Table 1

Hemodynamic, 6 min walking test, and pulmonary function parameters before and after combination therapy of  pirfenidone and macitentan.

Before 12 monthsBefore 6 monthsAt administrationAfter 3 monthsAfter 6 monthsAfter 12 months
PAP (S/D/M) (mm Hg)NDND33/17/2431/11/2223/12/1527/9/17
PCWP (S/D/M) (mm Hg)NDND13/12/916/14/1310/8/813/9/9
PVR (dyne/s/cm5)NDND185.5118.098.394.1
CO (L/min)NDND6.476.15.76.8
BNP (U/mL)39.575.267.447.330.144.4
6MWD (m)420315302325391330
Lowest SpO2 (%)908992939290
%FVC (%)77.770.165.881.774.377.8
%DLco (%)49.450.540.940.954.139.3
PaO2 (mm Hg)80.672.567.574.581.373.9
  • 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.