Patients n | 13 | 13 | |
Age years | 43 (27–57) | 48 (29–66) | 0.5 |
Female | 7 (54) | 10 (77) | 0.2 |
History of vaso-occlusive disease | 11 (85) | 13 (100) | 0.1 |
>2 vaso-occlusive crises per year | 7 (54) | 9 (69) | 0.4 |
History of acute chest syndrome | 11 (85) | 9 (69) | 0.3 |
History of leg ulcers | 4 (31) | 4 (31) | 1 |
History of acute thromboembolic disease | 4 (31) | 2 (15) | 0.3 |
Hydroxyurea therapy | 11 (85) | 6 (43) | 0.02 |
History of splenectomy | 1 (8) | 2 (15) | 0.5 |
Laboratory values | | | |
Haemoglobin g·dL−1 | 8.3 (7–9.6) | 8.5 (6.6–11.5) | 0.6 |
Lactate dehydrogenase IU·L−1 | 937 (415–3034) | 785 (678–1464) | 0.8 |
Total bilirubin µmol·L−1 | 35 (22–92) | 24 (11–41) | 0.06 |
Creatinine µmol·L−1 | 84 (36–137) | 71 (44–89) | 0.2 |
Pulmonary function | | | |
FEV1 % pred | 65 (29–76) | 61 (50–95) | 0.9 |
FVC % pred | 65 (29–81) | 67 (50–97) | 0.7 |
TLC % pred | 69 (36–95) | 71 (51–99) | 0.2 |
DLCO % pred | 37 (25–68) | 44 (29–78) | 0.8 |
PaO2 mmHg | 68 (43–100) | 75 (64–83) | 0.6 |
PaCO2 mmHg | 37 (27–46) | 38 (30–43) | 0.8 |
NYHA FC | | | |
I/II | 2 (15) | 7 (54) | 0.04 |
III/IV | 11 (85) | 6 (46) | |
6MWD m | 234 (0–501) | 395 (210–527) | 0.06 |
Tricuspid regurgitant velocity m·s−1 | 3.95 (3.01–4.8) | 3.16 (2.50–4.05) | 0.026 |
Haemodynamics | | | |
Right atrial pressure mmHg | 9 (7–22) | 3 (2–8) | 0.001 |
Mean pulmonary artery pressure mmHg | 36 (30–48) | 30 (25–44) | 0.02 |
Pulmonary capillary wedge pressure mmHg | 10 (4–14) | 10 (5–14) | 0.6 |
Cardiac index L·min−1·m−2 | 5.5 (2.4–7.5) | 4.9 (3.2–6.6) | 0.5 |
Pulmonary vascular resistance WU | 2.7 (1.9–9.3) | 2.7 (1.4–4) | 0.7 |
SvO2 % | 60 (32–80) | 67 (57–77) | 0.2 |