- Rebecca Cogswell, MD
Rebecca Cogswell, MD
- Assistant Professor of Medicine
- University of Minnesota
- Nelson B Schiller, MD
Nelson B Schiller, MD
- Section Editor — Noninvasive Cardiac Imaging and Stress Testing
- Professor of Medicine and Anesthesia
- University of California, San Francisco
- Harry Acquatella, MD, FACC, FAHA
Harry Acquatella, MD, FACC, FAHA
- Professor of Medicine
- Universidad Central de Venezuela
Endomyocardial fibrosis (EMF) is a disease that is characterized by fibrosis of the apical endocardium of the right ventricle (RV), left ventricle (LV), or both. The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure.
EMF refers to a specific syndrome with characteristic epidemiologic features. The epidemiology, pathophysiology, clinical manifestations, diagnosis, and treatment of EMF are reviewed here. Other cardiomyopathy syndromes with similar pathologies, including hypereosinophilia and/or fibrotic changes of the endocardium are addressed separately. (See "Carcinoid heart disease" and "Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis", section on 'Cardiac disease' and "Treatment and prognosis of myocarditis in adults", section on 'Eosinophilic myocarditis'.)
Endomyocardial fibrosis (EMF) was first recognized in Uganda during the 1940s and accounts for as much as 20 percent of cardiac cases sent for echocardiography in that country in contemporary series [1,2]. Although accurate epidemiologic data are lacking , EMF is estimated to the most common form of restrictive cardiomyopathy worldwide.
Most studies of EMF occur in tropical regions where there is a high prevalence of disease such as Uganda , Nigeria , Ivory Coast, south India , and Brazil . EMF also occurs in subtropical regions. An echocardiographic screening study in Mozambique found a population prevalence of 20 percent; however, this study included patients with early, subclinical disease . Within endemic countries, there appears to be a regional variation [8,9].
EMF is primarily a disease of the young, occurring in children, adolescents, and young adults. In Uganda, a bimodal peak at ages 10 and 30 has been observed , and a similar pattern was found in Mozambique . The differences between genders in the frequency of disease have been variable [7,10,11].
- Sliwa K, Damasceno A, Mayosi BM. Epidemiology and etiology of cardiomyopathy in Africa. Circulation 2005; 112:3577.
- BALL JD, WILLIAMS AW, DAVIES JN. Endomyocardial fibrosis. Lancet 1954; 266:1049.
- Connor DH, Somers K, Hutt MS, et al. Endomyocardial fibrosis in Uganda (Davies' disease). 1. An epidemiologic, clinical, and pathologic study. Am Heart J 1967; 74:687.
- Andy JJ, Ogunowo PO, Akpan NA, et al. Helminth associated hypereosinophilia and tropical endomyocardial fibrosis (EMF) in Nigeria. Acta Trop 1998; 69:127.
- Valiathan MS, Balakrishnan KG, Sankarkumar R, Kartha CC. Surgical treatment of endomyocardial fibrosis. Ann Thorac Surg 1987; 43:68.
- Moraes F, Lapa C, Hazin S, et al. Surgery for endomyocardial fibrosis revisited. Eur J Cardiothorac Surg 1999; 15:309.
- Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH. A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med 2008; 359:43.
- Kutty VR, Abraham S, Kartha CC. Geographical distribution of endomyocardial fibrosis in south Kerala. Int J Epidemiol 1996; 25:1202.
- Radhakumary C, Kumari TV, Kartha CC. Endomyocardial fibrosis is associated with selective deposition of type I collagen. Indian Heart J 2001; 53:486.
- Rutakingirwa M, Ziegler JL, Newton R, Freers J. Poverty and eosinophilia are risk factors for endomyocardial fibrosis (EMF) in Uganda. Trop Med Int Health 1999; 4:229.
- Falase AO. Endomyocardial fibrosis in Africa. Postgrad Med J 1983; 59:170.
- Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994; 83:2759.
- Spry CJF. A Comprehensive Review and Guide to the Scientific and Medical Literature, Oxford Medical Publications, Oxford 1988.
- Kendell KR, Day JD, Hruban RH, et al. Intimate association of eosinophils to collagen bundles in eosinophilic myocarditis and ranitidine-induced hypersensitivity myocarditis. Arch Pathol Lab Med 1995; 119:1154.
- Monsuez JJ, de Kerviler E, Barboteu M, et al. Non-Hodgkin's lymphoma related eosinophilic endomyocardial disease. Eur Heart J 1994; 15:1423.
- Donhuijsen K, Haedicke C, Hattenberger S, et al. Granulocyte-macrophage colony-stimulating factor-related eosinophilia and Loeffler's endocarditis. Blood 1992; 79:2798.
- Freers J, Masembe V, Schmauz R, Mayanja-Kizza H. Endomyocardial fibrosis syndrome in Uganda. Lancet 2000; 355:1994.
- Valiathan SM, Kartha CC. Endomyocardial fibrosis--the possible connexion with myocardial levels of magnesium and cerium. Int J Cardiol 1990; 28:1.
- Patel AK, Ziegler JL, D'Arbela PG, Somers K. Familial cases of endomyocardial fibrosis in Uganda. Br Med J 1971; 4:331.
