TABLE 1.
Patient characteristics
| All (1745) | (I/D/H)PAH (n=994) | CTD-PAH (n=751) | p-value | |
| Female sex % | 73 | 66 | 82 | <0.0001 |
| Age at diagnosis years | 59±17 | 55±18 | 64±13 | <0.0001 |
| Incident patients % (n=618) | 35 | 29 | 44 | <0.0001 |
| FEV1 % predicted (n=1457) | 82±21 | 84±19 | 80±23 | 0.0001 |
| FVC % predicted (n=1459) | 92±23 | 95±20 | 89±27 | <0.0001 |
| FEV1/FVC (n=1459) | 73±13 | 74±13 | 73±14 | 0.078 |
| Mean RAP mmHg (n=1503) | 9±6 | 10±6 | 8±5 | <0.0001 |
| Mean PAP mmHg(n=1573) | 48±13 | 53±13 | 41±11 | <0.0001 |
| PAWP mmHg (n=1496) | 9±4 | 9±4 | 9±3 | 0.56 |
| PVR Wood units (n=1378) | 10.5±5.8 | 12.0±5.7 | 8.7±5.4 | <0.0001 |
| Cardiac output L·min−1 (n=1465) | 4.2±1.5 | 4.0±1.5 | 4.3±1.5 | <0.0005 |
| Cardiac index L·min−1·m−2 (n=1305) | 2.4±0.8 | 2.2±0.8 | 2.5±0.8 | <0.0001 |
| EmPHasis-10 score | 29 (18–38) | 28 (17–37) | 30 (19–38) | 0.001 |
| WHO functional class# % (n=1725) | ||||
| Class I | 3 | 4 | 1 | |
| Class II | 23 | 26 | 20 | |
| Class III | 61 | 57 | 67 | |
| Class IV | 13 | 13 | 12 | |
| 6MWD# (n=659) | 310 (180–408) | 340 (192–432) | 241 (141–360) | <0.0001 |
| ISWD# (n=797) | 150 (70–270) | 160 (80–350) | 140 (60–228) | 0.001 |
Data are presented as n, mean±sd, or median (1st–3rd quartile). PAH: pulmonary arterial hypertension; CTD: connective tissue disease; IPAH: idiopathic PAH; DPAH: drug-induced PAH; HPAH: heritable PAH; CTD-PAH: CTD-associated PAH; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; RAP: right atrial pressure; PAP: pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; WHO: World Health Organization; 6MWD: 6-min walk distance; ISWD: incremental shuttle walk distance; sd: standard deviation. #: variables were recorded at the time of baseline emPHasis-10 measurement (other variables were recorded at diagnosis). Baseline 6MWD and ISWD were available in 38% and 46% of patients, respectively, with no overlap.