eMedicine Specialties > Cardiology > Myocardial Disease and Cardiomyopathies

Endomyocardial Fibrosis

Ali A Sovari, MD, Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology, University of Illinois at Chicago
Abraham G Kocheril, MD, FACC, FACP, Professor of Medicine, Director of Clinical Electrophysiology, University of Illinois at Chicago

Updated: Jan 21, 2010

Introduction

Background

Endomyocardial fibrosis (EMF) is an idiopathic disorder of the tropical and subtropical regions of the world that is characterized by the development of restrictive cardiomyopathy.

The nosology of EMF coincides with some related disorders. EMF is sometimes considered part of a spectrum of a single disease process that includes Löeffler endocarditis (nontropical eosinophilic endomyocardial fibrosis or fibroplastic parietal endocarditis with eosinophilia).

Tropical EMF and Löeffler endocarditis should be distinguished from endocardial fibroelastosis, which is characterized by cartilaginous thickening of the mural endocardium, chiefly of the left ventricle. This disease is most common in the first 2 years of life and, in some patients, appears to be an inherited disorder that is associated with congenital cardiac malformations.

Pathophysiology

In EMF, the underlying process produces patchy fibrosis of the endocardial surface of the heart, leading to reduced compliance and, ultimately, restrictive physiology as the endomyocardial surface becomes more generally involved. Endocardial fibrosis principally involves the apices of the right and left ventricles and may affect the atrioventricular valves mainly by tethering the papillary muscles, leading to tricuspid and mitral regurgitation.

The earliest changes of EMF are not well described because most patients do not present with symptoms until relatively late in the clinical course. Olsen described 3 phases of EMF. The first phase involves eosinophilic infiltration of the myocardium with necrosis of the subendocardium and a pathologic picture consistent with acute myocarditis. This is reportedly present in the first 5 weeks of the illness. The second stage, typically observed after 10 months, is associated with thrombus formation over the initial lesions, with a decrement in the amount of inflammatory activity present. Ultimately, after several years of disease activity, the fibrotic phase is reached, when the endocardium is replaced by collagenous fibrosis. This pathomorphologic schema is not observed uniformly and has not been consistently supported by other investigators.

Myocardial fibrosis consists of collagen deposition and fibroblast proliferation. These changes can potentially explain most of the symptoms in patients with EMF. Fibrosis increases the stiffness of the heart, resulting in the restrictive physiology. Ventricular stiffness along with atrioventricular valvular regurgitation results in atrial enlargement, which has been linked to atrial arrhythmias such as atrial fibrillation. Fibrosis also reduces conduction velocity, impairs activation pattern and may provide the substrate for wave breaks and reentry.^{[1 ]}Recently, fibrosis has been suggested to facilitate focal activity by fibroblast-myocyte coupling as well.^{[2 ]}Atrial fibrillation has been reported in more than 30% of patients with EMF followed by other rhythm or conduction abnormalities like junctional rhythm, heart blocks, and intraventricular conduction delay.^{[3 ]}

While in general, fibrosis in cardiac tissue has been mainly linked to increased level of a cytokine, transforming growth factor-β1^{[4 ]}; the underlying mechanisms of myocardial fibrosis in this specific entity remain unclear. Hypotheses include infectious, inflammatory, and nutritional processes. EMF is frequently associated with concomitant parasitic infections (eg, helminths) and their attendant eosinophilia, although the role of parasitic infections and/or the eosinophil remains speculative. The development of EMF as a sequela to toxoplasma-related myocarditis has also been described, as has a relationship of malarial infection to development of EMF. However, no specific organism has been consistently associated with EMF.

EMF is most frequently observed in the socially disadvantaged and in children and young women. These groups frequently have malnutrition, and in regions of sub-Saharan Africa where the disease is most prevalent, the typical diet is high in a tuber called cassava, which contains relatively high concentrations of the rare earth element cerium (Ce). The combination of high Ce levels and hypomagnesemia has been shown to produce EMF-like lesions in laboratory animals.

A familial tendency has rarely been noted in Uganda and Zambia.

