All patients n=192 | IPAH/HPAH n=93 | PAH-CHD n=60 | PAH-CTD n=28 | |||||||||
Baseline | 3–4 months | p-value | Baseline | 3–4 months | p-value | Baseline | 3–4 months | p-value | Baseline | 3–4 months | p-value | |
NYHA FC III/IV | 49 | 35 | <0.001 | 51 | 32 | <0.001 | 38 | 28 | 0.146 | 71 | 64 | 0.687 |
6MWD m | 428 (339–496) | 465 (380–545) | <0.001 | 450 (302–531) | 498 (376–570) | <0.001 | 431 (394–498) | 456 (425–546) | <0.001 | 364 (214–426) | 397 (310–434) | 0.030 |
RAP mmHg | 9 (7–12) | 8 (6–11) | 0.003 | 8 (6–11) | 7 (6–10) | 0.003 | 9 (7–12) | 9 (7–11) | 0.405 | 12 (7–16) | 10 (6–15) | 0.321 |
mPAP mmHg | 62 (51–76) | 57 (48–70) | <0.001 | 59 (49–69) | 54 (46–63) | <0.001 | 78 (65–90) | 73 (59–90) | <0.001 | 50 (43–56) | 49 (42–54) | 0.011 |
CI L·min−1·m−2 | 2.3 (1.9–2.6) | 2.5 (2.2–2.9) | <0.001 | 2.3 (1.9–2.7) | 2.7 (2.4–3.0) | <0.001 | 2.1 (1.8–2.6) | 2.4 (2.1–2.9) | 0.003 | 2.2 (1.9–2.5) | 2.5 (2.0–2.8) | 0.010 |
PVR WU | 13 (10–18) | 11 (8–15) | <0.001 | 12 (9–16) | 10 (7–12) | <0.001 | 17 (13–27) | 14 (9–21) | <0.001 | 13 (8–14) | 9 (7–14) | 0.003 |
Data are presented as % or median (interquartile range), unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; HPAH: heritable PAH; CHD: congenital heart disease; CTD: connective tissue disease; NYHA FC: New York Heart Association functional class; 6MWD: 6-min walking distance; RAP: right atrial pressure; mPAP: mean pulmonary arterial pressure; CI: cardiac index; PVR: pulmonary vascular resistance; WU: Wood units. The p-values were determined using a non-parametric Wilcoxon–Mann–Whitney test for all comparisons, except for NYHA that was evaluated by McNemar test.