home *** CD-ROM | disk | FTP | other *** search
- $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.
-
-