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 |