| BackgroundCongenital vascular ring(CVR)refers to the congenital abnormal development of aorta and its branches or pulmonary artery and its branches,forming a vascular ring with themselves or with the ductus arteriosus(DA).The CVR compress the trachea and / or esophagus,causing varying degrees of respiratory and digestive symptoms.CVR is a rare congenital cardiovascular malformation accounting for 0.8% ~ 1.3% in congenital heart diseases.In recent years,with the development of medical examination equipment and prenatal diagnosis technology,CVR,used to be diagnosed only after birth or even after symptoms appear,can now be diagnosed in fetal period,providing better guidance for postpartum care and follow-up treatment.However,the difference in prenatal diagnostic criteria of CVR causes divergence in prognosis evaluation,which results in excessive induced abortion of CVR fetuses.Appropriate clinical decision calls for more accurate prenatal diagnosis in CVR.This study retrospectively analyzes the prenatal ultrasound images of CVR,summarizes the prenatal echocardiographic features of CVR,and improves the diagnostic efficiency.The clinical prognosis was compared with prenatal ultrasound to analyze the value of prenatal ultrasound diagnosis for clinical prognosis.Purpose1.To reclassify CVR that can be detected in prenatal diagnosis,summarize the echocardiographic features of each type of CVR,simplify the diagnostic process,and improve the diagnostic efficiency.2.To analyze the value of prenatal ultrasound diagnosis in predicting clinical prognosis by following up present cases,and to compare their clinical prognosis with prenatal ultrasound.Materials and MethodsPatients diagnosed as fetal CVR in Xinqiao hospital were included from January 1,2013 to June 30,2019,consisting of double aortic arch(DAA),mirror right aortic arch-left arterial duct(MRAA-LDA),right aortic arch-left arterial duct-aberrant left subclavian artery(RAA-LDA-ALSA),aberrant right subclavian artery-left aortic arch-Left arterial duct(ARSA-LAA-LDA)and pulmonary Artery sling(PAS).Gestation ages-matched normal fetuses served as controls.The ethics committee of Xinqiao hospital approved this study and all subjects signed the informed consent.Cases with poor image quality and insufficient diagnostic data were excluded from this study.Philips IE33 color doppler ultrasound diagnostic instrument and EPIQ 7C color doppler ultrasonic diagnostic instrument with convex array probe(frequency:2.0~ 5.0MHz),C5-1 abdominal ultrasound probe(frequency: 2.0 ~ 5.0MHz),and C5-3 abdominal ultrasound probe(frequency: 2.0 ~ 5.0MHz)were used.DC-8 color doppler ultrasonic diagnostic instrument manufactured by Mindray company in Shenzhen with convex array probe C5-2s was used.Fetal cardiac examination was performed by two-dimensional ultrasound examination mode,Color Doppler Flow Imaging(CDFI)mode,and Color Power Imaging(CPA)mode.The pregnant women lied in supine or lateral position,and fetal cardiac examination conditions were selected for fetal echocardiography.According to "China fetal echocardiography specification guide",fetal cardiac four-chamber view,left and right ventricular outflow view,three blood vessels-trachea view(3-VT),pulmonary artery view,long axis of the aortic arch view,descending aorta coronary view and ductus bow long axis plane were obtained and fetal heart development situation was evaluated.Echocardiography images were stored.The transverse diameters of left and right atria,right and left ventricular diameters,oval foramen size,inner diameters and peak flow rates of ascending aorta and pulmonary artery,inner diameters of pulmonary artery branches and ductus arteriosus(DA),systolic and diastolic flow rates of DA were measured and recorded.The data of pregnancy examination,fetal examination during pregnancy,and clinical decision-making plan were followed up.The fetus was followed up at 1 day,1 month and 3 months after birth,and every year after that.The end point of the follow-up study was fetal induction,postpartum death from various causes,or surgical treatment.for the cases underwent termination of pregnancy due to severe intracardial malformation,pathological specimens of fetuses were obtained with the consent of patients.The pathological results were compared with the database data of fetal complex congenital heart disease cases in our hospital.SPSS 22.0.0.0 statistical software was used,and measurement data were expressed as mean ± SD.Independent sample t-test was conducted.P< 0.05 was considered statistically significant.Results15796 cases received fetal echocardiography examination.A total of 229 fetuses with CVR combined with or without other cardiac malformations were diagnosed.The age range of pregnant women was 19 ~ 42 years old,and the average age was of 27.91±4.54 years old.Gestational age ranges from 21 to 38 weeks and with averages of(26.63±2.93)weeks.The case group include ARSA(LAA-LDA)in 112 cases(pure heart deformity of 13 cases,complex heart deformity of 5 cases),MRAA-LDA in 27 cases(pure heart deformity in 4 case,complex heart malformations in 6 cases),RAA LDA-with ALSA 70 cases(pure heart malformations in 4 cases,complex heart malformation in 2 fetuses),DAA 17(pure heart deformity in 1 case),and 3 cases with pulmonary artery sling.Among the 17 cases of DAA,11 cases were found to have right-arch dominant type and 6 cases with double-arch symmetric type.The RAA-LDA(mirror branch)group combined with most complicated intracardiac malformations,and 6(22.2%).The most common kind of simple intracardiac malformations CVR combined with CVR was double superior vena cava with dilated coronary sinus,with ventricular septal defect followed.The most common kind of complex intracardiac malformations CVR combined with CVR was Tetralogy of Fallot.The CVR with poorest prognosis was PAS,with DAA and MRAA-LDA followed.ARSA(LAA-LDA)and RAA-LDA-ALSA had better prognosis.Conclusion1.Fetal echocardiography plays a very important role in prenatal examination of CVR.3-VT section is the most important section to find and determine the type of vascular ring.Coronary section of aortic arch and long axial section of aortic arch are very important to judge DAA and its classification.3-VT section of pulmonary artery bifurcation is very important for judging PAS and its classification.2.Classification of CVR is very important for determining fetal prognosis.The prognosis evaluation of fetuses with heart malformation should consider intracardiac malformation and chromosome abnormality.In addition to classification,the blood vessel structures of isolated type of CVR also need to be considered.The development of the blood vessels after the birth of the ring structure is significant in prognosis evaluation. |