Table 2

Baseline characteristics and haemodynamics among patients with CTD-PAH and SSc-PAH

Combination therapy (CTD)
(n=103)
Monotherapy pooled (CTD)
(n=84)
Combination therapy (SSc)
(n=71)
Monotherapy pooled (SSc)
(n=47)
Median (Q1, Q3) time from diagnosis to study drug administration (days)20 (8, 49)21.5 (8, 49)20 (8, 45)16 (8, 35)
Mean (SD) age (years)58.4 (12.3)57.9 (13.1)62.0 (9.2)60.7 (10.7)
Sex, n (%)
 Female89 (86)76 (90)58 (82)41 (87)
 Male14 (14)8 (10)13 (18)6 (13)
WHO functional class, n (%)
 II26 (25)22 (26)16 (23)11 (23)
 III77 (75)62 (74)55 (77)36 (77)
6-min walk distance (m)
 Mean (SD)324.8 (86.3)327.9 (98.0)312.8 (87.7)328.9 (98.2)
 Median333.1346.8313.2352.0
Median (Q1, Q3) NT-proBNP (ng/L)1321 (372, 2995) n=981128 (337, 2357) n=821713 (332, 3294) n=681152 (382, 2576) n=46
Mean (SD) % of predicted normal TLC89.0 (15.7) n=10288.6 (17.6) n=8389.8 (16.4) n=7090.0 (18.4)
Mean (SD) % of predicted normal FEV1 84.3 (18.8)81.4 (16.4)86.2 (19.8)85.9 (17.4)
Prior medications, n (%)
 Immunosuppressants14 (14)13 (15)4 (6)5 (11)
 Steroids18 (17)17 (20)10 (14)7 (15)
Mean (SD) mean right atrial pressure (mm Hg)7.4 (4.2) n=1027.7 (4.5)7.4 (4.2)7.6 (4.7)
Mean (SD) cardiac index (L/min/m2)2.55 (0.63)2.67 (0.73) n=832.57 (0.60)2.54 (0.62) n=46
Mean (SD) mean pulmonary arterial pressure (mm Hg)43.5 (10.4)45.1 (10.0)43.7 (10.3)45.7 (10.1)
Mean (SD) pulmonary capillary wedge pressure (mm Hg)8.2 (3.0) n=1029.1 (3.3) n=828.3 (3.0)8.6 (3.3) n=45
Mean (SD) pulmonary vascular resistance (dyne·s/cm5)672.3 (274.5)667.1 (257.8)674.7 (267.8)702.2 (265.3)
  • Post hoc summary.

  • CTD, connective tissue disease; FEV1, forced expiratory volume in 1 s; NT-proBNP, N-terminal pro-B-type natriuretic peptide; PAH, pulmonary arterial hypertension; Q1, quartile 1; Q3, quartile 3; SSc, systemic sclerosis; TLC, total lung capacity.