Endomyocardial Fibrosis

Endomyocardial Fibrosis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Endomyocardial Fibrosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • EMF
  • Davies' disease
  • fibroelastic endocarditis
  • Loeffler endomyocardial fibrosis with eosinophilia
  • Loeffler fibroplastic parietal endocarditis
  • Loeffler's disease

Disorder Subdivisions

  • biventricular fibrosis
  • left ventricular fibrosis
  • right ventricular fibrosis

General Discussion

Endomyocardial fibrosis (EMF) is a progressive disease of unknown origin (idiopathic) that may seriously affect the heart. Its most obvious feature is a gross change in the makeup of the lining of the heart cavities (the endocardium) of one or both of the lower chambers of the heart (the ventricles) leading to the replacement of normal cells with fibrous tissue (fibrosis). This process is progressive and leads to the narrowing (constriction) of the right or left ventricular cavities. It may involve the valves between the chambers of the heart as well as the tendon-like cords that fix the valves to the ventricles (chordae tendineae).



Loeffler's disease is a disease of the heart much like endomyocardial fibrosis. Some clinicians regard it as an early stage of EMF, although this idea remains controversial. Loeffler's disease is a rare disorder of unknown origin, characterized by abnormal increases in the number of particular white blood cells (eosinophilia), and like EMF, gross fibrosis of the endocardium, and inflammation of small blood vessels (arteritis).

Symptoms

The main microscopic feature of endomyocardial fibrosis (as well as of Loeffler's disease) is fibrosis of the inner lining of the heart cavities (the endocardium). This means that the normal endocardium is replaced by a thick, inelastic tissue. The fibrotic lesions may be over 1 cm thick and may extend finger-like projections into the heart muscle (the myocardium).



Fibrosis frequently affects the heart asymmetrically. It may specifically involve one or more of the following areas: the top (apex) of the left ventricle, the back (posterior) wall of the left ventricle including the fibrous cords that connect the valves to the ventricles (the chordae tendineae), and the top (apex) of the right ventricle, extending backwards to encase the muscle and cords (chordae tendineae) attaching the heart valve (tricuspid).



If fibrosis of the left ventricle is predominant, then blood flow from the right side of the heart is reduced often with mitral valve failure causing back-flow (regurgitation) of blood. The results may include pulmonary venous hypertension and left ventricular enlargement. Abnormal heartbeat patterns (atrial fibrillation or atrial arrhythmia) are common. Difficulty in breathing (dyspnea) especially, but not exclusively, on physical exertion is the major physical sign.



If fibrosis of the right ventricle is predominant, then circulation is restricted often with tricuspid valve failure, causing backflow (tricuspid regurgitation). Enlargement of the heart (cardiomyopathy) because of right atrial dilatation is often seen. Facial swelling (edema), swelling of the legs, enlargement of the spleen and liver (hepatosplenomegaly), and an accumulation of fluid in the abdominal cavity (ascites) are to be expected.



Biventricular fibrosis with circulation features is a mixture of the two forms listed above. That is, the symptoms are a combination of left and right ventricular fibrosis.



The extracardiac manifestations of Loeffler's disease include emboli to the brain (stroke), spotty (petechial) hemorrhages, and an enlarged liver (hepatomegaly).

Causes

At this time, clinicians believe that an as yet unknown immunological process is the preferred explanation for the cause of most cases of endomyocardial fibrosis and Loeffler's disease. In the past, the cause of both conditions was attributed to the presence of the filaria worm in patients or to poor nutrition. Widespread infection with such worms and poor diets are typical in the tropical regions in which these disorders are more common. Eosinophils have been observed in some cases of endomyocardial fibrosis, suggesting a form of hypersensitivity may play a role in select cases. In children, endomyocardial fibrosis has been associated with the mumps virus.

Affected Populations

Endomyocardial fibrosis is principally an endemic disease of the equatorial tropics. It is exceedingly rare in Europe and North America. It affects all races, mostly children and young adults. The disease has been described in a few patients over 60 years of age and, rarely, in patients younger than 5 years of age.

