Patient characteristics
| Baseline | Study enrolment | p-value | |
| Male/female | 3/29 | ||
| Age years | 28.5±10.3 | 33.0±10.1 | <0.01 |
| Body mass index kg·m−2 | 20.7±3.0 | 19.8±2.6 | 0.09 |
| WHO functional class# (I/II/III/IV) | III (0/6/14/12) | II (7/25/0/0) | <0.01 |
| PAH-specific therapy | |||
| Intravenous epoprostenol | 1 (3) | 26 (81) | <0.01 |
| Beraprost | 11 (34) | 5 (16) | 0.18 |
| Endothelin receptor antagonist | 9 (28) | 30 (94) | <0.01 |
| Phosphodiesterase type 5 inhibitor | 6 (19) | 21 (66) | <0.01 |
| Soluble guanylate cyclase stimulator | 0 (0) | 1 (3) | |
| Combination therapy | |||
| Dual therapy | 2 (6) | 13 (41) | <0.01 |
| Triple therapy | 4 (13) | 19 (59) | <0.01 |
| Laboratory parameters | |||
| BNP pg·mL−1 | 334.5 (8.7–1687.6) | 10.3 (5.8–131.7) | <0.01 |
| Respiratory parameters | |||
| Vital capacity % predicted | 89.9±17.2 | 93.7±14.4 | 0.03 |
| FEV1/FVC % | 84.1±7.3 | 82.5±7.2 | 0.24 |
| DLCO % predicted | 58.5±12.6 | 66.3±10.8 | <0.01 |
| Pulmonary haemodynamic parameters | |||
| PAWP mmHg | 8.3±3.4 | 7.9±3.2 | 0.57 |
| mPAP mmHg | 61.8±18.3 | 28.7±12.2 | <0.01 |
| Cardiac index L·min−1·m−2 | 2.17±0.66 | 3.59±0.89 | <0.01 |
| PVR Wood units | 18.3±8.0 | 4.3±2.9 | <0.01 |
| Exercise capacity | |||
| 6MWD m | 287.3±117.1 | 449.7±69.2 | <0.01 |
Data are presented as n, mean±sd, n (%) or median (range), unless otherwise stated. WHO: World Health Organization; PAH: pulmonary arterial hypertension; BNP: brain natriuretic peptide; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; PAWP: pulmonary artery wedge pressure; mPAP: mean pulmonary arterial pressure; PVR: pulmonary vascular resistance; 6MWD: 6-min walk distance. #: median (n).