Elevated myocardial extracellular volume fraction in Duchenne muscular dystrophy

Myocardial T₁-mapping and Extracellular Volume Quantification in Patients and Putative Carriers of Muscular Dystrophy: Early Experience

“…Previous studies have shown the utility of myocardial T 1 values and ECV for detecting myocardial fibrosis in patients with Duchenne and Becker muscular dystrophy. 10,15,17,18 In the present study, elevated ECV was observed even in female putative carriers without LGE. Furthermore, mild diffuse fibrosis detected ECV appears to precede the development of LV dysfunction or substantial LGE, suggesting that myocardial tissue characterization by T 1 -mapping and ECV may be more sensitive to detect cardiac involvement in muscular dystrophy compared to conventional CMR techniques.…”

“…Given the fact that ECV mapping requires the administration of gadolinium-based contrast material, it was ethically and practically difficult to obtain the reference value of ECV in healthy children. 15 For this reason, the cut-off values of ECV for men and women were derived from the previous study in healthy volunteers using 3T CMR, 16 in which women (mean ± SD of age, 55 ± 17 years) had a higher ECV as compared to age-matched men (mean ± SD; 28.4 ± 3.4% vs. 25.1 ± 2.2%, P < 0.001). ECV higher than the mean + 2SD of the reference values (>29.5% for men and >35.2% for women) was considered elevated.…”