Bosentan-treated PPH patients | NIH Registry PPH patients# | |
Subjects n | 169 | 187 |
Sex male/female % | 21/79 | 37/63 |
Age yrs | ||
Mean±sd | 46±16 | 36±15 |
Range | 13–80 | 1–81 |
WHO functional class % | ||
I/II | 1/8¶ | 0/29 |
III/IV | 82/9 | 71 |
Time from diagnosis months+ | ||
Mean±sd | 32±41 | |
Range | 0.3–326 | |
Haemodynamics mean±sd+ | ||
Cardiac index L·min−1·m−2 | 2.35±0.80 | 2.3±0.9 |
PVR Wood units | 12.9±8.4 | |
mPAP mmHg | 57.1±16.0 | 60±18 |
mRAP mmHg | 10.1±5.9 | 9.7±6.3 |
Walk test m | ||
Mean±sd | 345±87 |
PPH: primary pulmonary hypertension; NIH: National Institutes of Health; WHO: World Health Organization; PVR: pulmonary vascular pressure; mPAP: mean pulmonary arterial pressure; mRAP: mean right atrial pressure. #: data from Rich et al. 10 provided for context, NIH Registry data were not directly used in any analysis; ¶: all patients entered the pivotal studies in functional class III or IV, but 15 (9%) patients in the placebo group improved before being switched to bosentan in the extension study; +: time from diagnosis and haemodynamic data were available for 157–169 bosentan-treated patients, and for most of these patients were available only at the start of the placebo-controlled study.