Table 2.
CTEPH risk factors, symptoms, hemodynamics, and functional class at initial visit or diagnosis
| Characteristic | Patients (N = 212) |
|---|---|
| Risk factors for CTEPH documented at initial visita, n (%) | |
| VTE | 89 (42) |
| Antiphospholipid antibody syndrome | 29 (14) |
| Antiphospholipid antibodies | 25 (12) |
| Lupus anticoagulant | 11 (5) |
| Thyroid hormone replacement therapy | 10 (5) |
| Large thrombus mass | 9 (4) |
| Splenectomy | 6 (3) |
| Elevated plasma concentrations of factor VIII | 3 (1) |
| Ventriculoatrial shunt | 0 (0) |
| Infected pacemaker | 0 (0) |
| None | 52 (25) |
| No assessment of risk factors | 49 (23) |
| Symptoms documented at diagnosis/initial visita, n (%) | |
| Shortness of breath | 196 (92)/155 (73) |
| Fatigue | 90 (42)/83 (39) |
| Edema | 74 (35)/49 (23) |
| Chest discomfort | 63 (30)/52 (25) |
| Cyanosis | 24 (11)/24 (11) |
| No symptoms documented | 1 (< 1)/10 (5) |
| History of VTE, n (%) | 145 (68) |
| Length of hospitalization for past VTE before initial visit (days), mean ± SD | 22 ± 51 (n = 64) |
| Time between symptoms and diagnosis (years), mean ± SD | 2.2 ± 3.0 |
| 6MWD (m), mean ± SD | 337.1 ± 118.1 |
| Hemodynamic values at diagnosis, mean ± SD | |
| RAP (mmHg) | 12.9 ± 11.0 (n = 123) |
| CO (L/min) | 4.0 ± 1.5 (n = 109) |
| CI (L/min/m2) | 2.3 ± 0.7 (n = 104) |
| PCWP (mmHg) | 11.7 ± 5.4 (n = 133) |
| mPAP (mmHg) | 52.7 ± 16.3 (n = 149) |
| PVR (WU) | 10.5 ± 7.8 (n = 111) |
| WHO functional class, n (%) | |
| Not assessed | 32 (15) |
| I | 23 (11) |
| II | 59 (28) |
| III | 84 (40) |
| IV | 14 (7) |
Percentages are for all 212 patients unless indicated
6MWD 6-minute walk distance, CI cardiac index, CO cardiac output, CTEPH chronic thromboembolic pulmonary hypertension, mPAP mean pulmonary arterial pressure, PCWP pulmonary capillary wedge pressure, PVR pulmonary vascular resistance, RAP right atrial pressure, SD standard deviation, VTE venous thromboembolism, WHO World Health Organization, WU Wood unit
aMultiple ethnicity, risk factors, or symptoms could be reported