$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.