Frequency

United States

EMF is rarely encountered in patients who have not traveled from the subtropical regions of Africa and tropical and subtropical regions elsewhere in the world, including areas in India and South America that are within 15° of the equator. Löeffler endocarditis (also called nontropical eosinophilic endocarditis) is a related condition that is observed in the United States and is considered by some authors to be a different stage of a similar process related to eosinophilia.

International

EMF occurs primarily in the subtropical regions of Africa but is also encountered in tropical and subtropical regions elsewhere in the world, including areas in India and South America that are within 15° of the equator.

More than 90% of reported cases of EMF have occurred in geographic locations that are within 15° of the equator. In equatorial African nations, such as Nigeria, EMF is the fourth most common cause of cardiac disease in adults, and EMF accounts for 22% of cases of heart failure in Nigerian children. EMF is the most common type of restrictive cardiomyopathy in tropical countries and worldwide.

In a screening study in a rural area in Mozambique, approximately 20% of a random sample of 1063 subjects of all age groups had echocardiographic evidence of this disease.^{[6 ]}In the same study, the prevalence was highest among persons aged 10-19 years, and the most common form was biventricular endomyocardial fibrosis followed by right-side dominant and then left-side dominant disease. Most other studies also reported higher prevalence of biventricular pattern while in some studies left ventricular dominant pattern has been more common than the right ventricular dominant pattern.^{[7 ]}

Mortality/Morbidity

• The overall prognosis of patients with EMF is poor and depends on the extent and distribution of disease within the various chambers and valves of the heart. The disease is usually progressive but the time course of decline varies.
• Most patients have extensive disease at the time of presentation; therefore, survival after diagnosis is relatively brief. In one study, 95% of a group of patients had died at 2 years. In a second study, 44% of patients died within 1 year after the onset of symptoms, and another 40% of patients died 1-3 years after onset. Death usually occurs as a result of progressive heart failure or associated arrhythmia and sudden cardiac death.

Race

EMF is most commonly reported in individuals living in Nigeria and Uganda. Among residents of these countries, EMF appears to be more prevalent in certain ethnic groups. One study in Uganda showed that EMF is more common in individuals with Rwanda/Burundi ethnic origins.^{[8 ]}

Sex

In general, women of reproductive age and children are more commonly affected than men. However, a recent screening study in a rural area of Mozambique reported a higher rate among male than female subjects (23.0% vs 17.5%, P =0.03).^{[6 ]}This study was based on a screening echocardiography and many of these patients were not symptomatic.  

Age

• EMF is not generally observed in children younger than 4 years, although the typical pathology for EMF has recently been described in a 4-month-old infant with left ventricular inflow tract obstruction.
• The people most commonly affected are usually older children (aged 5-15 y) and young adults, but cases have been reported in individuals aged 70 years.

Clinical

History

Typically, endomyocardial fibrosis (EMF) has an insidious onset, and symptoms relate to the specific chambers and valves where the disease is most extensive.

• When right ventricular involvement or tricuspid regurgitation predominates, lower extremity swelling, increasing abdominal girth, and nausea may be expected.
• With left ventricular involvement, dyspnea is the predominant symptom, especially exertional dyspnea. Additionally, fatigue, paroxysmal nocturnal dyspnea, and orthopnea may be present.
• Thromboembolic complications may occur in EMF.
• Rarely, patients may present early in the course of the disease with an acute febrile illness with symptoms of cardiac insufficiency mimicking myocarditis.
• Angina-like chest pain was reported in a patient with EMF involving the left ventricle. Patients with EMF may also present with arrhythmia symptoms such as syncope, near syncope, and palpitations.

Physical

Physical findings are also dependent on the extent and distribution of disease.

• In those with right ventricular involvement, jugular venous pressure elevation, ascites, and edema may be present.
• The presence of ascites may appear out of proportion to the amount of peripheral edema. This may occur because of the concomitant presence of a protein-losing enteropathy and subsequent hypoalbuminemia. In 1 retrospective study of patients with symptomatic EMF, ascites was observed in approximately half of the cases and was associated with greater involvement of the right ventricle and with a longer duration of the disease, thus being a characteristic of a worse prognosis.^{[9 ]}
• Patients with tricuspid regurgitation may have giant V waves observed in the jugular venous pulsations.
• A third or fourth heart sound and tachycardia may be present.
• Signs of pulmonary congestion are present in patients with left-sided disease.

