STUDY OBJECTIVES: we performed an open-label national study to evaluate the effects of Bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS AND METHODS: fifteen patients with CTEPH not eligible or waiting for surgery were enrolled. The primary endpoint was the change in pulmonary vascular resistance (PVR). Secondary endpoints included quality of life (measured by the Minnesota living with heart failure questionnaire, MLHF), 6 minute walk distance (6MWD), World Health Organization (WHO) functional class, Borg dyspnoea scale, plasma endothelin, serum values of disease severity such as uric acid, N-terminal-pro brain natriuretic peptide (NTproBNP), C-reactive protein measured by a highly sensitive method (CRPs) and other serum and haemodynamic parameters. RESULTS: after six months of treatment with bosentan, the PVR decreased from 852 (319) to 657(249) dyn*s*m-5 (p = 0.02). Quality of life considerably improved from a mean total score of 48(14) to 35(17) (p = 0.003) with improvements in the physical (from 25(5) to 17(7)) and emotional (from 11(6) to 6(5)) subscores (p = 0.005 and 0.011), respectively. The 6MWD improved from 389(78) to 443(79) meters (p = 0.005). 4 patients (27%) improved and 11 patients (73%) maintained their WHO class with no deterioration during the six months of bosentan treatment (p = 0.02). Uric acid serum levels declined from 525(145) to 453(151) micromol/l (p = 0.006), NTproBNP and CRPs declined insignificantly. Endothelin serum levels increased from 4.3(1.5) to 5.9(2.2) pg/ml (p = 0.025). Patients tolerated the treatment well, and there were no severe adverse events or deaths. CONCLUSION: this open-label study suggests a beneficial effect of bosentan therapy not only on pulmonary haemodynamics, but also on quality of life and exercise capacity for patients with severe CTEPH.
STUDY OBJECTIVES: we performed an open-label national study to evaluate the effects of Bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS AND METHODS: fifteen patients with CTEPH not eligible or waiting for surgery were enrolled. The primary endpoint was the change in pulmonary vascular resistance (PVR). Secondary endpoints included quality of life (measured by the Minnesota living with heart failure questionnaire, MLHF), 6 minute walk distance (6MWD), World Health Organization (WHO) functional class, Borg dyspnoea scale, plasma endothelin, serum values of disease severity such as uric acid, N-terminal-pro brain natriuretic peptide (NTproBNP), C-reactive protein measured by a highly sensitive method (CRPs) and other serum and haemodynamic parameters. RESULTS: after six months of treatment with bosentan, the PVR decreased from 852 (319) to 657(249) dyn*s*m-5 (p = 0.02). Quality of life considerably improved from a mean total score of 48(14) to 35(17) (p = 0.003) with improvements in the physical (from 25(5) to 17(7)) and emotional (from 11(6) to 6(5)) subscores (p = 0.005 and 0.011), respectively. The 6MWD improved from 389(78) to 443(79) meters (p = 0.005). 4 patients (27%) improved and 11 patients (73%) maintained their WHO class with no deterioration during the six months of bosentan treatment (p = 0.02). Uric acid serum levels declined from 525(145) to 453(151) micromol/l (p = 0.006), NTproBNP and CRPs declined insignificantly. Endothelin serum levels increased from 4.3(1.5) to 5.9(2.2) pg/ml (p = 0.025). Patients tolerated the treatment well, and there were no severe adverse events or deaths. CONCLUSION: this open-label study suggests a beneficial effect of bosentan therapy not only on pulmonary haemodynamics, but also on quality of life and exercise capacity for patients with severe CTEPH.
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: Neeraj Dhaun; Vanessa Melville; Scott Blackwell; Dinesh K Talwar; Neil R Johnston; Jane Goddard; David J Webb Journal: J Am Soc Nephrol Date: 2012-12-14 Impact factor: 10.121
Authors: Divay Chandra; Robert A Wise; Hrishikesh S Kulkarni; Roberto P Benzo; Gerard Criner; Barry Make; William A Slivka; Andrew L Ries; John J Reilly; Fernando J Martinez; Frank C Sciurba Journal: Chest Date: 2012-12 Impact factor: 9.410
Authors: Christian Nagel; Felix Prange; Stefan Guth; Jochen Herb; Nicola Ehlken; Christine Fischer; Frank Reichenberger; Stephan Rosenkranz; Hans-Juergen Seyfarth; Eckhard Mayer; Michael Halank; Ekkehard Grünig Journal: PLoS One Date: 2012-07-25 Impact factor: 3.240
Authors: Nicola Bonner; Linda Abetz; Juliette Meunier; Mirko Sikirica; Stephen C Mathai Journal: Health Qual Life Outcomes Date: 2013-10-03 Impact factor: 3.186