Prenatal diagnosis of isolated mirror-image right aortic arch with vascular ring by fetal echocardiography

Objective To explore the value of three-vessel and trachea(3VT) view plus aortic arch coronal section in ultrasonic diagnosis and classification of fetal right aortic arch(RAA). Methods Echocardiography data and follow-up results of 44 fetuses with RAA were retrospectively analyzed. Sonographic features on 3VT view and aortic arch coronal section were summarized. Results Among 44 fetuses with RAA, 27 cases(61.36%) were RAA with aberrant left subclavian artery(ALSA) and the left ductus arteriosus(LDA) connecting the ALSA and U-shaped vascular ring was formed; 17 cases(38.64%) were mirror right aortic arch(MRAA). Among 17 cases with MRAA, 3 cases were with LDA connecting the descending aorta(DAO) and U-shaped vascular ring was formed; 1 case was with LDA connecting the left innominate artery(LINA), 8 cases were with absent ductus arteriosus(ADA), 5 case were with right ductus arteriosus(RDA), no vascular ring was formed. Parts of MRAA were combined with other complex intracardiac abnormalities. RAA-ALSA-LDA were rarely combined with intracardiac abnormalities. Conclusions 3VT view is simple and intuitive for diagnosing RAA. Aortic arch coronal section has great clinical value in determining the concrete type of RAA. Key words: Echocardiography; Fetus; Aortic disease; Three-vessel and trachea view; Aortic arch coronal section

Objective By comparing the results of prenatal diagnosis of vascular rings by ultrasound and MRI, chromosome screening and postnatal follow-ups, to explore the clinical significance of prenatal diagnostic technology for the diagnosis of vascular rings and prognostic assessment. Methods Ninety-five fetuses that were diagnosed by fetal echocardiography were followed up regularly, of which 72 cases had chromosome screening, 31 cases had MRI examination. These results were compared with the postnatal follow-up outcome. Results Ninety-five cases of vascular rings included 85 cases of right aortic arch(RAA) and left ductus arteriosus(LDA), 4 cases of pulmonary artery sling(LPAS), 4 cases of double aortic arch(DAA), 1 case of RAA and double ductus arteriosus(DDA), 1 case of left aortic arch(LAA) and aberrant right subclavian artery(ARSA). Sixty-six cases of isolated vascular rings and 29 cases of complex vascular rings(combined with other cardiovascular abnormalities) were detecded. Fifty-four cases were born(2 infants died), 8 cases were ongoing pregnancy, 6 cases were lost to follow-up, 27 cases were aborted. Prenatal ultrasound and MRI examination indicated 6 cases of trachea compression. However, only 3 infants showed clinical symptoms due to trachea compression from born 5 cases. Conclusions Isolated vascular ring is the most common format and its diagnosis and type can be determined by prenatal ultrasound. Dynamic follow-up using ultrasound in pregnancy combined with MRI is helpful to assess the compression degree of trachea induced by vascular ring and prognosis. Key words: Ultrasonography, prenatal; Magnetic resonance imaging; Fetal heart; Vascular ring

Objective To explore the ultrasonographic features, diagnostic clues and methods of fetal aortic arch anomalies, and to improve the diagnosis rate of prenatal ultrasonography for fetal aortic arch anomalies. Methods Fetuses with aortic arch anomalies diagnosed by prenatal ultrasound were selected as the study objects. The prenatal ultrasonographic features of the three views of the aortic arch were carefully observed. The three views included the followings: the transverse view(the view of three vessels and trachea), the sagittal view (the longitudinal view of the aortic arch), and the coronal view (the coronal view of the descending aorta). The prenatal diagnostic clues and diagnostic techniques of aortic arch anomalies were summarized and analyzed. Results Eighty-one cases with aortic arch anomalies were followed up. Fifteen cases were left-sided aortic arch and left-sided ductus arteriosus with aberrant right subclavian artery, 22 cases with right aortic arch and left-sided ductus arteriosus with aberrant left subclavian artery, 4 cases were mirror right aortic arch and left-sided ductus arteriosus, 2 cases were mirror right aortic arch and right-sided ductus arteriosus, 11 cases were double aortic arch, 23 cases were coarctation of the aorta, and 4 cases were interruption of aortic arch. Among them, 27 cases were complicated with other cardiac and extra-cardiac malformations and 13 cases with chromosomal abnormalities. Prenatal ultrasonographic clues for diagnosis of coarctation of the aorta included the three vessels and trachea view showing small diameter and disproportion to the diameter of the ductus arteriosus. And the longitudinal view of the aortic arch was helpful for showing the position and the extent of coarctation of the aorta. Prenatal ultrasonographic clues for diagnosis of interruption of aortic arch were that the three vessels and trachea view and the longitudinal view of the aortic arch showed the interruption between aortic arch and descending aorta. And the abnormal combination cases of the aortic arch segments also had their corresponding diagnostic clues. These clues included that the three vessels and trachea view showed abnormal position, arrangement and running of the branches of the aortic arch. And the coronal view of the descending aorta also showed abnormal changes in the branches of aortic arch. Conclusions Various types of congenital aortic arch anomalies have characteristic ultrasonography and their corresponding diagnostic clues and methods. The combined use of the three views of the aortic arch is effective in prenatal diagnosis for aortic arch anomalies. Key words: Ultrasonography, prenatal; Aortic arch anomalies; Diagnostic clues