Causes

A specific single etiology of EMF has not been established. Suggested potential causes include the following:

• Infectious causes
  • Parasites (eg, helminths)
  • Protozoans (eg, toxoplasmosis, malaria)
• Inflammatory causes - Eosinophilia
• Nutritional causes
  • General malnutrition
  • High-tuber diet
  • Ce toxicity
  • Hypomagnesemia

Differential Diagnoses

C-11 Hydroxylase Deficiency

Other Problems to Be Considered

Anthracycline toxicity
Carcinoid heart disease
Drug-induced cardiotoxicity (eg, serotonin, methysergide, ergotamine, mercurial agents, busulfan)
Fabry disease
Fatty infiltration
Gaucher disease
Glycogen storage disease
Hurler disease
Idiopathic cardiomyopathy
Metastatic cancers
Radiation
Rheumatic heart disease
Occasionally, a masslike lesion seen in endomyocardial fibrosis masquerades as an intracardiac tumor.

Workup

Laboratory Studies

Complete blood cell count may show anemia and eosinophilia.

Imaging Studies

• Chest radiography 
  • The cardiac silhouette in endomyocardial fibrosis (EMF) may be normal in size, and generalized cardiomegaly is unusual because the ventricles are not typically dilated.
  • The roentgenographic image may exhibit significant enlargement of the atria, and significant right atrial enlargement creates a cardiac silhouette in the shape of the African continent, which is a specific heart shadow sign that has been termed the heart of Africa.
• Echocardiography 
  • Echocardiography is a useful tool and the diagnostic modality of choice when making the diagnosis of EMF and has been demonstrated to successfully differentiate EMF and other processes such as rheumatic heart disease and congenital heart disease.
  • The presence and location of fibrosis as determined by echocardiography correlates well with autopsy findings.
  • Findings include thickening of the inferior and basal left ventricular wall, apical obliteration, and thrombi adherent to the endocardial surface
  • A pericardial effusion is frequently present and may be large.
  • While parameters of diastolic function by Doppler echocardiography tend to correlate with the functional status of the patient, because most patients present with later stages of EMF, a restrictive filling pattern in the left ventricle is most common.
  • Decreased flow propagation velocity (Vp) has been demonstrated in a large percentage of patients with EMF.
  • Color-flow imaging frequently exhibits tricuspid and mitral regurgitation, believed to be due to retraction or adherence of the atrioventricular valvular apparatus. Spectral Doppler analysis of tricuspid regurgitation frequently reflects an increased pulmonary artery systolic pressure.
• Angiography
  • Traditionally, angiography has been considered the criterion standard when making the diagnosis of EMF.
  • Left and right ventriculography exhibits distortion of chamber morphology by fibrosis and obliteration and variable degrees of mitral and tricuspid regurgitation.
  • The mushroom sign has been used to describe the shape of the affected ventricle when the apex is obliterated completely by fibrosis.
• Electron beam computed tomography scanning
  • Features of EMF observed with this modality were described in the mid 1990s.
  • The fibrotic process is delineated as a band of low attenuation within the endocardium.
  • Obliteration of the apex and inflow tract, when present, is also demonstrated.
  • This method reportedly assists in distinguishing EMF from constrictive pericarditis.
• Cardiovascular magnetic resonance imaging (MRI): Recently, the use of cardiovascular MRI has been shown to demonstrate obliterative changes in the ventricles, atrial dilation, and regurgitant atrioventricular valve lesions in patients with EMF. Recent studies have evaluated the role of contrast-enhanced MRI in detecting myocardial fibrosis, which can potentially be a useful diagnostic tool in patients with EMF.^{[10 ]}However, clinical use of MRI is limited by access to this technology in endemic areas.

