Table 1.
Stanford Cohort Characteristics at Baseline Measurement of NE and Elafin
| Variable | Control Participants (N = 106) | All PAH (n = 249) | PAH Subgroup With Follow-up NE and Elafin Measurements (n = 70) |
|---|---|---|---|
| Age, y | 58 (48-72) | 49 (38-59) | 50 (40-58) |
| Sex | |||
| Female | 58 (54.7) | 191 (76.7) | 53 (75.7) |
| Male | 48 (45.3) | 58 (23.3) | 17 (24.3) |
| PAH cause | |||
| IPAH + HPAHa | — | 69 (27.7) | 20 (28.6) |
| D&T-associated PAH | — | 47 (18.9) | 17 (24.3) |
| CTD-associated PAH | — | 79 (31.7) | 18 (25.7) |
| PoPH | — | 16 (6.4) | 7 (10.0) |
| CHD-associated PAH | — | 38 (15.3) | 8 (11.4) |
| NYHA FC | |||
| I | — | 14 (5.6) | 5 (7.1) |
| II | — | 93 (37.3) | 33 (47.1) |
| III | — | 114 (45.8) | 24 (34.3) |
| IV | — | 28 (11.2) | 8 (11.4) |
| 6MWD, m | — | 423 (341-513) | 433 (366-512) |
| Therapy extent | |||
| Naïve | — | 87 (35.0) | 19 (27.1) |
| Monotherapy | — | 70 (28.1) | 21 (30.0) |
| Dual therapy | — | 69 (27.7) | 24 (34.3) |
| Triple therapy | — | 23 (9.2) | 6 (8.6) |
| Therapy class | |||
| PDE-5 inhibitor | — | 119 (47.8) | 36 (51.4) |
| ERA | — | 74 (29.7) | 22 (31.4) |
| Prostacyclin | — | 84 (33.7) | 29 (41.4) |
| Dlco, % predicted | — | 71 (54-87) | 73 (64-89) |
| NT-proBNP, pg/mL | — | 289 (80-1238) | 216 (75-934) |
| Hemodynamics | |||
| Right atrial pressure, mm Hg | — | 7 (5-11) | 7 (4-12) |
| mPAP, mm Hg | — | 50 (40-60) | 52 (42-60) |
| Cardiac index, L/min/m2 | — | 2.09 (1.76-2.43) | 2.07 (1.70-2.36) |
| PVR, dynes/sec/cm5 | — | 821 (506-1197) | 842 (596-1177) |
| NE, ng/mL | 97.6 (74.4-126.6) | 205.1 (123.6-387.3) | 169.9 (119.1-310.3) |
| Elafin, ng/mL | 45.5 (28.1-92.8) | 32.0 (15.3-59.1) | 42.4 (33.1-72.2) |
| NE to elafin ratio | 2.0 (0.8-3.8) | 6.2 (3.0-17.3) | 4.3 (2.0-7.2) |
Data are presented as No. (%) or median (interquartile range). Missing data: all PAH group, 6MWD (n = 5), Dlco (n = 20), RAP (n = 4), CI and PVR (n = 2); follow-up group: Dlco (n = 10). 6MWD = 6-min walk distance; APAH = associated pulmonary arterial hypertension; CHD = congenital heart disease; CTD = connective tissue disease; D&T = drugs and toxins; Dlco = diffusion capacity of lung for carbon monoxide; — = data not available for healthy control participants; ERA = endothelin receptor antagonist; HPAH = heritable pulmonary arterial hypertension; IPAH = idiopathic pulmonary arterial hypertension; mPAP = mean pulmonary arterial pressure; NE = neutrophil elastase; NT-proBNP = N-terminal B-type natriuretic peptide; NYHA FC = New York Heart Association functional class; PAH = pulmonary arterial hypertension; PDE-5 = phosphodiesterase-5; PoPH = portopulmonary hypertension; PVR = pulmonary vascular resistance.
HPAH: n = 3 patients (1.2% of cohort), who had confirmed BMPR2 mutations. Mutation status was not otherwise evaluated routinely in the cohort.