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752例胎儿心脏病临床预后分级与产前产后一体化管理的中期随访结果

The Mid-term Follow-up Results of 752 Cases of Fetal Heart Disease With Clinical Prognosis Stratification and Integrated Prenatal and Postnatal Management
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摘要 目的:分析胎儿心脏病(FHD)临床预后分级与产前产后一体化管理的中期随访结果。方法:回顾性分析2018年2月8日至2022年4月30日在中国医学科学院阜外医院(简称阜外医院)经胎儿超声心动图诊断的FHD胎儿817例。根据中国先天性心脏病(先心病)胎儿预后评分专家共识及阜外医院小儿外科中心评分将FHD分为Ⅰ级(0分)、Ⅱ级(1~3分)、Ⅲ级(4~6分)和Ⅳ级(7~9分)。需在新生儿期救治的FHD胎儿,完成孕产-出生-新生儿监护-外科手术-随访的全程闭环管理与救治,不保留的FHD胎儿引产。分析FHD胎儿的临床资料及中期随访结果。结果:共752例胎儿完成产前产后一体化管理的中期随访,平均随访(18.5±2.3)个月。FHDⅠ级胎儿111例(14.8%)、Ⅱ级251例(33.4%)、Ⅲ级275例(33.6%)、Ⅳ级115例(15.3%)。393例(52.2%)胎儿保留、1例(0.2%)胎死宫内、358例(47.6%)胎儿引产。与引产胎儿比,保留胎儿的孕妇年龄偏大,受检孕周更大,接受无创DNA及羊水穿刺检查的比率更低,发现染色体异常或基因异常的病例占比更小,FHD预后评分更低,差异均有统计学意义(P均<0.05)。FHDⅠ级胎儿中1例(0.9%)胎死宫内,110例(99.1%)出生并痊愈;Ⅱ级胎儿164例(65.3%)出生,并完成外科手术治愈或自行痊愈;Ⅲ级胎儿118例(42.9%)出生,其中117例完成双心室根治术,1例完成分期手术等待二期双心室根治术;Ⅳ级胎儿1例(0.9%)出生,并完成二期双向格林手术。FHDⅡ级引产胎儿中,8例(9.2%)因染色体或基因检测结果异常选择引产,8例(9.2%)合并其他脏器异常引产。FHDⅢ级引产胎儿中,5例(3.2%)合并染色体异常,5例(3.2%)基因检测异常,9例(5.7%)合并其他脏器异常。FHDⅣ级胎儿中只有1例(0.9%)左心发育不良综合征,因是双胎之一选择保留并顺利出生,生后新生儿期完成Norwood一期手术,6个月完成二期双向格林手术,术后恢复良好,其余胎儿均由孕产妇及家属选择引产。结论:根据临床预后进行分级管理有利于科学指导FHD胎儿的一体化救治,提高预后良好型FHD胎儿的救治成功率。 Objectives:To analyze the clinical prognosis stratification of fetal heart disease(FHD) and the mid-term follow-up results following integrated prenatal and postnatal management.Methods:Present retrospective analysis was performed on 817 fetuses diagnosed with FHD by fetal echocardiography in Fuwai Hospital,Chinese Academy of Medical Sciences(Fuwai Hospital) from the 8th February 2018 to 30th April 2022.According to the Chinese expert consensus on fetal prognosis score of congenital heart disease(CHD) and Fuwai Hospital Pediatric Center score,FHD was divided into grade Ⅰ(0 point),grade Ⅱ(1-3 points),grade Ⅲ(4-6 points) and grade Ⅳ(7-9points).For FHD fetuses that need to be treated in the neonatal period,the whole closed-loop management and treatment were completed,including pregnancy,birth,neonatal monitoring,surgery,and follow-up.The clinical data and mid-term follow-up results of FHD fetuses were analyzed.Results:A total of 752 fetuses completed the mid-term follow-up.The mean follow-up time was(18.5±2.3) months.There were 111 cases(14.8%) of FHD grade Ⅰ,251 cases(33.4%) of FHD grade Ⅱ,275 cases(33.6%) of FHD grade Ⅲ and 115 cases(15.3%) of FHD grade Ⅳ.There were 393 cases(52.2%) of fetal preservation,1 case(0.2%) of intrauterine death,and 358 cases(47.6%) of induced abortion.Compared with the induced fetus,the pregnant women in the reserved fetus group were older,the gestational age was longer,the rate of receiving noninvasive DNA and amniocentesis was lower,the proportion of chromosomal abnormalities or genetic abnormalities was smaller,and the FHD prognostic score was lower(all P <0.05).One case(0.9%) of FHD grade Ⅰ fetus experienced intrauterine fetal death,and 110 cases(99.1%) were born and recovered.164 cases(65.3%) of grade Ⅱ fetuses were born,and all of them were cured by surgery or spontaneously.118 cases(42.9%)of grade Ⅲ fetuses were born,117 cases completed biventricular radical operation,and 1 case completed two-stage operation waiting for the second stage biventricular radical operation.One case(0.9%) of grade Ⅳ was born and completed the twostage bidirectional Glenn procedure.Among the induced FHD grade Ⅱ fetuses,8(9.2%) had abnormal chromosomal or genetic testing results,and 8(9.2%) had other organ abnormalities.Among the induced FHD grade Ⅲ fetuses,5 cases(3.2%)had chromosomal abnormalities,5 cases(3.2%) had genetic abnormalities,and 9 cases(5.7%) had other organ abnormalities.There was only one case(0.9%) of FHD Ⅳ with hypoplastic left heart syndrome,which was retained as one of the twins and was born successfully.Norwood one-stage procedure was performed in the neonatal period,and two-stage bidirectional Greene procedure was performed at 6 months after birth.Other fetuses were induced following the choice of maternity and their family members.Conclusions:Hierarchical management based on clinical prognosis is helpful to scientifically guide the integrated treatment of FHD fetuses and improve the success rate of treatment of FHD fetuses with good prognosis.
作者 徐楠 张丽 邢佳怡 张婷婷 裴秋艳 逄坤静 XU Nan;ZHANG Li;XING Jiayi;ZHANG Tingting;PEI Qiuyan;PANG Kunjing(Ultrasound Imaging Center,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China;Department of Obstetrics,Peking University People's Hospital,Beijing 100044,China)
出处 《中国循环杂志》 CSCD 北大核心 2024年第9期871-876,共6页 Chinese Circulation Journal
基金 中国医学科学院临床与转化医学研究基金(2020-I2M-C&T-A-013)。
关键词 胎儿心脏病 临床预后 分级管理 随访 fetal heart disease clinical prognosis stratified management follow-up
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