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. 2008 Feb 27;2(2):e97.
doi: 10.1371/journal.pntd.0000097.

Endomyocardial fibrosis: still a mystery after 60 years

Gene Bukhman  1 , John Ziegler, Eldryd Parry
Affiliations

Endomyocardial fibrosis: still a mystery after 60 years

Gene Bukhman et al. PLoS Negl Trop Dis. .

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.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Top, echocardiogram in a 25-y-old man with predominantly right ventricular EMF from eastern Rwanda.
Apical four-chamber view. Note the marked dilatation of the right atrium. RV = right ventricle, RA = right atrium, LV = left ventricle, LA = left atrium. Bottom, massive ascites in the same patient.
Figure 2
Figure 2. Number of publications in MEDLINE between 1950 and 2006 with either “endomyocardial fibrosis” or “endomyocardial sclerosis” in the title.
Figure 3
Figure 3. Distribution by country of published cases of endomyocardial fibrosis between 1950 and 2006.
Includes only those cases diagnosed at autopsy, or confirmed by surgery or cardiac imaging. Within-country variation depicted for Brazil, China, India, Mozambique, and Nigeria.
See this image and copyright information in PMC

References

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    1. Connor DH, Somers K, Hutt MS, Manion WC, D'Arbela PG. Endomyocardial fibrosis in Uganda (Davies' disease). 1. An epidemiologic, clinical, and pathologic study. Am Heart J. 1967;74:687–709. - PubMed
    1. Williams A. Heart disease in the native population of Uganda. East African Medical Journal. 1938;15:279.
    1. Hutt MS. Epidemiology aspects of endomyocardial fibrosis. Postgrad Med J. 1983;59:142–146. - PMC - PubMed

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