Baseline characteristics at initiation of combination therapy
| All patients | IPAH/HPAH | PAH-CHD | PAH-CTD | |
| Patients n | 181 | 102 | 61 | 29 |
| Male % | 40 | 48 | 38 | 17 |
| Age at initiation of combination therapy years | 50 (36–62) | 50 (36–62) | 45 (31–54) | 66 (58–72) |
| Duration of monotherapy months | 16 (5–39) | 14 (4–35) | 26 (8–51) | 13 (6–27) |
| New York Heart Association functional class III/IV % | 52 | 54 | 39 | 72 |
| 6-min walking distance m | 425 (307–491) | 441 (271–516) | 428 (383–494) | 360 (219–425) |
| Right atrial pressure mmHg | 9 (7–13) | 8 (7–12) | 9 (7–12) | 12 (7–16) |
| Mean pulmonary arterial pressure mmHg | 62 (51–75) | 59 (49–69) | 78 (65–90) | 50 (44–56) |
| Pulmonary wedge pressure mmHg | 10 (8–12) | 10 (8–12) | 11 (9–12) | 9 (6–12) |
| Mean systemic arterial pressure mmHg | 85 (77–93) | 85 (77–91) | 82 (75–94) | 89 (82–97) |
| Cardiac index L·min−1·m−2 | 2.2 (1.9–2.6) | 2.3 (1.9–2.7) | 2.1 (1.8–2.6) | 2.1 (1.9–2.4) |
| Pulmonary vascular resistance WU | 13 (10–18) | 12 (9–16) | 17 (13–27) | 13 (8–14) |
| Systemic vascular resistance WU | 20 (16–24) | 18 (16–23) | 21 (16–27) | 20 (18–25) |
| Arterial oxygen saturation % | 93 (90–96) | 95 (92–97) | 90 (84–95) | 94 (92–97) |
| Pulmonary arterial oxygen saturation % | 64 (57–69) | 62 (56–67) | 68 (61–73) | 60 (51–64) |
Data are presented as median (interquartile range) unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; CHD: congenital heart disease; CTD: connective tissue disease; WU: Wood units.