Abstract

Three-dimensional (3D) speckle-tracking echocardiography (STE) is a new imaging modality used for quantitative analysis of left ventricular (LV) function. The aim of this study is to assess the value of 3D STE in early detection of subclinical myocardial involvement in children with Duchenne muscular dystrophy (DMD). Fifty-six children with DMD (mean age, 8.8 ± 1.9years) and 31 age-matched control subjects were studied. Patients were subdivided into two groups by age: ≤ 8 or > 8years. Standard echocardiography examinations were performed to measure LV size and ejection fraction (EF). 3D STE was performed to assess LV 3D global strain and LV end-diastolic volume (EDV), end-systolic volume (ESV), and EF. Standard and 3D echocardiography measures were compared between children with DMD and those in the control group as well as between different patient groups. The areas under the receiver-operating characteristic (ROC) curve were calculated to determine the capability of 3D global strain indices to discriminate between patients and control subjects. No significant difference was detected in either LVEF derived from M-mode or 3D echocardiography between the two groups, and they were both within the normal range. Compared with control subjects, children with DMD had significantly reduced LV 3D global longitudinal strain (GLS; - 16.6 ± 4.7 vs. - 19.5 ± 3.7, p = 0.003), global circumferential strain (GCS; - 13.7 ± 2.9 vs. - 15.8 ± 2.6, p = 0.001), global radial strain (GRS; 42.5 ± 9.7 vs. 50.3 ± 10.4, p = 0.001), and global area strain (GAS; - 25.3 ± 4.9 vs. - 30.7 ± 4.1, p = 0.000). The older DMD children (age > 8years) had lower GLS (- 15.1 ± 4.43 vs. - 18.6 ± 4.35, p < 0.05), GCS (- 12.8 ± 3.48 vs. - 14.8 ± 2.83, p < 0.001), GAS (- 23.8 ± 4.7 vs. - 29.0 ± 5.4, p < 0.001), and GRS (40.7 ± 8.8 vs. 47.3 ± 11.5, p < 0.05) than younger patients (age ≤ 8years). The AUC of GAS was 0.80, and the cutoff value of - 29.5 had a sensitivity of 85.7% and a specificity of 71.0% for differentiating DMD patients from control. 3D speckle-tracking echocardiography is useful for detecting subclinical myocardial dysfunction and stratifying cardiomyopathy in children with DMD.

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