Table 4– Characteristics of the validation cohort
Derivation cohortValidation cohortp-value
Subjects n18299
Age yrs62 (48–73)53 (42–69)0.010
Females125 (69)72 (73)0.479
Aetiology
 Idiopathic PAH97 (53)96 (97)
 Heritable PAH2 (1)3 (3)
 CTD-PAH
 SSc associated33 (18)
 non-SSc associated26 (14)
 WHO Group I PAH others
  PoPH15 (8)
  HIV1 (0.5)
  PVOD8 (4)
Lung function % pred
 FEV187 (75–98)#84±150.038
 FVC99 (86–112)#97±170.094
DL,CO42 (28–60)59±22##<0.001
Pulmonary haemodynamics
Pra mmHg7 (4–11)9±60.013
 Mean Ppa mmHg47 (39–55)50 (42–59)0.016
 CO L·min13.6 (2.9–4.7)3.2 (2.6–3.8)0.002
 PVR Wood units10.8 (7.4–15.5)13.3±5.40.011
Sv,O2 %64 (57–70)63±80.889
WHO FC
 I and II25 (14)18 (18)0.572
 III131 (72)66 (67)
 IV26 (14)15 (15)
6MWD m260±109+267±1210.669
NT-pro-BNP pg·mL−11026 (298–2637)§2029 (330–4407)¶¶0.134
CAMPHOR38 (27–57)ƒ38 (26–57)++0.804
Year of diagnosis
 Prior to 200551 (28)45 (45)0.003
 2005 onwards131 (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.