Table 2– Univariate Cox proportional hazards analysis in the derivation cohort
HR (95% CI)p-value
Age at diagnosis per decade increase yrs1.32 (1.12–1.57)0.010
 Female0.76 (0.47–1.23)0.260
 Male (reference)
Aetiology subgroups0.001
 WHO Group I PAH others#3.21 (1.79–5.76)<0.001
 CTD-PAH-SSc1.74 (0.98–3.09)0.057
 CTD-PAH non-SSc1.29 (0.67–2.50)0.445
 Idiopathic or heritable PAH (reference)
Lung function % pred
 FEV1 per 10% increase0.07 (0.00–3.02)0.165
 FVC per 10% increase0.32 (0.02–5.32)0.892
DL,CO per 10% increase0.80 (0.70–0.91)<0.001
Pulmonary haemodynamics
Pra per 5 mmHg1.30 (1.08–1.57)<0.001
 Mean Ppa per 5 mmHg1.03 (0.96–1.10)0.480
Sv,O2 per 5%0.95 (0.83–1.09)0.464
 PVR per 5 Wood units1.13 (0.95–1.33)0.170
 CO per L·min−10.83 (0.68–1.01)0.058
WHO FC0.010
 I and II0.30 (0.12–0.72)0.007
 III0.49 (0.28–0.85)0.010
 IV (reference)
6MWD per 100 m increase0.61 (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 increase1.16 (1.04–1.29)0.008
Year of diagnosis
 2005 onwards1.15 (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.