多参数心脏MR在Danon病随访应用价值及相关文献回顾  

Application value of multi-parameter cardiovascular MR in the follow-up of Danon disease and related literature review

作　　者：田萍 尉胜男 李磊磊 郑建民 郑敏文 刘莹 TIAN Ping;WEI Shengnan;LI Leilei;ZHENG Jianmin;ZHENG Minwen;LIU Ying(Department of Radiology,Xijing Hospital,the Air Force Medical University,Xi'an 710032,China)

机构地区：[1]空军军医大学西京医院放射科,陕西西安710032

出　　处：《实用放射学杂志》2023年第8期1258-1261,共4页Journal of Practical Radiology

摘　　要：目的探讨多参数心脏磁共振(CMR)在Danon病随访中的应用价值。方法纳入1例Danon病患者,采用1.5T MR对患者进行2次(2015年、2019年)CMR扫描。结果2015年首次CMR检查发现左心室心肌肥厚,左心室腔内径变窄,左心室容积变小,左心室收缩功能正常,左心室侧壁出现少量心肌纤维化,初始T1值1147ms,细胞外容积(ECV)值35.3%;2019年复查CMR左室及室间隔心肌肥厚持续性加重,同时累及左室乳头肌和右室,左心室容积进一步缩小,心肌质量明显增加,初始T1值1271ms,ECV值71.5%。结论多参数CMR在诊断及随访中发现Danon病患者晚期心肌受损严重,出现广泛的心肌纤维化,快速进展为心衰,提示预后不良。Objective To investigate the application value of multi-parameter cardiovascular magnetic resonance(CMR)in the follow-up of Danon disease.Methods One child with Danon disease diagnosed was included.The patient underwent two times CMR scans in 2015 and 2019,respectively,using 1.5T MR system.Results The first CMR scanning in 2015 showed left ventricular hypertrophy,narrow left ventricular luminal diameter,small left ventricular volume,normal left ventricular systolic function,and a small amount of myocardial fibrosis in the left ventricular lateral wall.The native T,value was 1147 ms,and the extracellular volume(ECV)was 35.3%.The continuously aggravation of the left ventricular and septal myocardial hypertrophy was detected 2019 CMR scanning,and the left ventricular papillary muscle and right ventricle were also involved,with further reduced volume of left ventricular and significantly increased myocardial mass.The native Ti value was 1271 ms,and ECV was 71.5%.Conclusion In the diagnosis and follow-up,multi-parameter CMR could be available to reveal the severe myocardial damage,extensive myocardial fibrosis and rapid progression to heart failure in child with Danon disease,indicating poor prognosis.

关 键 词：Danon病 心脏磁共振 肥厚型心肌病 细胞外容积 

分 类 号：R596.1[医药卫生—内科学] R445.2[医药卫生—临床医学] R542.2