摘要
目的:通过比较特纳综合征(TS)患者与健康志愿者的心肌初始T1(Native T1)值及细胞外间质容积分数(ECV)值,探讨基于T1-mapping技术的心脏MRI(cMRI)对特纳综合征心血管并发症的诊断价值.方法:纳入2019年10月—2020年12月在四川大学华西第二医院经临床诊断为TS的38例患者,同期招募20例年龄、性别相匹配的健康志愿者(正常对照组).所有受试者采用T1-mapping技术行cMRI检查.利用后处理软件对T1-mapping图像进行分析,得到左心室基底部、中间部、心尖部及整体心肌的初始T1和ECV值.根据染色体核型分析检测结果将TS患者分为45X型和非45X型,根据生长发育阶段将所有受试者按年龄分为3～11岁组、12～18岁组和18岁以上组.采用独立样本t检验或非参数检验比较TS组与正常对照组、45X型与非45X型TS组之间基本临床资料、实验室检查以及cMRI定量参数的组间差异.TS组中cMRI定量参数(初始T,和ECV)与临床资料之间的相关性采用Pearson或Sperman相关性分析,并采用二元logistic 回归分析将所有临床指标和cMRI定量参数变量纳入并筛选升主动脉扩张的独立影响因素.结果:TS组中最常见的临床表现是身材矮小(n=23,60.5％),伴有主动脉缩窄者4例(10.5％)、升主动脉扩张者10例(26.3％).TS组左心室基底部心肌的初始T1值明显高于正常对照组[(1296±54)vs.(1256±43)ms,P=0.006)];45X型组的左心室心肌初始T1值明显高于非45X型组:基底部为(1324±50)vs.(1272±47)ms(P=0.002);中间部为(1323±48)vs.(1278±42)ms(P=0.014);心尖部为(1313±61)vs.(1266±75)ms(P=0.032);心肌整体为(1320±41)vs.(1272±39)ms(P=0.001).相较于非45X型患者,ECV值在45X型患者中具有增高的趋势(27.9％±2.2％vs.26.4％±2.6％;P=0.056).此外,升主动脉扩张与45X核型呈弱相关(r=0.391,P=0.015);45X 核型分别与基底部(r=0.495,P=0.002)、中间部(r=0.475,P=0.004)、心尖部(r=0.418,P=0.009)和心肌整体的 T1 值(r=0.572,P＜0.001)以及 ECV 值(r=0.351,P=0.031)均存在不同程度的相关关系.相较于非45X型患者,45X型患者发生升主动脉扩张的风险显著增加(OR=7.87,95％CI:1.19～13.9,P=0.032).结论:基于 T1-mapping 技术的 cMRI 可定量评估 TS患者心肌组织的特征,45X型TS患者左心室心肌的初始T1值增高,对于45X型TS患者临床上应重点关注其潜在的心血管异常改变.
Abstract
Objective:This study was aimed to exploring the diagnostic value of cardiac magnetic resonance imaging(cMRI)using T1-mapping technique for cardiovascular complications in Turner syndrome(TS)by comparing myocardial native T1 and extracellular volume fraction(ECV)between patients with TS and healthy volunteers.Methods:This study included 38 patients diagnosed with TS at West China Second Hospital of Sichuan University from October 2019 to December 2020,and re-cruited 20 age-and gender-matched healthy volunteers as normal controls during the same period.All participants underwent cMRI examination using T1-mapping technology.T1-mapping images were ana-lyzed utilizing post-processing software;the native T1 values of the left ventricular base,middle,apex,and overall left ventricular myocardium and ECV values were obtained.TS patients were divided into 45X-type and non-45X-type based on results of chromosome karyotype analysis.Based on the age and development stage of the patients,all subjects were divided into three groups:3～11,12～18,and＞18 years old.The differences of clinical data,laboratory examinations and cMRI parameters between TS patients and normal controls,45X-type and non-45X-type TS patients were compared using indepen-dent sample t-test or non parametric test.The correlation between quantitative parameters of cMRI(native T,and ECV values)and clinical data in TS patients was detected using Pearson or Spearman correlation analysis,and binary logistic regression analysis was used to explore the independent in-fluencing factors of ascending aortic dilation.Results:The most common clinical manifestation of TS patients was short stature(n=23,60.5％),and some patients have aortic constriction(n=4,10.5％)and ascending aortic dilation(n=10,26.3％).The left ventricular basal myocardial native T1 value of TS patients was significantly higher than that of the normal controls[(1296±54)vs.(1256±43)ms,P=0.006],while the left ventricular myocardial native T1 value of 45X-type TS patients was signifi-cantly higher than that of non-45X-type TS patients:basal myocardial native T1 value[(1324±50)vs.(1272±47)ms,P=0.002],middle myocardial native T1 value[(1323±48)vs.(1278±42)ms,P=0.014],apical myocardial native T1 value[(1313±61)vs.(1266±75)ms,P=0.032],and overall myo-cardial native T1 value[(1320±41)vs.(1272±39)ms,P=0.001].Compared to non-45X-type TS pa-tients,there is an increasing trend in ECV values among 45X-type TS patients(27.9％±2.2％vs.26.4％±2.6％,P=0.058).In addition,ascending aortic dilation was weakly correlated with the 45X karyotype(r=0.391,P=0.015);The 45X karyotypes were correlated to varying degrees with basal myocardial native T1 value(r=0.495,P=0.002),intermediate myocardial native T1 value(r=0.475,P=0.004),apical myocardial native T1 value(r=0.418,P=0.009),overall myocardial native T1 value(r=0.572,P＜0.001),and ECV value(r=0.351,P=0.031),respectively.Compared to non-45X-type,45X-type TS patients showed a significantly increased risk of ascending aortic dilation(OR=7.87,95％CI:1.19～13.9,P=0.032).Conclusion:cMRI T1-mapping technique can quantitatively evaluate the myocardial tissue characteristics of TS patients.45X-type TS patients have increased left ventricular myocardial native T1 value.In clinical practice,special attention should be paid to their potential cardio-vascular abnormalities as for 45X-type TS patients.
万方数据