Blood leaves the heart through a large blood vessel called the pulmonary artery. It flows through the lungs, absorbing oxygen, and then returns to the heart to be pumped through another large vessel, the aorta, to the body. Just as a person has a normal blood pressure for the body, he or she also has a normal blood pressure for the blood passing through the lungs. In a person with pulmonary hypertension, the pressure becomes abnormally elevated. This happens because the small arteries in the lung become abnormally narrow. As a consequence, blood cannot pass easily through the lungs to absorb oxygen, compromising the delivery of oxygen-rich blood to the tissues of the body. Because the heart is working harder against the high pressure, heart failure can develop. Pulmonary hypertension can be idiopathic (the cause is unknown) or inherited (runs in families and is often linked to a specific gene) or may be associated with other medical issues. The October 3, 2012, issue of JAMA contains an article about pulmonary hypertension. This Patient Page is based on one previously published in the October 7, 2009, issue of JAMA.
Connective tissue diseases (autoimmune diseases such as scleroderma, sarcoidosis, or lupus)
Low oxygen levels due to heart or lung disease (such as chronic obstructive pulmonary disease [COPD]) or high altitude
Portal hypertension (resulting from liver disease)
Drugs and toxins (such as appetite suppressants, cocaine, or amphetamines)
Myeloproliferative disorders (overproduction of red blood cells or white blood cells)
Hemoglobinopathies (abnormal oxygen-carrying proteins in red blood cells, such as found in sickle cell anemia)
Blood clots in the pulmonary arteries
Obesity with obstructive sleep apnea
Initial symptoms may be very mild and diagnosis may be delayed for several years until symptoms worsen. Some symptoms include
Shortness of breath with exertion
Dizziness and fainting
Bluish color of lips and skin
If your doctor suspects pulmonary hypertension, he or she may perform a series of tests to measure the blood pressure in the pulmonary arteries and to determine how well the lungs and heart are working, as well as tests to rule out other diseases. There is not one specific test to diagnose pulmonary hypertension.
The treatment of choice for pulmonary hypertension depends on the cause and severity of the condition in a particular patient. Treatments range from therapy for the underlying condition (eg, treating sleep apnea or blood clots) to medicines that lower the blood pressure in the lungs. Treatment of pulmonary hypertension generally requires the care of a specialist.
American Lung Association www.lung.org
National Heart, Lung, and Blood Institute www.nhlbi.nih.gov/health/health -topics/topics/pah
To find this and previous JAMA Patient Pages, go to the Patient Page link on JAMA 's website at www.jama.com. Many are available in English and Spanish.
Zeller JL, Goodman DM, Lynm C, Livingston EH. Pulmonary Hypertension. JAMA. 2012;308(13):1390. doi:10.1001/2012.jama.11701