完全型大动脉转位胎儿孕期及围产期一体化管理及预后分析

Integrated management and prognosis analysis of fetus with complete transposition of the great arteries during pregnancy and perinatal period

目的探讨孕期超声检查发现的完全型大动脉转位(D-TGA)胎儿的产前诊断、孕期及围产期一体化管理方法及其预后。方法回顾性分析2014年1月至2019年6月在北京大学人民医院孕期超声检查发现的19例D-TGA胎儿的产前诊断情况、孕期及围产期一体化管理情况及预后。结果5年余,孕期超声检查诊断的胎儿D-TGA的发生率为0.12%(19/16028)。19例D-TGA胎儿中,单纯D-TGA 7例(7/19),D-TGA合并室间隔缺损7例(7/19),D-TGA合并其他心内畸形5例(5/19)。19例D-TGA胎儿中合并心外畸形2例(2/19),合并胎儿生长受限1例(1/19),胎儿颈部透明层增厚3例(3/19)。19例超声检查发现的D-TGA胎儿中,18例(18/19)进行了胎儿或新生儿的染色体核型分析,发现染色体异常2例,均于孕中期终止妊娠。对D-TGA胎儿进行孕期及围产期一体化管理和多学科联合诊治,9例(9/19)孕中期引产,10例(10/19)足月分娩,分娩孕周为(38.3±0.7)周,其中产科因素剖宫产术分娩6例(6/10),阴道分娩4例(4/10)。10例新生儿出生时血氧饱和度为(69.2±11.3)%,根据出生时情况予以常压吸氧或前列地尔6.00~13.00 ng·min^-1·kg^-1泵入,新生儿转出时血氧饱和度为(77.8±6.7)%。10例患儿除1例失访外,其余9例均进行手术治疗,手术时间为出生后(21.8±22.1)d,8例一次手术完成,1例行二次手术。9例手术治疗的患儿随访至今均预后良好。结论孕期超声检查发现的D-TGA胎儿应行产前诊断,并进行个体化评估和孕期及围产期一体化管理。胎儿D-TGA并非剖宫产术指征,患儿出生后必要时药物维持动脉导管的开放,通过手术可获得良好预后。

Objective To investigate the prenatal diagnosis,integrated management and prognosis of fetal complete transposition of the great arteries(D-TGA)detected by ultrasonography.Methods The prenatal diagnosis,integrated management and prognosis of 19 D-TGA fetuses found by ultrasound during pregnancy in Peking University People′s Hospital from January 2014 to June 2019 were analyzed retrospectively.Results The incidence of D-TGA was 0.12%(19/16028)among fetuses diagnosed by ultrasound during 5 years.Among the 19 cases,there were 7 cases(7/19)of D-TGA alone,7 cases(7/19)of D-TGA combined with ventricular septal defect(VSD),5 cases(5/19)of D-TGA combined with other cardiac malformations;2 cases(2/19)of D-TGA combined with extra cardiac malformations,and 1 case(1/19)of fetal growth restriction.Nuchal translucency(NT)thickening was found in 3 cases(3/19)at the first trimester of pregnancy.Among the 19 D-TGA fetuses found by ultrasound examination,18(18/19)had chromosome karyotype analysis of fetuses or newborns,and chromosomal abnormalities were found in 2 cases,all of which were terminated in the second trimester of pregnancy.The integrated management and multidisciplinary diagnosis and treatment of D-TGA fetuses during pregnancy and perinatal period were carried out.Nine cases(9/19)had induction in the second trimester of pregnancy,10 cases(10/19)were delivered at term,and the gestational week of delivery was(38.3±0.7)weeks,among which 6 cases(6/10)were delivered by caesarean section due to obstetric factors,and 4 cases(4/10)were delivered by vaginal birth.The oxygen saturation was(69.2±11.3)%at birth and(77.8±6.7)%when transferred to the department of pediatrics.Except for one case lost to follow-up,the other 9 newborns received operation.The average operation time was(21.8±22.1)days after birth,8 cases(8/9)completed one operation and 1 case(1/9)performed two operations.All of the 9 cases treated by surgery were followed up well.Conclusions Prenatal diagnosis,individualized evaluation and integrated management during pregnancy and perinatal period should be carried out for the patients with fetal D-TGA detected by ultrasound.Fetal D-TGA is not an indication of cesarean section.The open of ductus arteriosus can be maintained with drugs when necessary after birth,and a good prognosis could be obtained through surgery.