Objective:To investigate the mode and timing of delivery in pregnant women with complex fetal congenital heart disease (CHD) detected by prenatal ultrasound.Methods:The clinical data of 123 fetuses with complex CHD detected by prenatal ultrasound in Peking University People′s Hospital from January 2016 to December 2023 were retrospectively analyzed. Pregnant women with indications for prenatal diagnosis underwent G-banding karyotype analysis, single nucleotide polymorphism array (SNP arrry) or whole exome sequencing after informed consent. Integrated managements were provided for pregnant women with complex CHD during pregnancy and perinatal period, and to determine the mode and timing of delivery. Infants with complex CHD received timely treatment or referral after birth.Results:The gestational age at ultrasound diagnosis of the 123 fetuses with complex CHD was (23.7±3.4) weeks. There were 11 cases (8.9%) of total anomalous pulmonary venous connection (TAPVC), 14 cases (11.4%) of anomalous pulmonary valve (PVA), 7 cases (5.7%) of right ventricle double outlet (RVDO), 13 cases (10.6%) of anomalous aortic arch, 69 cases (56.1%) of transposition of the great arteries (TGA), 9 cases (7.3%) of other types. All cases were treated with fetal preservation after prenatal consultation. Among the 72 cases undergoing prenatal diagnosis, 9 cases (12.5%) had chromosomal variations. Fifty-five cases (44.7%, 55/123) underwent trial of labor, of which 46 cases (37.4%, 46/123) had successful vaginal delivery, including 6 cases of forceps delivery, and other 9 cases of failed trial of labor transferred to cesarean section. A total of 77 cases of pregnant women underwent cesarean section. Except for the 21 cases of pregnant women who asked for cesarean section, the cesarean section rate of pregnant women with complex CHD was 45.5% (56/123), which was not significantly different from the average cesarean section rate of the same period in our hospital (40.2%; χ2=7.34, P=0.270). The gestational age at delivery of the 123 fetuses with complex CHD was (37.9±1.4) weeks, the birth weight of the neonates was (3 099±480) g, and the umbilical artery blood pH value was 7.31±0.05. The oxygen saturation of 86 cases before and after alprostadil infusion were (72.8±6.0)% and (80.5±5.0)%, respectively, and the difference was statistically significant ( t=4.38, P<0.001). One hundred and fourteen children underwent surgical treatment, 112 of them (98.2%) had good postoperative reexamination. Only 2 cases (1.8%) died after surgery, and 14.0% (16/114) had the possibility of secondary surgery. Conclusions:Fetal complex CHD is not an indication for cesarean section, and the delivery mode could be selected according to the obstetric situation. If the mother and child are in stable condition, the delivery is planned after 39 weeks of gestation. For children with low oxygen saturation after birth, alprostadil could be pumped to maintain the open ductus arteriosus, and timely referral to the pediatric cardiac surgery for subsequent surgical treatment, which could achieve a good prognosis.

