Table 2.
Demographic and Baseline Characteristics of Subjects Enrolled in USPHSR With Associated PAH According to Subgroup
| Characteristic | APAH Subgroup |
|||||
|---|---|---|---|---|---|---|
| CTD | CHD | PoPH | Drugs/Toxins | HIV | Othera | |
| Patients | 171 (67) | 30 (12) | 22 (9) | 24 (9) | 5 (2) | 4 (1) |
| Age at enrollment, y | 61.3 ± 13.7 | 52.1 ± 17.4 | 60.8 ± 10.2 | 54.1 ± 12.9 | 50.4 ±5.6 | 54.4 ± 13.6 |
| Age at diagnosis, y | 57.0 ± 13.8 | 48.1 ± 17.1 | 57.5 ± 9.8 | 50.1 ± 11.8 | 46.4 ± 4.4 | 47.8 ± 14.9 |
| Age group | ||||||
| 16-64 y | 110 (64.3) | 22 (73.3) | 17 (77.3) | 21 (87.5) | 5 (100) | 4 (100) |
| 65-74 y | 51 (29.8) | 7 (23.3) | 4 (18.2) | 2 (8.3) | 0 | 0 |
| ≥ 75 y | 10 (5.8) | 1 (3.3) | 1(4.5) | 1 (4.2) | 0 | 0 |
| Female sex | 154 (90.1) | 21 (70.0) | 9 (40.9) | 19 (79.2) | 3 (60.0) | 4 (100.0) |
| Race | ||||||
| Asian | 7 (4.1) | 3 (10) | 0 | 0 | 0 | 0 |
| Biracial | 1 (0.6) | 0 | 0 | 0 | 0 | 0 |
| Black | 15 (8.8) | 0 | 2 (9.1) | 0 | 2 (40) | 0 |
| Native American/Alaskan | 1 (0.6) | 0 | 0 | 0 | 0 | 0 |
| Pacific Islander | 1 (0.6) | 0 | 0 | 0 | 0 | 0 |
| White | 146 (85.4) | 27 (90) | 20 (90.9) | 24 (100) | 3 (60) | 4 (100) |
| BMI, kg/m2 | 27.2 ± 6.6 | 26.9 ± 6.5 | 29.4 ± 7.3 | 31.5 ± 8.6 | 29.2 ± 6.8 | 36.5 ± 6.7 |
| Time from diagnosis to enrollment, mo | ||||||
| Mean ± SD | 52.1 ± 31.8 | 49.0 ± 29.6 | 39.2 ± 31.7 | 47.9 ± 33.7 | 48.2 ± 44.5 | 80.2 ± 18.1 |
| Median | 51.5 | 44.1 | 32.1 | 44.1 | 21.9 | 84.1 |
| IQR | 28.2, 73.4 | 29.6, 66.8 | 19.3, 54.7 | 17.4, 70.7 | 18.9, 86.8 | 69.8, 94.5 |
| Newly diagnosedb | 15 (8.8) | 2 (6.7) | 2 (9.1) | 0 | 0 | 0 |
| Previously diagnosed | 156 (91.2) | 28 (93.3) | 20 (90.9) | 24 (100) | 5 (100) | 4 (100) |
Data are presented as No. (%) or mean ± SD unless otherwise indicated. CHD = congenital heart disease; CTD = connective tissue disease; PAH = pulmonary arterial hypertension; PoPH = portopulmonary hypertension. See Table 1 legend for expansion of other abbreviations.
a
Includes hereditary hemorrhagic telangiectasia (1), exercise PAH (2), and neurofibromatosis (1).
b
Patients classified as newly diagnosed with PAH if the diagnostic right heart catheterization occurred within 180 days of USPHSR enrollment.