Parameter | MD2 | FSHD1 | ||||
---|---|---|---|---|---|---|
Baseline (n = 31) | Follow-up n = 22 | p-value | Baseline n = 52 | Follow-up n = 41 | p value | |
LVEF (%) | 68 ± 6 | 62 ± 6 |  < 0.01 | 63 ± 5 | 60 ± 3 | 0.762 |
LVEDV (ml) | 126 ± 22 | 124 ± 29 | 0.605 | 128 ± 21 | 139 ± 34 | 0.131 |
LVEDVI (ml/cm) | 0.80 ± 0.11 | 0.73 ± 0.15 | 0.275 | 0.70 ± 0.10 | 0.79 ± 0.17 |  < 0.01 |
LV mass (g) | 104 ± 27 | 92 ± 24 | 0.124 | 99 ± 25 | 102 ± 24 | 0.630 |
LV mass index (g/cm) | 0.60 ± 0.14 | 0.54 ± 0.12 | 0.110 | 0.56 ± 0.13 | 0.57 ± 0.12 | 0.524 |
LV stroke volume (ml) | 85 ± 13 | 78 ± 17 | 0.113 | 80 ± 15 | 84 ± 18 | 0.084 |
LV stroke volume index (ml/m2) | 46 ± 6 | 42 ± 6 | 0.063 | 45 ± 8 | 48 ± 9 | 0.245 |
RVEF (%) | 59 ± 7 | 54 ± 4 |  < 0.01 | 51 ± 6 | 49 ± 5 | 0.104 |
RVEDV (ml) | 140 ± 29 | 146 ± 33 | 0.444 | 160 ± 31 | 169 ± 28 | 0.470 |
RVEDVI (ml/m2) | 76 ± 15 | 79 ± 14 | 0.456 | 83 ± 16 | 88 ± 14 | 0.422 |
RV stroke volume (ml) | 82 ± 18 | 80 ± 19 | 0.932 | 76 ± 18 | 83 ± 18 | 0.082 |
RV stroke volume index (ml/m2) | 44 ± 89 | 43 ± 8 | 0.852 | 40 ± 8 | 43 ± 8 | 0.063 |
LAEF (%) | 60 ± 8 | 57 ± 7 | 0.392 | 62 ± 8 | 60 ± 7 | 0.452 |
LAEDV (ml) | 63 ± 18 | 71 ± 25 | 0.275 | 54 ± 14 | 61 ± 14 | 0.021 |
LAEDVI(ml/m2) | 35 ± 8 | 38 ± 25 | 0.288 | 28 ± 7 | 32 ± 8 | 0.027 |
LA (cm2) | 21 ± 3 | 24 ± 5 |  < 0.01 | 19 ± 3 | 22 ± 3 |  < 0.01 |
RA (cm2) | 21 ± 3 | 24 ± 5 | 0.040 | 20 ± 3 | 23 ± 4 | 0.029 |
Native T1 basal (ms) | 1029 ± 30 | 1015 ± 38 | p = 0.066 | 1010 ± 26 | 991 ± 24 |  < 0.01 |
Native T1 mid (ms) | 1012 ± 38 | 998 ± 30 | p = 0.258 | 991 ± 39 | 989 ± 30 | p = 0.102 |
Native T1 apical (ms) | 1018 ± 50 | 999 ± 32 | p = 0.163 | 983 ± 41 | 970 ± 40 | p = 0.203 |
ECV basal (%) | 26 ± 3 | 24 ± 3 |  < 0.01 | 26 ± 3 | 22 ± 2 |  < 0.01 |
ECV mid (%) | 26 ± 2 | 24 ± 2 |  < 0.01 | 26 ± 3 | 23 ± 3 |  < 0.01 |
ECV apical (%) | 29 ± 3 | 26 ± 2 |  < 0.01 | 27 ± 3 | 24 ± 2 |  < 0.01 |
T2 basal (ms) | 51 ± 2 | 49 ± 2 |  < 0.01 | 50 ± 4 | 47 ± 2 |  < 0.01 |
T2 mid (ms) | 52 ± 3 | 49 ± 3 |  < 0.01 | 51 ± 3 | 47 ± 2 |  < 0.01 |
T2 apical (ms) | 55 ± 4 | 50 ± 2 |  < 0.01 | 53 ± 3 | 48 ± 2 |  < 0.01 |
Cell volume basal (ml) | 72 ± 18 | 68 ± 17 | p = 0.409 | 71 ± 19 | 74 ± 22 | p = 0.308 |
Cell volume mid (ml) | 70 ± 19 | 76 ± 17 | p = 0.553 | 71 ± 20 | 73 ± 22 | p = 0.361 |
Fibrosis volume basal (ml) | 25 ± 9 | 20 ± 7 | p = 0.055 | 24 ± 8 | 20 ± 7 | p = 0.023 |
Fibrosis volume mid (ml) | 26 ± 7 | 21 ± 6 |  < 0.01 | 23 ± 7 | 22 ± 7 | p = 0.032 |
GLS (%) LGE(-) patients | -17.9 ± 1 | -16.8 ± 1 |  < 0.01 | -18.3 ± 1 | -16.4 ± 1 | p < 0.01 |