Table 1.
Demographic and characteristics of PAH‐Biobank cohort
| n | 2017 |
|---|---|
| Age (years), median (IQR) | 56 (45–66) |
| Female sex, n (%) | 1611 (80) |
| Race (Caucasian), n (%) | 1662 (82) |
| PAH subgroup, n (%) | |
| IPAH | 870 (43) |
| FPAH | 81 (4) |
| APAH | 961 (48) |
| APAH–CTD | 623 (31) |
| APAH–CHD | 171 (8) |
| PoPH | 111 (5.5) |
| APAH–HIV | 42 (2) |
| Other | 106 (5) |
| 6MWD (m), median (IQR) | 348 (258.5–426) |
| NYHA functional class, n (%) | |
| I | 90 (5) |
| II | 451 (22) |
| III | 789 (39) |
| IV | 118 (6) |
| Unknown | 569 (28) |
| Laboratory chemistry, median (IQR) | |
| HDGF (ng/ml) | 0.86 (0.51–1.37) |
| NT‐proBNP (pg/ml) | 672 (217–2164) |
| Hemodynamics, median (IQR) | |
| RAP (mmHg) | 8 (5–12) |
| mPAP (mmHg) | 49 (40–58) |
| mPCWP (mmHg) | 10 (7–13) |
| PVR (wood unit) | 8.61 (5.61–12.75) |
| CI (L/min/m2) | 2.52 (1.98–3.16) |
| REVEAL score‐category, n (%) | |
| 1 | 1251 (62.2) |
| 2 | 342 (17) |
| 3 | 217 (10.8) |
| 4 | 175 (8.7) |
| 5 | 25 (1.2) |
Abbreviations: 6MWD, six‐min walk distance; APAH, PAH associated with other conditions; CHD, congenital heart disease; CI, cardiac index; CTD, connective tissue disease; FPAH, familial PAH; HDGF, hepatoma‐derived growth factor; IPAH, idiopathic PAH; IQR, interquartile range; mPAP, mean pulmonary artery pressure; mPCWP, mean pulmonary capillary wedge pressure; mRAP, mean right atrial pressure; NT‐proBNP, N‐terminal brain natriuretic peptide prohormone; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; PoPH; portopulmonary hypertension; PVR, pulmonary vascular resistance; REVEAL, Registry to Evaluate Early and Long‐Term PAH Disease Management.