TABLE 2

Symptomatic and haemodynamic parameters at baseline and after 3–4months of combination therapy

All patients n=192IPAH/HPAH n=93PAH-CHD n=60PAH-CTD n=28
Baseline3–4 monthsp-valueBaseline3–4 monthsp-valueBaseline3–4 monthsp-valueBaseline3–4 monthsp-value
NYHA FC III/IV4935<0.0015132<0.00138280.14671640.687
6MWD m428 (339–496)465 (380–545)<0.001450 (302–531)498 (376–570)<0.001431 (394–498)456 (425–546)<0.001364 (214–426)397 (310–434)0.030
RAP mmHg9 (7–12)8 (6–11)0.0038 (6–11)7 (6–10)0.0039 (7–12)9 (7–11)0.40512 (7–16)10 (6–15)0.321
mPAP mmHg62 (51–76)57 (48–70)<0.00159 (49–69)54 (46–63)<0.00178 (65–90)73 (59–90)<0.00150 (43–56)49 (42–54)0.011
CI L·min−1·m−22.3 (1.9–2.6)2.5 (2.2–2.9)<0.0012.3 (1.9–2.7)2.7 (2.4–3.0)<0.0012.1 (1.8–2.6)2.4 (2.1–2.9)0.0032.2 (1.9–2.5)2.5 (2.0–2.8)0.010
PVR WU13 (10–18)11 (8–15)<0.00112 (9–16)10 (7–12)<0.00117 (13–27)14 (9–21)<0.00113 (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.