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目的探讨 MRI 在儿童心肌病诊断中的价值。方法搜集心肌病患儿33例,其中肥厚型心肌病13例,扩张型心肌病10例,限制型心肌病7例,致心律不齐性右心室发育不良型心肌病3例。MR 采用不同序列不屏气扫描,13例肥厚型心肌病中9例选取左心室8个节段进行室壁厚度测量及心肌增厚率计算,并测定左心室收缩功能。对10例扩张型心肌病中7例测定左心室收缩功能。上述2组左心室收缩功能结果与心脏超声(ECHO)结果比较。7例限制型心肌病对其右心室流人道、流出道、心尖、右心房及下腔静脉的 MRI 表现与心血管造影进行比较。对3例致心律不齐性右心室发育不良型心肌病结合解剖特点、临床特点综合评估,并测量左右心室收缩功能。结果 13例肥厚型心肌病中9例 MRI 显示基底部游离壁前外侧段心肌增厚率(16%)及心尖部前段心肌增厚率(16%)下降明显,左心室收缩功能略下降,射血分数为57%,心肌质量有所增高(114 g/m~2),与ECHO 相比,左心室舒张末容量及每搏量上差异有统计学意义(P 值分别为0.0255和0.0341)。10例扩张型心肌病中7例左心室收缩功能下降,射血分数为20.7%,与 ECHO 比较在每搏量测定上差异有统计学意义(P=0.0106)。7例限制型心肌病 MRI 显示右心房明显扩大,上下腔静脉、肝静脉扩张,右心室腔变形,流人道缩短,心尖闭塞,流出道扩张。3例致心律不齐性右心室发育不良型心肌病中显示右心室球形扩张,右心室室壁局部变薄,1例见脂肪浸润,3例左右心室收缩功能均下降。结论 MPI 是目前诊断儿童心肌病中较好的非创伤性检查方法,其高分辨率可提供儿童心肌病解剖上的信息并能较 ECHO 提供更为准确的左右心室功能评估。
Objective To investigate the value of MRI in diagnosis of cardiomyopathy in children. Methods 33 cases of cardiomyopathy were collected, including 13 cases of hypertrophic cardiomyopathy, 10 cases of dilated cardiomyopathy, 7 cases of restrictive cardiomyopathy and 3 cases of arrhythmogenic right ventricular dysplastic cardiomyopathy. MR scan with different series of non-breath-hold, 13 cases of hypertrophic cardiomyopathy in 9 cases selected left ventricular segment 8 thickness measurement and myocardial thickening rate calculation, and determination of left ventricular systolic function. In 10 cases of dilated cardiomyopathy in 7 cases measured left ventricular systolic function. The above two groups of left ventricular systolic function results compared with the results of echocardiography (ECHO). Seven cases of restricted cardiomyopathy were compared with MRI findings of right ventricular flow tract, outflow tract, apex, right atrium and inferior vena cava. Three cases of arrhythmia caused by right ventricular dysplastic cardiomyopathy combined with anatomical features, clinical features of a comprehensive assessment and measurement of left ventricular systolic function. Results Thirteen patients with hypertrophic cardiomyopathy showed that the myocardial thickening rate (16%) in the anterolateral segment of basilar free wall and the myocardial thickening rate (16%) in the anterior part of the apical portion decreased significantly and the left ventricular systolic function decreased slightly Compared with ECHO, the left ventricular end-diastolic volume and stroke volume were significantly different (P values were 0.0255 and 0.0341 respectively), with a 57% increase in blood and an increase in myocardial mass (114 g / m 2). In 10 cases of dilated cardiomyopathy, left ventricular systolic function was decreased in 7 cases and the ejection fraction was 20.7%. There was significant difference in stroke volume between ECHO and control group (P = 0.0106). 7 cases of restrictive cardiomyopathy MRI showed a significant enlargement of the right atrium, superior and inferior vena cava, hepatic vein dilatation, right ventricular cavity deformation, shortening flow tract, apical occlusion, outflow tract dilatation. 3 cases of arrhythmic right ventricular dysplastic cardiomyopathy showed right ventricular spherical dilatation, local thinning of the right ventricular wall, fat infiltration in 1 case, 3 cases of left ventricular systolic function decreased. Conclusion MPI is a better noninvasive method for the diagnosis of childhood cardiomyopathy. Its high resolution provides anatomical information on cardiomyopathy in children and provides more accurate assessment of left and right ventricular function than ECHO.