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Endomyocardial fibrosis (EMF) was described as a distinct clinicopathological entity in 1948 in Uganda.w1 Initially several terms were used to describe the disease. These included tropical endomyocardial disease, endocarditis parietalis fibroplastica, endocardial fibrosis, constrictive endocarditis and endocardial fibroelastose.
EMF is thought to be the most common type of restrictive cardiomyopathy worldwide.1 Although most studies have been reported from Uganda, Ivory Coast, Nigeria, Brazil and India, the disease has been occasionally encountered outside the tropics.2 Geographic distribution of EMF is not uniform both in Africa and Asia.3 4 In the endemic areas of Africa, EMF is the second cause of admission for acquired cardiovascular disease in children and young adults, after rheumatic heart disease,5 w2 accounting for up to 20% of all causes of heart failure.
To date our knowledge of the prevalence of the disease is derived from hospital based studies with absence of data from systematic studies in the community. EMF affects predominantly children and adolescents, usually from low socioeconomic background. More than half the cases are seen in the first decade of life. Male preponderance was found in Kerala and Nigeria, while female preponderance has been described in Brazil and Uganda. A bimodal age distribution has been reported in some studies.5 w3
AETIOLOGY AND PATHOGENESIS
The aetiology of EMF remains unknown. Several hypotheses have been proposed and explored including cardiotoxicity of the eosinophil, infectious agents, autoimmune processes, genetic predisposition, ethnicity, diet, climate and poverty.
EMF appears to share some pathogenic mechanisms with the hypereosinophilic syndromes, which comprise a heterogenous group of disorders characterised by peripheral eosinophilia for at least 6 months and end organ damage related to eosinophil infiltrations. These syndromes have been classified as idiopathic, clonal and reactive. The similarity of mechanisms responsible for EMF and the hypereosinophilic syndromes could have exciting therapeutic implications. …
Competing interests: In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The authors have no competing interests.
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