Table 1.
Demographic Characteristics and Clinical Features of Patients With IPAH and SSc-PAH
| Characteristic | Discovery Cohort (Cohort 1) |
Validation Cohort (Cohort 2) |
||||
|---|---|---|---|---|---|---|
| IPAH (n = 864) | SSc-PAH (n = 310) | P Value | IPAH (n = 213) | SSc-PAH (n = 91) | P Value | |
| Female | 663 (76.7) | 267 (86.1) | .001 | 166 (77.9) | 82 (90.1) | .019 |
| Age, y | 51.90 ± 18.47 | 64.10 ± 11.03 | < .001 | 53.22 ± 14.95 | 63.74 ± 10.29 | < .001 |
| BMI, kg/m2 | 30.36 ± 19.14) | 28.24 ± 11.40 | .068 | 30.78 ± 9.23 | 27.32 ± 8.17 | .002 |
| Renal insufficiency | 32 (3.7) | 28 (9.0) | < .001 | 11 (5.2) | 7 (7.7) | .555 |
| Cirrhosis | 13 (1.5) | 5 (1.6) | 1 | 2 (0.9) | 4 (4.4) | .125 |
| WHO functional class | .093 | .51 | ||||
| I | 46 (7.1) | 14 (5.7) | 5 (3.9) | 0 (0.0) | ||
| II | 182 (28.3) | 81 (33.1) | 42 (32.6) | 20 (36.4) | ||
| III | 345 (53.6) | 135 (55.1) | 72 (55.8) | 31 (56.4) | ||
| IV | 71 (11.0) | 15 (6.1) | 10 (7.8) | 4 (7.3) | ||
| 6MWD, m | 353.91 ± 138.36 | 312.44 ± 118.06 | .001 | 348.80 ± 125.44 | 312.30 ± 130.54 | .121 |
| mRAP, mm Hg | 9.16 ± 5.85 | 8.33 ± 5.06 | .028 | 8.38 ± 4.99 | 7.48 ± 4.94 | .154 |
| mPAP, mm Hg | 50.82 ± 14.25 | 43.30 ± 11.32 | < .001 | 52.73 ± 13.8 | 41.53 ± 9.47 | < .001 |
| PAWP, mm Hg | 9.58 ± 3.24 | 9.38 ± 3.35 | .36 | 9 ± 3.52 | 8.38 ± 3.39 | .15 |
| PVR, Woods units | 10.74 ± 6.84 | 8.50 ± 5.13 | < .001 | 12.18 ± 6.38 | 8.48 ± 4.06 | < .001 |
| Cardiac index, L/min/m | 2.48 ±1 | 2.60 ± 0.78 | .08 | 2.26 ± 0.83 | 2.49 ± 0.74 | .03 |
| SVI, mL/m2 | 32.65 ± 13.88 | 33.01 ± 11.70 | .77 | 29.64 ± 12.15 | 32.72 ± 10.48 | .17 |
| Prostanoid use | 415 (48.0) | 130 (41.9) | .075 | 83 (39.0) | 25 (27.5) | .074 |
Data are expressed as No. (%) or mean ± SD. 6MWD = 6-min walk distance; IPAH = idiopathic pulmonary arterial hypertension; mPAP = mean pulmonary artery pressure; mRAP = mean right atrial pressure; PAWP = pulmonary artery wedge pressure; PVR = pulmonary vascular resistance; SSc-PAH = systemic sclerosis-associated pulmonary arterial hypertension; SVI = stroke volume index.