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

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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%(19/16 028)。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·minn -1·kgn -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。n 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% (19/16 028) 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.
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