Other Tests

Electrocardiography 

• Atrial fibrillation in approximately one third of these patients^{[3 ]}
• Low QRS voltage due to myocardial fibrosis
• Atrioventricular blocks
• Intraventricular conduction delay and right or left bundle branch blocks
• Evidence of left (and/or right) atrial enlargement

Procedures

• Cardiac catheterization likely exhibits hemodynamic findings consistent with restrictive cardiomyopathy.
• Findings from endomyocardial biopsy may be diagnostic, but this procedure is typically not needed. Biopsy findings may be nondiagnostic when the disease is patchy and sampling sites do not correlate with areas of disease. Because biopsy (especially from the left ventricle) carries some risk, reserve the use of this technique until other diagnostic approaches have been used.
• Diagnostic and therapeutic paracentesis, thoracentesis and pericardiocentesis may be indicated in patients with significantly large ascites, pleural effusion, and pericardial effusion, respectively, who do not respond to medical therapy.

Histologic Findings

The heart size is not usually enlarged in EMF. The ventricular cavities are frequently laden with thrombi and, in severe cases, may be nearly totally obliterated by endocardial thickening and thrombosis. The histologic findings of EMF are characterized by reactive fibrosis associated with a selective increase in type I collagen deposition, subendocardial infarction and fibrosis, and thrombus formation. Additionally, specific features of other diseases, such as those associated with hemochromatosis or glycogen storage disease, are notably absent.

Staging

Mocumbi and colleagues provided a set of echocardiographic criteria that is useful in staging the disease, studying its progression, and comparing the results of different epidemiologic studies.^{[6 ]}In this classification, there are 6 major criteria and 7 minor criteria. The diagnosis is considered when 2 major criteria or 1 major and 2 minor criteria are present. A score has been assigned to each criterion and the severity of the disease is measured by this score; a total score of less than 8 indicates mild endomyocardial fibrosis, a score of 8-15 indicates moderate disease, and a score of more than 15 indicates severe disease.

Major criteria

1. Endomyocardial plaques >2 mm in thickness; score: 2
2. Thin (≤1 mm) endomyocardial patches affecting more than one ventricular wall; score: 3
3. Obliteration of the right ventricular or left ventricular apex; score: 4
4. Thrombi or spontaneous contrast without severe ventricular dysfunction; score: 4
5. Retraction of the right ventricular apex (right ventricular apical notch); score: 4
6. Atrioventricular valve dysfunction due to adhesion of the valvular apparatus to the ventricular wall; score: 1–4 (depending on the severity of the regurgitation)

Minor criteria

1. Thin endomyocardial patches localized to 1 ventricular wall; score: 1
2. Restrictive flow pattern across mitral or tricuspid valves; score: 2
3. Pulmonary-valve diastolic opening; score: 2
4. Diffuse thickening of the anterior mitral leaflet; score: 1
5. Enlarged atrium with normal size ventricle; score: 2
6. M-movement of the interventricular septum and flat posterior wall; score: 1
7. Enhanced density of the moderator or other intraventricular bands; score: 1

Treatment

Medical Care

• In general, the response to medical therapy is unproven due to lack of well-designed studies.
• Because most patients with endomyocardial fibrosis (EMF) present long after any possible period of early active myocarditis may have existed, little, if any, role exists for immunosuppressive therapy as is used in some patients with Löeffler disease. If the patient presents with acute myocarditis, prednisone may be considered as medical therapy.
• Symptomatic therapy with diuretics has been shown to be useful. Similar to other patients with diastolic dysfunction, these patients may also benefit from ACE inhibitors, angiotensin receptor blockers, and beta-blockers, but only to some degree.
• For patients with severe symptoms, consider surgical therapy because the prognosis for these patients with continued medical therapy alone is dismal.
• There is no reliable evidence on the rate of the embolic events in these patients and whether anticoagulation and antiplatelet therapy are effective in their prevention. It is reasonable to anticoagulate patients who have thrombus on echocardiography and are compliant with medical treatments. Antiplatelet therapy with aspirin or clopidogrel can be considered as an alternative approach. Those patients with atrial fibrillation should be anticoagulated, although the ability to do this safely and effectively is again limited by availability of local services in endemic regions.

