Abstract
Endomyocardial fibrosis (EMF) is a neglected idiopathic disorder, predominant in tropical and subtropical regions of the developing world. It is characterized by fibrotic thickening of the endocardium and myocardium of one or both ventricles. EMF was an important cause of heart failure which accounted for up to 20% of the cases in endemic areas of Africa (rural community in Mozambique), but during the last few years, incidents of the disease have decreased considerably. Although its pathogenesis and etiology are not fully understood, its pathology resembles conditions such as eosinophilic cardiomyopathy and hypereosinophilic syndrome. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling, and valvular insufficiency defines the disease. Confined to peculiar and limited geographical areas, the etiology remains blurred and it carries a grim prognosis. Medical care currently remains very challenging as one-third to half of patients with an advanced disease die within 2 years. Surgery in the correct setting can increase survival and especially in patients with advanced heart failure.
Similar content being viewed by others
References
Mocumbi AO, Yacoub S, Yacoub MH (2008) Neglected tropical cardiomyopathies: II. Endomyocardial fibrosis: myocardial disease. Heart 94(3):384–390. https://doi.org/10.1136/hrt.2007.136101
Knorr D, Scheppe KJ (1958) Endocarditis parietalis fibroplastica mit Bluteosinophilie (Löffler) bei einem siebenjährigen Kind (Erstbeschreibung im Kindesalter). Zeitschrift für Kinderheilkunde 81(1):102–112. https://doi.org/10.1007/bf00438369
Bedford DE, Konstam GL (1946) Heart failure of unknown aetiology in Africans. Br Heart J 8(4):236. https://doi.org/10.1136/hrt.8.2.87
Davies JNP (1948) Endomyocardial fibrosis in Uganda. East Afr Med J 25:10
Kumar P, Chandrashekhara SH, Kumar S, Malhi AS (2019) Loeffler’s endocarditis with isolated left ventricular involvement on cardiac MRI. BMJ Case Rep 12(4):e227642. https://doi.org/10.1136/bcr-2018-227642
Lachaud M, Lachaud C, Sidi D, Menete A, Jouven X, Marijon E, Ferreira B (2018) Tropical endomyocardial fibrosis: perspectives. Ann Cardiol Angeiol (Paris) 67(2):74–81. https://doi.org/10.1016/j.ancard.2018.01.001
Grimaldi A, Mocumbi AO, Freers J, Lachaud M, Mirabel M, Ferreira B, Narayanan K, Celermajer DS, Sidi D, Jouven X, Marijon E (2016) Tropical endomyocardial fibrosis: natural history, challenges, and perspectives. Circulation 133(24):2503–2515. https://doi.org/10.1161/CIRCULATIONAHA.115.021178
Bukhman G, Ziegler J, Parry E (2008) Endomyocardial fibrosis: still a mystery after 60 years. PLoS Negl Trop Dis 2(2):e97. https://doi.org/10.1371/journal.pntd.0000097
Mocumbi AO (2012) Endomyocardial fibrosis: a form of endemic restrictive cardiomyopathy. Glob Cardiol Sci Pract 2012(1):11. https://doi.org/10.5339/gcsp.2012.11
Mathai A, Kartha CC, Balakrishnan KG (1986) Serum immunoglobulins in patients with endomyocardial fibrosis. Indian Heart J 38(6):470–472
Gupta PN, Kunju SM, Rajan B, Koshy AG, Vishwanathan S, George PS, Velappan P (2018) Geographical variation in the clinical presentation of endomyocardial fibrosis in India? Indian Heart J 70(1):56–65. https://doi.org/10.1016/j.ihj.2016.12.015
Hakim JG, Matenga JA, Ternouth I (1996) Endomyocardial fibrosis in Zimbabwe--how rare is it? A report of two cases. Cent Afr J Med 42(9):262–265
Grimaldi A, Alfieri O, Camici PG, La Canna G, Zoppei G, Olivotto I (2011) “African sickness” and the heart: the mystery of endomyocardial fibrosis. G Ital Cardiol (Rome) 12(7–8):484–491. https://doi.org/10.1714/915.10074
Rutakingirwa M, Ziegler JL, Newton R, Freers J (1999) Poverty and eosinophilia are risk factors for endomyocardial fibrosis (EMF) in Uganda. Tropical Med Int Health 4(3):229–235
Vijayaraghavan G, Sivasankaran S (2012) Tropical endomyocardial fibrosis in India: a vanishing disease! Indian J Med Res 136(5):729–738
