Table 1.
Study population characteristics and CMR parameters.
| MD-carrier | Female controls | p-value∗∗ | |
|---|---|---|---|
| N = 29 | N = 24 | ||
| Female, n (%) | 29 (100) | 24 (100) | 1.00 |
| Age, years [mean ± SD] | 45 ± 14 | 41 ± 11 | 0.38 |
| DMD/BMD, n (%) | 18 (62)/11 (38) | – | |
| BMI, [mean ± SD] kg/m2 | 25 ± 4 | 24 ± 3 | 0.09 |
| Skeletal muscle symptoms, n (%) | 4 (14) | 0 (0) | 0.12 |
| Cardiac symptoms, n (%) | 9 (31) | 0 (0) | 0.003 |
| ACE inhibitor/ARB, n (%) | 2 (7) | 0 (0) | 0.49 |
| Beta blocker, n (%) | 3 (10) | 0 (0) | 0.24 |
| CMR findings | |||
| LV-EDVi, [mean ± SD] (ml/m2) | 71 ± 18 | 65 ± 11 | 0.15 |
| LV-ESVi, [mean ± SD] (ml/m2) | 26 ± 9 | 23 ± 5 | 0.20 |
| LV-massi, [mean ± SD] (g/m2) | 48 ± 9 | 43 ± 6 | 0.022 |
| LV-EF, [mean ± SD] (%) | 64 ± 7 | 65 ± 4 | 0.47 |
| RV-EF, [mean ± SD] (%) | 55 ± 11 | 56 ± 8 | 0.87 |
| LV-EF < 55%, n (%) | 4 (14) | 0 (0) | 0.12 |
| RV-EF < 45%, n (%) | 4 (14) | 0 (0) | 0.12 |
| LGE presence, n (%) | 13 (45) | 0 (0) | <0.0001 |
| LGE extent, n (%) | 0 (0–7) | – | |
| Abnormal CMR, n (%) | 14 (48) | 0 (0) | <0.0001 |
| Serum measurements | |||
| CK, U/L [median (IQR)] | 168 (111–595) | 103 (80–149) | 0.004 |
| Elevated CK, n (%)∗ | 14 (48) | 1 (4) | <0.0001 |
| Elevated hs-Trop, n (%)† | 2 (7) | 0 (0) | 0.49 |
| Elevated NT-proBNP, n (%)‡ | 2 (7) | 0 (0) | 0.49 |
∗ >190 U/l. † >14 pg/ml. ‡ >450 pg/ml. ∗∗Student’s t-test, Mann–Whitney U test, Kruskal–Wallis test with Bonferroni post hoc correction or Chi-square test were used appropriate. Bold values indicate significant p < 0.05.