Hemodynamic, 6 min walking test, and pulmonary function parameters before and after combination therapy of pirfenidone and macitentan.
| Before 12 months | Before 6 months | At administration | After 3 months | After 6 months | After 12 months | |
| PAP (S/D/M) (mm Hg) | ND | ND | 33/17/24 | 31/11/22 | 23/12/15 | 27/9/17 |
| PCWP (S/D/M) (mm Hg) | ND | ND | 13/12/9 | 16/14/13 | 10/8/8 | 13/9/9 |
| PVR (dyne/s/cm5) | ND | ND | 185.5 | 118.0 | 98.3 | 94.1 |
| CO (L/min) | ND | ND | 6.47 | 6.1 | 5.7 | 6.8 |
| BNP (U/mL) | 39.5 | 75.2 | 67.4 | 47.3 | 30.1 | 44.4 |
| mMRC | 3 | 2 | 3 | 2 | 1 | 1 |
| 6MWD (m) | 420 | 315 | 302 | 325 | 391 | 330 |
| Lowest SpO2 (%) | 90 | 89 | 92 | 93 | 92 | 90 |
| %FVC (%) | 77.7 | 70.1 | 65.8 | 81.7 | 74.3 | 77.8 |
| %DLco (%) | 49.4 | 50.5 | 40.9 | 40.9 | 54.1 | 39.3 |
| PaO2 (mm Hg) | 80.6 | 72.5 | 67.5 | 74.5 | 81.3 | 73.9 |
6MWD, 6 min walking distance; BNP, brain natriuretic peptide; CO, cardiac output; DLco, diffusion capacity for carbon monoxide; FVC, forced vital capacity; mMRC, modified Medical Research Council; ND, not determined; PaO2, arterial oxygen tension; PAP, pulmonary artery pressure; PCWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; S/D/M, systolic/diastolic/mean; SpO2, peripheral capillary oxygen saturation.