Derivation cohort | Validation cohort | p-value | |
Subjects n | 182 | 99 | |
Age yrs | 62 (48–73) | 53 (42–69) | 0.010 |
Females | 125 (69) | 72 (73) | 0.479 |
Aetiology | |||
Idiopathic PAH | 97 (53) | 96 (97) | |
Heritable PAH | 2 (1) | 3 (3) | |
CTD-PAH | |||
SSc associated | 33 (18) | ||
non-SSc associated | 26 (14) | ||
WHO Group I PAH others | |||
PoPH | 15 (8) | ||
HIV | 1 (0.5) | ||
PVOD | 8 (4) | ||
Lung function % pred | |||
FEV1 | 87 (75–98)# | 84±15 | 0.038 |
FVC | 99 (86–112)# | 97±17 | 0.094 |
DL,CO | 42 (28–60)¶ | 59±22## | <0.001 |
Pulmonary haemodynamics | |||
Pra mmHg | 7 (4–11) | 9±6 | 0.013 |
Mean Ppa mmHg | 47 (39–55) | 50 (42–59) | 0.016 |
CO L·min1 | 3.6 (2.9–4.7) | 3.2 (2.6–3.8) | 0.002 |
PVR Wood units | 10.8 (7.4–15.5) | 13.3±5.4 | 0.011 |
Sv,O2 % | 64 (57–70) | 63±8 | 0.889 |
WHO FC | |||
I and II | 25 (14) | 18 (18) | 0.572 |
III | 131 (72) | 66 (67) | |
IV | 26 (14) | 15 (15) | |
6MWD m | 260±109+ | 267±121 | 0.669 |
NT-pro-BNP pg·mL−1 | 1026 (298–2637)§ | 2029 (330–4407)¶¶ | 0.134 |
CAMPHOR | 38 (27–57)ƒ | 38 (26–57)++ | 0.804 |
Year of diagnosis | |||
Prior to 2005 | 51 (28) | 45 (45) | 0.003 |
2005 onwards | 131 (72) | 54 (55) |
Data are expressed as median (interquartile range), n (%) or mean±sd, unless otherwise stated. PAH: pulmonary arterial hypertension; CTD-PAH: connective tissue disease associated PAH; SSc: systemic sclerosis; WHO: World Health Organization; PoPH: portopulmonary hypertension; PVOD: pulmonary veno-occlusive disease; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; Pra: right atrial pressure; Ppa: pulmonary artery pressure; CO: cardiac output; PVR: pulmonary vascular resistance; Sv, O2: mixed venous saturation; FC: functional class; 6MWD: 6 min walk distance; NTproBNP: N-terminal pro-brain natriuretic peptide; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; #: n=171; ¶: n=158; +: n= 177; §: n=96; ƒ: n=76; ##: n=81; ¶¶: n=45; ++: n=49.