Demographic and clinical characteristics overall and by use of early therapy(<6 years of age)
| . | Overall (n = 31), n (%) or median (IQR) . | No early therapy (n = 8), n (%) or median (IQR) . | Early therapy (<6 y) (n = 20), n (%) or median (IQR) . | Normal values . |
|---|---|---|---|---|
| Age at CMR (y) | 16.7 (14.7-18.0) | 16.8 (15.2-19.0) | 17.0 (14.4-17.9) | |
| Sex | ||||
| Male | 16 (51.6) | 2 (25.0) | 12 (60.0) | |
| Female | 15 (48.4) | 6 (75.0) | 8 (40.0) | |
| Weight (kg) | 62.0 (46.2-69.5) | 64.8 (50.0-73.1) | 58.2 (45.6-70.1) | |
| Height (cm) | 162.5 (152.9-171.8) | 161.9 (155.8-166.2) | 164.4 (152.9-176.1) | |
| BSA (m2) | 1.7 (1.4-1.8) | 1.7 (1.5-1.8) | 1.6 (1.4-1.9) | |
| Received hydroxyurea in the past 2 y | ||||
| Yes | 11 (35.5) | 3 (37.5) | 8 (40.0) | |
| No | 20 (64.5) | 5 (62.5) | 12 (60.0) | |
| Received chronic transfusions in the past 2 y | ||||
| Yes | 14 (45.2) | 3 (37.5) | 11 (55.0) | |
| No | 17 (54.8) | 5 (62.5) | 9 (45.0) | |
| Receiving beta-blockers | ||||
| Yes | 10 (32.3) | 0 (0.0) | 8 (40.0) | |
| No | 21 (67.7) | 8 (100.0) | 12 (60.0) | |
| Laboratory data | ||||
| Hemoglobin (g/dL)∗ | 8.9 (8.2-9.9) | 9.1 (8.2-9.7) | 9.0 (8.2-10.0) | 12-16 |
| Hematocrit (%) | 25.6 (23.6-27.8) | 26.0 (23.0-30.2) | 25.8 (23.7-27.4) | 36-46 |
| Reticulocyte absolute (/mm3)∗ | 0.208 (0.139-0.338) | 0.157 (0.125-0.236) | 0.252 (0.155-0.358) | 0.021-0.085 |
| LDH∗ | 473 (333-573) | 452 (389-504) | 464 (329-629) | 94-260 |
| Bilirubin (total)∗ | 2.7 (1.9-3.5) | 1.9 (1.5-2.5) | 3.2 (2.1-3.6) | 0.0-1.0 |
| Cardiac MRI data | ||||
| RVEDVi (mL/m2) | 108.1 (95.3-121.3) | 91.4 (84.2-101.2) | 110.3 (104.4-123.4) | 82 ± 17 (48-123)11 |
| RVESVi (mL/m2) | 44.3 (36.7-54.9) | 38.0 (35.3-41.0) | 46.7 (39.1-56.4) | 34 ± 11 (13-59)11 |
| RVEF (%) | 56.8 (53.7-61.4) | 58.3 (55.5-61.8) | 56.7 (53.9-61.6) | 58 ± 8 (42-74)11 |
| RVCi (L/min/m2) | 4.2 (3.7-5.4) | 3.8 (3.3-4.2) | 4.7 (4.1-5.7) | 2.9 ± 0.7 (1.5-4.5)11 |
| LVEDVi (mL/m2) | 109.2 (95.4-123.1) | 93.8 (75.7-113.4) | 118.7 (99.8-125.9) | 74 ± 15 (47-107)11 |
| LVESVi (mL/m2) | 48.2 (37.8-52.1) | 38.2 (31.8-50.6) | 50.3 (43.1-55.1) | 27 ± 9 (10-47)11 |
| LV mass indexed value (g/m2) | 64.5 (54.0-75.5) | 56.2 (44.9-70.4) | 65.5 (59.9-77.2) | 51 ± 10 (30-75)11 |