Surgical Care

• Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional-therapeutic class III or IV. The operative mortality rate is high (15-20%), but successful surgery has a clear benefit on symptoms and seems to favorably affect survival as well.
• The most commonly used approach is endocardiectomy, combined with mitral and/or tricuspid repair or replacement (when indicated), using a midline thoracotomy and cardiopulmonary bypass.
  • Depending on the location of the disease (right or left ventricle, apex or inflow tract), a transapical or transventricular approach can be used.
  • Because a well-defined plane of cleavage usually exists between the endocardium and myocardium, endocardiectomy is most frequently feasible.
  • Because the myocardium is not usually affected, the severe hemodynamic derangement associated with EMF is relieved with successful resection of the endocardium.
  • Common postoperative complications include low cardiac output, heart block, and ventricular arrhythmias.

Diet

No specific diet is recommended for this condition.

Activity

No activity restrictions are recommended because activity restrictions have not been proven to alter the prognosis of this condition, and no specific activity has been shown to hasten the onset of fatal arrhythmias or sudden death.

Follow-up

Transfer

• Transfer suitable patients with severe symptoms for consideration of endomyocardial resection. Although the operative mortality rate is high (approximately 20%), prognosis is favorably altered in those that survive the surgery.
• Consider heart transplant in patients who are classified in function class III or IV.

Deterrence/Prevention

No specific preventive measures have been proven effective.

Complications

Common postoperative complications include low cardiac output, heart block, and ventricular arrhythmias.

Prognosis

• Prognosis for this condition is poor. Incidence of sudden cardiac death from fatal arrhythmias or from progressive cardiac failure is high.
• Most patients have extensive disease at the time of presentation; therefore, survival after diagnosis is relatively brief. In one study, 95% of a group of patients had died at 2 years. In a second study, 44% of patients died within 1 year after the onset of symptoms, and another 40% of patients died 1-3 years after onset.

Patient Education

Inform patients about the poor prognosis and increased likelihood of fatal and nonfatal arrhythmias.

Miscellaneous

Medicolegal Pitfalls

As with other forms of restrictive cardiomyopathy, distinguish endomyocardial fibrosis (EMF) from constrictive pericarditis.

References

1. Rossi S, Baruffi S, Bertuzzi A, Miragoli M, Corradi D, Maestri R, et al. Ventricular activation is impaired in aged rat hearts. Am J Physiol Heart Circ Physiol. Dec 2008;295(6):H2336-47. [Medline].

2. Miragoli M, Salvarani N, Rohr S. Myofibroblasts induce ectopic activity in cardiac tissue. Circ Res. Oct 12 2007;101(8):755-8. [Medline].

3. Gupta PN, Valiathan MS, Balakrishnan KG, et al. Clinical course of endomyocardial fibrosis. Br Heart J. Dec 1989;62(6):450-4. [Medline].

4. Sovari AA, Morita N, Weiss JN, Karagueuzian HS. Serum transforming growth factor-beta1 as a risk stratifier of sudden cardiac death. Med Hypotheses. Aug 2008;71(2):262-5. [Medline].

5. Valiathan SM, Kartha CC. Endomyocardial fibrosis--the possible connexion with myocardial levels of magnesium and cerium. Int J Cardiol. Jul 1990;28(1):1-5. [Medline].

6. Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH. A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med. Jul 3 2008;359(1):43-9. [Medline].

7. Hassan WM, Fawzy ME, Al Helaly S, Hegazy H, Malik S. Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest. Dec 2005;128(6):3985-92. [Medline].

8. Rutakingirwa M, Ziegler JL, Newton R, Freers J. Poverty and eosinophilia are risk factors for endomyocardial fibrosis (EMF) in Uganda. Trop Med Int Health. Mar 1999;4(3):229-35. [Medline].

9. Barretto AC, Mady C, Oliveira SA, et al. Clinical meaning of ascites in patients with endomyocardial fibrosis. Arq Bras Cardiol. Feb 2002;78(2):196-9. [Medline].

10. Iles L, Pfluger H, Phrommintikul A, Cherayath J, Aksit P, Gupta SN, et al. Evaluation of diffuse myocardial fibrosis in heart failure with cardiac magnetic resonance contrast-enhanced T1 mapping. J Am Coll Cardiol. Nov 4 2008;52(19):1574-80. [Medline].

