Clinical, laboratory, and imaging variables across study time points
| Variable . | Baseline . | Year 1 . | Year 2 . | Annualized change . |
|---|---|---|---|---|
| Laboratory values | ||||
| Hb (g/dL) | 9.8 (1.4) | 10 (1.4) | 10.2 (1.4) | 0.16 (0.6) |
| Fetal Hb (%) | 18 (13) | 16 (13) | 15 (12) | −2.4 (7) |
| Reticulocyte count (%) | 8.1 (5) | 7.3 (4) | 6.2 (3) | −0.3 (1.6) |
| NT-proBNP (pg/mL) | 126 (187) | 104 (116) | 85 (78) | −21 (82) |
| eGFR (mL/min per 1.73 m2) | 141 (36) | 142 (38) | 144 (40) | 1.2 (14) |
| Total bilirubin (mg/dL) | 2.4 (1.5) | 2.5 (1.7) | 2.3 (1) | −0.04 (0.7) |
| AST (U/L) | 48 (28) | 46 (25) | 53 (47) | 2.1 (25) |
| Lactate dehydrogenase (U/L) | 550 (271) | 500 (217) | 547 (371) | −1.6 (210) |
| White blood cell (×103/μL) | 9.5 (3.5) | 9.2 (3) | 9.6 (4.1) | 0.02 (1.7) |
| Platelet count (×103/μL) | 343 (96) | 330 (83) | 309 (85) | −20 (39) |
| Aldosterone (ng/dL) | 8.4 (6) | 7.1 (4) | 10.2 (8) | 1.1 (4) |
| Plasma renin activity (ng/mL per h) | 1.9 (1.4) | 1.9 (1.9) | 2 (2.4) | −0.02 (1.3) |
| Aldosterone-to-renin ratio | 6.2 (13) | 10.5 (13) | 13.6 (36) | 4.8 (21) |
| Echocardiographic and diastolic parameters | ||||
| TRV (m/s) | 2.5 (0.5) | 2.7 (0.6) | 2.4 (0.5) | −0.05 (0.25) |
| E:A ratio | 1.9 (0.6) | 2.2 (0.5) | 2.1 (0.6) | 0.09 (0.3) |
| Tricuspid E:A ratio | 1.6 (0.4) | 1.7 (0.4) | 1.6 (0.4) | 0.02 (0.3) |
| Lateral e' (cm/s) | 15 (3.8) | 14.8 (3.4) | 14.3 (3.1) | −0.5 (1.5) |
| Lateral E:e' ratio | 7.3 (2.2) | 7.7 (2.1) | 7.7 (2.6) | 0.21 (0.9) |
| Diastolic classification | ||||
| DD | 5/24 | 6/24 | 5/24 | N/A |
| Normal | 6/24 | 6/24 | 6/24 | N/A |
| CMR parameters | ||||
| LAVi (mL/m2) | 44 (11) | 46 (9) | 44 (10) | 0.0 (4) |
| LVEDVi (mL/m2) | 110 (26) | 109 (20) | 110 (22) | 1.03 (6) |
| LVESVi (mL/m2) | 48 (14) | 46 (11) | 47 (13) | −0.31 (3) |
| LVEF (%) | 58 (6) | 58 (4) | 58 (5) | 0.1 (3) |
| Cardiac index (L/min per m2) | 4.5 (1) | 4.4 (1) | 4.6 (1.2) | 0.06 (0.4) |
| LV mass index (g/m2) | 61 (13) | 61 (12) | 61 (11) | 0.5 (4.1) |
| Native T1 (ms) | 1020 (71) | 1052 (33) | 1037 (38) | 5.5 (33) |
| ECV (%) | 32.7 (5) | 33.3 (4) | 32.2 (4) | −1.1 (2.7) |
| ΔECV % | N/A | −0.8 (14) | −1.3 (11) | −2.9 (7) |
| Extracellular matrix volume index (mL/m2) | 19.2 (6) | 18.9 (5) | 18.2 (4.2) | −0.9 (3) |
| Cellular volume index (mL/m2) | 39.1 (9) | 38.1 (7) | 39 (7) | 1.1 (4) |
| Clinical variables | ||||
| Acute care visits, median (range) | 1.5 (0-35) | 1.5 (0-28) | 1.5 (0-20) | N/A |
| Acute chest syndrome, median (range) | 1 (0-4) | 0 (0-3) | 0 (0-5) | N/A |
| Transfusion incidents, median (range) | 1.5 (0-12) | 0 (0-16) | 0 (0-14) | N/A |
| Prescribed hydroxyurea (%) | 19 (79) | 19 (79) | 17 (71) | N/A |
| Variable . | Baseline . | Year 1 . | Year 2 . | Annualized change . |
|---|---|---|---|---|
| Laboratory values | ||||
| Hb (g/dL) | 9.8 (1.4) | 10 (1.4) | 10.2 (1.4) | 0.16 (0.6) |
