Table 1.
Demographic data and mean hemodynamic parameters from the US/UK PAH cohort*
| All | IPAH | APAH** | FPAH | Other*** | |
|---|---|---|---|---|---|
| Total, n (%) | 4241 | 2319 (54.6) | 1479 (34.8) | 252 (5.9) | 191 (4.6) |
| Sex, n (%) | |||||
| F | 3187 (75.1) | 1721 (74.2) | 1156 (78.2) | 173 (68.6) | 137 (71.7) |
| M | 1054 (24.9) | 598 (25.8) | 323 (21.8) | 79 (31.3) | 54 (28.3) |
| F:M ratio | 3:1 | 2.9:1 | 3.6:1 | 2.3:1 | 2.5:1 |
| Age-of-onset, n (%) | |||||
| Adult (≥ 18 years) | 3780 (89.2) | 2126 (91.7) | 1256 (85.2) | 213 (84.5) | 185 (96.9) |
| Child (< 18 years) | 457 (10.8) | 193 (8.3) | 219 (14.8) | 39 (15.4) | 6 (3.1) |
| Mean ± SD | 45.9 ± 20.0 | 47.0 ± 19.5 | 45.2 ± 21.3 | 36.8 ± 16.8 | 47.7 ± 15.0 |
| Ancestry, n (%) | |||||
| EUR | 3108 (74.5) | 1798 (77.5) | 988 (67.0) | 166 (86.9) | 156 (81.7) |
| HISP | 359 (8.6) | 145 (6.3) | 186 (12.6) | 13 (6.8) | 15 (7.9) |
| AFR | 365 (8.7) | 166 (7.2) | 189 (12.8) | 2 (1.0) | 8 (4.2) |
| EAS | 104 (2.5) | 42 (1.8) | 57 (3.9) | 1 (0.5) | 4 (2.1) |
| SAS | 116 (2.8) | 82 (3.5) | 28 (1.9) | 4 (2.1) | 2 (1.0) |
| Other/unknown | 123 (2.9) | 85 (3.7) | 27 (1.8) | 5 (2.6) | 6 (3.1) |
| Hemodynamic parameters, mean ± SD (n) | |||||
| MPAP, mmHg | 51 ± 14 (3594) | 53 ± 17 (2045) | 48 ± 14 (1235) | 58 ± 14 (158) | 51 ± 12 (156) |
| BMPR2+ | 59 ± 12 (320) | 60 ± 12 (197) | 56 ± 16 (11) | 58 ± 12 (109) | 53 ± 5 (3) |
| BMPR2− | 50 ± 14 (3394) | 52 ± 14 (1928) | 48 ± 14 (1257) | 57 ± 17 (65) | 51 ± 13 (144) |
| MPCWP, mmHg | 10 ± 4 (3407) | 10 ± 24 (1912) | 10 ± 4 (1197) | 10 ± 4 (151) | 11 ± 4 (147) |
| BMPR2+ | 10 ± 4 () | 10 ± 4 () | 10 ± 3 () | 10 ± 4 () | 8 ± 4 () |
| BMPR2− | 10 ± 4 () | 10 ± 4 () | 10 ± 4 () | 10 ± 4 () | 11 ± 4 () |
*US/UK PAH cohort: 2572 PAH Biobank; 1134 UK NIHR BioResource; 534 CUIMC
**APAH: PAH associated with connective tissue diseases, congenital heart disease, HHT, HIV
***Other: diet- and toxin-induced PAH, non-familial pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis and one case of persistent pulmonary hypertension of the newborn
Abbreviations: AFR, African; EAS, East Asian; EUR, European; HISP, Hispanic; SAS, Southeast Asian; MPAP, mean pulmonary artery pressure; MPCWP, mean pulmonary capillary wedge pressure