BACKGROUND Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Speckle-tracking echocardiographic (STE) imaging is emerging as a noninvasive functional biomarker to consider in the early detection of DMD-related cardiomyopathy. However, STE analysis has not been assessed in a prospectively controlled study, especially in presymptomatic children with DMD, and no study has used STE analysis in all three displacements (longitudinal, radial, and circumferential) and for both ventricles. METHODS This prospective controlled study enrolled 108 boys, 36 of whom had DMD (mean age, 11 ± 3.8 years) and 72 of whom were age-matched control subjects in a 1:2 case-control design. Conventional echocardiographic variables were collected for the left and right ventricles. STE analyses were performed in the longitudinal, radial, and circumferential displacements for the left ventricle and in the free wall longitudinal displacement for the right ventricle. The effect of age on the evolution of two-dimensional strain in children with DMD was studied by adding an interaction term, DMD × age, in the models. RESULTS Conventional echocardiographic measures were normal in both groups. Left ventricular (LV) ejection fraction ranged from 45% to 76% (mean, 63 ± 6%) in the DMD group and from 55% to 76% (mean, 64 ± 5%) in the control group. Global LV strain mean measures were significantly worse in the DMD group for the longitudinal (-16.8 ± 3.9% vs -20.6 ± 2.6%, P < .0001), radial (22.7 ± 11.3% vs 31.7 ± 14%, P = .002), and circumferential (-16.5 ± 3.8% vs -20.3 ± 3.1%, P < .0001) displacements. The decrease of global LV longitudinal strain with age in children with DMD was 0.34% per year more marked than that in control subjects. The LV inferolateral and anterolateral segments were specifically impaired, especially in the basal area. Right ventricular function evaluated using conventional echocardiography and STE analysis was normal and not different between children with DMD and control subjects. CONCLUSIONS The existence of altered LV strain despite normal LV function in children with DMD represents an important perspective for future pediatric drug trials in DMD-related cardiomyopathy prevention.

中文翻译：

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小儿杜氏肌营养不良症的斑点追踪超声心动图：前瞻性多中心对照横断面研究。

背景技术杜氏肌营养不良症（DMD）的预后与心脏功能障碍有关。斑点跟踪超声心动图（STE）成像已成为一种无创功能性生物标志物，可在DMD相关性心肌病的早期检测中加以考虑。但是，尚未在前瞻性对照研究中评估STE分析，尤其是在有症状的DMD前症状儿童中，并且没有研究在所有三个位移（纵向，径向和圆周）和两个心室中都使用STE分析。方法该前瞻性对照研究纳入了1：2病例对照设计中的108名男孩，其中36名患有DMD（平均年龄，11±3.8岁），其中72名是年龄匹配的对照对象。常规超声心动图变量收集左，右心室。STE分析是在纵向，径向，左心室的圆周位移和右心室的自由壁纵向位移。通过在模型中添加交互项DMD×年龄，研究了年龄对DMD儿童二维应变演变的影响。结果两组常规超声心动图检查均正常。在DMD组中，左心室（LV）射血分数的范围为45％至76％（平均63±6％），而在对照组中，其左心室射血分数的范围为55％至76％（平均64±5％）。DMD组的纵向（-16.8±3.9％vs -20.6±2.6％，P <.0001），放射状（22.7±11.3％vs 31.7±14％，P = 0.002）的整体LV应变平均测量显着更差）和圆周位移（-16.5±3.8％vs -20.3±3.1％，P <.0001）。与对照组相比，DMD患儿的总体左心室纵向应变随年龄的降低每年增加0.34％。左下外侧和前外侧节特别受损，尤其是在基底区域。使用常规超声心动图和STE分析评估的右心室功能正常，DMD儿童与对照组之间无差异。结论尽管DMD儿童的LV功能正常，但LV应变改变的存在为将来在DMD相关性心肌病预防中的儿科药物试验提供了重要的观点。使用常规超声心动图和STE分析评估的右心室功能正常，DMD儿童与对照组之间无差异。结论尽管DMD儿童的LV功能正常，但LV应变改变的存在为将来在DMD相关性心肌病预防中的儿科药物试验提供了重要的观点。使用常规超声心动图和STE分析评估的右心室功能正常，DMD儿童与对照组之间无差异。结论尽管DMD儿童的LV功能正常，但LV应变改变的存在为将来在DMD相关性心肌病预防中的儿科药物试验提供了重要的观点。