Main cohort characteristics
| HC | DC | CTED | IPAH/HPAH | CTEPH-discovery | CTEPH-replication | Patients sampled at three locations | |
| Subjects | 121 | 132 | 63 | 433 | 108 | 92 | 86 |
| Demographics | |||||||
| Age at sampling years | 51 (37–57) | 59 (43–69) | 60 (45–70) | 54 (41–67) | 68 (56–76) | 66 (53–77) | 64 (50–71) |
| Female | 78 (64) | 90 (68) | 27 (43) | 305 (70) | 41 (38) | 56 (61) | 42 (49) |
| Ethnicity: European | 66 (55) | 57 (43) | 33 (66) | 358 (83) | 82 (76) | 68 (74) | 74 (86) |
| BMI kg·m−2 | 25 (24–30) | 27 (24–30) | 30 (26–34) | 28 (24–32) | 27 (24–30) | 28 (25–32) | 29 (25–34) |
| WHO functional class | |||||||
| I | ND | ND | 4 (17) | 28 (7) | 2 (2) | 7 (9) | 5 (6) |
| II | ND | ND | 12 (52) | 105 (26) | 23 (22) | 13 (16) | 30 (35) |
| III | ND | ND | 7 (30) | 238 (58) | 73 (69) | 53 (67) | 48 (56) |
| IV | ND | ND | 0 (0) | 38 (9) | 8 (8) | 6 (8) | 3 (3) |
| 6-min walk distance m | ND | ND | 387 (312–452) | 336 (187–420) | 282 (146–384) | 218 (96–352) | 352 (260–436) |
| Creatinine mmol·L−1 | ND | 71 (63–89) | 78 (72–86) | 83 (69–104) | 84 (70–106) | 88 (74–103) | 88 (72–103) |
| Bilirubin μmol·L−1 | ND | 9 (7–14) | 9 (7–13) | 11 (8–17) | 12 (9–19) | 12 (9–20) | 11 (8–14) |
| Albumin g·L−1 | ND | 40 (38–42) | 40 (39–42) | 40 (37–44) | 38 (35–40) | 38 (36–40) | 38 (36–40) |
| CRP mg·L−1 | ND | 3 (1–6) | 2 (1–3) | 4 (2–7) | 3 (2–7) | 5 (3–11) | 2 (1–7) |
| Haemodynamics at diagnosis | |||||||
| mRAP mmHg | ND | 6 (4–9) | 6 (4–8) | 9 (6–13) | 9 (6–12) | 9 (6–13) | 8 (5–11) |
| mPAP mmHg | ND | 20 (16–23) | 20 (17–22) | 53 (44 - 62) | 41 (33–54) | 45 (34–54) | 38 (33–44) |
| mPAWP mmHg | ND | 11 (9–14) | 10 (8–13) | 10 (7–13) | 12 (9–14) | 11 (8–13) | 10 (8–13) |
| PVR WU | ND | 1.7 (1.1–2.5) | 1.8 (1.2–2.3) | 11.1 (6.8–15.7) | 8.0 (4.9–11.3) | 7.9 (4.8–11.6) | 5.5 (4.0–8.1) |
| Cardiac output L·min−1 | ND | 4.8 (3.7–5.9) | 5.2 (4.5–6.0) | 3.8 (3.0–4.8) | 3.9 (3.3–4.6) | 4.0 (3.0–4.8) | 4.6 (4.1–5.7) |
| Comorbidities and medication | |||||||
| COPD | 0 (0) | 20 (15) | 4 (6) | 65 (15) | 8 (7) | 12 (13) | 8 (9) |
| Diabetes | 0 (0) | 15 (11) | 5 (8) | 82 (19) | 11 (10) | 8 (9) | 9 (10) |
| Atherosclerosis | 0 (0) | 14 (11) | 2 (4) | 59 (14) | 32 (37) | 17 (18) | 20 (23) |
| Atrial arrhythmia | 0 (0) | 22 (17) | 3 (5) | 57 (13) | 20 (19) | 22 (24) | 8 (9) |
| Hypertension | 0 (0) | 39 (30) | 12 (19) | 103 (24) | 36 (33) | 26 (28) | 29 (34) |
| Dyslipidaemia | 0 (0) | 16 (12) | 13 (21) | 44 (10) | 22 (20) | 14 (15) | 25 (29) |
| PDE-5i | 0 (0) | 0 (0) | 1 (2) | 283 (65) | 40 (39) | 34 (37) | 27 (31) |
| ERA | 0 (0) | 0 (0) | 0 (0) | 232 (54) | 28 (27) | 21 (23) | 15 (17) |
| Riociguat | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 3 (3) |
| Prostanoid | 0 (0) | 0 (0) | 0 (0) | 78 (18) | 2 (2) | 1 (1) | 4 (5) |
| Anticoagulation | 0 (0) | 44 (33) | 27 (84) | 291 (67) | 104 (96) | 87 (95) | Stopped for RHC |
| Loop diuretic | 0 (0) | 24 (18) | 5 (8) | 228 (53) | 47 (44) | 46 (50) | 46 (53) |
| Potassium-sparing diuretic | 0 (0) | 6 (5) | 2 (3) | 104 (24) | 24 (22) | 26 (28) | 17 (20) |
| Statin | 0 (0) | 40 (30) | 11 (22) | 112 (26) | 38 (42) | 35 (38) | 25 (29) |
| CCB | 0 (0) | 22 (17) | 5 (8) | 75 (17) | 4 (4) | 9 (10) | 12 (14) |
| Digoxin | 0 (0) | 11 (8) | 1 (2) | 68 (16) | 7 (6) | 8 (9) | 3 (3) |
| Antidiabetic drugs | 0 (0) | 13 (10) | 4 (9) | 62 (14) | 11 (13) | 8 (9) | 9 (10) |
| Iron supplementation | 0 (0) | 7 (5) | 1 (2) | 50 (12) | 8 (10) | 6 (7) | 7 (8) |
| ACEi | 0 (0) | 44 (33) | 11 (17) | 100 (23) | 30 (28) | 26 (28) | 12 (14) |
Data are presented as n, median (interquartile range) or n (%), unless otherwise stated. Ethnicity is shown for subjects who self-declared. A further group of 82 CTEPH patients sampled only after pulmonary endartectomy surgery are detailed insupplementary table S1. HC: healthy controls; DC: disease controls; CTED: chronic thromboembolic disease; IPAH/HPAH: idiopathic and heritable pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; BMI: body mass index; WHO: World Health Organization; CRP: C-reactive protein; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; mPAWP: mean pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; WU: Wood unit; PDE-5i: phosphodiesterase-5 inhibitor; ERA: endothelin receptor antagonist; CCB: calcium channel blocker; ACEi: angiotensin-converting enzyme inhibitor; ND: not determined; RHC: right-heart catheterisation.