disease has suggested a connection with serotonin or eosinophil toxicity.

Given the difficulty of cardiovascular research in resource-poor settings, the supply of theories about EMF has exceeded the reach of investigation. The disease accounts for a striking proportion of heart failure in some regions. The dissemination of echocardiography in tropical countries should facilitate prospective studies that clarify case definition and generate new insights into the mechanisms of heart failure in sub-Saharan Africa. At the same time, molecular techniques could bring new life to old ideas.



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