Table 1.
PAH Biobank cohort demographic and hemodynamic data
| All | IPAH | APAH | FPAH | Other* | |
|---|---|---|---|---|---|
| Total (%) | 2572 | 1110 (43.2) | 1239 (48.2) | 101 (3.9) | 122 (4.7) |
| Age of onset, n (%) | |||||
| Child (dx age < 19) | 226 (8.8) | 94 (8.5) | 112 (9.0) | 15 (14.9) | 5 (4.1) |
| Adult (dx age > =19) | 2345 (91.2) | 1015 (91.4) | 1127 (91.0) | 86 (85.1) | 117 (95.9) |
| Mean age | 48 ± 19 | 48 ± 18 | 49 ± 19 | 37 ± 15 | 47 ± 15 |
| Gender, n (%) | |||||
| Female | 2023 (78.7) | 868 (78.2) | 996 (80.4) | 69 (68.3) | 90 (73.8) |
| Male | 549 (21.3) | 242 (21.8) | 243 (19.6) | 32 (31.7) | 32 (26.2) |
| Female to male ratio | 3.7:1 | 3.6:1 | 4.1:1 | 2.2:1 | 2.8:1 |
| Ancestry, n (%) | |||||
| European | 1852 (72) | 809 (73.0) | 855 (69.0) | 89 (88.1) | 99 (81.2) |
| Hispanic | 315 (12.3) | 137 (12.3) | 156 (12.6) | 10 (9.9) | 12 (9.8) |
| African | 292 (11.4) | 117 (10.5) | 168 (13.6) | 1 (1) | 6 (4.9) |
| East Asian | 70 (2.7) | 25 (2.2) | 41 (3.3) | 0 | 4 (3.3) |
| South Asian | 28 (1.1) | 12 (1.1) | 15 (1.2) | 1 (1) | 0 |
| Others | 15 (0.58) | 10 (0.9) | 4 (0.3) | 0 | 1 (0.8) |
| Hemodynamic parameters | |||||
| MPAP (mmHg) | 50 ± 14 | 52 ± 14 | 48 ± 14 | 58 ± 14 | 52 ± 13 |
| MPCW (mmHg) | 10 ± 4 | 10 ± 4 | 10 ± 4 | 10 ± 4 | 11 ± 4 |
| CO, Fick (L/min) | 4.5 ± 1.8 | 4.5 ± 1.7 | 4.6 ± 1.9 | 3.6 ± 1.0 | 4.2 ± 1.3 |
| PVR (Woods units) | 10.7 ± 7.0 | 11.2 ± 7.0 | 10.0 ± 7.1 | 14.9 ± 6.3 | 11.0 ± 6.6 |
| MAP (mmHg) | 90 ± 19 | 91 ± 20 | 90 ± 19 | 88 ± 16 | 94 ± 19 |
| MAP:MPAP | 1.9 ± 0.7 | 1.9 ± 0.7 | 2.0 ± 0.7 | 1.6 ± 0.5 | 1.9 ± 0.5 |
Abbreviations: MPAP mean pulmonary arterial pressure, MPCW mean pulmonary capillary wedge pressure, CO cardiac output by Frick’s method, PVR pulmonary vascular resistance, MAP mean arterial pressure
*Other included 110 diet- and toxin-induced PAH, 11 non-familial pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis, and 1 persistent pulmonary hypertension of the newborn