Table 1.
Baseline characteristics of patients with IPAH.
| Variables | Control (n = 338) | Total (n = 338) | Male (n = 105) | Female (n = 233) | P-value* |
|---|---|---|---|---|---|
| Age, years | 41 ± 9 | 40 ± 16 | 44 ± 19 | 39 ± 14 | 0.003 |
| BMI, kg/m2 | 22 (20–24) | 22 (20–24) | 22 (20–24) | 22 (20–24) | 0.834 |
| 6MWD, meters† | - | 380 (305–445) | 398 (308–471) | 374 (305–440) | 0.112 |
| WHO FC, n (%) | 0.586 | ||||
| Class I/II | - | 106 (31) | 32 (30) | 74 (32) | |
| Class III | - | 210 (62) | 64 (61) | 146 (62) | |
| Class IV | - | 22 (7) | 9 (9) | 13 (6) | |
| BMPR2 mutation, n (%)‡ | - | 48 (24) | 21 (35) | 27 (19) | 0.017 |
| NT-proBNP, ng/L | - | 1157 ± 1212 | 1339 ± 1532 | 1075 ± 1031 | 0.113 |
| Liver function | |||||
| GGT, U/L | 23.8 ± 13.3# | 64.2 ± 67.1 | 89.3 ± 82.2 | 52.6 ± 55.4 | <0.001 |
| ALT, U/L | 22.7 ± 16.2 | 29.4 ± 22.8 | 31.3 ± 19.9 | 28.6 ± 24.0 | 0.286 |
| AST, U/L | 20.0 ± 5.5 | 28.8 ± 20.0 | 28.8 ± 16.9 | 28.8 ± 21.3 | 1.000 |
| Renal function | |||||
| Creatinine, μmol/L | 61.2 ± 12.4 | 68.7 ± 24.0 | 74.5 ± 33.3 | 61.9 ± 14.1 | <0.001 |
| BUN, mmol/L | 4.7 ± 1.2 | 5.5 ± 2.1 | 6.5 ± 2.6 | 5.1 ± 1.7 | <0.001 |
| UA, μmol/L | 285.0 ± 74.2# | 409.4 ± 125.7 | 446.6 ± 131.3 | 393.4 ± 120.0 | 0.001 |
| Hemodynamic variables | |||||
| mRAP, mmHg | - | 7.0 ± 5.2 | 7.4 ± 5.7 | 6.8 ± 5.0 | 0.398 |
| mPAP, mmHg | - | 58.2 ± 14.7 | 60.5 ± 16.6 | 57.1 ± 13.6 | 0.044 |
| PAWP, mmHg | - | 7.6 ± 3.3 | 8.0 ± 3.1 | 7.4 ± 3.3 | 0.031 |
| CI, L/min/m2 | - | 2.6 ± 0.8 | 2.5 ± 0.8 | 2.6 ± 0.8 | 0.486 |
| PVR, Wood units | - | 13.8 ± 6.5 | 15.3 ± 6.5 | 12.6 ± 6.6 | 0.034 |
| SVO2, % | 63 (57–69) | 62 (56–70) | 64 (57–69) | 0.278 | |
| Cardiopulmonary exercise testing§ | |||||
| Peak VO2, mL/min/kg | - | 13 (10–16) | 13 (10–17) | 13 (11–15) | 0.784 |
| VE/VCO2 slope | - | 56 ± 33 | 60 ± 29 | 54 ± 36 | 0.336 |
| Echocardiography | |||||
| RA area, cm2∥ | - | 25 ± 13 | 30 ± 15 | 23 ± 11 | 0.001 |
| PAH-Specific therapies, n (%) | 0.394 | ||||
| ERA | - | 39 (13) | 16 (17) | 23 (11) | |
| PDE5-i | - | 141 (46) | 46 (47) | 95 (45) | |
| Prostanoids | - | 17 (5) | 4 (4) | 13 (6) | |
| Combination therapy | - | 112 (36) | 31 (32) | 81 (38) | |
Data were presented as numbers of patients (n, %), mean ± standard deviations or median (interquartile range).
Comparison between male and female.
Comparison between controls and total patients, # represented p < 0.05.
Data on 6MWD was available from 275 individuals.
Data on BMPR2 mutation measurement was available from 200 individuals.
Data on cardiopulmonary exercise testing was available from 100 individuals.
RA area was measured in 222 individuals.
ALT, glutamate pyruvate transaminase; AST, glutamic oxalacetic transaminase; BMI, body mass index; BMPR2, bone morphogenetic protein receptor type 2; BUN, blood urea nitrogen; CI, cardiac index; ERA, endothelin receptor antagonist; GGT, gamma-glutamyltranspeptidase; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary arterial pressure; PAWP, mean pulmonary artery wedge pressure; mRAP, mean right atrial pressure; 6MWD, 6minute walking distance; NT-proBNP, N-terminal pro B-type natriuretic peptide; PDE5-i, phosphodiesterase type 5 inhibitors; Peak VO2, peak oxygen uptake; PVR, pulmonary vascular resistance; RA area, right atrial area; SvO2, mixed venous oxygen saturation; UA, uric acid; VE/VCO2 slope, ventilatory equivalent for carbon dioxide slope; WHO FC, World Health Organization functional class.