HR (95% CI) | p-value | |
Age at diagnosis per decade increase yrs | 1.32 (1.12–1.57) | 0.010 |
Sex | ||
Female | 0.76 (0.47–1.23) | 0.260 |
Male (reference) | ||
Aetiology subgroups | 0.001 | |
WHO Group I PAH others# | 3.21 (1.79–5.76) | <0.001 |
CTD-PAH-SSc | 1.74 (0.98–3.09) | 0.057 |
CTD-PAH non-SSc | 1.29 (0.67–2.50) | 0.445 |
Idiopathic or heritable PAH (reference) | ||
Lung function % pred | ||
FEV1 per 10% increase | 0.07 (0.00–3.02) | 0.165 |
FVC per 10% increase | 0.32 (0.02–5.32) | 0.892 |
DL,CO per 10% increase | 0.80 (0.70–0.91) | <0.001 |
Pulmonary haemodynamics | ||
Pra per 5 mmHg | 1.30 (1.08–1.57) | <0.001 |
Mean Ppa per 5 mmHg | 1.03 (0.96–1.10) | 0.480 |
Sv,O2 per 5% | 0.95 (0.83–1.09) | 0.464 |
PVR per 5 Wood units | 1.13 (0.95–1.33) | 0.170 |
CO per L·min−1 | 0.83 (0.68–1.01) | 0.058 |
WHO FC | 0.010 | |
I and II | 0.30 (0.12–0.72) | 0.007 |
III | 0.49 (0.28–0.85) | 0.010 |
IV (reference) | ||
6MWD per 100 m increase | 0.61 (0.50–0.67) | <0.001 |
NT-pro-BNP per 200 pg·mL−1 increase | 1.02 (1.00–1.04) | 0.036 |
CAMPHOR per 5 points increase | 1.16 (1.04–1.29) | 0.008 |
Year of diagnosis | ||
2005 onwards | 1.15 (0.71–1.86) | 0.572 |
2000–2005 (reference) |
WHO: World Health Organization; PAH: pulmonary arterial hypertension; CTD-PAH: connective tissue disease associated PAH; SSc: systemic sclerosis; 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; Sv,O2: mixed venous saturation; PVR: pulmonary vascular resistance; CO: cardiac output; FC: functional class; 6MWD: 6-min walk distance; NT-pro-BNP: N-terminal-pro-brain natriuretic peptide; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review. #: includes portopulmonary hypertension, HIV and pulmonary veno-occlusive disease.