Please note: This information was current at the time of publication but now may be out of date. This handout provides a general overview and may not apply to everyone. 

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Am Fam Physician. 2001;63(9):1800

What is pulmonary hypertension?

Pulmonary hypertension is a disorder of the blood vessels in the lungs. “Pulmonary” means “in the lungs,” and “hypertension” means “high blood pressure.”

Pulmonary hypertension happens when the blood pressure in the pulmonary arteries (the vessels that carry blood from the heart to the lungs) becomes higher than normal. This puts strain on the right side of the heart. Pulmonary hypertension is a serious problem.

What are the signs of pulmonary hypertension?

Common signs of pulmonary hypertension are shortness of breath with activity, feeling tired, fainting and chest pain. Symptoms usually limit exercise and other activities.

What causes pulmonary hypertension?

Pulmonary hypertension can have many causes. Sometimes the cause is not known. When the cause of pulmonary hypertension isn't known, the disorder is called “primary” pulmonary hypertension.

“Secondary” pulmonary hypertension has a known cause. Common causes are emphysema and chronic bronchitis (breathing problems). Other causes are congestive heart failure, birth defects in the heart, chronic pulmonary thromboembolism (old blood clots in the pulmonary arteries), HIV infection (AIDS), and certain medicines, including the diet drugs fenfluramine and dexfenfluramine. (These diet drugs are no longer available.)

Both primary and secondary types are usually permanent conditions, although treatment can improve symptoms and long-term outcomes in some people.

How does my doctor know that I have pulmonary hypertension?

The signs of pulmonary hypertension can be like the signs of many health problems. To find out if you have pulmonary hypertension, your doctor may do an echocardiogram. For this test, an instrument called a “probe” is placed on the outside of your chest. The probe sends out sound waves. These sound waves are used to form pictures of your heart and estimate the blood pressure in your pulmonary arteries.

How is pulmonary hypertension treated?

If the cause of your pulmonary hypertension is known, treating the cause may help. Sometimes, medicines called calcium-channel blockers, breathing oxygen from a tank, or a blood-thinning medicine may help. In primary pulmonary hypertension, a medicine that is given through a vein, called prostacyclin (brand name: Flolan), may lower the blood pressure in your pulmonary arteries.

Where can I get more information about pulmonary hypertension?

For more information, you can call the Pulmonary Hypertension Association at 1-800-748-7274. You can also visit their Web site athttp://www.phassociation.org.

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