Key result
Myocardial T2 heterogeneity marks Duchenne muscular dystrophy and correlates with impaired circumferential strain.
Why the study?
The association of myocardial T2 distribution with the severity of Duchenne Muscular Dystrophy was not well characterized.
Cross-Sectional (n=34)
The transverse relaxation time (T2) of water molecules differ between tissues. Because the molecular motion of water is significantly affected by macro molecules, tissue containing fibrous polymers such as collagen has shorter T2 values. Patients with Duchenne Muscular Dystrophy (DMD) develop myocardial fibrosis in the late stage of the disease. The goal of this study was to determine the association of myocardial T2 distribution to the severity of DMD. Twenty six DMD patients and eight normal subjects were studied. T2 maps of the left ventricle were generated using a black blood dual spin echo method. (TE 1 = 6 ms, TE 2 = 34 ms) DMD patients were grouped according to the ejection fraction (EF) and the circumferential strain (ε cc ) as: A: EF ≥ 56%, ε cc ≥ 12%, mean age = 10.5 yrs, B: EF ≥ 56%, ε cc ≤ 12%, mean age = 17.5 yrs, C: EF ≤ 56%, mean age = 17.5 yrs. T2 values were plotted for each subject as a histogram. The normalized mean histograms from each group were compared by the Full Width of Half Maximum (FWHM). The FWHM of the T2 histogram was significantly higher in group B and C compared to that of group A and the normal group, indicating significantly high heterogeneity in T2 in Group B and C. FWHM/T2mean is significantly (p < .0001) higher in group B (0.42 ± 0.06) compared to Group A (0.54 ± 0.08). Regression analysis show moderate association between FWHM/T2mean and |Ecc| in group A and B. (Pearson correlation coefficient r = .51). The distribution of T2 in the LV of the DMD subjects were remarkably heterogeneous compared to that of normal subjects. These characteristics may suggest early signs of diffused collagen accumulation in the LV in DMD. The T2 maps of a normal (a) and a DMD subject (b) showing the left ventricle in the short axis view . Association between FWHM/T2mean and ε cc in group A and B . Pearson correlation coefficient r = .51.
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Wansapura et al. (2009) conducted a cross-sectional in Duchenne Muscular Dystrophy (n=34). Duchenne Muscular Dystrophy vs. Normal subjects was evaluated on Heterogeneity in left ventricular T2 distribution (FWHM of the T2 histogram) (r = 0.51, p=<.0001). The left ventricular myocardial T2 distribution was remarkably heterogeneous in Duchenne Muscular Dystrophy patients compared to normal subjects, correlating with circumferential strain (r=0.51).
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