Endomyocardial Fibrosis: Still a Mystery after 60 Years

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.

Endomyocardial fibrosis associated with mansoni schistosomiasis

“…The eosinophilic endomyocardial disease seems to be a result of prolonged release products of degranulating eosinophils 8 . Rasshwan et al, in an extensive echocardiographic investigation involving 10,000 patients with schistosomiasis, had raised a supposed relationship between schistosomiasis and endomyocardial fibrosis in 15 patients 9 .…”

“…The patient presented progressive improvement of pulmonary congestion after conventional treatment for heart failure and was discharged after 5 days of hospitalization. Endomyocardial fibrosis is characterized by fibrotic involvement of the endocardium and adjacent myocardium, determining a restrictive syndrome [1][2]8 . Several theories are being considered to better understand the cause and pathogenesis of the disease.…”

“…Several theories are being considered to better understand the cause and pathogenesis of the disease. The accepted mechanisms are a pancarditis with eosinophilia, primarily by helminthiasis, or by immunological factors, genetic, nutritional deficiencies, or post-radiation treatment 8 . Endomyocardial fibrosis is an uncommon disease in Brazil, where the prevalence is higher among female than (among) male subjects.…”

Endomyocardial Fibrosis (EMF) in a Ugandan Child with Advanced Hepatosplenic Schistosomiasis: Coincidence or Connection?

“…It represents a significant local health burden, although its etiology remains enigmatic. 3 EMF can feature right and/or left ventricular endocardial fibrosis extending to the inner third of the myocardium. It typically affects the apex and inflow regions, 3 leading to restrictive cardiomyopathy and cardiac failure.…”

Right Ventricular Endomyocardial Fibrosis (2013 Grover Conference Series)

“…1 It is the most prevalent form of restrictive cardiomyopathy worldwide, showing marked variation in geographic distribution. [2][3][4] The hypotheses postulated to explain the etiology of EMF have not been proven and cannot explain its occurrence worldwide.…”