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Eur Respir J 2005; 25:244-249
Copyright ©ERS Journals Ltd 2005

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

V. V. McLaughlin1, O. Sitbon2, D. B. Badesch3, R. J. Barst4, C. Black5, N. Galiè6, M. Rainisio7, G. Simonneau2 and L. J. Rubin8

1 University of Michigan, Ann Arbor, MI, USA. 2 University Paris-Sud, Clamart, France. 3 University of Colorado Health Sciences Center, Denver, CO, USA. 4 Columbia University College of Physicians and Surgeons, New York, NY, USA. 5 Royal College of Physicians, London, UK. 6 University of Bologna, Bologna, Italy. 7 Actelion Pharmaceuticals Ltd, Allschwil, Switzerland. 8 University of California, San Diego, CA, USA

CORRESPONDENCE: V. V. McLaughlin, University of Michigan, 1500 East Medical Center Drive, Women's Hospital, Room L3119, Ann Arbor, MI, USA. Fax: 1 7347637390. E-mail: vmclaugh@med.umich.edu

Keywords: Bosentan, endothelin receptor antagonists, primary pulmonary hypertension, pulmonary hypertension, survival

Received: May 7, 2004
Accepted October 4, 2004

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.




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