Objective:To investigate the mode and timing of delivery in pregnant women with complex fetal congenital heart disease (CHD) detected by prenatal ultrasound.Methods:The clinical data of 123 fetuses with complex CHD detected by prenatal ultrasound in Peking University People′s Hospital from January 2016 to December 2023 were retrospectively analyzed. Pregnant women with indications for prenatal diagnosis underwent G-banding karyotype analysis, single nucleotide polymorphism array (SNP arrry) or whole exome sequencing after informed consent. Integrated managements were provided for pregnant women with complex CHD during pregnancy and perinatal period, and to determine the mode and timing of delivery. Infants with complex CHD received timely treatment or referral after birth.Results:The gestational age at ultrasound diagnosis of the 123 fetuses with complex CHD was (23.7±3.4) weeks. There were 11 cases (8.9%) of total anomalous pulmonary venous connection (TAPVC), 14 cases (11.4%) of anomalous pulmonary valve (PVA), 7 cases (5.7%) of right ventricle double outlet (RVDO), 13 cases (10.6%) of anomalous aortic arch, 69 cases (56.1%) of transposition of the great arteries (TGA), 9 cases (7.3%) of other types. All cases were treated with fetal preservation after prenatal consultation. Among the 72 cases undergoing prenatal diagnosis, 9 cases (12.5%) had chromosomal variations. Fifty-five cases (44.7%, 55/123) underwent trial of labor, of which 46 cases (37.4%, 46/123) had successful vaginal delivery, including 6 cases of forceps delivery, and other 9 cases of failed trial of labor transferred to cesarean section. A total of 77 cases of pregnant women underwent cesarean section. Except for the 21 cases of pregnant women who asked for cesarean section, the cesarean section rate of pregnant women with complex CHD was 45.5% (56/123), which was not significantly different from the average cesarean section rate of the same period in our hospital (40.2%; χ2=7.34, P=0.270). The gestational age at delivery of the 123 fetuses with complex CHD was (37.9±1.4) weeks, the birth weight of the neonates was (3 099±480) g, and the umbilical artery blood pH value was 7.31±0.05. The oxygen saturation of 86 cases before and after alprostadil infusion were (72.8±6.0)% and (80.5±5.0)%, respectively, and the difference was statistically significant ( t=4.38, P<0.001). One hundred and fourteen children underwent surgical treatment, 112 of them (98.2%) had good postoperative reexamination. Only 2 cases (1.8%) died after surgery, and 14.0% (16/114) had the possibility of secondary surgery. Conclusions:Fetal complex CHD is not an indication for cesarean section, and the delivery mode could be selected according to the obstetric situation. If the mother and child are in stable condition, the delivery is planned after 39 weeks of gestation. For children with low oxygen saturation after birth, alprostadil could be pumped to maintain the open ductus arteriosus, and timely referral to the pediatric cardiac surgery for subsequent surgical treatment, which could achieve a good prognosis.

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(7-9 points).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 two-stage 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.

Humans ; Pregnancy ; Female ; Umbilical Cord/diagnostic imaging* ; Pregnancy Outcome ; Ultrasonography ; Ultrasonography, Prenatal

Congenital portosystemic venous shunts (CPSVS) are defined as abnormal vascular communications between the portal and the systemic venous circulation,which is a rare vascular malformations,but with potentially devastating complications.In order to better understand the diagnosis and prenatal counselling,here we summarized some literatures about the embryologic origins,ultrasound image features,prenatal diagnosis,classifications and postnatal clinical presentations,treatment and prognosis of CPSVS.

Objective To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis.Methods Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan.The clinical data,ultrasonographic features,genetic information and prognosis were collected. Results Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567)of 28 567 patients.Three fetuses(14%,3/21)complicated with heart and extracardiac malformations, including endocardiac cushion defect,single atrium and single ventricle,double superior vena cava,dextrocardia, asplenia syndrome,visceral heterotaxy,duodenal atresia;six fetuses(29%,6/21)were associated with cardiac anomalies, such as hypoplastic left heart syndrome, double outlet right ventricle, pulmonary stenosis, ventricular septal defect,persistent left superior vena cava,endocardiac cushion defect and transposition of the great arteries;six cases(29%,6/21)were only combined with extracardiac malformations,includingasplenia syndrome, visceral heterotaxy, duodenal atresia. Three fetuses (14%,3/21) were nonorganic abnormalities included thickening of the right ventricle wall, fetal bradycardia, pericardial effusion, hydrops abdominis, increased peak systolic velocity/end diastolic velocity and single umbilical artery.Three fetuses(14%,3/21) were isolated interrupted inferior vena cava with azygous continuation,but without other anomalies and 2 of them had normal fetal karyotype.Five cases(24%,5/21)were successfully vaginal delivery,1 case(5%,1/21) had cesarean section. After 12-40 months follow-up, we didn′t obeserve obviously abnormality, nor any chromosomal abnormality.Ten patients(48%,10/21)opted for termination of the pregnancy and the autopsies were not done.Five cases(24%,5/21)were lost to follow up.Conclusions Interrupted inferior vena cava with azygous continuation are associated with cardiovascular and extracardiac anomalies, cardiac malformation and visceral heterotaxy are the most common anomalies. Visceral heterotaxy should be considered and fetal karyotype should be suggested. In the cases of isolated interrupted inferior vena cava with azygous continuation and normal karyotype,the outcome is favorable.