11. Brockington IF, Edington GM. Adult heart disease in western Nigeria: a clinicopathological synopsis. Am Heart J. Jan 1972;83(1):27-40. [Medline].

12. Chopra P, Narula J, Talwar KK, et al. Histomorphologic characteristics of endomyocardial fibrosis: an endomyocardial biopsy study. Hum Pathol. Jun 1990;21(6):613-6. [Medline].

13. Eling WM, Jerusalem CR, Heinen-Borries UJ, et al. Is malaria involved in the pathogenesis of tropical endomyocardial fibrosis?. Acta Leiden. 1988;57(1):47-52. [Medline].

14. Falase AO. Aetiological considerations in Nigeria. In: Valiathan MS, Somers K, Kartha CC, eds. Endomyocardial Fibrosis. Oxford, England:. Oxford Univ Press;1993:88, 94.

15. Freers J, Mayanja-Kizza H, Rutakingirwa M, Gerwing E. Endomyocardial fibrosis: why is there striking ascites with little or no peripheral oedema?. Lancet. Jan 20 1996;347(8995):197. [Medline].

16. Freers J, Mayanja-Kizza H, Ziegler JL, Rutakingirwa M. Echocardiographic diagnosis of heart disease in Uganda. Trop Doct. Jul 1996;26(3):125-8. [Medline].

17. Goo HW, Han NJ, Lim TH. Endomyocardial fibrosis mimicking right ventricular tumor. AJR Am J Roentgenol. Jul 2001;177(1):205-6. [Medline].

18. Jatene MB, Contreras IS, Lameda LC, et al. Endomyocardial fibrosis in infancy. Arq Bras Cardiol. Apr 2003;80(4):438-45. [Medline].

19. Lowenthal MN. Endomyocardial fibrosis: familial and other cases from northern Zambia. Med J Zambia. 1978 Feb-Mar;12(1):2-7. 1978;12:2-7.

20. Metras D. Endomyocardial fibrosis and its surgical treatment: Ivory Coast experience. In: Valiathan MS, Somers K, Kartha CC, eds. Endomyocardial Fibrosis. Oxford, England:. Oxford Univ Press;1993:207-19.

21. Metras D, Coulibaly AO, Ouattara K. The surgical treatment of endomyocardial fibrosis: results in 55 patients. Circulation. Sep 1985;72(3 Pt 2):II274-9. [Medline].

22. Moraes CR, Buffolo E, Victor E, et al. Endomyocardial fibrosis: report of 6 patients and review of the surgical literature. Ann Thorac Surg. Mar 1980;29(3):243-8. [Medline].

23. Mousseaux E, Hernigou A, Azencot M, et al. Endomyocardial fibrosis: electron-beam CT features. Radiology. Mar 1996;198(3):755-60. [Medline].

24. Nieveen J, Huber J. Familial myocardial fibrosis. Acta Med Scand. Nov 1970;188(5):439-45. [Medline].

25. Niino T, Shiono M, Yamamoto T, et al. A case of left ventricular endomyocardial fibrosis. Ann Thorac Cardiovasc Surg. Jun 2002;8(3):173-6. [Medline].

26. Parry EH, Abrahams DG. The natural history of endomyocardial fibrosis. Q J Med. Oct 1965;34(136):383-408. [Medline].

27. Patel AK, Ziegler JL, D''Arbela PG, Somers K. Familial cases of endomyocardial fibrosis in Uganda. Br Med J. Nov 6 1971;4(783):331-4. [Medline].

28. Puvaneswary M, Joshua F, Ratnarajah S. Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. Nov 2001;45(4):524-7. [Medline].

29. Radhakumary C, Kumari TV, Kartha CC. Endomyocardial fibrosis is associated with selective deposition of type I collagen. Indian Heart J. Jul-Aug 2001;53(4):486-9. [Medline].

30. Roberts WC, Liegler DG, Carbone PP. Endomyocardial disease and eosinophilia. A clinical and pathologic spectrum. Am J Med. Jan 1969;46(1):28-42. [Medline].

31. Salemi VM, Picard MH, Mady C. Assessment of diastolic function in endomyocardial fibrosis: value of flow propagation velocity. Artif Organs. Apr 2004;28(4):343-6. [Medline].

