Table 2.
Registry (ref.) | All patients (n = 7,844) | Patients with CTD‐PAH (n = 2,113) | |||||||
---|---|---|---|---|---|---|---|---|---|
Age, mean ± SD years |
Female, % |
WHO functional class I–II, % |
6MWD, mean ± SD meters |
CTD, % |
Age, mean ± SD years |
Female, % |
WHO functional class I–II, % |
6MWD, mean ± SD meters |
|
REHAP (31) | 45 ± 17 | 71 | 31 | 363 ± 120 | 18 | 54 ± 15 | 90 | 21 | 309 ± 115 |
PAH‐QuERI (32) | 55 ± 16 | 77 | 47 | NR | 29 | NR | NR | NR | NR |
COMPERA (5) | 64 ± 16 | 64 | 11 | 298 ± 126 | 22 | 66 ± 13 | 78 | 11 | 273 ± 130 |
French PAH Network Registry (4) | 50 ± 15 | 66 | 25 | 329 ± 109 | 15 | 56 ± 15 | 80 | 26 | 315 ± 111 |
REVEAL (33) | 50 ± 17 | 77 | NR | NR | 28 | NR | NR | NR | NR |
Turkish registry (34) | 46 ± 17 | 77 | 21 | NR | 22 | NR | NR | NR | NR |
Chinese Registry‐PAH (35) | 36 ± 15 | 76 | 46 | 390 ± 111 | 37 | 42 ± 14 | 85 | 45 | 384 ± 107 |
BPR (36) | 59 ± 17 | 77 | 11 | NR | 42 | 62 ± 11 | 85 | 6 | NR |
KORPAH (37) | 50 ± 17 | 78 | 53 | 363 ± 116 | 58 | 54 ± 17 | 85 | 63 | 358 ± 114 |
All registries | 51 ± 2.7† | 74 | 28 | 348 ± 16.4† | 29 | 56 ± 3.3† | 84 | 24 | 328 ± 20.1† |
PAH = pulmonary arterial hypertension; CTD‐PAH = connective tissue disease–associated PAH; WHO = World Health Organization; 6MWD = 6‐minute walk distance; REHAP = Spanish Registry of PAH; PAH‐QuERI = PAH Quality Enhancement Research Initiative; NR = not reported; COMPERA = Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; REVEAL = Registry to Evaluate Early and Long‐term PAH Disease Management; BPR = Bosentan Patient Registry; KORPAH = Korean Registry of PAH.
Values are the estimated mean ± SEM from the random‐effects model.