Lung function testing and cardiopulmonary exercise test parameters
| Placebo | Bosentan | p-value | |||
| Baseline | 12 weeks | Baseline | 12 weeks | ||
| Subjects n | 10 | 9 | 20 | 17 | |
| Body plethysmography | |||||
| Total lung capacity post-BD L | 6.89±0.91 | 6.70±1.08 | 7.23±1.45 | 7.32±1.30 | 0.372 |
| Total lung capacity post-BD % | 120±22 | 114±14 | 126±15 | 124±14 | 0.927 |
| Residual volume post-BD L | 3.80±1.07 | 3.46±0.69 | 4.18±1.11 | 4.40±0.97 | 0.095 |
| Residual volume post-BD % | 174±68 | 151±28 | 184±51 | 188±38 | 0.094 |
| FEV1 post-BD L | 1.05±0.39 | 1.07±0.38 | 0.92±0.27 | 0.86±0.26 | 0.379 |
| FEV1 post-BD % | 41±14 | 42±14 | 38 ±13 | 35±12 | 0.482 |
| FEV1/VC post-BD % | 35±8 | 35±11 | 32±10 | 31±9 | 0.320 |
| Diffusion capacity % | 44±13 | 42±13 | 37±18 | 35±18 | 0.842 |
| Cardiopulmonary exercise testing | |||||
| Respiratory quotient rest | 0.86±0.07 | 0.89±0.11 | 0.85±0.11 | 0.85±0.07 | 0.282 |
| Respiratory quotient max | 0.88±0.04 | 0.86±0.06 | 0.85±0.07 | 0.80±0.06 | 0.056 |
| fC rest beats·min−1 | 85±13.3 | 87±16 | 91.9±15 | 91±11 | 0.474 |
| fC max beats·min−1 | 115±20 | 115±14 | 108±21 | 114±21 | 0.626 |
| fC max % | 75±13 | 75±8 | 72±15 | 76±15 | 0.489 |
| O2 pulse at rest mL·beat−1 | 3.81±1.60 | 3.27±1.68 | 3.07±0.77 | 3.41±1.38 | 0.088 |
| O2 pulse max mL·beat−1 | 9.02±2.91 | 9.98±3.11 | 8.69±4.56 | 7.90±3.02 | 0.156 |
| O2 pulse max % | 75±15 | 75±18 | 93±52 | 82±31 | 0.293 |
| Minute ventilation rest L | 13.6±3.6 | 13.8±5.0 | 14.0±2.8 | 15.4±4.8 | 0.498 |
| Minute ventilation max L | 34.6±10.7 | 36.0±12.2 | 31.4±9.1 | 30.3±7.2 | 0.114 |
| Minute ventilation max % | 47±13 | 48±14 | 45±15 | 43±15 | 0.276 |
| V′O2 at rest mL·min·kg−1 | 4.1±1.0 | 4.9±1.9 | 4.3±0.8 | 3.5±1.7 | 0.288 |
| V′O2 max mL·min·kg−1 | 13.4±2.4 | 13.9±2.8 | 13.8±3.0 | 13.4±3.4 | 0.113 |
| V′O2 max % | 63±11 | 64±15 | 54±12 | 50±12 | 0.304 |
| O2 saturation at rest % | 92±4 | 93±2 | 93±3 | 91±4 | 0.035 |
| O2 saturation at max % | 77±17 | 84±5 | 83±6 | 79±13 | 0.842 |
Data are presented as mean±sd, unless otherwise stated. BD: bronchodilator; FEV1: forced expiratory volume in one second; VC: vital capacity; fC: cardiac frequency; V′O2: maximal oxygen uptake.