Table 1.
Patient characteristics at baseline
| Characteristic | Mean + SD or n(%) |
|---|---|
| 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%) |
* date of PAH diagnosis is the date of right heart catheterisation
6MWD, six minute walk distance; CI, cardiac index; CTD, connective tissue disease; MCTD, mixed connective tissue disease; mPAP, mean pulmonary arterial pressure; mRAP, mean right atrial pressure; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; RA, rheumatoid arthritis; SD, standard deviation; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; WHO, World Health Organization