Number of patients n (%) | 58 | 58 (100) | 44 (76) | 14 (24) | |
Female n (%) | 58 | 32 (55) | 27 (61) | 5 (36) | 0.09 |
Age years | 58 | 44 (18–67) | 43 (18–61) | 48 (18–67) | 0.05 |
Body mass index kg·m−2 | 58 | 22 (16–34) | 21 (16–34) | 26 (20–32) | 0.0002 |
History of acute chest syndrome n (%) | 58 | 36 (62) | 31 (70) | 5 (36) | 0.02 |
Current smokers n (%) | 58 | 6 (10) | 6 (14) | 0 (0) | - |
Former smokers n (%) | 58 | 6 (10) | 6 (14) | 0 (0) | - |
Reported history of renal complications n (%) | 58 | 17 (29) | 15 (34) | 2 (14) | 0.2 |
History of leg ulcers n (%) | 58 | 13 (22) | 13 (30) | 0 (0) | - |
History of pulmonary embolism n (%) | 58 | 21 (36) | 14 (32) | 7 (50) | 0.2 |
History of deep vein thrombosis n (%) | 58 | 5 (9) | 4 (9) | 1 (7) | 0.8 |
Treatment regimens at time of PH diagnosis | 58 | | | | |
Hydroxyurea therapy n (%) | | 21 (36) | 19 (43) | 2 (14) | 0.05 |
Regular transfusion programme n (%) | | 11 (19) | 10 (23) | 1 (7) | 0.2 |
PH-associated risk factor n (%) | 58 | | | | |
Interstitial lung disease n (%) | | 11 (19) | 7 (16) | 4 (29) | 0.3 |
Portal hypertension n (%) | | 2 (3) | 2 (5) | 0 (0) | - |
Biology |
Haemoglobin g·dL−1 | 57 | 9 (6.5–14.2) | 8.4 (6.5–11.5) | 10.6 (9–14.2) | <0.0001 |
Creatinine µmol·L−1 | 54 | 83 (36–167) | 83 (36–167) | 85 (59–114) | 0.8 |
Lactate dehydrogenase IU·L−1 | 35 | 944 (317–3034) | 1052 (334–3034) | 458 (317–1457) | 0.03 |
Total bilirubin µmol·L−1 | 46 | 52 (8–233) | 63 (11–233) | 19 (8–37) | <0.0001 |
Arterial blood gases |
PaO2 mmHg | 46 | 69 (43–100) | 68 (43–100) | 73 (52–90) | 0.2 |
PaCO2 mmHg | 46 | 37 (25–56) | 37 (25–46) | 38 (29–56) | 0.8 |
Pulmonary function tests |
FVC % | 51 | 63 (25–97) | 60 (25–94) | 73 (44–97) | 0.03 |
FEV1 % | 52 | 67 (23–107) | 63 (23–107) | 76 (44–107) | 0.06 |
TLC % | 53 | 71 (36–99) | 69 (36–99) | 74 (52–96) | 0.3 |
DLCO % | 48 | 48 (16–81) | 46 (25–78) | 58 (43–81) | 0.02 |
DLCO adjusted for Hb % | 48 | 57 (22–98) | 58 (22–81) | 56 (51–98) | 0.24 |
NYHA-FC n (%) | 58 | | | | |
I or II | | 19 (33) | 15 (34) | 4 (29) | 0.7 |
III or IV | | 39 (67) | 29 (66) | 10 (71) | |
6-min walk distance m | 51 | 335 (0–569) | 308 (0–544) | 423 (0–569) | 0.01 |
Haemodynamics |
RAP mmHg | 58 | 8 (0–34) | 7 (0–34) | 8 (3–11) | 0.1 |
mPAP mmHg | 58 | 36 (25–62) | 35 (25–62) | 38 (26–54) | 0.2 |
PAWP mmHg | 58 | 9 (1–5) | 9 (1–14) | 10 (1–15) | 0.2 |
Cardiac index, L·min−1·m−2 | 58 | 4.1 (1.8–7.5) | 4.4 (1.8–7.5) | 3.2 (2.1–4.9) | 0.002 |
PVR Wood units | 58 | 4.7 (1.4–21) | 4.6 (1.4–21) | 5.3 (1.8–10.7) | 0.04 |
Mixed venous saturation % | 40 | 62 (32–86) | 63 (32–86) | 61 (44–73) | 0.6 |
BNP >100 ng·L−1 or NT-proBNP >300 ng·L−1 | 50 | 23 (46) | 19 (51) | 4 (31) | 0.3 |