TY - JOUR
T1 - Survival in primary pulmonary hypertension
T2 - The impact of epoprostenol therapy
AU - McLaughlin, Vallerie V.
AU - Shillington, Alicia
AU - Rich, Stuart
PY - 2002/9/17
Y1 - 2002/9/17
N2 - 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.
AB - 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.
KW - Prostaglandins
KW - Pulmonary heart disease
KW - Survival
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U2 - 10.1161/01.CIR.0000029100.82385.58
DO - 10.1161/01.CIR.0000029100.82385.58
M3 - Article
C2 - 12234951
AN - SCOPUS:0037126044
SN - 0009-7322
VL - 106
SP - 1477
EP - 1482
JO - Circulation
JF - Circulation
IS - 12
ER -