Table. 1—

1 Demographics and baseline characteristics

Bosentan-treated PPH patientsNIH Registry PPH patients#
Subjects n169187
Sex male/female %21/7937/63
Age yrs
 Mean±sd46±1636±15
 Range13–801–81
WHO functional class %
 I/II1/80/29
 III/IV82/971
Time from diagnosis months+
 Mean±sd32±41
 Range0.3–326
Haemodynamics mean±sd+
 Cardiac index L·min−1·m−22.35±0.802.3±0.9
 PVR Wood units12.9±8.4
 mPAP mmHg57.1±16.060±18
 mRAP mmHg10.1±5.99.7±6.3
Walk test m
 Mean±sd345±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.