Endemic Agrestic Cardiomyopathy: Where Negligence leads to Heart Failure, While Pain and Sudden Death Become the New Normal

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.