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$Unique_ID{BRK04154}
$Pretitle{}
$Title{Pulmonary Hypertension, Secondary}
$Subject{Pulmonary Hypertension, Secondary Pulmonary Arterial Hypertension Cor
Pulmonale Interstitial Pneumonia Pulmonary Hypertension, Primary}
$Volume{}
$Log{}
Copyright (C) 1989, 1990 National Organization for Rare Disorders, Inc.
671:
Pulmonary Hypertension, Secondary
** IMPORTANT **
It is possible that the main title of the article (Secondary Pulmonary
Hypertension) is not the name you expected. Please check the SYNONYM listing
to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
Pulmonary Arterial Hypertension
Information on the following diseases can be found in the Related
Disorders section of this report:
Cor Pulmonale
Interstitial Pneumonia
Pulmonary Hypertension, Primary
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.
Secondary Pulmonary Hypertension is a disorder of the blood vessels in
the lungs. It usually is the result of other lung diseases or related
diseases in other organs. The disorder is characterized by breathing
difficulties, especially after exertion.
Symptoms
Secondary Pulmonary Hypertension is characterized by symptoms of
breathlessness, anxiety, rapid breathing (tachpnea), chest pain and in
extreme cases heart failure. Measurements of right and left descending
pulmonary artery diameter can provide a correct diagnosis in 98% of patients
with suspected Pulmonary Hypertension. In the right lung artery a diameter
over 16.7mm and in the left lung artery a diameter of over 16.9mm, is an
indication of excessively high lung pressure.
Causes
There can be a number of causes of Secondary Pulmonary Hypertension. Lung
disease such as Interstitial Lung Disease, blood clots in the pulmonary
arteries, decrease in the size of blood vessels (pulmonary vasoconstriction),
a form of Scleroderma called CREST Syndrome (characterized by Calcenosis,
Raynaud's phenomenon, Esophageal dysfunction, Sclerodactylia and
Telangiectosis) may cause Pulmonary Hypertension. Other causes may be living
at high altitude, thickening of the blood, and portal hypertension.
Secondary Pulmonary Hypertension may also occur for unknown reasons.
Affected Population
Secondary Pulmonary Hypertension is a disease that affects males and females
in equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Secondary
Pulmonary Hypertension. Comparisons may be useful for a differential
diagnosis:
Cor Pulmonale is a term that denotes enlargement of the right ventricle
of the heart that occurs as a result of disease in the lungs. It is used as
a synonym for pulmonary heart disease. The most common cause of Cor
Pulmonale is massive clotting in the lungs which results in increased
pressure in lungs and heart, usually resulting in heart failure. Other
causes may be chronic bronchitis, emphysema, and extensive loss of lung
tissue from surgery or injury. Symptoms usually include right heart
enlargement, difficulty breathing, fainting spells on exertion, and
substernal angina pain.
Interstitial Pneumonia is a type of primary pneumonia. It involves the
spaces and tissues in the lining of the lungs with abnormal increases in
these tissues. Major symptoms may include shortness of breath on exertion,
cough and loss of appetite. The symptoms may vary from mild to severe
according to the extent of involvement. The patient usually has no fever,
and there is usually not an over production of mucous. (For more information
on this disorder, choose "Interstitial Pneumonia" as your search term in the
Rare Disease Database).
Primary Pulmonary Hypertension is a rare lung disorder of unknown cause.
It occurs most often in young women. it is characterized by high pulmonary
artery pressure, right ventricular failure, breathing difficulty, chest pain
and fainting. it is progressive and usually follows a rapid course. Drug
therapy may slow the progress of the disease including the intravenous drug
prostacyclin, vasodilators, alpha-adrenergic blocking agents, beta agonists
and prostaglandins. There is no known cure for this disease and the patient
may eventually need a heart-lung transplant.
Therapies: Standard
Prior to treatment of Secondary Pulmonary Hypertension, tests to confirm the
diagnosis and degree of this disorder should be carried out. This may be
done by a right-sided cardiac catheterization or use of echo-cardiography.
After diagnosis, physical activity should be limited and any underlying
causes such as: heart disease, clogged arteries or living at high altitude
should be treated. Drug therapy to treat Pulmonary Hypertension may include
the use of vasodilators such as: epoprostenol, hydralazine and nifedipine.
Therapies: Investigational
In extreme cases a Heart/Lung transplant may be necessary for people with
Secondary Pulmonary Hypertension.
This disease entry is based upon medical information available through
January 1990. 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 Secondary Pulmonary Hypertension, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Pathlight is a quarterly newsletter for patients with Pulmonary
Hypertension.
PATHLIGHT
1060 Pembroke Ave., NE
Palm Bay, FL 32907
The Foundation for Pulmonary Hypertension, Inc.
P.O. Box 61540
New Orleans, LA 70130
American Heart Association
7320 Greenville Ave.
Dallas, TX 75231
(214) 750-8700
American Lung Association
1740 Broadway
New York, NY 10019
(212) 315-8700
NIH/National Heart, Blood and Lung Institute
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4236
References
PULMONARY DISEASES AND DISORDERS, 2nd edition, Alfred P. Fishman, McGraw-Hill
Book Co., 1988, Pp. 999-1025.
CURRENT APPROACH TO TREATMENT OF PRIMARY PULMONARY HYPERTENSION, B.M.
Groves, et al,; Chest (March, 1988, issue 93 (3 Suppl)). Pp. 175S-178S.
FUNCTIONAL TRICUSPID REGURGITATION AND RIGHT VENTRICULAR DYSFUNCTION IN
PULMONARY HYPERTENSION. D.A. Morrison, et al,; Am J Cardiol, (July, 1988, 62
(1)). Pp. 108-112.
PREDICTION OF FAVOURABLE RESPONSES TO LONG-TERM VASODILATOR TREATMENT OF
PULMONARY HYPERTENSION BY SHORT TERM ADMINISTRATION OF EPOPROSTENOL
(PROSTACYCLIN) OR NIFEDIPINE. A. Rozkovec, et al,; Br Heart J (June, 1988, 59
(6)). Pp. 696-705.