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Endomyocardial fibrosis: past, present, and future

  • Andre Rodrigues DuraesEmail author
  • Yasmin de Souza Lima Bitar
  • Leonardo Roever
  • Mansueto Gomes Neto
Article
  • 36 Downloads

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.

Keywords

Endomyocardial fibrosis Heart failure Restrictive cardiomyopathy 

Notes

References

  1. 1.
    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 CrossRefGoogle Scholar
  2. 2.
    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 CrossRefGoogle Scholar
  3. 3.
    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 Google Scholar
  4. 4.
    Davies JNP (1948) Endomyocardial fibrosis in Uganda. East Afr Med J 25:10Google Scholar
  5. 5.
    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 CrossRefGoogle Scholar
  6. 6.
    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 CrossRefGoogle Scholar
  7. 7.
    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 CrossRefGoogle Scholar
  8. 8.
    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 CrossRefGoogle Scholar
  9. 9.
    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 CrossRefGoogle Scholar
  10. 10.
    Mathai A, Kartha CC, Balakrishnan KG (1986) Serum immunoglobulins in patients with endomyocardial fibrosis. Indian Heart J 38(6):470–472Google Scholar
  11. 11.
    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 CrossRefGoogle Scholar
  12. 12.
    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):262–265Google Scholar
  13. 13.
    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 Google Scholar
  14. 14.
    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):229–235CrossRefGoogle Scholar
  15. 15.
    Vijayaraghavan G, Sivasankaran S (2012) Tropical endomyocardial fibrosis in India: a vanishing disease! Indian J Med Res 136(5):729–738Google Scholar
  16. 16.
    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 CrossRefGoogle Scholar
  17. 17.
    Radhakumary C, Kumari TV, Kartha CC (2001) Endomyocardial fibrosis is associated with selective deposition of type I collagen. Indian Heart J 53(4):486–489Google Scholar
  18. 18.
    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 CrossRefGoogle Scholar
  19. 19.
    Bhatti K, Lopez-Mattei J (2019) Endomyocardial fibrosis. In: StatPearls. Treasure Island (FL),Google Scholar
  20. 20.
    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 CrossRefGoogle Scholar
  21. 21.
    Ramos Filho J, Souza CAF, Magrini E, Macedo M, Isolato RB (2005) Endomiocardiofibrose biventricular associada à amiloidose renal. Arq Bras Cardiol 85:275–278CrossRefGoogle Scholar
  22. 22.
    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 CrossRefGoogle Scholar
  23. 23.
    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 CrossRefGoogle Scholar
  24. 24.
    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–I182Google Scholar
  25. 25.
    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 CrossRefGoogle Scholar
  26. 26.
    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):269–272Google Scholar
  27. 27.
    Tharakan JA (2011) Electrocardiogram in endomyocardial fibrosis. Indian Pacing Electrophysiol J 11(5):129–133Google Scholar
  28. 28.
    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):19–25CrossRefGoogle Scholar
  29. 29.
    Basile U, Carlisle R, Falase AO (1974) E.C.G. in endomyocardial fibrosis. East Afr Med J 51(6):444–448Google Scholar
  30. 30.
    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 CrossRefGoogle Scholar
  31. 31.
    Brindeiro Filho D, Cavalcanti C (1996) Value of Doppler echocardiography in the diagnosis and management of endomyocardial fibrosis. Arq Bras Cardiol 67(4):279–284Google Scholar
  32. 32.
    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):251–255Google Scholar
  33. 33.
    Tarun K, Naresh K, Khullar RK, Daga MK (2009) Endomyocardial fibrosis. Southeast Asian J Trop Med Public Health 40(2):327–329Google Scholar
  34. 34.
    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 CrossRefGoogle Scholar
  35. 35.
    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 CrossRefGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Medical School, FAMEBFederal University of Bahia, UFBASalvadorBrazil
  2. 2.Federal University of UberlândiaUberlândiaBrazil
  3. 3.Federal University of Bahia, UFBASalvadorBrazil

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