| LVEF (%) | 57.4 (55.3-59.8) | 58.0 (57.0-59.6) | 57.2 (55.1-59.8) | 64 ± 7 (51-79)11 |
| LVCi (L/min/m2) | 4.2 (3.9-5.5) | 3.9 (3.6-4.2) | 4.8 (4.1-5.8) | 3.1 ± 0.6 (1.9-4.3)11 |
| LA volume (mL/m2) | 40.5 (30.0-51.5) | 36.8 (24.8-46.0) | 42.4 (32.4-59.0) | 34 ± 10 (13-53)12 |
| Native T1 (ms) | 1029 (1004-1051) | 1020 (986-1057) | 1028 (1011-1036) | 1005 ± 25 (959-1052)† |
| Native T2 (ms)∗ | 46.5 (44.9-48.3) | 46.6 (45.9-47.3) | 46.6 (45.0-48.5) | 44 ± 2 (40-50)† |
| Native T2 > 50 | 2 (6.7) | 1 (12.5) | 1 (5.3) | |
| ECV (%) | 32.0 (29.0-34.0) | 31.0 (29.8-34.0) | 31.5 (29.0-34.0) | ≥18 y = 25 ± 313; <18 y = 21 ± 29 |
| ECV > 25 | 31 (100.0) | 8 (100.0) | 20 (100.0) | |
| ECV ≥ 30 | 22 (71.0) | 6 (75.0) | 14 (70.0) | |
| T2∗ (ms) | 35.0 (32.2-39.0) | 33.8 (31.2-34.8) | 35.9 (33.6-40.4) | >20 ms |
| . | Overall (n = 31), n (%) or median (IQR) . | No early therapy (n = 8), n (%) or median (IQR) . | Early therapy (<6 y) (n = 20), n (%) or median (IQR) . | Normal values . |
|---|---|---|---|---|
| Age at CMR (y) | 16.7 (14.7-18.0) | 16.8 (15.2-19.0) | 17.0 (14.4-17.9) | |
| Sex | ||||
| Male | 16 (51.6) | 2 (25.0) | 12 (60.0) | |
| Female | 15 (48.4) | 6 (75.0) | 8 (40.0) | |
| Weight (kg) | 62.0 (46.2-69.5) | 64.8 (50.0-73.1) | 58.2 (45.6-70.1) | |
| Height (cm) | 162.5 (152.9-171.8) | 161.9 (155.8-166.2) | 164.4 (152.9-176.1) | |
| BSA (m2) | 1.7 (1.4-1.8) | 1.7 (1.5-1.8) | 1.6 (1.4-1.9) | |
| Received hydroxyurea in the past 2 y | ||||
| Yes | 11 (35.5) | 3 (37.5) | 8 (40.0) | |
| No | 20 (64.5) | 5 (62.5) | 12 (60.0) | |
| Received chronic transfusions in the past 2 y | ||||
| Yes | 14 (45.2) | 3 (37.5) | 11 (55.0) | |
| No | 17 (54.8) | 5 (62.5) | 9 (45.0) | |
| Receiving beta-blockers | ||||
| Yes | 10 (32.3) | 0 (0.0) | 8 (40.0) | |
| No | 21 (67.7) | 8 (100.0) | 12 (60.0) | |
| Laboratory data | ||||
| Hemoglobin (g/dL)∗ | 8.9 (8.2-9.9) | 9.1 (8.2-9.7) | 9.0 (8.2-10.0) | 12-16 |
| Hematocrit (%) | 25.6 (23.6-27.8) | 26.0 (23.0-30.2) | 25.8 (23.7-27.4) | 36-46 |
| Reticulocyte absolute (/mm3)∗ | 0.208 (0.139-0.338) | 0.157 (0.125-0.236) | 0.252 (0.155-0.358) | 0.021-0.085 |
| LDH∗ | 473 (333-573) | 452 (389-504) | 464 (329-629) | 94-260 |
| Bilirubin (total)∗ | 2.7 (1.9-3.5) | 1.9 (1.5-2.5) | 3.2 (2.1-3.6) | 0.0-1.0 |
