Table 1.
Demographic and clinical characteristics
| Variable | IPAH (n = 58) | NIPAH(n = 55) | Control (n = 33) |
|---|---|---|---|
| Mean age ± SD, years | 51 ± 15 | 50 ± 11 | 27 ± 6 |
| Female sex | 45 (77) | 40 (72) | 10 (30) |
| Race/ethnicity | |||
| White (not of Hispanic origin) | 47 (81) | 42 (76) | 25 (76) |
| Black (not of Hispanic origin) | 6 (10) | 7 (13) | 4 (12) |
| Hispanic | 4 (7) | 5 (9) | 1 (3) |
| Asian | 1 (2) | 1 (2) | 3 (9) |
| NIPAH | |||
| Heritable PAH | 5 (9) | ||
| Portopulmonary hypertension | 13 (24) | ||
| Drugs and toxins | 6 (11) | ||
| Connective tissue diseases | 18 (33) | ||
| Other | 13 (23) | ||
| WHO functional class | |||
| I | 3 (5) | 0 (0) | |
| II | 19 (33) | 16 (29) | |
| III | 33 (57) | 37 (67) | |
| IV | 3 (5) | 2 (4) | |
| Hemodynamics | |||
| Mean right atrial pressure ± SD, mmHg | 10 ± 7 | 10 ± 6 | |
| Mean pulmonary artery pressure ± SD, mmHg | 47 ± 14 | 45 ± 13 | |
| Mean pulmonary capillary wedge pressure ± SD, mmHg | 11 ± 6 | 10 ± 4 | |
| Mean pulmonary vascular resistance ± SD, dyn s cm−5 | 789 ± 439 | 631 ± 366 | |
| Mean cardiac index ± SD, L/min/m2 | 2.4 ± 0.9 | 2.7 ± 0.9 | |
| Treatment | |||
| Calcium channel blocker | 19 (33) | 14 (25) | |
| Inhaled iloprost | 13 (22) | 7 (13) | |
| Intravenous epoprostenol | 12 (21) | 10 (18) | |
| Ambrisentan | 1 (2) | 0 (0) | |
| Bosentan | 29 (50) | 25 (45) | |
| Sildenafil | 4 (7) | 5 (9) |
Note
Data are no. (%) of patients unless otherwise noted. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; NIPAH: nonidiopathic PAH; WHO: World Health Organization.