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Pulmonary arterial hypertension markedly worsens a person's quality of life, but medication may help

Pulmonary arterial hypertension (PAH) is a rare blood vessel disorder in which pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels. In PAH, the pulmonary artery has narrowed, making the heart work harder to pump blood into the lungs (where it is oxygenated) and into the body. The result is a devastating disease that primarily strikes women in the prime of their life (20-40 years of age).

A recent study of patients with PAH found significantly impaired quality of life—so much so, that on average, patients were willing to accept a 29 percent risk of death in order to be cured of PAH. Shirin Shafazand, M.D., M.S., F.C.C.P., of the Stanford University School of Medicine, and fellow researchers studied health-related quality of life (HRQOL) in 53 patients who had PAH for nearly 2 years and were being treated at a university hospital-based pulmonary hypertension (PH) clinic. Overall, 83 percent were women, and 53 percent received epoprostenol (a medication that opens up the pulmonary artery to reduce hypertension). The epoprostenol and non-epoprostenol groups were similar in age, marital status, education, and ethnicity.

The researchers administered a Nottingham Health Profile (NHP), Congestive Heart Failure Questionnaire (CHQ), and Hospital Anxiety and Depression Scale to the participants. Participants reported moderate to severe impairment in all NHP domains, including energy, emotional reaction, pain, physical mobility, sleep, and social isolation. They also showed moderate impairment in all CHQ domains, including shortness of breath, fatigue, emotional function, and mastery. Also, 20.5 percent and 7.5 percent reported moderate or severe levels of anxiety and depression, respectively. Despite severe PH at presentation, longer duration of illness, and adverse effects associated with therapy, those receiving epoprostenol described more energy, less fatigue, less emotional distress, less anxiety or depression, and greater control over their disease than the non-drug group. This research was supported in part by the Agency for Healthcare Research and Quality (F33 HS11767).

See "Health-related quality of life in patients with pulmonary arterial hypertension," by Dr. Shafazand, Mary K. Goldstein, M.D., M.Sc., Ramona L. Doyle, M.D., F.C.C.P., and others, in the November 2004 Chest 126(5), pp. 1452-1459.

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