Table 1 Patient characteristics at baseline

Total number of patients

117

Female

105 (89.7%)

Age at PAH diagnosis*, years

61.5 ± 11.4

Disease duration at PAH diagnosis*, years

11.7 ± 11.3

Race

Caucasian

101 (86.3%)

Asian

10 (8.5%)

Aboriginal/Torres Strait Islander

3 (2.6%)

Hispanic

1 (0.9%)

Other

2 (1.7%)

Underlying CTD

Limited SSc

79 (72.5%)

Diffuse SSc

25 (21.4%)

MCTD

5 (4.5%)

SLE

3 (2.8%)

RA

3 (2.8%)

Undifferentiated CTD

2 (1.8%)

Anti-centromere antibody positive

56 (47.9%)

Anti-Scl-70 antibody positive

9 (7.7%)

Anti-phospholipid antibodies

27 (23.1%)

WHO functional class

Class I

9 (7.7%)

Class II

14 (12.0%)

Class III

88 (75.2%)

Class IV

6 (5.1%)

Baseline 6MWD, m

325 ± 127

Baseline mRAP, mm Hg

6.9 ± 4.2

Baseline mPAP, mm Hg

35.9 ± 12.4

Baseline PCWP, mmHg

9.7 ± 3.9

Baseline CI, L/min/m²

2.6 ± 0.8

Baseline PVR, Wood units

6.9 ± 6.1

Pericardial effusion

14 (12.0%)

Warfarin therapy

36 (30.8%)

Pulmonary vasodilator therapy

   Monotherapy

70 (59.8%)

   Sequential monotherapy

12 (10.3%)

   Combination therapy

34 (29.0%)