Baseline and follow-up incremental shuttle walking test distance (ISWD) and emPHasis-10 quality-of-life score (E-10)
| Subjects | IPAHno-LD | Subjects | IPAHmild-LD | Subjects | IPAHDLCO<45 | Subjects | IPAHDLCO≥45 | |
| Baseline and first follow-up beyond 90 days | ||||||||
| ISWD | ||||||||
| Baseline m | 279 | 210 (80–360) | 159 | 80 (40–180)**** | 71 | 80 (30–210) | 170 | 260 (130–430)#### |
| ΔISWD m | 215 | 40 (−10–120) | 124 | 0 (−32–30)**** | 47 | 20 (−13–70) | 139 | 50 (−10–160)# |
| ΔISWD p-value | <0.0001 | 0.90 | <0.05 | <0.0001 | ||||
| E-10 score | ||||||||
| Baseline | 84 | 32 (20–40) | 83 | 32 (26–41) | 25 | 38 (33–44) | 55 | 27 (0–35)## |
| ΔE-10 | 64 | −4 (−12–3) | 65 | 0 (−5–8)* | 19 | −4 (−13–2) | 43 | −4 (−11–3) |
| ΔE-10 p-value | 0.005 | 0.57 | 0.08 | 0.03 | ||||
| Baseline and 1-year follow-up: patients treated with combination oral treatment within 6 months of diagnosis | ||||||||
| ISWD | ||||||||
| Baseline ISWD m | 125 | 200 (80–340) | 76 | 60 (20–140)**** | ||||
| ΔISWD m | 79 | 40 (−10–140) | 32 | −20 (−50–50)** | ||||
| ΔISWD p-value | <0.0001 | 0.83 | ||||||
| E-10 score | ||||||||
| Baseline E-10 | 57 | 34 (22–42) | 41 | 35 (28–41) | ||||
| ΔE-10 | 27 | −4 (−18–5) | 17 | −4 (−11–3) | ||||
| ΔE-10 p-value | 0.03 | 0.19 |
Data are presented as n or median (interquartile range). IPAH: idiopathic pulmonary arterial hypertension; IPAHno-LD: IPAH with no lung disease; IPAHmild-LD: IPAH with mild lung disease; IPAHDLCO<45: IPAHno-LD with DLCO <45% pred; IPAHDLCO≥45: IPAHno-LD with DLCO ≥45% pred; Δ: change. *: p<0.05; **: p<0.01; ****: p<0.0001 compared to IPAHno-LD; #: p<0.05; ##: p<0.01; ####: p<0.0001 compared to IPAHDLCO<45.