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$Title{Dilatation of the Pulmonary Artery, Idiopathic}
$Subject{Dilatation of the Pulmonary Artery Idiopathic IDPA}
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Copyright (C) 1986 National Organization for Rare Disorders, Inc.

225:
Dilatation of the Pulmonary Artery, Idiopathic

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

Synonyms

     IDPA

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.


Idiopathic Dilatation of the Pulmonary Artery (IDPA) is a rare congenital
defect characterized by a wider than normal main pulmonary artery in the
absence of any apparent anatomical or physiological cause.

Symptoms

Idiopathic Dilatation of the Pulmonary Artery commonly doesn't produce
symptoms because there is no circulation abnormality.  Clinical signs are
minimal and consist of a palpable pulmonary ejection sound which disappears
when one inhales, a soft pulmonary ejection systolic murmur (abnormal heart
sound) and splitting of the second sound on breathing in.  Idiopathic
Dilatation of the Pulmonary Artery does not cause pulmonary valve disease,
nor does bacterial endocarditis occur in patients with this condition.  The
electrocardiogram is normal, and diagnosis is made when chest X-rays reveal a
dilated pulmonary artery without cardiac chamber enlargement.

Causes

The cause of Idiopathic Dilatation of the Pulmonary Artery is unknown.  A
defect in the normal development of pulmonary artery elastic tissue before or
after birth has been postulated.  The dilatation may also be a consequence of
a generalized connective tissue disease as it is occasionally found in
Marfan's syndrome or Ehlers-Danlos syndrome.  (For more information on these
disorders, choose "Marfan" and Ehlers-Danlos" as your search terms in the
Rare Disease Database.

Therapies:  Standard

Treatment for Idiopathic Dilatation of the Pulmonary Artery is not required.
People with this condition have a normal life expectancy, provided they have
no cardiac lesions.

Therapies:  Investigational

This disease entry is based upon medical information available through March
1987.  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 Idiopathic Dilatation of the Pulmonary Artery,
please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

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

     American Lung Association
     1740 Broadway
     New York, New York 10019
     (212) 315-8700

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 376.