Abstract
BackgroundProgressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients. Thus, our aim was to comprehensively characterize myocardial involvement by investigating the heterogeneity of native T1 mapping in DMD patients using global and regional (including segmental and layer-specific) analysis across a large cohort.MethodsWe prospectively enrolled 99 DMD patients (8.8 ± 2.5 years) and 25 matched male healthy controls (9.5 ± 2.5 years). All subjects underwent cardiovascular magnetic resonance (CMR) with cine, T1 mapping and late gadolinium enhancement (LGE) sequences. Native T1 values based on the global and regional myocardium were measured, and LGE was defined.ResultsLGE was present in 49 (49%) DMD patients. Global native T1 values were significantly longer in LGE-positive (LGE +) patients than in healthy controls, both in basal slices (1304 ± 55 vs. 1246 ± 27 ms, p < 0.001) and in mid-level slices (1305 ± 57 vs. 1245 ± 37 ms, p < 0.001). No significant difference in global native T1 was found between healthy controls and LGE-negative (LGE−) patients. In segmental analysis, LGE + patients had significantly increased native T1 in all analyzed segments compared to the healthy control group. Meanwhile, the comparison between LGE− patients and healthy controls showed significantly elevated values only in the basal anterolateral segment (1273 ± 62 vs. 1234 ± 40 ms, p = 0.034). Interestingly, the epicardial layer had a significantly higher native T1 in LGE− patients than in healthy controls (p < 0.05), whereas no such pattern was noticed in the global myocardium. Epicardial layer native T1 resulted in the highest diagnostic performance for distinguishing between healthy controls and DMD patients in receiver operating curve analyses (area under the curve [AUC] 0.84 for basal level and 0.85 for middle level) when compared to global native T1 and endocardial layer native T1.ConclusionsMyocardial regional native T1, particularly epicardial native T1, seems to have potential as a novel robust marker of very early cardiac involvement in DMD patients.Trial registration: Chinese Clinical Trial Registry (http://www.chictr.org.cn/index.aspx) ChiCTR1800018340, 09/12/2018, Retrospectively registered.
Highlights
Progressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients. our aim was to comprehensively characterize myocardial involvement by investigating the het‐ erogeneity of native T1 mapping in DMD patients using global and regional analysis across a large cohort
Cardiovascular magnetic resonance (CMR) offers an accurate and highly reproducible technique for assessing left ventricular (LV) function together with the ability to detect focal fibrosis based on late gadolinium enhancement (LGE) imaging, which plays an increasingly vital role in the diagnosis and clinical care of boys with DMD-associated cardiomyopathies [6] and is recommended as the preferred noninvasive imaging modality for patients with DMD [7]
We found three significant demographic differences between the two groups: DMD patients were shorter and had faster resting heart rates and lower body surface area (BSA) values than healthy controls (Table 1)
Summary
Progressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients. our aim was to comprehensively characterize myocardial involvement by investigating the het‐ erogeneity of native T1 mapping in DMD patients using global and regional (including segmental and layer-specific) analysis across a large cohort. Native T1 mapping, a novel CMR parametric mapping technique, has provided a potential tool for quantifying tissue alterations without the administration of contrast medium and assessing early, subclinical cardiac involvement in focal and diffuse myocardial fibrosis [9,10,11]. The aim of our study was to comprehensively characterize myocardial involvement by investigating the heterogeneity of native T1 mapping in a large young DMD population using global and regional (including segmental and layer-specific) analysis and to further assess the feasibility and specificity of native T1 mapping in the early detection of myocardial involvement in DMD
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