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Endomyocardial Fibrosis Treatment & Management

  • Author: Ali A Sovari, MD, FACP; Chief Editor: Henry H Ooi, MD, MRCPI  more...
 
Updated: Nov 24, 2014
 

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.

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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.

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Diet

No specific diet is recommended for this condition.

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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.

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Contributor Information and Disclosures
Author

Ali A Sovari, MD, FACP Fellow in Clinical Cardiac Electrophysiology, Cedars Sinai Medical Center/Heart Institute

Ali A Sovari, MD, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Physician Scientists Association, American Physiological Society, Biophysical Society, Heart Rhythm Society, Society for Cardiovascular Magnetic Resonance

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, Central Society for Clinical and Translational Research, Heart Failure Society of America, Cardiac Electrophysiology Society, American College of Physicians, American Heart Association, American Medical Association, Illinois State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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 Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Texas Medical Association, Southern Society for Clinical Investigation, American Federation for Clinical Research, Association of Professors of Medicine, Association of Professors of Cardiology, American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Medical Research, American Heart Association

Disclosure: Received honoraria from Pfizer for speaking and teaching; Received honoraria from Merck for speaking and teaching; Received consulting fee from Orthoclinica Diagnostica for consulting.

Chief Editor

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

Disclosure: Nothing to disclose.

Acknowledgements

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

References
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