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$Unique_ID{BRK03709}
$Pretitle{}
$Title{Endomyocardial Fibrosis}
$Subject{Endomyocardial Fibrosis Davies' Disease EMF Loeffler's Disease
Fibroelastic Endocarditis Loeffler Fibroplastic Parietal Endocarditis Loeffler
Endomyocardial Fibrosis with Eosinophilia Left Ventricular Fibrosis Right
Ventricular Fibrosis Biventricular Fibrosis}
$Volume{}
$Log{}
Copyright (C) 1986 National Organization for Rare Disorders, Inc.
232:
Endomyocardial Fibrosis
** IMPORTANT **
It is possible the main title of the article (Endomyocardial Fibrosis) is
not the name you expected. Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Davies' Disease
EMF
Loeffler's Disease
Fibroelastic Endocarditis
Loeffler Fibroplastic Parietal Endocarditis
Loeffler Endomyocardial Fibrosis with Eosinophilia
DISORDER SUBDIVISIONS
Left Ventricular Fibrosis
Right Ventricular Fibrosis
Biventricular Fibrosis
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Endomyocardial Fibrosis is a heart disease of unknown origin in which the
most characteristic feature is a gross fibrosis of the lining of the heart
cavities (the endocardium) of one or both ventricles. Fibrosis progresses
towards constriction of the ventricular cavities and involvement of the
chordae tendinae and atrioventricular valves. Loeffler's disease is a
disease of the heart and small arteries, of unknown origin, characterized by
eosinophilia, gross fibrosis of the endocardium, small vessel arteritis and
infiltration of other organs. Endomyocardial Fibrosis is thought to be a
late stage of Loeffler's disease by some authorities.
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
dense collagen tissue, almost devoid of elastic fibers. 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
involves one or more of the following areas:
1. the top of the left ventricle
2. the posterior wall of the left ventricle including the fibrous cords
that connect the valves to the ventricles (the chordae tendinae)
3. the apex of the right ventricle, extending backwards along the inflow
tract and encasing the papillary muscles and chordae tendinae of the
tricuspid valve.
Thrombosis (blood clot) often develops on the surface of the fibrotic
lesions. Calcification also may occur. Different forms of fibrosis may be
distinguished depending on the area of the heart predominantly involved.
Left ventricular fibrosis is characterized by restriction of circulation
to the right ventricle and often mitral valve incompetence. This results in
mitral regurgitation, left atrial dilatation, pulmonary venous hypertension,
left ventricular enlargement, and first degree arteriovenous block. Atrial
fibrillation, an atrial arrhythmia, is common. Chest x-ray may reveal a
normal or only mildly enlarged heart silhouette except for left atrial
enlargement and signs of pulmonary venous hypertension. The electrocardiogram
(ECG) shows a low QRS voltage, non-specific ST segment and T-wave changes.
Right ventricular fibrosis is characterized by restriction of circulation
to the right ventricle often associated with tricuspid valve incompetence.
This results in tricuspid regurgitation, right atrial dilatation and systemic
venous hypertension. Symptoms include the clinical signs of tricuspid
regurgitation associated with those of constrictive pericarditis such as
stasis in the jugular veins with facial swelling (edema), enlarged spleen and
liver (hepatosplenomegaly), and an accumulation of fluid in the abdominal
cavity (ascites). Similar changes in the ECG as occur in left ventricular
fibrosis are seen. On chest X-rays, right atrial enlargement will usually be
seen.
Biventricular fibrosis with circulation features are 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
The cause of Endomyocardial Fibrosis is unknown. Both the presence of the
filaria worm and diet have been incriminated in the past. An immunological
mechanism is currently the most accepted explanation for most cases of
Endomyocardial Fibrosis and Loeffler's disease.
Affected Population
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, although the disease has been
described in patients over 60 years of age.
Therapies: Standard
The treatment of Endomyocardial Fibrosis is open heart surgery with a
three-pronged attack:
1. endomyocardiectomy to allow normal diastolic ventricular filling
2. repair or replacement of the mitral or tricuspid valve (or both), to
ensure valvular competence
3. leaving in place a portion of fibrous endocardium on the left
ventricular septum to prevent postoperative heart block.
Therapies: Investigational
This disease entry is based upon medical information available through
September 1989. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Endomyocardial Fibrosis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
American Heart Association
7320 Greenville Avenue
Dallas, TX 75231
(214) 750-5300
NIH/National Heart, Lung and Blood Institute
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4236
Centers for Disease Control (CDC)
1600 Clifton Road, NE
Atlanta, GA 30333
(404) 639-3534
References
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.: W.B. Saunders Co., 1988. Pp. 361-2.