Baseline clinical, laboratory and haemodynamic characteristics of the study cohort by PAH-type
| IPAH | CTD-PAH | Total | p-value | |
| Subjects n | 305 | 197 | 502 | |
| Demography and clinical data | ||||
| Age years | 68 (20) | 68 (11) | 68 (16) | 0.769 |
| Female gender % | 57 | 78 | 65 | <0.001 |
| BMI kg·m−2 | 26 (6.5) | 24 (5.8) | 25 (6) | <0.001 |
| WHO-FC (I/II/III/IV) | 1/15/74/10 | 1/19/67/13 | 1/17/71/11 | 0.307 |
| 6MWD m | 250 (211) | 288 (207) | 267 (210) | <0.001 |
| SBP mmHg | 130 (32) | 131 (31) | 130 (31) | 0.906 |
| DBP mmHg | 75 (21) | 75 (15) | 75 (19) | 0.722 |
| eGFR mL/min/1.73 m2 | 64 (35) | 67 (34) | 65 (35) | 0.796 |
| Hb g·L−1 | 147 (24) | 132 (23) | 141 (27) | <0.001 |
| NTproBNP ng·L−1 | 1792 (3354) | 1245 (3081) | 1573 (3077) | 0.004 |
| DLCO % pred mean±sd | 49±22 | 41±15 | 46 ±20 | <0.001 |
| Comorbidity (%) | ||||
| Systemic hypertension | 44 | 35 | 40 | 0.035 |
| Diabetes mellitus | 25 | 10 | 19 | <0.001 |
| Ischaemic stroke | 5 | 5 | 5 | 0.843 |
| Ischaemic heart disease | 14 | 16 | 15 | 0.712 |
| Atrial fibrillation | 14 | 11 | 13 | 0.435 |
| Interstitial lung disease | 0 | 32 | 13 | <0.001 |
| Obesity | 25 | 11 | 19 | <0.001 |
| Kidney dysfunction | 51 | 48 | 49 | 0.547 |
| Haemodynamics | ||||
| MRAP mmHg | 8 (6) | 5 (7) | 7 (7) | <0.001 |
| MPAP mmHg | 48 (13) | 38 (15) | 45 (16) | <0.001 |
| PCWP mmHg | 9 (6) | 8 (6) | 8 (5) | 0.012 |
| CI L·min−1·m−2 | 2.2 (0.8) | 2.4 (1.0) | 2.3 (0.8) | <0.001 |
| PVR Wood units | 10 (5) | 7 (6) | 9 (6) | <0.001 |
| SaO2 % | 91 (8) | 93 (6) | 92 (7) | 0.001 |
| SvO2 % | 60 (13) | 64 (14) | 61 (11) | <0.001 |
| Heart rate bpm | 76 (19) | 79 (22) | 77 (22) | 0.042 |
| PAH-targeted therapy (%)# | ||||
| Single | 65 | 69 | 67 | 0.383 |
| Dual | 21 | 19 | 20 | 0.733 |
| Triple | 1 | 1 | 1 | 0.710 |
| No treatment registered | 13 | 11 | 12 | 0.573 |
| Supportive therapy (%) | ||||
| Anticoagulants | 58 | 42 | 51 | 0.003 |
| Diuretics | 67 | 51 | 61 | <0.001 |
| Supplemental oxygen | 32 | 25 | 29 | 0.169 |
| Risk group | ||||
| Low/medium/high % | 12/68/20 | 19/69/12 | 14/69/17 | 0.003 |
| Transplantation-free survival | ||||
| 1 year, total (female/male), % | 83 (87/79) | 80 (81/74) | 82 (84/78) | 0.343 |
Data are presented as median (interquartile range) or %, unless otherwise indicated. Bold indicates statistical significance at p<0.05. PAH: pulmonary arterial hypertension; IPAH: idiopathic/hereditary pulmonary arterial hypertension; CTD-PAH: connective tissue disease-associated pulmonary arterial hypertension; BMI: body mass index; WHO-FC: World Health Organisation functional class; 6MWD: 6-min walk distance; SBP: systolic blood pressure; DBP: diastolic blood pressure; eGFR: estimated glomerular filtration rate; Hb: haemoglobin; NTproBNP: N-terminal pro-brain natriuretic peptide; DLCO: diffusing capacity of the lung for carbon monoxide; mRAP: mean right atrial pressure; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; SaO2: arterial oxygen saturation; SvO2: mixed venous oxygen saturation. # Started within 3 months from diagnosis.