All patients | Aetiology reference subgroup | p-value | |
Subjects n | 182 | 125 | |
Age yrs | 62 (48–73) | 61 (45–71) | 0.592 |
Females | 125 (69) | 81 (65) | 0.477 |
Aetiology | |||
Idiopathic PAH | 97 (53) | 97 (78) | |
Heritable PAH | 2 (1) | 2 (2) | |
CTD-PAH | |||
SSc associated | 33 (18) | ||
non-SSc associated | 26 (14) | 26 (21) | |
WHO Group I PAH others | |||
PoPH | 15 (8) | ||
HIV | 1 (0.5) | ||
PVOD | 8 (4) | ||
Lung function % pred | |||
FEV1 | 87 (75–98)# | 86±17## | 0.605 |
FVC | 99 (86–112)# | 98±19## | 0.628 |
DL,CO | 42 (28–60)¶ | 44 (28–63)¶¶ | 0.645 |
Pulmonary haemodynamics | |||
Pra mmHg | 7 (4–11) | 6 (4–10) | 0.421 |
Mean Ppa mmHg | 47 (39–55) | 48 (40–58) | 0.459 |
CO L·min−1 | 3.6 (2.9–4.7) | 3.6 (2.9–4.7) | 0.929 |
PVR Wood units | 10.8 (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 II | 25 (14) | 19 (15) | 0.899 |
III | 131 (72) | 87 (70) | |
IV | 26 (14) | 19 (15) | |
6MWD m | 260±109+ | 273±110++ | 0.342 |
NT-pro-BNP pg·mL−1 | 1026 (298–2637)§ | 972 (316–2258)§§ | 0.856 |
CAMPHOR | 38 (27–57)ƒ | 40±18ƒƒ | 0.933 |
Year of diagnosis | |||
Prior to 2005 | 51 (28) | 36 (29) | 0.882 |
2005 onwards | 131 (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.