32. Shaper AG. The etiology of endomyocardial fibrosis. In: Valiathan MS, Somers K, Kartha CC, eds. Endomyocardial Fibrosis. Oxford, England: Oxford University Press;1993:113.

33. Shaper AG, Hutt MS, Coles RM. Necropsy study of endomyocardial fibrosis and rheumatic heart disease in Uganda 1950-1965. Br Heart J. May 1968;30(3):391-401. [Medline].

34. 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. Dec 2004;20(6):517-22. [Medline].

35. Somers K, Hutt MS, Patel AK, D''Arbela PG. Endomyocardial biopsy in diagnosis of cardiomyopathies. Br Heart J. Nov 1971;33(6):822-32. [Medline].

36. Somsen GA, Duren DR. [Heart failure in a Ghanese woman due to endomyocardial fibrosis]. Ned Tijdschr Geneeskd. Aug 26 1995;139(34):1746-8. [Medline].

37. Urhoghide GE, Falase AO. Degranulated eosinophils, eosinophil granule basic proteins and humoral factors in Nigerians with endomyocardial fibrosis. Afr J Med Med Sci. Sep 1987;16(3):133-9. [Medline].

38. Valiathan MS, Balakrishnan KG, Sankarkumar R, Kartha CC. Surgical treatment of endomyocardial fibrosis. Ann Thorac Surg. Jan 1987;43(1):68-73. [Medline].

39. Valiathan MS, Kartha CC. Geochemical basis of tropical endomyocardial fibrosis. In: Valiathan MS, Somers K, Kartha CC, eds. Endomyocardial Fibrosis. Oxford, England:. Oxford University Press;1993:98.

40. Valiathan MS, Kartha CC, Panday VK, et al. A geochemical basis for endomyocardial fibrosis. Cardiovasc Res. Sep 1986;20(9):679-82. [Medline].

41. Wynne J, Braunwald E. The cardiomyopathies and myocarditides. In: Braunwald E, ed. Heart Disease. 5th ed. Philadelphia, Pa:. WB Saunders and Co;1997:1433-4.

42. Zabsonre P, Renambot J, Adoh-Adoh M, et al. [Conduction disorders in chronic parietal endocarditis or endomyocardial fibrosis.170 cases at the Cardiology Institute of Abidjan.]. Dakar Med. 2000;45(1):15-9. [Medline].

Keywords

endomyocardial fibrosis, EMF, endomyocardial disease, hypereosinophilic syndrome, obliterative myocardial disease, tropical eosinophilic endomyocardial fibrosis, Davies disease, endocardial fibroelastosis, endomyocardial fibroelastosis, Löffler endocarditis, Loeffler endocarditis, restrictive cardiomyopathy, fibrosis of the endocardial surface of the heart, acute myocarditis, parasites, helminths, protozoans, toxoplasmosis, malaria, eosinophilia, malnutrition, high-tuber diet, cerium toxicity, Ce toxicity, hypomagnesemia, constrictive pericarditis

Contributor Information and Disclosures

Author

Ali A Sovari, MD, Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology, University of Illinois at Chicago
Ali A Sovari, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Abraham G Kocheril, MD, FACC, FACP, Professor of Medicine, Director of Clinical Electrophysiology, University of Illinois at Chicago
Abraham G Kocheril, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, Cardiac Electrophysiology Society, Central Society for Clinical Research, Heart Failure Society of America, and Illinois State Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Hanumant Deshmukh, MD †, Former Chief of Cardiology, Veterans Affairs Medical Center; Former Associate Professor, Department of Medicine, Rosalind Franklin University of Medicine and Science
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marschall S Runge, MD, PhD, Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine
Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association
Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Henry H Ooi, MBBCh, BAO, MRCPI, Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans Affairs Medical Center; Cardiologist, Heart Failure and Cardiac Transplant Program, Vanderbilt University Medical Center
Henry H Ooi, MBBCh, BAO, MRCPI is a member of the following medical societies: American College of Cardiology, American Heart Association, Heart Failure Society of America, and International Society for Heart and Lung Transplantation
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author James L Furgerson, MD to the development and writing of this article.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)