Functional class, echocardiography, PFT, and hemodynamic variables at baseline.
| Variables | Monotherapy | Sequential Combination | Upfront Combination | Global p | p, Monotherapy vs Sequential | p, Monotherapy vs Upfront |
|---|---|---|---|---|---|---|
| Functional class | ||||||
| I | 6 (18) | 4 (17) | 2 (13) | 0.897 | 1.000 | 1.000 |
| II | 9 (26) | 6 (25) | 6 (38) | 0.654 | 1.000 | 0.514 |
| III | 13 (38) | 11 (46) | 8 (50) | 0.701 | 0.598 | 0.543 |
| IV | 2 (5.9) | 1 (4.2) | 0 | 0.616 | 1.000 | 1.000 |
| FVC, % | 67.6 ± 19.6 | 71.7 ± 22.6 | 59.7 ± 23.8 | 0.500 | 0.661 | 0.403 |
| DLCO, % | 25.3 ± 13.5 | 43.6 ± 18.6 | 38.1 ± 16.9 | 0.183 | 0.080 | 0.218 |
| FEV1, % | 63.4 ± 6.8 | 63.3 ± 6.8 | 64.7 ± 6.7 | 0.894 | 0.988 | 0.664 |
| FVC/DLCO | 2.9 ± 1.1 | 1.8 ± 0.4 | 2.3 ± 0.8 | 0.085 | 0.043 | 0.365 |
| sPAP by echocardiography, mmHg | 62.2 ± 21.5 | 64.0 ± 14.3 | 79.3 ± 31.9 | 0.260 | 0.834 | 0.152 |
| TRV, m/s | 3.7 ± 0.7 | 3.7 ± 0.6 | 3.9 ± 1.0 | 0.798 | 0.973 | 0.532 |
| TAPSE, mm | 19.5 ± 1.3 | 20.5 ± 2.1 | 13.1 ± 9.6 | 0.319 | 0.495 | 0.275 |
| Pericardial effusion | 3 (19) | 2 (22) | 4 (50) | 0.248 | 1.000 | 0.167 |
| Right ventricular dilation | 7 (44) | 5 (56) | 4 (67) | 0.608 | 0.688 | 0.635 |
| mPAP, mmHg | 37.2 ± 8.7 | 40.8 ± 8.8 | 46.0 ± 15.9 | 0.026 | 0.120 | 0.046 |
| PCWP, mmHg | 12.8 ± 6.2 | 11.1 ± 4.1 | 14.2 ± 5.0 | 0.254 | 0.303 | 0.467 |
| Cardiac output, l/min | 4.2 ± 1.3 | 4.3 ± 1.7 | 4.2 ± 1.7 | 0.963 | 0.775 | 0.898 |
| PVR, Wood units | 8.2 ± 5.9 | 8.1 ± 3.8 | 8.3 ± 4.9 | 0.994 | 0.947 | 0.966 |
Values are mean ± SD or n (%) unless otherwise specified. PFT: pulmonary functional tests; FVC: forced vital capacity; FEVI: forced expiratory volume in the first second; sPAP: systolic pulmonary arterial pressure; TRV: tricuspid regurgitation velocity; TAPSE: tricuspid annular plane systolic excursion; mPAP: mean PAP; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance.