Primary pulmonary hypertension (PPH) is a rare disease of unknown cause that develops when the arteries that carry blood from the heart to the lungs become narrowed, resulting in high pressures in these arteries. A serious disease, it occurs in young adults and is approximately twice as common in women as in men.
There are some known causes of pulmonary hypertension; for example, the use of the appetite suppressant “fen-phen” (dexfenfluramine and phentermine). Although these appetites suppressants have been taken off the market, former fen-phen users have a 23-fold increase risk of developing PPH, possibly years later. The disease can also result from chronic liver disease, some rheumatologic disorders, congenital heart malformations or illicit drug use.
Many people do not realize they have pulmonary hypertension until they become short-winded doing common things, such as climbing stairs. The diagnosis is supported by an abnormal echocardiogram of the heart and confirmed with the direct measurement of blood pressure in the lung arteries during a cardiac catheterization.
How is PPH treated?
PPH is difficult to treat, although intravenous prostacyclin has proven effective in many patients. Now, this treatment is proving effective in some patient with pulmonary hypertension from known causes. Physicians at CCF are evaluating new treatment approaches for pulmonary hypertension in clinical trials.
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