STUDY OBJECTIVES: We performed an open-label multicenter study to evaluate the safety and efficacy of the dual endothelin receptor antagonist bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS: Nineteen patients with inoperable CTEPH were enrolled. MEASUREMENTS: The primary end point was a change in pulmonary vascular resistance (PVR). Secondary end points included 6-min walk test, peak oxygen uptake (V(O2)), New York Heart Association functional class, serum levels of N-terminal-pro brain natriuretic peptide (NT-pro-BNP), and various other hemodynamic parameters. RESULTS: After 3 months of treatment with bosentan, PVR decreased from 914 +/- 329 to 611 +/- 220 dyne.s.cm(-5) (p < 0.001). Functional class and peak V(O2) remained unchanged, but 6-min walk distance increased from 340 +/- 102 to 413 +/- 130 m (p = 0.009), and serum NT-pro BNP levels improved from 2,895 +/- 2,620 to 2,179 +/- 2,301 (p = 0.027). One patient died, presumably from influenza A infection, and another patient experienced progressive fluid retention despite reduction of PVR. Other than that, treatment was well tolerated by all patients. CONCLUSIONS: This open-label pilot trial suggests that bosentan may offer a therapeutic option for patients with inoperable CTEPH. Randomized controlled trials are warranted to confirm these findings.
STUDY OBJECTIVES: We performed an open-label multicenter study to evaluate the safety and efficacy of the dual endothelin receptor antagonist bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS: Nineteen patients with inoperable CTEPH were enrolled. MEASUREMENTS: The primary end point was a change in pulmonary vascular resistance (PVR). Secondary end points included 6-min walk test, peak oxygen uptake (V(O2)), New York Heart Association functional class, serum levels of N-terminal-pro brain natriuretic peptide (NT-pro-BNP), and various other hemodynamic parameters. RESULTS: After 3 months of treatment with bosentan, PVR decreased from 914 +/- 329 to 611 +/- 220 dyne.s.cm(-5) (p < 0.001). Functional class and peak V(O2) remained unchanged, but 6-min walk distance increased from 340 +/- 102 to 413 +/- 130 m (p = 0.009), and serum NT-pro BNP levels improved from 2,895 +/- 2,620 to 2,179 +/- 2,301 (p = 0.027). One patient died, presumably from influenza A infection, and another patient experienced progressive fluid retention despite reduction of PVR. Other than that, treatment was well tolerated by all patients. CONCLUSIONS: This open-label pilot trial suggests that bosentan may offer a therapeutic option for patients with inoperable CTEPH. Randomized controlled trials are warranted to confirm these findings.
Authors: Sanjay Mehta; Doug Helmersen; Steeve Provencher; Naushad Hirani; Fraser D Rubens; Marc De Perrot; Mark Blostein; Kim Boutet; George Chandy; Carole Dennie; John Granton; Paul Hernandez; Andrew M Hirsch; Karen Laframboise; Robert D Levy; Dale Lien; Simon Martel; Gerard Shoemaker; John Swiston; Justin Weinkauf Journal: Can Respir J Date: 2010 Nov-Dec Impact factor: 2.409
Authors: Mark R Toshner; Deepa Gopalan; Jay Suntharalingam; Carmen Treacy; Elaine Soon; Karen K Sheares; Nicholas W Morrell; Nicholas Screaton; Joanna Pepke-Zaba Journal: J Heart Lung Transplant Date: 2010-03-15 Impact factor: 10.247
Authors: M G J Duffels; M N van der Plas; S Surie; M M Winter; B Bouma; M Groenink; A P J van Dijk; E Hoendermis; R M F Berger; P Bresser; B J M Mulder Journal: Neth Heart J Date: 2009-09 Impact factor: 2.380