ENDOMYOCARDIAL FIBROSIS

ALTERNATE NAMES

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

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. Signs of this disease include fibrotic heart lesions, calcification, restricted ventricle, and mitral regurgitation. 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 are.

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

Clinical examination describing physical findings; Laboratory studies include 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.

ICD-9: 288.3

ONSET AND 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.

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.

Suggested MER for Evaluation:

Clinical examination that describes diagnostic features of the disease and laboratory studies are needed to confirm the diagnosis.

Suggested Listings for Evaluation:

DETERMINATION

 LISTING 

REMARKS

Meets Listing

4.02

4.05

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