Demographic and clinical characteristics for stable (no observed disease progression over the 3 years of follow-up) and disease-progression patient groups as guideline-defined
| Characteristics | All PAH patients | Disease progression | Stable | p-value | |||
| n | Centrality | n | Centrality | n | Centrality | ||
| Age (years) | 55 | 57.8±15.3 | 25 | 58.1±16.5 | 30 | 57.6±14.7 | 0.915 |
| Female, n (%) | 55 | 35 (64) | 25 | 14 (56) | 30 | 21 (70) | 0.282 |
| Incident/prevalent, n (%) | 55 | 12 (22)/43 (78) | 25 | 6 (24)/19 (76) | 30 | 6 (20)/24 (80) | 0.720 |
| Aetiology, n (%) | 55 | 25 | 30 | 0.038 | |||
| Congenital | 1 (1.8) | 0 (0) | 1 (3.3) | ||||
| Histiocytosis | 2 (3.6) | 1 (4) | 1 (3.3) | ||||
| Idiopathic | 40 (72.7) | 22 (88) | 18 (60) | ||||
| CTEPH | 12 (21.8) | 2 (8) | 10 (33.3) | ||||
| PAH treatments, n (%) | 55 | 25 | 30 | 0.003 | |||
| No treatment | 1 (2) | 0 (0) | 1 (3.3) | ||||
| Monotherapy | 20 (36) | 4 (16) | 16 (53.3) | ||||
| Bi-therapy | 26 (47) | 18 (72) | 8 (26.7) | ||||
| Tri-therapy# | 8 (15) | 3 (12) | 5 (16.7) | ||||
| Oxygen, n (%) | 55 | 24 (44) | 25 | 13 (52) | 30 | 11 (36.67) | 0.253 |
| Comorbidities, n (%) | 55 | 38 (69) | 25 | 17 (68) | 30 | 21 (70) | |
| NYHA I/II/III/IV, n (%) | 55 | 8 (15)/25 (45)/19 (35)/3 (5) | 25 | 0 (0)/12 (48)/11 (44)/2 (8) | 30 | 8 (26.7)/13 (43.3)/8 (26.7)/1 (3.3) | 0.018 |
| Right heart catheterisation | |||||||
| Cardiac output (L·min−1) | 51 | 5.3±1.9 | 24 | 5.8±2.0 | 27 | 4.9±1.7 | 0.114 |
| mPAP (mmHg) | 52 | 44.5 (38.5–51) | 25 | 48 (40–64) | 27 | 43 (29–48) | 0.012 |
| PCWP (mmHg) | 44 | 9.3±3.6 | 21 | 10.1±3.4 | 23 | 8.7±3.8 | 0.221 |
| RAP (mmHg) | 34 | 8 (5–10) | 15 | 10 (7–12) | 19 | 8 (3–9) | 0.058 |
| PVR (Wood's units) | 50 | 8.6 (6.2–11.1) | 24 | 8.7 (7.0–12.3) | 26 | 8.4 (6.1–9.7) | 0.472 |
| 6-min walk test | |||||||
| Distance (6MWD) | 52 | 386±131 | 23 | 336±116 | 29 | 425±131 | 0.013 |
| 6MWD <160 m, n (%) | 3 (5.8) | 2 (8.7) | 1 (3.5) | 0.081 | |||
| 160 m≤6MWD≤400 m, n (%) | 29 (55.8) | 16 (69.6) | 13 (44.8) | ||||
| 6MWD >400 m, n (%) | 20 (38.5) | 5 (21.7) | 15 (51.7) | ||||
| Max 6MWD Borg score | 52 | 4.8±2.7 | 23 | 4.8±2.9 | 29 | 4.8±2.5 | 0.984 |
| Cardiac frequency (bpm) | |||||||
| Baseline | 17 | 77 (70–92) | 5 | 75 (69–92) | 12 | 78 (62–109) | 0.966 |
| End | 16 | 108.5 (96–123.5) | 6 | 121.5 (97–133) | 10 | 104 (95–115) | 0.209 |
| Oxygen saturation (%) | |||||||
| Baseline | 38 | 95.5 (92–97) | 14 | 92.5 (87–96) | 24 | 96 (94–97.5) | 0.039 |
| End | 43 | 88 (78–92) | 19 | 79 (73–88) | 24 | 89 (87–95.5) | 0.002 |
| Delta SpO2≥4% | 33 | 25 (76) | 12 | 11 (92) | 21 | 14 (67) | 0.282 |
Data are presented as mean±sd or median (interquartile range), unless otherwise stated. PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; NYHA: New York Heart Association; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; RAP: right arterial pressure; PVR: pulmonary vascular resistance; 6MWD: 6-min walking distance; SpO2: oxygen saturation measured by pulse oximetery. #: ERA, IPDE-5, PGI2.