- Ijaola O, Falase AO. Distribution of antibodies against Coxsackie B viruses, arboviruses and Toxoplasma gondii among patients with endomyocardial fibrosis (EMF) compared with normal subjects from EMF endemic and non-endemic zones of Nigeria. Afr J Med Med Sci 1990; 19:93.
- Shaper AG. Endomyocardial fibrosis and rheumatic heart-disease. Lancet 1966; 1:639.
- Eling WM, Jerusalem CR, Heinen-Borries UJ, et al. Is malaria involved in the pathogenesis of tropical endomyocardial fibrosis? Acta Leiden 1988; 57:47.
- Ive FA, Willis AJ, Ikeme AC, Brockington IF. Endomyocardial fibrosis and filariasis. Q J Med 1967; 36:495.
- Eling WM, Jerusalem CR, Heinen-Borries U. Role of macrophages in the pathogenesis of endomyocardial fibrosis in murine malaria. Trans R Soc Trop Med Hyg 1984; 78:43.
- Mocumbi AO, Songane M, Salomão C, et al. Lack of evidence of myocardial damage in children with Plasmodium falciparum severe and complicated malaria from an endemic area for endomyocardial fibrosis. J Trop Pediatr 2011; 57:312.
- Eapen JT, Kartha CC, Rathinam K, Valiathan MS. Levels of cerium in the tissues of rats fed a magnesium-restricted and cerium-adulterated diet. Bull Environ Contam Toxicol 1996; 56:178.
- Sivasankaran S. Restrictive cardiomyopathy in India: the story of a vanishing mystery. Heart 2009; 95:9.
- Brown CJ, Chenery SR, Smith B, et al. Environmental influences on the trace element content of teeth--implications for disease and nutritional status. Arch Oral Biol 2004; 49:705.
- Mocumbi AO, Latif N, Yacoub MH. Presence of circulating anti-myosin antibodies in endomyocardial fibrosis. PLoS Negl Trop Dis 2010; 4:e661.
- Lowenthal MN. Endomyocardial fibrosis: familial and other cases from northern Zambia. Med J Zambia 1978; 12:2.
- Connor DH, Somers K, Hutt MS, et al. Endomyocardial fibrosis in Uganda (Davies' disease). II. An epidemiologic, clinical, and pathologic study. Am Heart J 1968; 75:107.
- Mocumbi AO, Yacoub MH, Yokohama H, Ferreira MB. Right ventricular endomyocardial fibrosis. Cardiovasc Pathol 2009; 18:64.
- Chopra P, Narula J, Talwar KK, et al. Histomorphologic characteristics of endomyocardial fibrosis: an endomyocardial biopsy study. Hum Pathol 1990; 21:613.
- Freers J, Hakim J, Myanja-Kizza H, Parry E. The Heart. In: Principles of Medicine in Africa, Parry E, Godfrey R, Mabey D, Gill G (Eds), Cambridge University Press, Cambridge 2004. p.837.
- Freers J, Mayanja-Kizza H, Rutakingirwa M, Gerwing E. Endomyocardial fibrosis: why is there striking ascites with little or no peripheral oedema? Lancet 1996; 347:197.
- Barretto AC, Mady C, Oliveira SA, et al. Clinical meaning of ascites in patients with endomyocardial fibrosis. Arq Bras Cardiol 2002; 78:196.
- Marijon E, Hausse AO, Ferreira B. Typical clinical aspect of endomyocardial fibrosis. Int J Cardiol 2006; 112:259.
- Guimarães AC, Esteves JP, Filho AS, Macedo V. Clinical aspects of endomyocardial fibrosis in Bahia, Brazil. Am Heart J 1971; 81:7.
- Barretto AC, Mady C, Nussbacher A, et al. Atrial fibrillation in endomyocardial fibrosis is a marker of worse prognosis. Int J Cardiol 1998; 67:19.
- Berensztein CS, Piñeiro D, Marcotegui M, et al. Usefulness of echocardiography and doppler echocardiography in endomyocardial fibrosis. J Am Soc Echocardiogr 2000; 13:385.
- Hassan WM, Fawzy ME, Al Helaly S, et al. Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest 2005; 128:3985.
- Acquatella H, Schiller NB, Puigbó JJ, et al. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation 1983; 67:1219.
- Barretto AC, da Luz PL, de Oliveira SA, et al. Determinants of survival in endomyocardial fibrosis. Circulation 1989; 80:I177.
- Qureshi N, Amin F, Chatterjee D, et al. MR imaging of endomyocardial fibrosis (EMF). Int J Cardiol 2011; 149:e36.
- Smedema JP, Winckels SK, Snoep G, et al. Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging. Int J Cardiovasc Imaging 2004; 20:517.
- Salemi VM, Rochitte CE, Shiozaki AA, et al. Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging 2011; 4:304.
- Wynne J, Braunwald E.. The Cardiomyopathies. In: Braunwald's Heart Disease, Zipes DP, Libby P, Bonow RO, Braunwald E (Eds), Elsevier Saunders, Philadeplhia 2005. p.1659.
- Schneider U, Jenni R, Turina J, et al. Long-term follow up of patients with endomyocardial fibrosis: effects of surgery. Heart 1998; 79:362.
- Mocumbi AO, Yacoub S, Yacoub MH. Neglected tropical cardiomyopathies: II. Endomyocardial fibrosis: myocardial disease. Heart 2008; 94:384.