Baseline demographic, clinical and haemodynamic characteristics of the chronic thromboembolic pulmonary hypertension (CTEPH) population according to World Health Organization functional class (WHO FC)
| Total | WHO FC | p-value | ||||
| I | II | III | IV | |||
| Subjects | 68 | 3 | 18 | 34 | 13 | |
| Demographic | ||||||
| Age at diagnosis years | 39.7±11.8 | 28.3±7.5 | 42.0±11.2 | 39.8±12.8 | 39.1±10.2 | 0.328 |
| Sex | ||||||
| Female | 46 (67.6) | 0 (0.0) | 13 (72.2) | 23 (67.6) | 10 (76.9) | 0.113 |
| Male | 22 (32.4) | 3 (100.0) | 5 (27.8) | 11 (32.4) | 3 (23.1) | |
| Clinical | ||||||
| 6MWD m | 214.0 (145.0–424.5) | 451.5 (401.2–501.8) | 395.5 (299.0–448.5) | 189.0 (92.0–275.0) | 149.5 (103.5–179.0) | 0.009 |
| Borg dyspnoea Borg units | 3.5±2.1 | 2.0±2.8 | 2.6±1.5 | 4.4±2.2 | 3.8±2.6 | 0.131 |
| Borg fatigue Borg units | 3.4±2.1 | 2.0±2.8 | 2.3±1.1 | 4.4±2.2 | 3.8±2.6 | 0.062 |
| Syncope | 5 (7.4) | 0 (0.0) | 0 (0.0) | 3 (8.8) | 2 (15.4) | 0.457 |
| NT-proBNP pg·mL−1 | 702.5 (119.2–1645.2) | 129.0 (89.0–724.5) | 212.0 (67.0–981.0) | 702.0 (130.0–1215.0) | 1778.0 (1051.0–2610.0) | 0.031 |
| Heart failure | 1 (1.5) | 0 (0.0) | 0 (0.0) | 1 (2.9) | 0 (0.0) | 1.000 |
| Haemodynamics | ||||||
| Cardiac index L·min−1·m−2 | 2.0 (1.7–2.3) | 2.0 (1.8–2.0) | 1.9 (1.7–2.1) | 2.0 (1.8–2.3) | 1.8 (1.7–2.4) | 0.704 |
| Cardiac output L·min−1 | 3.7 (3.1–4.3) | 4.3 (3.4–4.3) | 3.7 (3.0–4.2) | 4.0 (3.4–4.3) | 3.2 (2.8–4.0) | 0.542 |
| mPAP, mmHg | 49.6±14.3 | 55.0±19.7 | 47.1±13.4 | 49.9±15.3) | 51.0±12.9 | 0.791 |
| Oxygen saturation baseline % | 96.2±2.3 | 96.5±2.1 | 96.0±2.0 | 97.2±2.2 | 94.0±2.0 | 0.047 |
| Oxygen saturation minimum % | 89.6±5.6 | 94.5±0.7 | 89.9±5.2 | 89.7±5.8 | 86.6±6.7 | 0.410 |
| PADP mmHg | 28.0 (23.0–38.0) | 32.0 (25.5–38.0) | 27.0 (22.0–36.0) | 30.5 (23.5–38.0) | 29.0 (23.0–35.0) | 0.944 |
| PASP mmHg | 78.9±26.1 | 92.7±32.0 | 74.9±24.0 | 77.6±28.8 | 84.0±21.2 | 0.622 |
| PCWP mmHg | 15.0 (11.0–18.0) | 10.0 (9.5–15.0) | 15.0 (11.0–19.0) | 14.0 (11.8–18.0) | 15.0 (11.0–18.8) | 0.836 |
| PVR Woods units | 9.7 (4.9–13.1) | 11.6 (8.5–15.6) | 9.6 (4.6–11.8) | 9.6 (5.2–13.2) | 9.4 (5.1–13.4) | 0.880 |
| RAP mmHg | 13.0±6.1 | 10.7±12.5 | 12.0±4.8 | 12.2±4.6 | 17.2±8.2 | 0.082 |
| Stroke volume mL·beat−1 | 50.9±18.0 | 51.9±15.1 | 51.7±14.3 | 52.3±19.7 | 46.3±20.0 | 0.810 |
| SVR WU | 18.2 (15.9–22.3) | 16.8 (16.0–22.2) | 18.3 (15.5–22.8) | 17.7 (15.8–20.5) | 18.6 (16.2–25.7) | 0.846 |
| TPR WU | 14.0 (8.0–17.4) | 16.1 (11.9–19.6) | 14.1 (8.2–16.4) | 13.4 (7.9–16.4) | 14.5 (13.0–19.1) | 0.701 |
Data are presented as n, mean±sd, n (%) or median (interquartile range), unless otherwise stated. 6MWD: 6-min walk distance; NT-proBNP: N-terminal pro-brain natriuretic peptide; mPAP: mean pulmonary arterial pressure; PADP: pulmonary artery diastolic pressure; PASP: pulmonary artery systolic pressure; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; RAP: right atrial pressure; SVR: systemic vascular resistance; TPR: total pulmonary resistance.