Baseline treatment-naive patient characteristics
| All | IPAH/HPAH/DPAH | CTD-PAH | CHD-PAH | |
| N, (%) | 725 (100) | 405 (55.9) | 173 (23.9) | 147 (20.2) |
| Male, n (%) | 226 (31) | 155 (38) | 16 (9) | 55 (37) |
| Age at diagnosis, years | 51 (32–67) | 51 (34–65) | 66 (55–73) | 30 (14–43) |
| Age at 1st Bologna evaluation, years | 53 (36–67) | 51 (35–65) | 66 (55–73) | 38 (26–52) |
| Aetiology, n (%) | IPAH 331 (82) HPAH 63 (15) DPAH 11 (3) | SSc 138 (80) Undiff/Mixed 16 (9) SLE 11 (6) Rheumatoid arthritis 5 (3) Sjögren’s syndrome 3 (2) | Eisenmenger 86 (59) Left-right shunt 23 (16) Small 8 (5) Corrected 30 (20) | |
| WHO FC III–IV, n (%) | 494 (68) | 279 (69) | 143 (83) | 72 (49) |
| 6MWD, m | 389 (290–468) n=686 | 402 (311–497) n=394 | 317 (232–402) n=152 | 421 (340–474) n=140 |
| BNP (ng/L) | 146 (53–313) n=72 | 135 (51–320) n=43 | 152 (47–471) n=24 | 175 (54–202) n=5 |
| NT-pro-BNP (ng/L) | 807 (268–2237) n=332 | 598 (247–1810) n=189 | 1771 (632–4054) n=79 | 720 (187–1283) n=64 |
| RAP, mm Hg | 7 (4–10) | 7 (4–10) | 7 (4–12) | 6 (4–9) |
| mPAP, mm Hg | 53 (42–64) | 52 (42–62) | 46 (38–55) | 69 (51–83) |
| PAWP, mm Hg | 8 (6–10) | 8 (6–10) | 8 (6–10) | 9 (7–11) |
| mBP, mm Hg | 90 (82–99) | 90 (82–99) | 92 (85–104) | 86 (78–93) |
| CI, L/min/m2 | 2.4 (1.9–2.9) | 2.3 (1.9–2.8) | 2.3 (1.9–2.9) | 2.4 (1.9–3.2) |
| PVR, Wood units | 11 (7–16) | 11 (8–16) | 9 (7–13) | 15 (10–23) |
| SVR, Wood units | 21 (17–26) | 21 (17–26) | 21 (17–28) | 21 (17–26) |
| Art O2 saturation, % | 95 (92–97) | 95 (93–97) | 95 (93–97) | 90 (83–95) |
| SvO2 saturation, % | 65 (57–71) | 64 (57–70) | 63 (54–69) | 70 (64–76) |
All non-percentage values are median (25th–75th percentile).
Art O2, arterial oxygen; BNP, brain natriuretic peptide; CHD, congenital heart disease; CI, cardiac index; CTD, connective tissue disease; DPAH, drug-induced PAH; HPAH, heritable PAH; IPAH, idiopathic PAH; mBP, mean blood pressure; mPAP, mean pulmonary arterial pressure; 6MWD, 6 min walk distance; NT-pro-BNP, N-terminal probrain natriuretic peptide; PAH, pulmonary arterial hypertension; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistanc; RAP, right atrial pressure; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; SvO2, mixed venous oxygen saturation; SVR, systemic vascular resistance; WHO FC, WHO functional class.