Table 1 Patients characteristics at Baseline.

Demographic and clinical characteristics

 Age (years)

41.5 ± 11.6

 Male gender, n (%)

15 (51.7)

 Body mass index (kg m⁻²)

23.4 ± 3.9

 Systolic arterial pressure (mmHg)

134 ± 19.1

 Diastolic arterial pressure (mmHg)

82.1 ± 12.7

 Mean arterial pressure (mmHg)

99.5 ± 14.1

 Heart rate (bpm)

77.8 ± 14.5

 Oxygen saturation (%)

96.7 ± 2.82

Treatments

 Hydroxyurea, n (%)

21 (72.4)

 Erythrapheresis, n (%)

10 (34.5)

 Beta-blocker, n (%)

9 (31)

Events and organ damage

 Occurrence of VOC and/or ATS, n (%)

28 (96.5)

 Intensive care unit admission, n (%)

11 (37.9)

 Pulmonary hypertension on RHC (n = 14), n (%)

7 (50)

 Cerebral vasculopathy, n (%)

10 (34.5)

 Retinopathy, n (%)

24 (82.8)

 Kidney damage without CKD*, n (%)

8 (27.6)

 Kidney damage with CKD**, n (%)

7 (24.1)

 Skin ulcers, n (%)

6 (20.7)

 Osteonecrosis, n (%)

9 (31.0)

 Priapism, n (%)

9 (60.0)

Biology

 Hemoglobin (g dL⁻¹)

8.7 ± 1.1

 Leukocytes (G L⁻¹)

7.9 ± 2.8

 Platelets (G L⁻¹)

287 ± 179

 Urea (mmol L⁻¹)

5.3 ± 3.6

 Creatinine (µmol L⁻¹)

68.5 [46.7; 90.2]

 Lactate dehydrogenase (IU L⁻¹)

490 ± 188

 Total bilirubin (µmol L⁻¹)

40.4 ± 23.7

 Aspartate transaminase (IU L⁻¹)

43.3 ± 16.7

 Alanine transaminase (IU L⁻¹)

24.9 ± 9.6

 Gamma-glutamyl transpeptidase (IU L⁻¹)

105 ± 115

 Alkaline phosphatase (IU L⁻¹)

96 ± 53.3

Echocardiography

 LVEF (%)

61.7 ± 5.2

 LVEDV_ind (mL m⁻²)

76.3 ± 16.4

 LVM_ind (g m⁻²)

93 ± 23

 GLS (%)

− 19 ± 3

 Cardiac index (L min⁻¹ m⁻²)

3.9 ± 0.7

 E (cm s⁻¹)

92 ± 23

 E deceleration time (ms)

200 ± 46

 A (cm s⁻¹)

66 ± 21

 E/A

1.5 ± 0.6

 e′ lateral (cm s⁻¹)

13.1 ± 3.1

 E/e′

7.3 ± 2.1

 LAV_ind (mL m⁻²)

57 ± 26

 TAPSE (mm)

27.7 ± 5.2

 Sdti (cm s⁻¹)

14.3 ± 3

 TRV (m s⁻¹)

2.5 ± 0.4

 Pulmonary acceleration time (ms)

122 ± 28

 Mild to moderate valve disease (grade 2–3), n (%)

2 (7)

 Ischemic heart disease, n (%)

1 (3.4)

Spirometry

 EV1 (L)

2.5 ± 0.7

 FEV1/FVC

82.7 ± 6.7