Tropical Endomyocardial Fibrosis: Natural History, Challenges, and Perspectives.

Antonio Grimaldi, Ana Olga Mocumbi, Juergen Freers, Matthias Lachaud, Mariana Mirabel, Beatriz Ferreira, Kumar Narayanan, David S Celermajer, Daniel Sidi, Xavier Jouven, Eloi Marijon

Journal: Circulation 2017;133(24):2503-15

PMID: 27297343

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Abstract

Tropical endomyocardial fibrosis (EMF) is a neglected disease of poverty that afflicts rural populations in tropical low-income countries, with some certain high-prevalence areas. Tropical EMF is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology. Since the first descriptions in Uganda in 1948, high-frequency areas for EMF have included Africa, Asia, and South America. Although there is no clear consensus on a unified hypothesis, it seems likely that dietary, environmental, and infectious factors may combine in a susceptible individual to give rise to an inflammatory process leading to endomyocardial damage and scar formation. The natural history of EMF includes an active phase with recurrent flare-ups of inflammation evolving to a chronic phase leading to restrictive heart failure. In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF. EMF carries a very poor prognosis. In addition to medical management of heart failure, early open heart surgery (endocardectomy and valve repair/replacement) appears to improve outcomes to some extent; however, surgery is technically challenging and not available in most endemic areas. Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies.

Address: From Cardiovascular and Thoracic Department, San Raffaele Hospital, Milan, Italy (A.G.); St. Raphael of St. Francis Nsambya Hospital, Kampala, Uganda (A.G.); Paris Cardiovascular Research Center (INSERM U970), Cardiology & Development, Paris, France (M.M., K.N., X.J., E.M.); Instituto Nacional de Saúde, Maputo, Mozambique (A.O.M.); Division of Cardiology, Department of Medicine, Makerere University, Kampala, Uganda (J.F.); Institut du Thorax, Nantes, France (M.L.); Maputo Heart Institute, Maputo, Mozambique (M.L., B.F., D.S., X.J., E.M.); European Georges Pompidou Hospital, Cardiology Department, Paris, France (M.M., X.J., E.M.); Necker Enfants Malades Hospital, Pediatric Cardiology, Paris, France (D.S.); Maxcure Hospitals, Hyderabad, India (K.N.); Sydney Medical School, Sydney, Australia (D.S.C.); and Paris Descartes University, Paris, France (D.S., X.J., E.M.).; From Cardiovascular and Thoracic Department, San Raffaele Hospital, Milan, Italy (A.G.); St. Raphael of St. Francis Nsambya Hospital, Kampala, Uganda (A.G.); Paris Cardiovascular Research Center (INSERM U970), Cardiology & Development, Paris, France (M.M., K.N., X.J., E.M.); Instituto Nacional de Saúde, Maputo, Mozambique (A.O.M.); Division of Cardiology, Department of Medicine, Makerere University, Kampala, Uganda (J.F.); Institut du Thorax, Nantes, France (M.L.); Maputo Heart Institute, Maputo, Mozambique (M.L., B.F., D.S., X.J., E.M.); European Georges Pompidou Hospital, Cardiology Department, Paris, France (M.M., X.J., E.M.); Necker Enfants Malades Hospital, Pediatric Cardiology, Paris, France (D.S.); Maxcure Hospitals, Hyderabad, India (K.N.); Sydney Medical School, Sydney, Australia (D.S.C.); and Paris Descartes University, Paris, France (D.S., X.J., E.M.). [email protected].

DOI: 10.1161/CIRCULATIONAHA.115.021178

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MeSH Terms: Endomyocardial Fibrosis, Humans, Prevalence, Prognosis