Patient characteristics
| PAH | PAH before treatment | PAH after treatment | CTD-PAH | IPAH/FPAH | |
| Sample size n (% female) | 48 (83) | 33 (88) | 33 (88) | 25 (92) | 23 (74) |
| Age years | 71.5 (64–76) | 71 (60.5–76.5) | 72 (61–76.5) | 71 (64.5–76) | 73 (57–77) |
| BSA m2 | 1.75 (1.6–1.95) | 1.73 (1.58–1.79) | 1.72 (1.58–1.78) | 1.7 (1.61–1.79) | 1.77 (1.6–1.98) |
| Comorbidities n | |||||
| Hypertension | 17 | 11 | NA | 5 | 12 |
| Diabetes mellitus | 12 | 8 | NA | 2 | 10 |
| Atrial fibrillation | 4 | 2 | NA | 2 | 2 |
| Stroke | 2 | 2 | NA | 0 | 2 |
| IHD | 7 | 5 | NA | 3 | 4 |
| Thyroid disease | 11 | 10 | NA | 6 | 5 |
| WHO-FC# n | |||||
| I | 1 | 1 | 2 | 0 | 1 |
| II | 9 | 6 | 10 | 6 | 3 |
| III | 28 | 22 | 15 | 12 | 16 |
| IV | 2 | 2 | 0 | 2 | 0 |
| 6MWD m | 242 (176.25–347.5) | 242 (191.25–344.5) | 270 (234–329.75)¶ | 267 (187.5–352) | 225 (162.5–277.5) |
| SvO2 % | 59.25 (51.35–66.12) | 62.3 (54.5–66.1) | 63.4 (59.3–72)¶ | 64.9 (54.6–71.2) | 55.2 (49.15–59.75)* |
| Haemodynamics | |||||
| mPAP mmHg | 43 (37–54.25) | 43 (37–55) | 36 (32–48)¶ | 39 (30–43) | 51 (42–56)* |
| PAWP mmHg | 8 (6–11) | 6 (5–9) | 8 (5–11) | 8 (5–10) | 9 (6–11.5) |
| mRAP mmHg | 7 (4–11) | 6 (3–9) | 6 (3–9) | 6 (3–9) | 9 (6–11)* |
| RVSP mmHg | 75.5 (62.75–86.75) | 76 (63–86) | 64 (50–77)¶ | 63 (55–76) | 83 (75–90.5)* |
| Cardiac index L⋅min−1⋅m−2 | 2.19 (1.77–2.77) | 2.25 (1.81–2.85) | 2.7 (2.17–3.34)¶ | 2.62 (1.96–3.05) | 1.9 (1.69–2.24)* |
| PVR Wood units | 9.5 (6.67–11.74) | 9.56 (7.02–11.88) | 5.79 (4.52–8.62)¶ | 6.88 (4.84–9.71) | 11.47 (9.15–13.46)* |
| SVR Wood units | 23.23 (19.29–28.34) | 23.13 (18.78–26.69) | 16.96 (13.16–21.41)¶ | 20.69 (18.19–25.41) | 26.01 (21.03–32.35)* |
| RVSWI mmHg⋅mL−1⋅m−2 | 990.48 (809.62–1228.42) | 981.18 (824.98–1219.54) | 1257.47 (928.66–1390.55)¶ | 958.15 (810.82–1140.7) | 1019.33 (809.58–1268.11) |
| LVSWI mmHg⋅mL−1⋅m−2 | 2488.28 (2047–3136.17) | 2579.42 (2059.81–3097.18) | 2779.15 (2486.08–3470.12)¶ | 2789.8 (2156.3–3474.15) | 2213.94 (1961.55–2978.13) |
Data are presented as median (interquartile range) or n, unless otherwise stated. PAH: pulmonary arterial hypertension; CTD: connective tissue disease; IPAH: idiopathic PAH; FPAH: familial PAH; BSA, body surface area; IHD, ischaemic heart disease; WHO-FC: World Health Organization functional class; 6MWD: 6-min walking distance; SvO2: mixed venous oxygen saturation; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; mRAP: mean right atrial pressure; RVSP: right ventricular systolic pressure; PVR: pulmonary vascular resistance; SVR: systemic vascular resistance; RVSWI: right ventricular stroke work index; LVSWI: left ventricular stroke work index; NA: not assessed. #: the total number of patients with functional class values does not match the sample size due to missing values;¶: indicates a significant difference before versus after treatment in PAH patients; *: indicates significant baseline differences between IPAH/FPAH and CTD-PAH.