Table 1. Baseline demographic characteristics of patients with chronic thromboembolic pulmonary hypertension.
| Variables | Mean ± SD or number (%) | Range |
| Female, n (%) | 19 (83%) | |
| Age at diagnosis, years | 48.4 ± 16.1 | 9-71 |
| BMI, kg/m2 | 24.4 ± 7.0 | 14.2-46.7 |
| SBP, mmHg | 125 ± 18 | 96-160 |
| DBP, mmHg | 81 ± 13 | 58-106 |
| HR, beats/min | 89 ± 12 | 54-122 |
| SpO2, % | 90 ± 8 | 77-100 |
| FC II/III/IV, n (%) | 1 (4%)/14 (61%)/8 (35%) | |
| 6MWD, m | 338 ± 86 | 180-474 |
| Non-O blood group, n (%) | 21 (91%) | |
| Symptoms to diagnosis, months | 27.7 ± 61.5 | 2-275 |
| VTE, n (%) | 19 (83%) | |
| Acute PE, n (%) | 17 (74%) | |
| DVT, n (%) | 8 (35%) | |
| Smoking, n (%) | 5 (22%) | |
| Estrogen replacement therapy, n (%) | 4 (17%) | |
| Comorbidities | ||
| Hypertension, n (%) | 7 (30%) | |
| Malignancy*, n (%) | 5 (22%) | |
| APS, n (%) | 4 (17%) | |
| AF, n (%) | 4 (17%) | |
| Asthma, n (%) | 4 (17%) | |
| CAD, n (%) | 3 (13%) | |
| Others#, n (%) | 7 (30%) | |
| Laboratory profiles | ||
| Hemoglobin, g/dL | 13.6 ± 2.4 | 9.4-18.5 |
| WBC, 103/μL | 9.0 ± 3.9 | 3.8-18.8 |
| Platelet, 103/μL | 243 ± 109 | 68-518 |
| D-dimer, ng/mL | 2853 ± 2869 | 170-10000 |
| Fibrinogen, mg/dL | 368 ± 117 | 186-640 |
| Protein C, % | 78.5 ± 22.7 | 43.7-124.1 |
| Protein S, % | 100.5 ± 36.3 | 22.9-185.3 |
| AST, U/L | 39 ± 26 | 16-120 |
| ALT, U/L | 40 ± 43 | 5-181 |
| Creatinine, mg/dL | 0.9 ± 0.3 | 0.4-1.6 |
| Uric acid, mg/dL | 7.2 ± 2.6 | 3.5-12.5 |
| Total cholesterol, mg/dL | 168 ± 46 | 108-280 |
| Fasting glucose, mg/dL | 106 ± 30 | 74-198 |
| BNP, pg/mL | 429 ± 500 | 19-1911 |
| 2D echocardiography | ||
| TRV, m/s | 4.2 ± 0.5 | 2.7-4.8 |
| SPAP, mmHg | 76 ± 18 | 32-102 |
| MPAP | 48 ± 11 | 22-64 |
| Tei index | 0.58 ± 0.24 | 0.06-1.00 |
| TRV/VTIRVOT ratio | 0.45 ± 0.18 | 0.14-0.78 |
| TAPSE, cm | 1.61 ± 0.41 | 1.10-2.43 |
| RA area, cm2 | 24 ± 8 | 10-43 |
| Systolic LVeI | 1.60 ± 0.53 | 1.10-3.18 |
| Diastolic LVeI | 1.42 ± 0.26 | 1.02-2.08 |
| RVFAC, % | 25 ± 8 | 17-43 |
| Pericardial effusion, n (%) | 8 (35%) | |
| Cardiac catheterization | ||
| RAP, mmHg | 12 ± 7 | 3-30 |
| SPAP, mmHg | 84 ± 20 | 51-129 |
| MPAP, mmHg | 51 ± 10 | 32-74 |
| PAWP, mmHg | 11 ± 5 | 3-21 |
| CO, L/min | 3.0 ± 1.0 | 1.6-4.8 |
| CI, L/min·m2 | 1.9 ± 0.6 | 1.2-3.1 |
| PVR, WU | 12.4 ± 6.8 | 4.5-28.1 |
| MvO2, % | 58 ± 13 | 37-76 |
| SaO2, % | 92 ± 6 | 75-100 |
| Treatment | ||
| PAH-specific drugs, n (%) | 8 (35%) | |
| PDE5i, n (%) | 7 (30%) | |
| ERA, n (%) | 2 (9%) | |
| sCGS, n (%) | 6 (26%) | |
| PEA, n (%) | 19 (83%) | |
| Bridging therapy, n (%) | 3 (16%) | |
| IVC filter implantation, n (%) | 5 (22%) | |
| BPA, n (%) | 4 (17%) |
Data are expressed as mean ± SD for continuous variables and as number and percentage for categorical variables.
* Malignancy included 2 colorectal cancer, 1 breast cancer, 1 ovarian with endometrial cancer, and 1 cervical cancer; # Others included 2 diabetes mellitus, 2 systemic lupus erythematosus, 1 embolic stroke, 1 chronic obstructive pulmonary disease, and 1 hemolytic anemia with splenectomy.
AF, atrial fibrillation; ALT, alanine aminotransferase; APS, antiphospholipid syndrome; AST, aspartate aminotransferase; BMI, body mass index; BNP, B-type natriuretic peptide; BPA, balloon pulmonary angioplasty; CAD, coronary artery disease; CI, cardiac index; CO, cardiac output; DBP, diastolic blood pressure; DVT, deep venous thrombosis; ERA, endothelin receptor antagonist; FC, functional class; HR, heart rate; IVC, inferior vena cava; LVeI, left ventricular eccentricity index; MPAP, mean pulmonary artery pressure; MvO2, mixed venous oxygen saturation; PAH, pulmonary arterial hypertension; PAWP, pulmonary artery wedge pressure; PDE5i, phosphodiesterase-5 inhibitor; PE, pulmonary embolism; PEA, pulmonary endarterectomy; PVR, pulmonary vascular resistance; RA, right atrial; RAP, right atrial pressure; RVFAC, right ventricular fractional area change; SaO2, arterial oxygen saturation; SBP, systolic blood pressure; sCGS, soluble guanylate cyclase stimulator; SPAP, systolic pulmonary artery pressure; SpO2, oxyhemoglobin saturation by pulse oximetry; TAPSE, tricuspid annular plane systolic excursion; TRV, peak tricuspid regurgitation velocity; VTIRVOT, velocity time integral at the right ventricular outflow tract; VTE, venous thromboembolism; WBC, white blood cell; 6MWD, 6-minute walk distance.