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1. 重庆市妇幼保健院超声科
2. 首都医科大学附属北京安贞医院超声科胎儿心脏病母胎医学北京市重点实验室心血管疾病精准医学北京实验室
3. 重庆市妇幼保健院遗传与生殖研究所
纸质出版日期:2020,
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[1]涂鹏,谷孝艳,张晓航,刘东云,林芸,张雪梅,董虹美,何怡华,冉素真.室间隔缺损胎儿产前超声及遗传学检查结果分析[J].临床超声医学杂志,2020,22(11):806-811.
TU Peng, GU Xiaoyan, ZHANG Xiaohang, et al. Analysis of prenatal ultrasound and genetic examination in fetuses with ventricular septal defect[J]. Journal of Clinical Ultrasound in Medicine, 2020,22(11):806-811.
[1]涂鹏,谷孝艳,张晓航,刘东云,林芸,张雪梅,董虹美,何怡华,冉素真.室间隔缺损胎儿产前超声及遗传学检查结果分析[J].临床超声医学杂志,2020,22(11):806-811. DOI: 10.16245/j.cnki.issn1008-6978.2020.11.003.
TU Peng, GU Xiaoyan, ZHANG Xiaohang, et al. Analysis of prenatal ultrasound and genetic examination in fetuses with ventricular septal defect[J]. Journal of Clinical Ultrasound in Medicine, 2020,22(11):806-811. DOI: 10.16245/j.cnki.issn1008-6978.2020.11.003.
目的探讨室间隔缺损(VSD)胎儿常见超声异常表型及遗传学结果
为产前咨询及预后评估提供依据。方法选取我院产前经超声心动图诊断
且染色体核型分析、染色体微阵列分析(CMA)、低深度全基因组测序(CNV-seq)、全外显子测序(WES)等遗传学检测资料完整并随访至妊娠终止的77例VSD胎儿
分析其心脏及心外超声结构异常表型、遗传学检查结果。结果 77例VSD胎儿中
孤立性VSD胎儿最多
为41例(53.2%)
其余为VSD同时合并心脏及心外畸形16例(20.8%)、VSD仅合并心脏畸形14例(18.2%)、VSD仅合并心外畸形6例(7.8%)。超声表型异常:合并心外畸形22例(28.6%)
以超声微小结构异常最常见;合并心脏畸形30例(39.0%)
均为圆锥动脉干畸形。遗传学异常者16例
检出率20.8%
其中VSD同时合并心脏及心外畸形胎儿的遗传学异常率最高
为43.8%(7/16)
其次为VSD仅合并心脏畸形胎儿[35.7%(5/14)]、VSD仅合并心外畸形胎儿[33.3%(2/6)]、孤立性VSD胎儿[4.9%(2/41)]。在核型正常VSD胎儿中检出12例异常
其中致病性CNVs 8例(6例微缺失和2例微重复)
4例单基因病
分子诊断率分别提高了15.1%、7.5%。结论产前超声检查发现胎儿VSD时
应仔细扫查心脏及心外异常
并进行遗传学检测;综合合理应用染色体核型分析、CMA、高通量测序(WES、CNV-seq)等方法能提高VSD胎儿遗传学异常检出率
为VSD胎儿的产前评估和预后咨询提供更全面的信息。
ObjectiveTo investigate the abnormal sonographic phenotypes and genetic examination in fetuses with ventricular septal defect(VSD)
and to provide information of prenatal counseling and prognosis assessment.MethodsSeventyseven fetuses with VSD diagnosed by echocardiography were selected
and their data of karyotype analysis
chromosome microarray analysis(CMA)
whole exome sequencing(WES)and copy number variation sequencing(CNV-seq)were complete
and follow-ups were performed until termination of pregnancy. The analysis of the heart
heart ultrasonic structure abnormal phenotype and genetic examination were conducted.ResultsAmong the 77 VSD fetuses
41 cases(53.2%)were isolated VSD which was the most
16 cases(20.8%)were VSD combined with cardiac and extracardiac malformations
14 cases(18.2%)were VSD combined with cardiac malformation
and 6 cases(7.8%)were VSD combined with extracardiac malformation. Ultrasound phenotypic abnormalities:22 cases(28.6%) were VSD combined with extracardiac malformations
in which ultrasonic microstructural abnormalities was the most common
and 30 cases(39.0%)were VSD combined with cardiac abnormalities
in which were all conus arteriosus malformations. There were 16 cases with genetic abnormalities
and the detection rate was20.8%. Among them
the highest rate was found in fetuses with VSD combined with cardiac and extracardiac malformations
which was 43.8%(7/16)
followed by 35.7%(5/14)with VSD combined with cardiac malformations
VSD combined withextracardiac malformation account for 33.3%(2/6)and isolated VSD account for 4.9%(2/41).12 abnormal cases were detected in normal karyotype VSD fetuses
including 8 cases of pathogenic CNVs(6 cases of microdeletions and 2 cases of microduplication)
and 4 cases of monogenic diseases. The molecular diagnostic rates were increased by 15.1% and 7.5%
respectively.ConclusionIntracardiac and extracardiac abnormalities should be carefully examined when VSD is detected in fetuses and perform genetic examination at the same time. Applying the methods including karyotype
CMA
WES
CNV-seq comprehensively and rationally can improve the detection rate of genetic abnormalities in VSD fetuses
and provide more information for prenatal evaluation and prognostic counseling.
超声检查室间隔缺损胎儿遗传学检测
UltrasonographyVentricular septal defectfetusGenetic examination
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