TN 19 (12-18)

DI 23022.550 Endomyocardial Fibrosis

COMPASSIONATE ALLOWANCE INFORMATION

ENDOMYOCARDIAL FIBROSIS

ALTERNATE NAMES

EMF; Davies Disease; Fibroelastic Endocarditis, Loeffler Endomyocardial Fibrosis with Eosinophilia; Loeffler Fibroplastic Parietal Endocarditis; Loeffler’s Disease

DESCRIPTION

Endomyocardial Fibrosis (EMF) is a rare progressive type of heart disease of unknown origin (idiopathic). It is illustrated by progressive fibrosis (the development of excess fibrous connective tissue) of the lining of one or both lower heart chambers. This results in constriction of the heart cavity and can involve the heart valves and other structures. The overall prognosis is poor and depends on the extent and distribution of disease within the various heart chambers and valves of the heart. EMF is an idiopathic disorder of the tropical and subtropical regions of the world characterized by the development of restrictive cardiomyopathy. Women and children are more commonly affected than men.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing:

  • Clinical examination describing physical findings;

  • Complete blood count showing anemia and eosinophilia;

  • Chest x-ray showing cardiomegaly;

  • Imaging studies showing enlargement of the atria;

  • Echocardiography;

  • Angiography showing distortion of the heart chambers;

  • Electron beam computed tomography; and

  • Cardiovascular magnetic resonance imaging (MRI) showing obliterative changes in the ventricles, atrial dilation, and regurgitant antrioventricular valves.

 Physical findings: Signs of this disease include:

  • Fibrotic heart lesions;

  • Calcification;

  • Restricted ventricle; and

  • Mitral regurgitation.

ICD-9: 288.3

TREATMENT

There is no definitive cure for this condition. Palliative treatment aimed at controlling symptoms, utilizing diuretics, digoxin, beta-blockers, endocardiectomy, cardiopulmonary bypass, and immunosuppressive therapy.

PROGRESSION

Most people have extensive disease at the time of diagnosis. The disease is usually fatal within one to three years after onset of symptoms. Prognosis for this condition is poor with high incidences of sudden cardiac death from fatal arrhythmias or from progressive cardiac failure.

SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
  • Clinical history and examination that describes the diagnostic features of the impairment;

  • Complete blood count (CBC);

  • Electrocardiogram (ECG);

  • Chest X-ray (CXR); or

  • Cardiac catheterization; and cardiovascular magnetic resonance imaging (MRI).

Suggested Listings for Evaluation:
DETERMINATION

LISTING

REMARKS
Meets 4.02
4.05
Equals
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

To Link to this section - Use this URL:
http://policy.ssa.gov/poms.nsf/lnx/0423022550
DI 23022.550 - Endomyocardial Fibrosis - 12/03/2018
Batch run: 12/03/2018
Rev:12/03/2018