| Cardiac MRI data | ||||
| RVEDVi (mL/m2) | 108.1 (95.3-121.3) | 91.4 (84.2-101.2) | 110.3 (104.4-123.4) | 82 ± 17 (48-123)11 |
| RVESVi (mL/m2) | 44.3 (36.7-54.9) | 38.0 (35.3-41.0) | 46.7 (39.1-56.4) | 34 ± 11 (13-59)11 |
| RVEF (%) | 56.8 (53.7-61.4) | 58.3 (55.5-61.8) | 56.7 (53.9-61.6) | 58 ± 8 (42-74)11 |
| RVCi (L/min/m2) | 4.2 (3.7-5.4) | 3.8 (3.3-4.2) | 4.7 (4.1-5.7) | 2.9 ± 0.7 (1.5-4.5)11 |
| LVEDVi (mL/m2) | 109.2 (95.4-123.1) | 93.8 (75.7-113.4) | 118.7 (99.8-125.9) | 74 ± 15 (47-107)11 |
| LVESVi (mL/m2) | 48.2 (37.8-52.1) | 38.2 (31.8-50.6) | 50.3 (43.1-55.1) | 27 ± 9 (10-47)11 |
| LV mass indexed value (g/m2) | 64.5 (54.0-75.5) | 56.2 (44.9-70.4) | 65.5 (59.9-77.2) | 51 ± 10 (30-75)11 |
| LVEF (%) | 57.4 (55.3-59.8) | 58.0 (57.0-59.6) | 57.2 (55.1-59.8) | 64 ± 7 (51-79)11 |
| LVCi (L/min/m2) | 4.2 (3.9-5.5) | 3.9 (3.6-4.2) | 4.8 (4.1-5.8) | 3.1 ± 0.6 (1.9-4.3)11 |
| LA volume (mL/m2) | 40.5 (30.0-51.5) | 36.8 (24.8-46.0) | 42.4 (32.4-59.0) | 34 ± 10 (13-53)12 |
| Native T1 (ms) | 1029 (1004-1051) | 1020 (986-1057) | 1028 (1011-1036) | 1005 ± 25 (959-1052)† |
| Native T2 (ms)∗ | 46.5 (44.9-48.3) | 46.6 (45.9-47.3) | 46.6 (45.0-48.5) | 44 ± 2 (40-50)† |
| Native T2 > 50 | 2 (6.7) | 1 (12.5) | 1 (5.3) | |
| ECV (%) | 32.0 (29.0-34.0) | 31.0 (29.8-34.0) | 31.5 (29.0-34.0) | ≥18 y = 25 ± 313; <18 y = 21 ± 29 |
| ECV > 25 | 31 (100.0) | 8 (100.0) | 20 (100.0) | |
| ECV ≥ 30 | 22 (71.0) | 6 (75.0) | 14 (70.0) | |
| T2∗ (ms) | 35.0 (32.2-39.0) | 33.8 (31.2-34.8) | 35.9 (33.6-40.4) | >20 ms |
Three patients in the cohort did not receive any DMT.
BSA, body surface area; LDH, lactate dehydrogenase; LVCi, left ventricular cardiac index; LVEDVi, left ventricular end-diastolic volume (indexed); LVEF, left ventricle ejection fraction; LVESVi, left ventricular end-systolic volume (indexed); MRI, magnetic resonance imaging; RVCi, right ventricular cardiac index; RVEDVi, right ventricular end-diastolic volume (indexed); RVEF, right ventricle ejection fraction; RVESVi, right ventricular end-systolic volume (indexed).
Two subjects are missing hemoglobin, LDH, and bilirubin; 3 subjects are missing reticulocyte absolute; 1 subject is missing native T2.
St. Jude Children’s Research Hospital 1.5 T normal references.