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JAMA Patient Page
January 23, 2008

Pulmonary Hypertension

JAMA. 2008;299(3):372. doi:10.1001/jama.299.3.372

The pulmonary artery is the main vessel that carries blood from the heart to the lungs. For a person at rest, blood pressure in a normal pulmonary artery is about 15 millimeters of mercury (mm Hg). In an individual with pulmonary hypertension, the average pressure in the pulmonary artery can be more than 25 mm Hg. This increase in pulmonary artery pressure occurs when the small arteries of the lungs become abnormally narrow. This condition can eventually lead to heart failure and death. Pulmonary hypertension can be idiopathic (unknown cause), familial (runs in families and is often linked to a genetic cause), or associated with other medical conditions. The January 23, 2008, issue of JAMA includes an article about the increase of pulmonary blood vessel disease in patients with some types of hereditary anemia (low red blood cell counts) and with human immunodeficiency virus (HIV) infection.


  • Connective tissue diseases (autoimmune diseases such as scleroderma, sarcoidosis, or lupus)

  • Congenital heart and lung disease (conditions that develop at or before birth, such as atrial septal defects)

  • Portal hypertension (resulting from liver disease)

  • HIV infection

  • Drugs and toxins (appetite suppressants, cocaine, amphetamines)

  • Thyroid disorders

  • Myeloproliferative disorders (overproduction of red or white blood cells)

  • Hemoglobinopathies (abnormal oxygen-carrying proteins in red blood cells, such as found in sickle cell anemia)

  • Blood clots in pulmonary arteries


Initial symptoms may be minor and diagnosis may be delayed for several years until symptoms worsen. Typical symptoms may include

  • Shortness of breath following exertion

  • Excessive fatigue

  • Dizziness and fainting

  • Ankle swelling

  • Bluish lips and skin

  • Chest pain


There is not one specific way to diagnose pulmonary hypertension. A doctor usually runs a series of tests to measure blood pressure in the pulmonary arteries, determine how well the heart and lungs are working, and rule out other diseases.



To find this and previous JAMA Patient Pages, go to the Patient Page link on JAMA's Web site at Many are available in English and Spanish. A previous Patient Page on the evaluation of lung function was published in the May 16, 2007, issue.

Sources: American Lung Association; National Heart, Lung, and Blood Institute; Pulmonary Hypertension Association

The JAMA Patient Page is a public service of JAMA. The information and recommendations appearing on this page are appropriate in most instances, but they are not a substitute for medical diagnosis. For specific information concerning your personal medical condition, JAMA suggests that you consult your physician. This page may be photocopied noncommercially by physicians and other health care professionals to share with patients. To purchase bulk reprints, call 203/259-8724.