Endomyocardial Fibrosis Workup

  • Author: Ali A Sovari, MD, FACP; Chief Editor: Henry H Ooi, MBBCh   more...
 
Updated: Jan 21, 2010
 

Laboratory Studies

Complete blood cell count may show anemia and eosinophilia.

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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.
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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
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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.
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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.

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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
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Contributor Information and Disclosures
Author

Ali A Sovari, MD, FACP  Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology, University of Illinois College of Medicine; Staff Physician and Hospitalist, St John Regional Medical Center, Cogent Healthcare, Inc

Ali A Sovari, MD, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Physiological Society, and Heart Rhythm Society

Disclosure: Nothing to disclose.

Coauthor(s)

Abraham G Kocheril, MD, FACC, FACP, FHRS  Professor of Medicine, University of Illinois College of Medicine

Abraham G Kocheril, MD, FACC, FACP, FHRS 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.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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

Amer Suleman, MD  Private Practice

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  Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans Affairs Medical Center; Assistant Professor of Medicine, Vanderbilt University School of Medicine

Henry H Ooi, MBBCh 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.

Acknowledgments

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.

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