| | Parameters | POPH (n = 30) | IPAH (n = 180) | value |
| | Demographics | | | | | Age at diagnosis (year) | 49.5 ± 13.1 | 39.1 ± 14.9 | <0.001 | | Female : male | 22 : 8 | 123 : 57 | 0.854 | | Height (cm) | 162.7 ± 7.6 | 161.9 ± 6.9 | 0.584 | | Weight (kg) | 63.0 ± 12.9 | 58.5 ± 9.9 | 0.032 | | Body surface area (m2) | 1.70 ± 0.29 | 1.62 ± 0.18 | 0.046 | | Onset of symptoms (mo) | 23 (10–37) | 19 (9–44) | 0.856 | | WHO FC (I : II : III : IV) | 1 : 12 : 14 : 3 | 2 : 61 : 107 : 10 | 0.571 | | Etiology of liver disease, n (%) | | | | | Cryptogenic | 7 (23.3%) | NA | | | Hepatitis B | 19 (63.4%) | NA | | | Hepatitis C | 1 (3.3%) | NA | | | Alcohol | 1 (3.3%) | NA | | | PBC | 2 (6.7%) | NA | | | Child-Pugh class, n (%) | | | | | A | 13 (43.3%) | NA | | | B | 14 (46.7%) | NA | | | C | 3 (10.0%) | NA | | | Hemodynamics | | | | | mRAP (mmHg) | 4.74 ± 3.54 | 8.48 ± 5.49 | <0.001 | | sPAP (mmHg) | 80.46 ± 21.71 | 99.25 ± 24.81 | <0.001 | | mPAP (mmHg) | 46.54 ± 11.95 | 60.58 ± 15.03 | <0.001 | | mPAWP (mmHg) | 8.23 ± 3.59 | 8.44 ± 3.16 | 0.755 | | TPG (mmHg) | 38.24 ± 11.21 | 52.38 ± 14.77 | <0.001 | | Cardiac index (L/min/m2) | 4.02 ± 1.59 | 2.47 ± 0.81 | <0.001 | | PVR (wood unit) | 7.08 ± 3.95 | 14.89 ± 7.11 | <0.001 | | SVR (wood unit) | 15.78 ± 7.37 | 22.01 ± 8.23 | 0.001 | | SvO2 (%) | 65.50 ± 9.73 | 60.97 ± 10.00 | 0.004 | | Target therapy, n (%) | | | | | ERAs | 3 (10.1%) | 29 (16.1%) | 0.389 | | PDE-5is | 19 (63.2%) | 77 (42.7%) | 0.036 | | Prostacyclin analogues | 1 (3.3%) | 14 (7.8%) | 0.382 | | Combined therapies | 3 (10.1%) | 39 (21.7%) | 0.139 | | No specific treatment | 4 (13.3%) | 21 (11.7%) | 0.794 | | Laboratory variables | | | | | Uric acid (μmol/L) | 366.57 ± 156.03 | 404.13 ± 125.95 | 0.149 | | BUN (mmol/L) | 5.20 ± 1.81 | 5.51 ± 1.96 | 0.415 | | Creatinine (μmol/L) | 61.57 ± 21.00 | 67.23 ± 17.43 | 0.114 | | D-dimer (ng/ml) | 399 (218, 1673) | 149 (106, 202) | <0.001 | | International normalized ratio | 1.31 ± 0.32 | 1.22 ± 0.43 | 0.315 | | NT-proBNP (pg/ml) | 324 (81, 893) | 739 (262, 1904) | 0.004 | | BNP (pg/ml) | 120 (80, 402) | 221 (55, 427) | 0.727 | | Arterial blood gas | | | | | PH | 7.45 ± 0.03 | 7.45 ± 0.03 | 0.522 | | PO2 (mmHg) | 72.16 ± 13.72 | 72.13 ± 18.26 | 0.994 | | PCO2 (mmHg) | 33.96 ± 11.68 | 28.43 ± 5.07 | 0.019 | | SO2 (%) | 93.35 ± 5.13 | 93.00 ± 5.62 | 0.780 | | Blood cell counts (109/L) | | | | | WBC | 4.18 ± 1.78 | 6.68 ± 2.09 | <0.001 | | Neutrophils | 2.26 ± 1.40 | 3.88 ± 1.79 | <0.001 | | RBC | 4.24 ± 0.85 | 4.92 ± 0.63 | <0.001 | | PLT | 114.73 ± 85.67 | 178.92 ± 72.54 | <0.001 | | MELD scores | 11 (8–13) | NA | |
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POPH, portopulmonary hypertension; IPAH, idiopathic pulmonary arterial hypertension; WHO FC, World Health Organization functional classification; PBC, primary biliary cirrhosis; sPAP, systolic pulmonary artery pressure; mRAP, mean right atrial pressure; mPAP, mean pulmonary artery pressure; mPAWP, mean pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; TPG, transpulmonary gradient; SVR, systemic vascular resistance; SvO2, mixed venous oxygen saturation; ERAs, endothelin receptor antagonists; PDE-5is, phosphodiesterase 5 inhibitors; BUN, blood urea nitrogen; NT-proBNP, N-terminal pro brain natriuretic peptide; BNP, brain natriuretic peptide; PH, power of hydrogen; PO2, partial pressure of oxygen; PCO2, partial pressure of carbon dioxide; SO2, oxygen content of arterial blood; WBC, white blood cell; RBC, red blood cell; PLT, platelet; MELD score, model for end-stage liver disease scores; NA, not applicable. Hemodynamics were measured via right heart catheterization.
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