Standard Therapies

Diagnosis

A combination of X-ray, echocardiography, and investigation of the state of the ventricles (ventriculography) is used to diagnose suspected cases of endomyocardial fibrosis or Loeffler's disease.



Treatment

Responses to medical treatment are generally poor and unproven. For patients with severe symptoms, surgical treatment may be applied when other treatments have not been successful. These procedures, however, are not without risk. Mortality as a consequence of surgery may run as high as 20%. Successful surgery reduces symptoms and increases survival times and rates.



Surgery is usually designed (1) to take out the fibrous endomyocardium so that the ventricles can be filled once more; (2) to repair or replace the mitral or tricuspid valve (or both), if one or another is involved; and (3) to leave a portion of fibrous endocardium in place to prevent postoperative heart block.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Braunwald E. Ed. Heart Disease: A Textbook of Cardiovascular Medicine. 8rd ed. W.B. Saunders Company, Philadelphia, PA; 2008.



Fauci AS, Braunwald E, Isselbacher KJ, et al. Eds. Harrison's Principles of Internal Medicine. 14th ed.McGraw-Hill Companies. New York, NY; 1998:1332.



REVIEW ARTICLES

Burlew BS, Weber KT. Cardiac fibrosis as a cause of diastolic dysfunction. Herz. 2002;27:92-98.



Andy JJ. Aetiology of endomyocardial fibrosis (EMF). West Afr J Med. 2001;20:199-207.



Lijnen PJ, Petrov VV, Fagard RH. Induction of cardiac fibrosis by transforming growth factor-beta (1). Mol Genet Metab. 2000;71:418-35.



Nicoletti A, Michel JB. Cardiac fibrosis and inflammation: interaction with hemodynamic and hormonal factors. Cardiovasc Res. 1999;41:532-43.



JOURNAL ARTICLES

Joshi R. Abraham S, Kumar AS. New approach for complete endocardiectomy in left ventricular endomyocardial fibrosis. J Thorac Cardiovasc Surg. 2003;125:40-42.



Pawlinski R, Fernandes A, Kehrle B, et al. Tissue factor deficiency causes cardiac fibrosis and left ventricular dysfunction. Proc Nat Acad Sci U S A. 2002;99:15333-338.



Barretto AC, Mady C, Oliveira SA et al. Clinical meaning of ascites in patients with endomyocardial fibrosis. Arq Bras Cardiol. 2002;78:196-99.



Radhakumary C, Kumari TV, Kartha CC. Endomyocardial fibrosis is associated with selective deposition of type I collagen. Indian Heart J. 2001;53:486-89.



Santos C.L., Moraes CR, Santos FL, et al. Endomyocardial fibrosis in children. Cardio Young. 2001;11:205-09.



INTERNET

Sovari, AA. Endomyocardial fibrosis. eMedicine. Last Update:January 21, 2010 www.emedicine.com/med/topic677.htm



Hassan SA. Loeffler endocarditis eMedicine. Last Update:December 6, 2011

www.emedicine.com/med/topic1318.htm

Resources

American Heart Association

7272 Greenville Avenue

Dallas, TX 75231

Tel: (214)784-7212

Fax: (214)784-1307

Tel: (800)242-8721

Email: Review.personal.info@heart.org

Internet: http://www.heart.org



Centers for Disease Control and Prevention

1600 Clifton Road NE

Atlanta, GA 30333

Tel: (404)639-3534

Tel: (800)232-4636

TDD: (888)232-6348

Email: cdcinfo@cdc.gov

Internet: http://www.cdc.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Myocarditis Foundation

2201 River Road #3401

Point Pleasant, NJ 08742

Tel: (732)295-3700

Fax: (732)295-3701

Tel: (866)846-1600

Email: candace@myocarditisfoundation.org

Internet: http://www.myocarditisfoundation.org/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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