Pulmonary Arterial Hypertension
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Pulmonary Arterial Hypertension is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- primary obliterative pulmonary vascular disease
- primary pulmonary hypertension
- precapillary pulmonary hypertension
- idiopathic pulmonary arterial hypertension
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by high blood pressure (hypertension) of the main artery of the lungs (pulmonary artery) for no apparent reason. The pulmonary artery is the blood vessel that carries blood from the heart through the lungs. Symptoms of PAH include shortness of breath (dyspnea) especially during exercise, chest pain, and fainting episodes. The exact cause of PAH is unknown and although treatable, there is no known cure for the disease.
PAH usually affects women between the ages of 20-50. Individuals with PAH may go years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise. However, it is important to treat PAH because without treatment high blood pressure causes the heart to work much harder, and over time, these muscles may weaken or fail. The progressive nature of this disease means that an individual may experience only mild symptoms at first, but will eventually require treatment and medical care to maintain a normal lifestyle.
Approximately 15-20% of patients with PAH have heritable PAH. People with heritable PAH have eitehr: (1) an autosomal dominant genetic condition associated with mutations in the BMPR2 gene or another gene in the TGFbeta pathway now associated with HPAH, or (2) are members of a family in which PAH is known to occur as primary disease.
The first reported case of PAH occurred in 1891, when the German doctor E. Romberg published a description of a patient who, at autopsy, showed thickening of the pulmonary artery but no heart or lung disease that might have caused the condition. In 1951, 3 cases were reported by Dr. D.T. Dresdale in the U.S. and the illness was originally called primary pulmonary hypertension.
PAH has been directly linked to diet drugs such as Fen Phen, Pondimin and Redux. These drugs were taken off the market in 1997, although cases related to diet drugs and toxins, such as methamphetamines do still appear.
Scleroderma Research Foundation
220 Montgomery Street
San Francisco, CA 94104
Pulmonary Hypertension Association
801 Roeder Road, Ste 1000
Silver Spring, MD 20910
American Heart Association
7272 Greenville Avenue
Dallas, TX 75231
American Lung Association
1301 Pennsylvania Ave NW
Washington, DC 20004
NIH/National Heart, Lung and Blood Institute
P.O. Box 30105
Bethesda, MD 20892-0105
1309 12th Avenue
San Francisco, CA 94122-2213
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
PHA EUROPE, European Pulmonary Hypertension Association
Tel: 43 1 402 37 25
Fax: 43 1 409 35 28
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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Last Updated: 10/20/2011
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