Drug improves survival in primary pulmonary hypertension patients

DALLAS, Aug. 27 Long-term use of a vasodilator drug can significantly increase survival and improve quality of life in patients with primary pulmonary hypertension (PPH), according to the first multi-year study of the agent. The research is published in today's rapid access publication of Circulation: Journal of the American Heart Association.

The intravenous use of epoprostenol increased three-year survival rates by up to 80 percent and nearly doubled exercise tolerance. Doctors have known that epoprostenol extends PPH survival, but its longer-term benefits had not been clearly defined.

"We now have clear evidence of the effectiveness of epoprostenol in treating this rare, fatal disease. Awareness needs to be raised among clinicians seeing patients with symptoms that could be PPH," says lead researcher Vallerie V. McLaughlin, M.D., an assistant professor of medicine at Rush-Presbyterian-St. Luke's Medical Center in Chicago.

Primary, or unexplained, pulmonary hypertension is a rare lung disorder in which the blood pressure in the pulmonary arteries rises far above normal levels for no apparent reason. This abnormally high pressure is associated with changes in the small blood vessels in the lungs that increase resistance to blood flow.

When the smaller blood vessels in the lungs become more resistant to blood flow, the heart's right ventricle must work harder to pump enough blood through the lungs.

Epoprostenol is marketed in the United States as Flolan by Glaxo-Smith-Kline and is also known as PGI 2. It works by relaxing blood vessels, thereby increasing the supply of blood to the lungs and reducing the heart's workload.

The National Institutes of Health established a PPH registry in 1980 describing clinical manifestations and survival rates. The registry demonstrated that the disease is difficult to diagnose and deadly. The average time from the start of symptoms until diagnosis was nearly three years

Contact: Carole Bullock
American Heart Association

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