eMedicine Specialties > Cardiology > Myocardial Disease and Cardiomyopathies

Endomyocardial Fibrosis: Differential Diagnoses & Workup

Author: James L Furgerson, MD, Consulting Staff, Cardiology Service, Brooke Army Medical Center; Clinical Assistant Professor, Department of Internal Medicine, University of Texas Health Science Center at San Antonio
Contributor Information and Disclosures

Updated: Jan 17, 2006

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 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, thrombi adherent to endocardial surface, mitral regurgitation, and tricuspid regurgitation.
    • 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 ventricular outflow tract is most common.
    • Recently, 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. 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: Recently, the use of cardiovascular magnetic resonance imaging has been shown to demonstrate obliterative changes in the ventricles, atrial dilation, and regurgitant atrioventricular valve lesions in patients with EMF. However, the use of contrast-enhanced imaging was not able to demonstrate fibrosis within the ventricles of these patients.

Other Tests

  • Electrocardiography
    • Atrial fibrillation
    • Low QRS voltage
    • First-degree atrioventricular block in up to 44% of patients
    • Incomplete right bundle-branch block in up to 30% of patients
  • Left 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.

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.

More on Endomyocardial Fibrosis

Overview: Endomyocardial Fibrosis
Differential Diagnoses & Workup: Endomyocardial Fibrosis
Treatment & Medication: Endomyocardial Fibrosis
Follow-up: Endomyocardial Fibrosis
References
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Further Reading

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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

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Contributor Information and Disclosures

Author

James L Furgerson, MD, Consulting Staff, Cardiology Service, Brooke Army Medical Center; Clinical Assistant Professor, Department of Internal Medicine, University of Texas Health Science Center at San Antonio
James L Furgerson, MD is a member of the following medical societies: American College of Cardiology and American College of Physicians
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: Nothing to disclose.

Managing Editor

Marschall S Runge, MD, PhD, Marion Covington Distinguished Professor of Medicine, Vice Dean for Clinical Affairs, Chairman, Department of Medicine, 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: Nothing to disclose.

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

Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice
Michael E Zevitz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, and Michigan State Medical Society
Disclosure: Nothing to disclose.

 
 
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