Ebstein畸形MRI表现及文献回顾  

Magnetic resonance imaging features of Ebstein anomaly and the literture review

作　　者：梁红琴 王健 范占明[2] LIANG Hongqin;WANG Jian;FAN Zhanming(Department of Radiology,the First Affiliated Hospital to Army Medical University,Chongqing 400038,China;Department of Radiology,Anzhen Hospital,Ca pital Medical University,Beijing100029,China)

机构地区：[1]陆军军医大学第一附属医院放射科,重庆400038 [2]首都医科大学附属安贞医院影像科,北京100029

出　　处：《实用放射学杂志》2021年第1期55-58,共4页Journal of Practical Radiology

摘　　要：目的总结分析Ebstein畸形(EA)的MRI表现.方法回顾性分析5例经手术证实EA患者资料,并对PubMed和中国生物医学数据库进行系统检索和筛选.对5例病例和文献报道病例进行汇总,分析其临床及MRI特征.结果5例EA中,三尖瓣隔瓣明显下移4例,后瓣明显下移1例,下移距离10~26mm,房化右心室(aRV)近室间隔局部有线样强化3例.按照纳入和排除标准,最终纳入文献19篇,病例总数651例,其中延迟强化病例223例,共同MRI特征是右心房与aRV连接,三尖瓣隔叶及后叶下移,功能右心室(fRV)减小,右心室收缩功能减低,aRV心肌可见线样、斑片状延迟强化信号影.结论巨大右心房、明显下移瓣膜、fRV减小及心肌局部纤维化是EA在MRI中的特征性表现.Ohjective To report the MRl features of Ebstein anomaly(EA).Methods The data of 5 EA patients proven by surgrywere analyzed retrospectively.PubMed and Chinese biomedical databases were systematically searehed and screened.The 5 cases andthe cases reported in the literature were summarized to comprehensively analyze their clinical and MRI characterstits.Results 4 cases ofthe tricuspid septal valve significantly descending,1 case of posterior valve significantly descending with the distance of descendingdistributed at 10—26 mm.3 cases wirelike enhancement of atrialized right ventricle(aRV)near ventricular septum.According to theinclusion and exclusion criteria,19 literatures were finally included,the total number of cases was 651,among which 23 were delayedenhancement.Common MRI features included right atrium enlargement and connection with aRV,descending tricuspid septum andposterior lobe,decreased functional right ventricle(fRV),decreased right ventricular systolic function,and linear and patchy delayedenhancement signal shadows of aRV near ventricular septum.Conclusion Huge right atrium,obviously descending valve,reduction of fRV and myocardial local fibrosis are the characteristic manifestations of EA in MRI.

关 键 词：EBSTEIN畸形 磁共振成像 延迟强化 房化右心室 

分 类 号：R542.53[医药卫生—心血管疾病] R445.2[医药卫生—内科学]