| All patients | Patients with IPAH | |||
| HR (95% CI) | p-value | HR (95% CI) | p-value | |
| Male sex | 1.38 (1.03–1.83) | 0.03 | 2.25 ( 1.31–3.72) | 0.002 |
| WHO FC III–IV | 1.32 (0.95–1.84) | 0.134 | 2.39 (1.64–4.92) | 0.018 |
| Pra 5 mmHg | 1.30 (1.15–1.47) | <0.001 | 1.46 (1.11–1.91) | 0.007 |
| Cardiac index 1 L·min−1·m−2 | 0.61 (0.51–0.75) | <0.001 | 0.68 (0.44–1.05) | 0.079 |
| Subtype of PH | 0.010 | |||
| IPAH | Reference | |||
| CTD | 1.78 (0.19–2.64) | 0.005 | ||
| CHD | 0.86 (0.51–1.46) | 0.583 | ||
| CTEPH | 1.08 (0.71–1.66) | 0.720 | ||
| HIV | 1.05 (0.57–1.94) | 0.883 | ||
| PoPH | 3.70 (2.28–6.03) | <0.001 | ||
| TOS | 0.69 (0.37–1.28) | 0.238 | ||
| PVOD | 3.11 (1.25–7.74) | 0.015 | ||
| ≥2 aetiologies | 1.21 (0.44–2.80) | 0.710 | ||
| Others | 1.49 (0.79–2.81) | 0.213 | ||
IPAH: idiopathic pulmonary arterial hypertension; HR: hazard ratio; WHO: World Health Organization; FC: functional class; Pra: right atrial pressure; PH: pulmonary hypertension; CTD: connective tissue disease; CHD: congenital heart disease; CTEPH: chronic thromboembolic pulmonary hypertension; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: pulmonary veno-occlusive disease.