Iglezias SD, Benvenuti LA, Calabrese F, Salemi VM, Silva AM, Carturan E, de Oliveira SA, Thiene G, De Brito T (2008) Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium. Virchows Arch 453(3):233–241. https://doi.org/10.1007/s00428-008-0652-3
Radhakumary C, Kumari TV, Kartha CC (2001) Endomyocardial fibrosis is associated with selective deposition of type I collagen. Indian Heart J 53(4):486–489
Sharma A, Pandey NN, Malhi AS, Kumar S (2019) Complex right atrial mass in endomyocardial fibrosis: a diagnostic dilemma. BMJ Case Rep 12(1):e227131. https://doi.org/10.1136/bcr-2018-227131
Bhatti K, Lopez-Mattei J (2019) Endomyocardial fibrosis. In: StatPearls. Treasure Island (FL),
Rashwan MA, Ayman M, Ashour S, Hassanin MM, Zeina AA (1995) Endomyocardial fibrosis in Egypt: an illustrated review. Br Heart J 73(3):284–289. https://doi.org/10.1136/hrt.73.3.284
Ramos Filho J, Souza CAF, Magrini E, Macedo M, Isolato RB (2005) Endomiocardiofibrose biventricular associada à amiloidose renal. Arq Bras Cardiol 85:275–278
Barretto AC, Mady C, Oliveira SA, Arteaga E, Dal Bo C, Ramires JA (2002) Clinical meaning of ascites in patients with endomyocardial fibrosis. Arq Bras Cardiol 78(2):196–199. https://doi.org/10.1590/s0066-782x2002000200008
Freers J, Masembe V, Schmauz R, Mayanja-Kizza H (2000) Endomyocardial fibrosis syndrome in Uganda. Lancet 355(9219):1994–1995. https://doi.org/10.1016/S0140-6736(05)72932-9
Barretto AC, da Luz PL, de Oliveira SA, Stolf NA, Mady C, Bellotti G, Jatene AD, Pileggi F (1989) Determinants of survival in endomyocardial fibrosis. Circulation 80(3 Pt 1):I177–I182
Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH (2008) A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med 359(1):43–49. https://doi.org/10.1056/NEJMoa0708629
Fernandes F, Mady C, Vianna Cde B, Barretto AC, Arteaga E, Ianni BM, Fujioka T, Ballas D, Bellotti G, Pileggi F (1997) Radiological findings in endomyocardial fibrosis. Arq Bras Cardiol 68(4):269–272
Tharakan JA (2011) Electrocardiogram in endomyocardial fibrosis. Indian Pacing Electrophysiol J 11(5):129–133
Barretto AC, Mady C, Nussbacher A, Ianni BM, Oliveira SA, Jatene A, Ramires JA (1998) Atrial fibrillation in endomyocardial fibrosis is a marker of worse prognosis. Int J Cardiol 67(1):19–25
Basile U, Carlisle R, Falase AO (1974) E.C.G. in endomyocardial fibrosis. East Afr Med J 51(6):444–448
Hassan WM, Fawzy ME, Al Helaly S, Hegazy H, Malik S (2005) Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest 128(6):3985–3992. https://doi.org/10.1378/chest.128.6.3985
Brindeiro Filho D, Cavalcanti C (1996) Value of Doppler echocardiography in the diagnosis and management of endomyocardial fibrosis. Arq Bras Cardiol 67(4):279–284
Tello R, Cuan V, Abundes A, Navarro J, Garcia Lara J, Astudillo R, Ariza H, Cuan M (1994) Doppler echocardiography in endomyocardial fibrosis. Arch Inst Cardiol Mex 64(3):251–255
Tarun K, Naresh K, Khullar RK, Daga MK (2009) Endomyocardial fibrosis. Southeast Asian J Trop Med Public Health 40(2):327–329
Kharabish A, Haroun D (2015) Cardiac MRI findings of endomyocardial fibrosis (Loeffler’s endocarditis) in a patient with rheumatoid arthritis. J Saudi Heart Assoc 27(2):127–131. https://doi.org/10.1016/j.jsha.2014.11.002
Moraes F, Lapa C, Hazin S, Tenorio E, Gomes C, Moraes CR (1999) Surgery for endomyocardial fibrosis revisited. Eur J Cardiothorac Surg 15(3):309–312; discussion 312-303. https://doi.org/10.1016/s1010-7940(99)00027-5
Author information
Authors and Affiliations
Corresponding author
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Duraes, A.R., de Souza Lima Bitar, Y., Roever, L. et al. Endomyocardial fibrosis: past, present, and future. Heart Fail Rev 25, 725–730 (2020). https://doi.org/10.1007/s10741-019-09848-4
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10741-019-09848-4