压力-应变环评价重型β地中海贫血患儿左室功能  

Pressure-strain Loop Evaluation of Left Ventricular Function in Children withβ-thalassemia Major

作　　者：林周勤 林妍冰 范舒旻[2] 陈伟玲[2] 于薇[2] 夏焙[1,2] LIN Zhou-qin;LIN Yan-bin;FAN Shu-min;CHEN Wei-ling;YU Wei;XIA Bei(Shenzhen Clinical College of Pediatrics,Shantou University School of Medicine,Shenzhen 518038,Guangdong Province,China;Department of Ultrasound,Shenzhen Children's Hospital,Shenzhen 518038,Guangdong Province,China)

机构地区：[1]汕头大学医学院深圳儿科临床学院,广东深圳518038 [2]深圳市儿童医院超声科,广东深圳518038

出　　处：《罕少疾病杂志》2023年第8期3-5,共3页Journal of Rare and Uncommon Diseases

基　　金：广东省高水平医院建设经费资助(深儿医科教【2023】7号);国家自然科学基金面上项目(62071309);深圳市科技计划项目(SGDX20201103095802007)。

摘　　要：目的应用压力-应变环(PSL)技术定量检测重型β地中海贫血(β-TM)患儿不同铁负荷的左室收缩功能,评价铁过载(IO)对心功能的影响。方法应用PSL技术定量检测192例β-TM患儿左室功能,分为大龄组与小龄组;以心脏MRI T2*评价心肌铁负荷为标准,分为铁过载组(IO组)与非铁过载组(非IO组);根据是否进行造血干细胞移植,分为移植组与未移植组;取同期体表面积相匹配的63例健康儿童为对照组。比较各组间超声心动图心肌做功指标包括GLS、GWI、GCW、GWW及GWE。结果192例β-TM患儿中男107例,女85例,中位年龄9.1(7.2,11.0)岁,年龄范围4.8岁-18岁。其中,心脏MRI T2*铁过载42例(21.8%)。①全部β-TM组患儿的GLS、GWI、GCW及GWE均减小于对照组(均P<0.05);②在小龄组患儿中,PSL结果在IO患儿与非IO患儿之间的差异均无统计学意义(均P>0.05);③在大龄组患儿中,大龄IO患儿的GLS、GWI、GCW和GWW结果较同组非IO患儿减低,其中GWI、GCW差异具有统计学意义(均P<0.05);④移植组与未移植者比较,PSL结果中仅有GWW减小(P<0.05)。结论β-TM患儿左室的压力-应变功能较正常儿童明显下降,患儿年龄越大者,心功能损伤越明显;移植后儿童期PSL功能损伤仍然存在。Objective This study aimed to quantify the cardiac function and evaluate the impact of iron overload(IO)using myocardial pressure-strain loop(PSL)in children withβ-thalassemia major(β-TM).Methods A total of 192 patients withβ-TM were selected and divided into two groups based on age:a younger group and an older group.Additionally,based on the cardiac MRI T2*standard,the patients were further classified into two groups:an iron overload(IO)group and a non-iron overload group(non IO).The patients were further categorized into two groups based on whether they had undergone hematopoietic stem cell transplantation:a transplanted group and a non-transplanted group.Sixty-three healthy children matched by body surface area during the same period were used as controls.The myocardial work parameters,including GLS,GWI,GCW,GWW and GWE were quantified for each group.Results Our study included a total of 192 children withβ-thalassemia major(β-TM),consisting of 107 males and 85 females.The median age of the participants was 9.1 years(with an interquartile range of 7.2 to 11.0),ranging from 4.8 to 18 years.There were 62 cases with genotypeβ0/β0 and 130 cases with genotypeβ0/β+;42 cases(21.8%)were found to have iron overload based on MRI T2*imaging.Compared with the control group,GLS,GWI,GCW,and GWE were all reduced in theβ-TM group(P<0.05).There was no statistically significant difference in PSL results between IO and non-IO children in the younger group(all P>0.05).In the older group,the GLS,GWI,GCW,and GWW results were lower in older IO patients compared to non-IO patients.The differences in GWI and GCW were statistically significant(all P<0.05).There was no significant difference in the myocardial PSL results between the transplanted and non-transplanted groups(P>0.05).However,there was a significant reduction in GWW in the transplanted group compared to the non-transplanted group(P<0.05).Conclusions The myocardial PSL function decreased in children withβ-TM,with a more pronounced impairment observed in older childr

关 键 词：重型Β地中海贫血 超声心动图 压力-应变环 儿童 

分 类 号：R720.5[医药卫生—儿科]