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(1．Department of Medical Imaging, Jinhua Municipal Center Hospital, Jinhua321000,China; 2. Department of Radiology, Sir Run Run Shaw Hospital,Zhejing University School of Medicine, Hangzhou 310016, China)

MRI characteristics of primary dilated cardiomyopathy and isolated left ventricular non-compaction

CHENXiaorong1,SHUJin'er1,PANYonghao1,HUHongjie2*

(1．DepartmentofMedicalImaging,JinhuaMunicipalCenterHospital,Jinhua321000,China; 2.DepartmentofRadiology,SirRunRunShawHospital,ZhejingUniversitySchoolofMedicine,Hangzhou310016,China)

Objective To explore the MRI characterisitics of primary dilated cardiomyopathy and isolated left ventricular non-compaction. Methods The patients who were diagnosed as primary dilated cardiomyopathy (n=18) and isolated left ventricular non-compaction (n=10) were enrolled, and the MRI was performed. The thickness of non-compaction myocardium (NC), compaction myocardium (C) in end-diastole, the feature of movement of myocardium, the number of non-compaction segment, the fraction shortening of non-compaction and the distribution were compared. Results Totally 823 segments were analyzed in primary dilated cardiomyopathy, in which 124 segments were non-compaction myocardium; 397 segments were analyzed in isolated left ventricular non-compaction, in which 115 segments were non-compaction myocardium. The NC, NC/C, NC/(NC+C), and the fraction shortening of the isolated left ventricular non-compaction patients were higher than those of primary dilated cardiomyopathy patients (allP<0.05). The features of distribution showed that the apical segment was mostly involved, and the basal segment was less involved or hardly involved. The anterior and lateral segments were more involved in the free wall, the septal was less involved. Conclusion The MRI characteristics of primary dilated cardiomyopathy and isolated left ventricular non-compaction are different, especially in the distribution, noncompacted segments, NC and NC/C, which is important for diagnosis and differential diagnosis of the two diseases.

Cardiomyopathy, dilated; Myocardium non-compaction; Magnetic resonance imaging

原发性扩张型心肌病(dilated cardiomyopathy, DCM)是一种常见的原发性心肌病，是指病因不明的左心室或全心扩张伴收缩功能受损的心脏病。心肌致密化不全是一类先天性心肌病，表现为心内膜下心肌肌小梁增多粗乱，呈海绵样改变，又称“海绵样心肌”。左心室心肌致密化不全常与先天性心脏病相伴出现，也可孤立存在，即为孤立性左心室心肌致密化不全 (isolated left ventricular non-cmpaction, ILVNC)。目前，DCM合并心内膜下心肌肌小梁异常的检出率越来越高，此时与ILVNC的鉴别尤为重要。鉴别DCM与ILVNC，临床首选超声心动图，但当其图像显示欠佳，诊断困难时，多借助心脏MRI进行诊断及鉴别诊断。本研究通过对比DCM与ILVNC的MRI特征，探讨MRI对两者的鉴别诊断价值。

1 资料与方法

1.1一般资料 回顾性分析2013年3月—2016年9月间在我院接受心脏MR检查的患者288例。入组标准：诊断为原发性DCM及ILVNC，诊断标准参考2008年欧洲心脏病协会心肌病分类[1]及2015年中华医学会心血管病分会的心肌病磁共振成像临床应用中国专家共识[2]。原发性DCM诊断标准：①左心室或左心或全心扩大；②左心室壁厚度普遍变薄或正常；③左心室壁运动弥漫性减弱；④除外冠心病、淀粉样变、甲状腺功能亢进、围产期、中毒性心肌病等继发性病因。ILVNC的诊断标准：病变心肌节段心内膜下心肌肌小梁增多、粗乱呈栅栏样改变，左心室舒张末期非致密心肌厚度(non-compaction myocardium, NC)与同节段致密心肌的厚度(compaction myocardium, C)的比值(NC/C)>2.5[3-4]，但除外同时合并有先天性心脏病者。排除标准：心律不齐致图像质量较差而无法测量的病例。根据最终诊断分为原发性DCM组18例，其中男14例，女4例，年龄29～70岁，中位年龄51.5岁；I……