on August 26, 2002
Circulation. 2002
Published online before print August 26, 2002, doi: 10.1161/01.CIR.0000029100.82385.58
Published online before print August 26, 2002, doi: 10.1161/01.CIR.0000029100.82385.58
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Submitted on April 22, 2002
From Rush-Presbyterian-St Luke's Medical Center, Department of Medicine, Section of Cardiology, Chicago, Ill (V.V.M., S.R.), and EPI-Q Inc, Oakbrook Terrace, Ill (A.S.). * To whom correspondence should be addressed. E-mail: vmclaugh{at}rush.edu.
Background—Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration-approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined. Methods and Results—One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure. Conclusions—Intravenous epoprostenol improves long-term survival in PPH.
Revised on June 24, 2002
Accepted on June 25, 2002
Survival in Primary Pulmonary Hypertension. The Impact of Epoprostenol Therapy
Vallerie V. McLaughlin MD*,
Key words: pulmonary heart disease • prostaglandins • survival
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