Survival with first-line bosentan in patients with primary pulmonary hypertension

被引:451
作者
McLaughlin, VV
Sitbon, O
Badesch, DB
Barst, RJ
Black, C
Gallè, N
Rainisio, M
Simonneau, G
Rubin, LJ
机构
[1] Univ Michigan, Womens Hosp, Ann Arbor, MI 48109 USA
[2] Univ Paris Sud, Clamart, France
[3] Univ Colorado, Hlth Sci Ctr, Denver, CO 80202 USA
[4] Columbia Univ Coll Phys & Surg, New York, NY 10032 USA
[5] Royal Coll Physicians, London NW1 4LE, England
[6] Univ Bologna, Bologna, Italy
[7] Actelion Pharmaceut Ltd, Allschwil, Switzerland
[8] Univ Calif San Francisco, San Francisco, CA 94143 USA
关键词
bosentan; endothelin receptor antagonists; primary pulmonary hypertension; pulmonary hypertension; survival;
D O I
10.1183/09031936.05.00054804
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Primary pulmonary hypertension (PPH) is a progressive disease with high mortality. Administration of i.v. epoprostenol has demonstrated improved exercise tolerance, haemodynamics, and survival. The orally active, dual endothelin receptor antagonist bosentan improves exercise endurance, haemodynamics, and functional class over the short term. To determine the effect of first-line bosentan therapy on survival, this study followed 169 patients with PPH treated with bosentan in two placebo-controlled trials and their extensions. Data on survival and alternative treatments were collected from September 1999 (start of the first placebo-controlled study) to December 31, 2002. Observed survival up to 36 months was reported as Kaplan-Meier estimates and compared with predicted survival as determined for each patient by the National Institutes of Health Registry formula. Kaplan-Meier survival estimates were 96% at 12 months and 89% at 24 months. In contrast, predicted survival was 69% and 57%, respectively. In addition, at the end of 12 and 24 months, 85% and 70% of patients, respectively, remained alive and on bosentan monotherapy. Factors that predicted a worse outcome included World Health Organization Functional Class IV and 6-min walk distance below the median (358 m) at baseline. First-line bosentan therapy was found to improve survival in patients with advanced primary pulmonary hypertension.
引用
收藏
页码:244 / 249
页数:6
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