| Fetal Hb (%) | 18 (13) | 16 (13) | 15 (12) | −2.4 (7) |
| Reticulocyte count (%) | 8.1 (5) | 7.3 (4) | 6.2 (3) | −0.3 (1.6) |
| NT-proBNP (pg/mL) | 126 (187) | 104 (116) | 85 (78) | −21 (82) |
| eGFR (mL/min per 1.73 m2) | 141 (36) | 142 (38) | 144 (40) | 1.2 (14) |
| Total bilirubin (mg/dL) | 2.4 (1.5) | 2.5 (1.7) | 2.3 (1) | −0.04 (0.7) |
| AST (U/L) | 48 (28) | 46 (25) | 53 (47) | 2.1 (25) |
| Lactate dehydrogenase (U/L) | 550 (271) | 500 (217) | 547 (371) | −1.6 (210) |
| White blood cell (×103/μL) | 9.5 (3.5) | 9.2 (3) | 9.6 (4.1) | 0.02 (1.7) |
| Platelet count (×103/μL) | 343 (96) | 330 (83) | 309 (85) | −20 (39) |
| Aldosterone (ng/dL) | 8.4 (6) | 7.1 (4) | 10.2 (8) | 1.1 (4) |
| Plasma renin activity (ng/mL per h) | 1.9 (1.4) | 1.9 (1.9) | 2 (2.4) | −0.02 (1.3) |
| Aldosterone-to-renin ratio | 6.2 (13) | 10.5 (13) | 13.6 (36) | 4.8 (21) |
| Echocardiographic and diastolic parameters | ||||
| TRV (m/s) | 2.5 (0.5) | 2.7 (0.6) | 2.4 (0.5) | −0.05 (0.25) |
| E:A ratio | 1.9 (0.6) | 2.2 (0.5) | 2.1 (0.6) | 0.09 (0.3) |
| Tricuspid E:A ratio | 1.6 (0.4) | 1.7 (0.4) | 1.6 (0.4) | 0.02 (0.3) |
| Lateral e' (cm/s) | 15 (3.8) | 14.8 (3.4) | 14.3 (3.1) | −0.5 (1.5) |
| Lateral E:e' ratio | 7.3 (2.2) | 7.7 (2.1) | 7.7 (2.6) | 0.21 (0.9) |
| Diastolic classification | ||||
| DD | 5/24 | 6/24 | 5/24 | N/A |
| Normal | 6/24 | 6/24 | 6/24 | N/A |
| CMR parameters | ||||
| LAVi (mL/m2) | 44 (11) | 46 (9) | 44 (10) | 0.0 (4) |
| LVEDVi (mL/m2) | 110 (26) | 109 (20) | 110 (22) | 1.03 (6) |
| LVESVi (mL/m2) | 48 (14) | 46 (11) | 47 (13) | −0.31 (3) |
| LVEF (%) | 58 (6) | 58 (4) | 58 (5) | 0.1 (3) |
| Cardiac index (L/min per m2) | 4.5 (1) | 4.4 (1) | 4.6 (1.2) | 0.06 (0.4) |
| LV mass index (g/m2) | 61 (13) | 61 (12) | 61 (11) | 0.5 (4.1) |
| Native T1 (ms) | 1020 (71) | 1052 (33) | 1037 (38) | 5.5 (33) |
| ECV (%) | 32.7 (5) | 33.3 (4) | 32.2 (4) | −1.1 (2.7) |
| ΔECV % | N/A | −0.8 (14) | −1.3 (11) | −2.9 (7) |
| Extracellular matrix volume index (mL/m2) | 19.2 (6) | 18.9 (5) | 18.2 (4.2) | −0.9 (3) |
| Cellular volume index (mL/m2) | 39.1 (9) | 38.1 (7) | 39 (7) | 1.1 (4) |
| Clinical variables | ||||
| Acute care visits, median (range) | 1.5 (0-35) | 1.5 (0-28) | 1.5 (0-20) | N/A |
| Acute chest syndrome, median (range) | 1 (0-4) | 0 (0-3) | 0 (0-5) | N/A |
| Transfusion incidents, median (range) | 1.5 (0-12) | 0 (0-16) | 0 (0-14) | N/A |
| Prescribed hydroxyurea (%) | 19 (79) | 19 (79) | 17 (71) | N/A |
All values are expressed as mean (standard deviation) except for clinical variables.
AST, aspartate aminotransferase; DD, diastolic dysfunction; E:A, ratio of peak early (E) to late (A) diastolic filling velocities; eGFR, estimated glomerular filtration rate; LVEDVi, left ventricular end-diastolic volume indexed to body surface area; LVESVi, left ventricular end-systolic volume indexed to body surface area; max, maximum; min, minimum; N/A, not applicable.