中文摘要：

目的探讨左心室起源的致心律失常性心肌病（ALVC）的临床及MRI影像特征。方法2012年至今连续搜集我院ALVC患者10例（ALVC组），同期随机选取16例致心律失常性右心室心肌病（ARVC组）作为对照组，对比分析2组患者的临床表现及MRI特征。MRI扫描序列包括快速自旋回波序列、电影序列及延迟强化序列，测量2组患者房室大小、左右心功能、脂肪及纤维替代情况。2组间比较采用独立样本￡检验及Mann-Whitney检验。结果2组患者年龄、性别差异无统计学意义，临床表现以胸闷、憋气、心悸常见。ALVC组和ARVC组左心室内径、左心室舒张末容积指数、左心室EF值及右心室EF值分别为（64．2±7．7）、（49．2±5．9）mm（t=5．551，P〈0．001），（113．9±24．0）、（69．2±30．0）ml／m^2（t=3．962，P〈0．001），（38．5±3．1）％、（56．0±8．4）％（t=-6．733，P〈0．001），（42．0±5．5）、（18．0±7．3）％（t=8．817，P〈0．001），差异均有统计学意义。在脂肪及纤维浸润方面，ALVC组左心室壁（包括室间隔）均有脂肪或纤维浸润，分别为11及14节段，而ARVC组均为0节段。结论左心室起源的致心律失常性心肌病临床表现类似ARVC，但心脏形态、功能及心肌病变部位不同，“一站式”MRI检查在左心室起源的致心律失常性心肌病诊断与鉴别诊断中具有重要的应用价值。

英文摘要：

Objective To investigate the characteristics of clinical and cardiovascular magnetic resonance imaging（CMR） of arrhythmogenic cardiomyopathy with left ventricular（ALVC） involvement. Methods Ten cases of arrhythmogenic cardiomyopathy with left ventricular involvement and sixteen randomly chosen cases of arrhythmogenic cardiomyopathy with right ventricular （ARVC） involvement were enrolled in this study. Clinical symptoms, cardiac electrophysiological changes as well as the cardiac morpharage, ventricular functions and delayed enhancement of myocardium assessed by CMR were compared between the two groups. The size of heart chambers, global ventricular functions and the fat/ fibrosis infiltration were evaluated by turbo fast spin echo, ture FISP cine and delayed enhancement. These were statistically analyzed by independent samples t test, respectively.Results There was no significant difference in age and gender between two groups. All the patients in two groups presented non-specific clinical manifestations with no significant differences （P〉0.05） except for short of breath（P=0.034）. The end-diastolic diameter of left ventricular, left ventricular end-diastolic volume index, left and right ejection fraction in ALVC and ARVC group were （64.2±7.7）, （49.2±5.9） mm（t=5.551, P〈0.001）, （113.9±24.0）, （69.2±30.0） ml/m^2 （t=3.962, P〈0.001 ）, （38.5±3.1）%, （56.0±8.4）%（t=- 6.733,P〈0.001）, （42.0±5.5） %, （18.0±7.3）% （t=8.817, P〈0.001） respectively. An medium of 11 and 14 segments of fat or fat/fibrosis were found in intramural wall of the LV myocardiumin patients with ALVC,while only 0 segment（all P〈0.001）was found in patients with ARVC.Condusions The clinical manifestations of arrhythmogenic cardiomyopathy with left ventricular involvement was similar to ARVC, however, with regarding to cardiac morphological, functional and myocardial lesions, these two diseases have different characteristics in CMR features. ＂one-stop-shop＂ MRI examin