TABLE 2

Univariate analysis of the overall study cohort

	Full cohort^#			IPAH^¶			PH-CLD⁺
Characteristic	HR	95% CI	p-value	HR	95% CI	p-value	HR	95% CI	p-value
CT – centrilobular ground-glass (CGG)	0.29	0.17–0.50	<0.001	0.44	0.25–0.78	0.005
CT – ground-glass opacification (GGO)	0.53	0.38–0.74	<0.001	0.52	0.32–0.86	0.010	0.72	0.46–1.14	0.2
CT – honeycombing	2.79	1.57–4.99	<0.001	3.72	1.37–10.1	0.010	2.11	1.04–4.30	0.039
CT – consolidation	0.84	0.50–1.40	0.5	1.10	0.45–2.68	0.8	0.60	0.32–1.13	0.11
CT – fibrosis	2.38	1.94–2.91	<0.001	2.48	1.76–3.50	<0.001	1.83	1.42–2.35	<0.001
CT – fibrosis (any present, ref: none)
None	—	—		—	—		—	—
Mild	1.94	1.46–2.58	<0.001	2.51	1.72–3.66	<0.001	1.73	1.11–2.71	0.016
Moderate	2.77	1.99–3.85	<0.001	4.53	2.07–9.92	<0.001	1.71	1.18–2.48	0.005
Severe	3.19	2.30–4.43	<0.001				1.98	1.40–2.80	<0.001
CT – emphysema	2.09	1.71–2.56	<0.001	2.74	1.96–3.81	<0.001	1.13	0.87–1.47	0.4
CT – emphysema (any present, ref: none)
None	—	—		—	—		—	—
Mild	1.78	1.30–2.43	<0.001	2.90	1.89–4.46	<0.001	0.89	0.56–1.42	0.6
Moderate	2.18	1.69–2.81	<0.001	3.16	2.01–4.97	<0.001	1.13	0.82–1.55	0.5
Severe	2.92	2.15–3.97	<0.001	11.1	3.92–31.6	<0.001	1.37	0.97–1.93	0.075
CT – CPFE	2.20	1.72–2.80	<0.001	2.09	1.33–3.29	0.001	1.82	1.36–2.44	<0.001
Age at diagnosis years	1.05	1.04–1.05	<0.001	1.06	1.05–1.08	<0.001	1.02	1.01–1.04	<0.001
Male sex	1.66	1.36–2.03	<0.001	1.59	1.16–2.18	0.004	1.42	1.10–1.83	0.007
WHO functional class III and IV (ref: I and II)	1.78	1.45–2.18	<0.001	1.88	1.34–2.64	<0.001	1.74	1.35–2.25	<0.001
WHO functional class
II	—	—		—	—		—	—
III	2.57	1.70–3.89	<0.001	3.00	1.51–5.96	0.002	2.45	1.46–4.13	<0.001
IV	5.08	3.31–7.79	<0.001	5.49	2.69–11.2	<0.001	4.80	2.81–8.21	<0.001
mPAP mmHg	0.99	0.98–1.00	0.028	0.98	0.97–0.99	0.006	1.04	1.03–1.05	<0.001
mRAP mmHg	1.01	0.99–1.03	0.4	1.03	1.00–1.06	0.023	1.03	1.00–1.05	0.057
PAWP mmHg	1.02	0.99–1.04	0.3	1.03	0.98–1.09	0.2	0.98	0.95–1.01	0.2
Cardiac output L·min⁻¹	0.92	0.86–0.98	0.009	0.88	0.78–0.99	0.035	0.79	0.72–0.86	<0.001
Cardiac index L·min⁻¹·m⁻²	0.87	0.76–0.98	0.028	0.80	0.63–1.01	0.063	0.61	0.51–0.74	<0.001
PVR (Wood Units)	1.04	1.00–1.08	0.036	0.98	0.91–1.05	0.5	1.10	1.05–1.16	<0.001
S_vO₂%	0.98	0.97–0.99	0.001	0.97	0.95–0.98	<0.001	0.96	0.94–0.97	<0.001
FEV₁ % predicted	1.00	0.99–1.00	0.032	0.99	0.98–1.00	0.11	1.01	1.01–1.02	<0.001
FVC % predicted	1.00	0.99–1.00	0.028	1.00	0.99–1.01	0.9	1.00	1.00–1.01	0.4
FEV₁/FVC ratio	0.99	0.99–1.00	0.038	0.97	0.95–0.98	<0.001	1.02	1.01–1.03	<0.001
D_LCO % predicted	0.95	0.95–0.96	<0.001	0.95	0.94–0.96	<0.001	0.96	0.95–0.97	<0.001

Bold text: meets statistical significance. IPAH: idiopathic pulmonary arterial hypertension; PH-CLD: PH due to chronic lung disease and/or hypoxia; HR: hazard ratio; CI: confidence interval; CT: computed tomography; WHO: World Health Organisation; CPFE: combined pulmonary fibrosis and emphysema; mPAP: mean pulmonary arterial pressure; mRAP: mean right atrial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; S_vO₂: mixed venous oxygen saturation; FEV₁: forced expiratory volume in 1 s; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide. ^#: n=660; ^¶: n=335; ⁺: n=325.