摘要
目的探讨肺动脉闭锁合并室间隔缺损(PA/VSD)产前超声诊断分型及预后评估。方法根据Boston分型将31例确诊为PA/VSD的胎儿分为Ⅰ~Ⅳ型,计算肺动脉McGoon指数,是否合并畸形及染色体异常,并进行随访。结果Ⅰ型PA/VSD共13例,其中合并畸形6例,染色体异常2例,7例McGoon指数>1.20,6例McGoon指数<1.20,8例行手术治疗(6例行根治术且术后随访良好,2例行姑息术且目前等待二次根治术),5例孕妇选择终止妊娠。Ⅱ型PA/VSD共6例,其中合并畸形5例,染色体异常1例,1例McGoon指数>1.20,5例McGoon指数<1.20,2例行手术治疗(1例行根治术且术后随访良好,1例行姑息术且目前等待二次根治术),4例孕妇选择终止妊娠。Ⅲ型PA/VSD共4例,其中合并畸形3例,无染色体异常,4例McGoon指数<1.20,1例行姑息术且目前等待二次根治术,3例孕妇选择终止妊娠。Ⅳ型PA/VSD共8例,其中合并畸形3例,染色体异常3例,1例行单源化手术,于术后1 d死亡,7例孕妇选择终止妊娠。胎儿McGoon指数提示PA/VSD产后行根治术的ROC曲线下面积为1.000(P=0.002,95%CI:1.0000~1.000),最佳分界值为1.255,相对应的敏感性和特异性分别为100%、85.7%。结论超声心动图能够对产前PA/VSD进行诊断分型,胎儿McGoon指数>1.255可以考虑产后行根治术,Ⅳ型PA/VSD、PA/VSD合并畸形及染色体异常预后较差。
Objective To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with ventricular septal defect(PA/VSD).Methods Thirty-one fetal pulmonary atresia with ventricular septal defect were classifiedⅠ-Ⅳtype by Boston classification,and the McGoon indexes were calculated,whether associated with malformation and chromosomal abnormalities,and follow-up.Results Thirteen fetuses were diagnosed typeⅠPA/VSD,6 fetuses were associated with malformation,2 fetuses were chromosomal abnormalities,7 fetuses′McGoon index>1.20,6 fetuses′McGoon index<1.20,8 cases had operation(6 cases had radical operation and had a good follow up,2 cases had palliative operation and were waiting for radical operation),5 cases received termination of pregnancy.Six fetuses were diagnosed as typeⅡPA/VSD,5 fetuses were associated with malformation,1 fetus was chromosomal abnormalities,1 fetus′s McGoon index>1.20,5 fetuses′McGoon index<1.20,2 cases had operation(1 case had radical operation and had a good follow up,1 case had palliative operation and was waiting for radical operation),4 fetuses received termination of pregnancy.Four fetuses were diagnosed as typeⅢPA/VSD,3 fetuses were associated with malformation,no fetus was chromosomal abnormalities,4 fetuses′McGoon index<1.20,1 case had palliative operation and was waiting for radical operation,3 cases received termination of pregnancy.Eight fetuses were diagnosed as typeⅣPA/VSD,3 fetuses were associated with malformation,3 fetuses were chromosomal abnormalities,1 case had unifocalization operation,but died after operation in one day,7 cases received termination of pregnancy.The area under the ROC curve of McGoon index in hinting PA/VSD postnatal radical operation was 1.000(P=0.002,95%CI:1.0000-1.000),the border value was 1.255,the sensitivity and specificity were 100%,85.7%,respectively.Conclusions Echocardiography can diagnose the classification of fetal PA/VSD.The radical operation for cases of McGoon index>1.255 is feasible,the cas
作者
刘琳
王红丹
崔存英
李亚南
刘园园
王莹
范太兵
彭帮田
Liu Lin;Wang Hongdan;Cui Cunying;Li Yanan;Liu Yuanyuan;Wang Ying;Fan Taibing;Peng Bangtian(Department of Echocardiography,Henan Provincial People's Hospital,Fuwai Central China Cardiovascular Hospital,People's Hospital of Zhengzhou University,Zhengzhou 450003,China;Gene Center,Henan Provincial People's Hospital,Fuwai Central China Cardiovascular Hospital,People's Hospital of Zhengzhou University,Zhengzhou 450003,China;Children's Heart Center,Henan Provincial People's Hospital,Fuwai Central China Cardiovascular Hospital,People's Hospital of Zhengzhou University,Zhengzhou 450003,China)
出处
《中华超声影像学杂志》
CSCD
北大核心
2019年第6期493-499,共7页
Chinese Journal of Ultrasonography
基金
国家自然科学基金项目(81401419)
河南省科技厅科技攻关项目(172102310003)
河南省卫生系统出国研修项目(2016047).