Objective To analyze the ultrasonic features of interrupted inferior vena cava (IVC) and its relationship with related deformities so as to improve the prenatal diagnosis of isomerism.Methods Pregnant women who received prenatal care in Peking University People's Hospital or who were referred to our hospital for suspected malformation of fetus between January 2010 and December 2013 were enrolled in this study.Fetal echocardiography and abdominal transverse section were performed routinely.Spatio-temporal image correlation technology was used to further clarify interrupted IVC if azygos vein was broadened or IVC disappeared in the fetal abdominal transverse sections.Based on the presence of cardiac structural abnormalities,interrupted IVC was divided into isolated IVC interruption and complex IVC interruption.Neonatal echocardiography was performed in women with continued pregnancies.In women with terminated pregnancy,fetus was autopsied after induced labor.Results In total,12 cases of interrupted IVC were diagnosed,including 5 cases of isolated IVC interruption with full-term delivery and 7 cases of complex IVC interruption.Neonatal echocardiography,abdominal ultrasound were all normal in the 5 cases of isolated IVC interruption except for 3 cases complicated with sistus inversus viscerum.All the 7 cases of complex IVC interruption had induced labor because of some congenital cardiac abnormalities.Autopsy showed that 6 cases had left isomerism,and 1 case had right isomerism.Conclusions Interrupted IVC can be easily identified in prenatal diagnosis due to the typical ultrasonic features.Complication with some congenital cardiac abnormalities often indicates isomerism,especially left isomerism.

Objective To investigate maternal risk factors for fetal congenital heart diseases (CHD). Methods A case-control study was conducted on 16 645 pregnant women who underwent cardiovascular malformation screening for fetal cardiovascular system,whose pregnancy outcomes were recorded,and whose newborns were scanned by an echocardiography in Peking University People's Hospital,Haidian,Changping,Mentougou and Daxing Maternal and Child Health Hospital from Nov.2006 to Oct.2009.One hundred and twelve pregnant women whose babies were found to be CHD (40 severe CHD and 72 simple CHD) before or after delivery were taken as study group.Women in control group (n =304) were randomly selected from those pregnant women who had infants without CHD.Logistic regression analysis and x2 test were used to analyze the maternal risk factors for fetal CHD. Results (1) The average age of women whose infants had severe CHD was 28.3 years (21-40 years),and it was 29.9 years (22-39 years) for women whose infants had simple CHD.There were no significant differences between the control group (29.5 years,20-44 years) and the above two groups (t=1.511 and -0.826,P=0.138 and 0.410 respectively).(2) Single factor analysis:during first trimester,the rate of upper respiratory infection (18/39,46.2 ％) and exposure to certain chemicals (13/40,32.5％) of severe CHD group were higher than those of control group [(14.9％ (45/303) and 2.0％ (6/304)] (x2 =22.399 and 62.678,OR=4.895 and 23.753,95％CI:2.419-9.905 and 8.358-67.506,P =0.000 respectively).Compared with control group (0.0％,0/304),the rate of pregnant women with CHD family history in simple CHD group was significantly higher (4.2％,3/72)(Fisher exact test,P=0.007).(3) Logistic regression analysis:maternal upper respiratory infections (OR =5.120,95％CI:2.340-11.206,P =0.000) and exposure to certain chemicals (f)R=23.030,95％CI:7.506-70.665,P=0.000) during first trimester were risk factors for fetal severe CHD. Conclusions Upper respiratory infection and exposure to certain chemicals during first trimester might play important roles in the occurrence of fetal severe CHD.Maternal family history of CHD might associate with fetal simple CHD.