Characteristics of the patients included in the baseline risk stratification group
| N | Low risk | Intermediate risk | High risk | All | |
| Subjects n | 196 | 1116 | 276 | 1588 | |
| Age years | 53±18 | 66±14 | 65±17 | 64±16 | |
| Female | 66 | 63 | 66 | 64 | |
| BMI kg·m−2 | 26±5 | 28±6 | 28±7 | 28±6 | |
| PAH aetiology | |||||
| I/D/H-PAH | 101 (52) | 760 (68) | 199 (72) | 1060 (67) | |
| CTD-PAH | 52 (27) | 234 (21) | 61 (22) | 347 (22) | |
| HIV-PAH | 4 (2) | 16 (1) | 2 (1) | 22 (1) | |
| PoPH | 20 (10) | 60 (5) | 9 (3) | 89 (6) | |
| CHD-PAH | 19 (10) | 46 (4) | 5 (2) | 70 (4) | |
| WHO FC class I/II/III/IV | 1530 | 2/47/43/0 (unknown n=8) | 0/8/78/11 (unknown n=3) | 0/1/53/43 (unknown n=3) | 0/11/70/15 (unknown n=4) |
| 6MWD m | 1262 | 442±100 | 299±109 | 186±103 | 298±126 |
| NT-proBNP ng·L−1 | 1003 | 151 (86–351) | 1404 (597–3042) | 4006 (2519–6743) | 1573 (526–3498) |
| BNP ng·L−1 | 249 | 44 (25–96) | 180 (93–377) | 549 (418–784) | 236 (101–523) |
| Haemodynamics | |||||
| Right atrial pressure mmHg | 1506 | 5±3 | 8±4 | 13±5 | 8±5 |
| mPAP mmHg | 1588 | 43±13 | 44 ±12 | 50±12 | 45±13 |
| PAWP mmHg | 1588 | 8±3 | 9±4 | 10±3 | 9±3 |
| Cardiac index L·min−1·m−2 | 1497 | 3.1±0.7 | 2.3±0.7 | 1.6±0.4 | 2.3±0.8 |
| PVR dyn·s·cm−5 | 1588 | 547±273 | 743±372 | 1149±522 | 784±431 |
| SvO2 % | 1420 | 72±5 | 64±8 | 53±8 | 63±9 |
| Initial therapy (within 3 months after diagnosis) | |||||
| CCB | 9% | 4% | 1% | 4% | |
| ERA | 43% | 36% | 45% | 39% | |
| PDE-5i/sGC | 63% | 72% | 78% | 72% | |
| PCA | 1% | 2% | 7% | 3% | |
| Monotherapy | 81% | 86% | 73% | 83% | |
| Combination therapy | 19% | 14% | 27% | 17% | |
| Anticoagulation | 31% | 44% | 48% | 43% |
Data are presented as mean±sd, n (%) or median (interquartile range), unless otherwise stated. BMI: body mass index; PAH: pulmonary arterial hypertension; I/D/H: idiopathic, drug-associated or hereditary; CTD: connective tissue disease; PoPH: portopulmonary hypertension; CHD: congenital heart disease; WHO FC: World Health Organization functional class; 6MWD: 6-min walking distance; NT-proBNP: N-terminal fragment of pro-brain natriuretic peptide; BNP: brain natriuretic peptide; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; CCB: calcium channel blocker; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase-5 inhibitor; sGC: stimulator of soluble guanylate cyclase; PCA: prostacyclin analogue.