$Unique_ID{BRK03740}
$Pretitle{}
$Title{Fibroelastosis, Endocardial}
$Subject{Fibroelastosis Endocardial EFE Fetal Endocarditis EMF Endocardial
Sclerosis Fetal Endomyocardial Fibrosis Subendocardial Sclerosis Endocardial
Dysplasia}
$Volume{}
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Copyright (C) 1986, 1988 National Organization for Rare Disorders, Inc.

219:
Fibroelastosis, Endocardial

** IMPORTANT **
It is possible the main title of the article (Endocardial Fibroelastosis)
is not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     EFE
     Fetal Endocarditis
     EMF
     Endocardial Sclerosis
     Fetal Endomyocardial Fibrosis
     Subendocardial Sclerosis
     Endocardial Dysplasia

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.


Endocardial Fibroelastosis is a heart disorder affecting infants which is
characterized by a thickened endocardium (lining of the heart cavities) that
shows proliferation of elastic tissue.  The cause is unknown at the present
time.

Symptoms

Endocardial Fibroelastosis is a common form of heart diseases in infancy and
early childhood with onset generally between 4 and 12 months of age.  Elastic
tissue proliferates in the tissue under the endocardium (the subendocardium),
causing a diffuse, milky-white thickening of the endocardium and the
subendocardium.  Ventricular failure due to altered distensibility of the
ventriculum sometimes develops.

Electrocardiogram (ECG) evidence of enlargement of the left ventricle of
the heart in an infant suggests a diagnosis of Endocardial Fibroelastosis.
Increased size of heart muscle fibers may accompany this disorder.  The
earliest signs of heart muscle (myocardial) damage are subtle S-T segment and
T-wave changes on the electrocardiogram.  Serial ECGs reveal progression or
regression of the disorder.  As the cardiomyopathy worsens, the patient
develops fatigue, difficulty breathing (dyspnea), palpitations, and
discomfort over the heart in the lower chest region.  Ventricular failure
with a fast heart beat (tachycardia) and atrial and ventricular arrhythmias
may also be encountered.

Causes

The cause of Endocardial Fibroelastosis is unknown.

Affected Population

Infants of both sexes between the ages of 4 months and 2 years are affected
by Endocardial Fibroelastosis.  A few adult cases have also been reported.

Related Disorders

Idiopathic Cardiomyopathy is characterized by an enlarged heart (hypertrophy).
The cause is unknown.

Viral Myocarditis is characterized by severe difficulty breathing
(dyspnea), an enlarged heart, excessively fast heart rate (tachycardia),
arrhythmias, and generalized swelling (edema).

Therapies:  Standard

Response to treatment is most favorable when the damage is noted early.
Absolute bed rest over a prolonged period of time may facilitate healing of
the myocardial lesions while the myocardium is working at a reduced load.
Further therapy of Endocardial Fibroelastosis is directed at control of
arrhythmias and treatment of congestive heart failure.

Therapies:  Investigational

This disease entry is based upon medical information available through
November 1988.  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 Endocardial Fibroelastosis, 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 Ave.
     Dallas, TX  75231
     (214) 750-5300

     NIH/National Heart, Lung and Blood Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-4236

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W.B. Saunders Co., 1988.  P. 351.