Fabry病患者的常规超声心动图及左心二维斑点追踪超声应变特征  

Conventional and two-dimensional speckle tracking echocardiography in Fabry disease

作　　者：于扬[1] 王崇慧 王涵 杨颖[1] 刘琳[1] 范芳芳[1] 赵亚雯 张巍[3] 马为[1] YU Yang;WANG Chonghui;WANG Han;YANG Ying;LIU Lin;FAN Fangfang;ZHAO Yawen;ZHANG Wei;MA Wei(Department of Cardiology,Peking University First Hospital,Beijing 100034,China;Department of Cardiology,Peking University International Hospital Beijing 100034,China;Department of Neurology,Peking University First Hospital Beijing 100034,China)

机构地区：[1]北京大学第一医院心内科,北京100034 [2]北京大学国际医院心内科,北京100034 [3]北京大学第一医院神经内科,北京100034

出　　处：《中华高血压杂志（中英文）》2024年第6期565-571,共7页Chinese Journal of Hypertension

基　　金：“十四五”国家重点研发计划(2021YFC2501106)。

摘　　要：目的通过常规超声心动图及左心二维斑点追踪超声心动图(2D-STE)分析Fabry病患者心肌受累情况,探讨Fabry病患者左心房、左心室应变特征。方法回顾性选取2014年8月至2023年2月在北京大学第一医院心内科、神经内科、肾内科就诊且在心内科行超声心动图检查的69例Fabry病患者作为Fabry病组,同期纳入年龄、性别匹配,无心血管、脑血管、肾脏、代谢及自身免疫性等疾病且超声心动图检查无明显异常的患者69例作为对照组。收集研究对象的临床资料、实验室检查,进行常规超声心动图测量和2D-STE分析左心房应变以及左心室纵向应变,比较组间相关参数的差异。结果与对照组相比,Fabry病组患者的体重指数、体表面积更小,心率更慢,估算的肾小球滤过率(eGFR)更低,脑利尿钠肽(BNP)、心肌肌钙蛋白I(cTnI)更高(均P<0.05)。Fabry病组的室间隔舒张早期反向峰(E'med)减低[(8.4±3.4)比(9.9±2.8)cm/s,t=2.821,P=0.006],而E峰、E峰减速时间、肺动脉收缩压升高,室间隔、左心室后壁厚度,左心室质量指数、二尖瓣口舒张早期峰值/室间隔舒张早期反向峰(E/E')升高(均P<0.05);Fabry病组左心房应变(包括储存期应变、管道期应变、泵血期应变)、左心室整体纵向应变减低,节段纵向应变中除下壁基底段、下壁中间段应变外,余节段纵向应变明显降低(均P<0.05)。结论Fabry病患者通常有肾脏、心脏等多器官受累。超声心动图通常表现为不同程度的心肌肥厚和舒张功能障碍,左室射血分数正常,而左心房应变、左心室整体及节段纵向应变均可出现不同程度的下降。Objective To analyze the myocardial involvement and explore the strain characteristics of the left atrium and the left ventricle in patients with Fabry disease(FD)using conventional echocardiography and two-dimensional speckle tracking echocardiography(2D-STE).Methods A retrospective study was conducted.Sixty-nine patients with FD who were treated in the Department of Cardiology,Neurology and Nephrology of Peking University First Hospital from August 2014 to February 2023 and underwent echocardiography examination in the Cardiology Depart-ment were collected(FD group).During the same time,69 age and sex matched individuals who had no cardiovascu-lar,cerebrovascular,renal,metabolic,and autoimmune diseases,and had no obvious abnormalities on echocardio-graphy were included in control group.The clinical data and laboratory tests were collected,and routine echocardio-graphy and 2D-STE were performed to analyze left atrial strain and left ventricular longitudinal strain.The differ-ences of relevant parameters between groups were compared.Results Compared with the control group,the pa-tients in the FD group had smaller body mass index(BMI)and body surface area,slower heart rate,poorer esti-mated glomerular filtration rate(eGFR),and significantly increased brain natriuretic peptide(BNP)and cardiac tro-ponin I(cTnl)(all P<0.05).Early diastolic reverse peak of interventricular septum(E'med)in the FD group was reduced[(8.4±3.4)us(9.9±2.8)cm/s,t=2.821,P=0.006]and E peak,E peak deceleration time,pulmona-ry artery systolic pressure increased,interventricular septum and left ventricular posterior wall thickened,left ventricular mass index and early diastolic transmitral inflow veloticy/septal mitral annular velocity(E/E')increased(all P<0.05).Compared with the control group,left atrial strains,including left atrial strain during reservoir phase(LASr),left atrial strain during contraction phase(LASct),left atrial strain during conduit phase(LAScd),and left ventricular average global longitudinal strain(GLSavg)were reduced in

关 键 词：FABRY病 左心室肥厚 应变 二维斑点追踪超声 

分 类 号：R596[医药卫生—内科学]