Table 1– Baseline characteristics of the derivation cohort (all patients versus patients in the aetiology reference subgroup)
All patientsAetiology reference subgroupp-value
Subjects n182125
Age yrs62 (48–73)61 (45–71)0.592
Females125 (69)81 (65)0.477
Aetiology
 Idiopathic PAH97 (53)97 (78)
 Heritable PAH2 (1)2 (2)
 CTD-PAH
  SSc associated33 (18)
  non-SSc associated26 (14)26 (21)
 WHO Group I PAH others
  PoPH15 (8)
  HIV1 (0.5)
  PVOD8 (4)
Lung function % pred
 FEV187 (75–98)#86±17##0.605
 FVC99 (86–112)#98±19##0.628
DL,CO42 (28–60)44 (28–63)¶¶0.645
Pulmonary haemodynamics
Pra mmHg7 (4–11)6 (4–10)0.421
 Mean Ppa mmHg47 (39–55)48 (40–58)0.459
 CO L·min−13.6 (2.9–4.7)3.6 (2.9–4.7)0.929
 PVR Wood units10.8 (7.4–15.5)11.2 (7.6–15.7)0.596
Sv,O2 %64 (57–70)65 (58–70)0.789
WHO FC
 I and II25 (14)19 (15)0.899
 III131 (72)87 (70)
 IV26 (14)19 (15)
6MWD m260±109+273±110++0.342
NT-pro-BNP pg·mL−11026 (298–2637)§972 (316–2258)§§0.856
CAMPHOR38 (27–57)ƒ40±18ƒƒ0.933
Year of diagnosis
 Prior to 200551 (28)36 (29)0.882
 2005 onwards131 (72)89 (71)
  • 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; S,vO2: mixed venous saturation; FC: functional class; 6MWD: 6-min walk distance; NT-pro-BNP: N-terminal-pro-brain natriuretic peptide; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review. #: n=171; : n=158; +: n=177; §: n=96; ƒ: n=76; ##: n=117; ¶¶: n=108; ++: n=122; §§: n=63; ƒƒ: n=51.