Prenatal diagnosis and counselling for congenital portosystemic venous shunts / 中华围产医学杂志

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.

Characteristics and prognosis of interrupted inferior vena cava with azygous continuation / 中华妇产科杂志

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.

Clinical value of interrupted inferior vena cava in prenatal diagnosis of isomerism / 中华围产医学杂志

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.

Case-control study of maternal risk factors for fetal congenital heart diseases / 中华围产医学杂志

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.