基于心血管磁共振的Danon病与肥厚型心肌病胸廓骨骼肌特征研究

Study on thoracic skeletal muscle characteristics of Danon disease and hypertrophic cardiomyopathy based on cardiovascular magnetic resonance imaging

目的应用心血管磁共振(CMR)定量评价Danon病和肥厚型心肌病(HCM)患者胸廓骨骼肌(TSM)形态和组织特征,以此提供辅助鉴别诊断信息。方法回顾性收集完成CMR检查的15例Danon病患者(Danon病组),并与性别、年龄配对的15例HCM患者(HCM组)及15例健康志愿者(对照组)进行对比研究,测量所有受试者4组TSM(胸大肌、胸小肌、竖脊肌及肩胛下肌)的面积指数(TSMAi)、2组骨骼肌(胸大肌及肩胛下肌)的T1弛豫时间及细胞外容积(ECV)。采用单因素方差分析、Kruskal-Wallis检验进行三组数值的比较。结果Danon病组总TSMAi低于HCM组和对照组[(15.37±3.28)cm^(2)/m^(2)vs(23.02±3.88)cm^(2)/m^(2)vs(22.33±4.67)cm^(2)/m^(2),P<0.001],且各肌肉的TSMAi亦均低于HCM组和对照组(均P<0.05),HCM组与对照组的各TSMAi差异均无统计学意义(均P>0.05)。Danon病组及HCM组胸大肌native T1值均大于对照组(均P<0.05),Danon病组胸大肌及肩胛下肌的ECV均高于HCM组和对照组,enhanced T1值均低于HCM组和对照组(均P<0.05);HCM组与对照组的ECV、enhanced T1差异无统计学意义(P>0.05)。结论应用CMR能有效评价Danon病患者TSM形态及组织特征改变。与HCM患者相比,Danon病患者TSM显著萎缩,伴细胞外容积增加。CMR为两者的鉴别诊断提供了TSM的定量参考依据。

Objective To apply cardiovascular magnetic resonance(CMR)to quantitatively evaluate the morphology and tissue characteristics of thoracic skeletal muscle(TSM)in patients with Danon disease and hypertrophic cardiomyopathy(HCM),in order to provide auxiliary differential diagnostic information.Methods A retrospective study was conducted on 15 Danon disease patients(Danon disease group)who completed CMR examination,and 15 HCM patients(HCM group)and 15 healthy volunteers(control group)who were matched by gender and age were compared.TSM(pectoralis major,pectoralis minor,erector spinae,and subscapularis)area index(TSMAi),T1 relaxation time of four groups,and extracellular volume(ECV)of skeletal muscles of two groups(pectoralis major and subscapularis)were measured for all subjects.Single factor analysis of variance and KruskalWallis test were used to compare three sets of values.Results The total TSMAi of the Danon disease group was lower than that of the HCM group and the control group[(15.37±3.28)cm^(2)/m^(2) vs(23.02±3.88)cm^(2)/m^(2) vs(22.33±4.67)cm^(2)/m^(2),P<0.001],and the TSMAi of each TSM was also lower than that of the HCM group and the control group(all P<0.05).There was no statistically significant difference in TSMAi between the HCM group and the control group(all P>0.05).The native T1 values of the pectoralis major muscle in the Danon disease group and HCM group were higher than those in the control group(all P<0.05).The ECV of the pectoralis major muscle and subscapularis muscle in the Danon disease group were higher than those in the HCM group and control group,and the enhanced T1 values were lower than those in the HCM group and control group(all P<0.05);There was no statistically significant difference in ECV and the enhanced T1 values between the HCM group and the control group(P>0.05).Conclusions The application of CMR can effectively evaluate the changes in TSM morphology and tissue characteristics in Danon disease patients.Compared with HCM patients,Danon disease patients showed significant atrophy of TSM with increased extracellular volume.CMR provides a quantitative reference for TSM in the differential diagnosis of the two.