磁共振成像在Duchenne型肌营养不良心肌病中的研究进展  

Research progress of magnetic resonance imaging in Duchenne muscular dystrophy cardiomyopathy

作　　者：许可 郭应坤[1] 许华燕 XU Ke;GUO Yingkun;XU Huayan(Department of Radiology,West China Second University Hospital,Sichuan University,Key Laboratory of Obstetric&Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education,Chengdu 610041,China)

机构地区：[1]四川大学华西第二医院放射科,出生缺陷与相关妇儿疾病教育部重点实验室,成都610041

出　　处：《磁共振成像》2023年第4期154-159,共6页Chinese Journal of Magnetic Resonance Imaging

基　　金：国家自然科学基金(编号:82271981、81901712)。

摘　　要：心肌病是目前导致Duchenne型肌营养不良(Duchenne muscular dystrophy, DMD)患者死亡的首要原因。心脏磁共振(cardiac magnetic resonance, CMR)为评估心脏结构和功能提供了一种准确且高度可重复的技术,其钆对比剂延迟增强成像(late gadolinium enhanced, LGE)在诊断和临床治疗DMD心肌病中发挥着越来越重要的作用。与此同时,T1 mapping、T2mapping、特征追踪等新技术在DMD心肌病中也得到了广泛的应用。当前DMD的CMR研究广泛地分析了心脏受累的模式,但尚未确定心脏受损随时间的演变以及CMR对超声心动图的附加价值及相关结果的预后意义。因此,笔者拟对DMD心肌病的病理生理学机制、常用的影像检查手段及CMR在DMD心肌病诊断和随访中的应用进展进行综述,旨在为DMD患者心脏损伤的早期诊断、定量评估及危险分层提供参考。Cardiomyopathy is the leading cause of death in patients with Duchenne muscular dystrophy(DMD)to date.Cardiac magnetic resonance(CMR)provides an accurate and highly reproducible technique for assessing cardiac structure and function,and late gadolinium enhanced(LGE)is playing an increasingly important role in the diagnosis and clinical treatment of DMD cardiomyopathy.Meanwhile,new technologies such as T1 mapping,T2 mapping,and feature tracking have also been widely used in DMD cardiomyopathy.Currently,the CMR studies of DMD population analysed the patterns of cardiac involvement,but have not confirmed the evolution of cardiac disease over time and established the added value of CMR to echocardiography and the prognostic significance of CMR findings.Thus,the authors reviewed the pathophysiology of DMD cardiomyopathy,commonly used imaging methods and the application progress of CMR in the diagnosis and follow-up of DMD cardiomyopathy,in order to serve as a reference for the early diagnosis,quantitative evaluation and risk stratification of cardiac injury in DMD patients.

关 键 词：心肌病 DUCHENNE型肌营养不良症 磁共振成像 T1 mapping T2 mapping 延迟强化 特征追踪 早期诊断 

分 类 号：R445.2[医药卫生—影像医学与核医学] R542.2[医药卫生—诊断学] R746.2[医药卫生—临床医学]