Endomyocardial fibrosis is a neglected tropical heart disease of poverty
which carries a poor prognosis with no specific treatment, and the most common
restrictive cardiomyopathy worldwide, affecting mainly children and young adults.
Since the first description(s) in Uganda in 1948, high-frequency areas for EMF have
included Africa, Asia, and South America and more recently, developed eastern
European countries. Although, its etiology, pathogenesis and natural history remain
unclear, its natural history includes both an active and chronic phase with recurrent
flare-ups of inflammation characterized by an impaired diastolic filling of either one or
both ventricles as a result of the fibrous tissue deposition on the endocardial surface,
variable degree of systolic dysfunction, atrioventricular valve dysfunction, and
associated intra-cavitary thrombi. This chapter aims to discuss the current knowledge
so far in clinical practice about the etiology, mechanism(s) and natural history of the
disease which would improve public awareness in rural endemic areas, leading to early
detection and diagnosis of the disease, enhancing therapeutic strategies/treatment,
biomolecular and other innovative technologies. The chapter would further go on to
explore and highlight critical potential gray areas for future translational research
which would enable both basic and clinical researchers, physicians and surgeons
recognize and address the many gaps, inconsistencies, flaws, and setbacks encountered
with this neglected cardiovascular disease.
Keywords: Africa, Asia, Adolescents, Alcohol, Cardiomyopathy, Causes,
Children, Death, Epidemiology, Endomyocardial fibrosis, Echocardiography,
Endemic disease, Health Services accountability, Heart failure, Latin America,
Mortality, Outcomes, Pathology, Physiology, Prognosis, Restrictive
cardiomyopathy, Sudden death, Surgical findings.