Objective To observe characters in isolated complete vascular ring (CVR) fetuses and to evaluate the trachea area (TA) and vascular ring area (VRA) changes by fetal echocardiography. Methods Ninety-two fetuses with isolated complete vascular ring were diagnosed by fetal echocardiography prenatally and confirmed by postnatal echocardiography, from August 2010 to November 2015 in the Department of Ultrasound in Beijing Anzhen Hospital and Fetal Heart Disease Maternal Fetal Medicine Research Important Laboratories. The VRA and TA were measured at the three vessel-trachea view, and the parameters were compared. Results The TA was decreased in vascular ring fetuses compared with normal fetuses [(5.27±1.49) mm2 vs (2.39±1.26) mm2]. The VRA of vascular ring fetuses was (48.92±19.10) mm2. Right aortic arch and left ductus arteriosus formed ʺUʺ shaped in 77 cases. Double aortic arch formed ʺOʺ shaped or ʺλʺ shaped in 15 cases. Conclusions Fetal echocardiography can accurately diagnosed isolated complete vascular ring. The TA decreased in vascular ring fetuses compared with normal fetuses. Key words: Echocardiography; Fetal; Vascular ring; Trachea

Re: Anomalies of the fetal aortic arch.

This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. isuog GUIDELINES ISUOG Practice Guidelines (updated): fetal cardiac screening The International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) is a scientific organization that encourages sound clinical practice and high-quality teaching and research related to diagnostic imaging in women's healthcare. The ISUOG Clinical Standards Committee (CSC) has a remit to develop Practice Guidelines and Consensus Statements as educational recommendations that provide healthcare practitioners with a consensus-based approach, from experts, for diagnostic imaging. They are intended to reflect what is considered by ISUOG to be the best practice at the time at which they are issued. Although ISUOG has made every effort to ensure that Guidelines are accurate when issued, neither the Society nor any of its employees or members accepts liability for the consequences of any inaccurate or misleading data, opinions or statements issued by the CSC. The ISUOG CSC documents are not intended to establish a legal standard of care, because interpretation of the evidence that underpins the Guidelines may be influenced by individual circumstances, local protocol and available resources. Approved Guidelines can be distributed freely with the permission of ISUOG (

Objective To investigate the value of prenatal echocardiographic diagnosis of anomalies of the right aortic arch(RAA) and its branch type, and provide rapid diagnostic methods. Methods A retrospective analysis of 55 cases of RAA of prenatal ultrasound diagnosis results, and their imaging characteristics were summarized. Results There were 55 cases of anomalies of the RAA, included 42 cases of RAA with aberrant left subclavian artery(RAA-ALSA) and 13 cases of RAA with mirror image branching pattern(RAA-MB), 1 case of double aortic arch(DAA) was misdiagnosed as RAA. The prenatal echocardiographic characteristics of RAA-ALSA: in three-vessel-aortic arch view, the first branch of the aortic arch was the left carotid artery, which ran in front of the trachea, and ALSA ran behind the trachea and esophagus. The prenatal echocardiographic characteristics of RAA-MB: the first branch of the aortic arch was the brachiocephalic trunk, which ran in front of the trachea and was divided to the left carotid artery and the left subclavian artery. Conclusions Prenatal ultrasound can qunickly and accurately diagnose anomalies of the right aortic arch and its branch type according to the direction, thickness and secondary branches of the first branch of the aortic arch. The location of arterial duct is helpful to judge the formation of vascular ring. Key words: Ultrasonography, prenatal; Right aortic arch; Vascular ring

Type B Aortic Dissection Involving an Isolated Right-Sided Aortic Arch

Objective To investigate the operation method for congenital tracheal stenosis(CTS) with vascular ring(VR), in order to minimize surgical risk. Methods The data of 53 VR patients diagnosed by CT imaging, fiber bronchoscope and color doppler echocardiography were analyzed retrospectively.The age ranged from 5.0 days to 2.5 yeas old, median age was 12.5 months, including pulmonary artery sling, double aortic arch, right arch combined with abnormal left subclavian artery and innominate artery oppression, et al, in which 12 patients(8 male, 4 female) associa-ted with CTS symptoms.All patients were treated by operations, 8 patients were dead, others were followed up for 3 to 5 years at 6-month interval. Results Fifty-three patients with VR were categorized into 2 treatment groups.There were 41 patients(25 male, 16 female) without CTS symptoms treated by VR repair(3 cases died), mortality was 7.3%.Of the 12 patients(8 male, 4 female) with VR+ CTS symptoms, 7 cases were treated by selective VR repair with 2 cases deaths, 5 cases underwent combined VR and CTS repairs with 3 cases died, for an overall mortality of 41.7%.Of the 41 patients with VR but no CTS, 7 cases had intracardiac anomalies with 2 cases died.In contrast, 3 of 12 patients with VR+ CTS and intracardiac anomalies with 2 cases died; 5 patients received cardiac and CTS repairs(3 cases died) and 7 cases in selective VR repairs(2 cases died). Conclusions Not all cases of TN associated with VR require combined correction.CTS and the combined cardiac abnormalities increased the risk of VR surgery in children.The individualized treatment program should be developed for such children. Key words: Vascular ring; Congenital tracheal stenosis; Individualized; Infant

The prenatal detection of a right-sided aortic arch achieved mainly by targeted visualization of the threevessel and three vessels and trachea (3VT) view, with or without color Doppler, has been discussed recently in this journal1–4. Two typical forms of a right aortic arch can be distinguished5,6. In one condition a vascular ring is found around the trachea, the so-called U-sign prenatally (Figure 1)2,3. The trachea and esophagus are entrapped between the right aortic arch and the left ductus arteriosus and this abnormality is often an isolated incidental finding prenatally2. In the other condition, both the aorta and ductus arteriosus lie to the right of the trachea without a vascular ring. This condition is very commonly associated with cardiac anomalies5. The branching pattern of the great vessels arising from the aortic arch in both conditions is of major interest in pediatric cardiology5,6. The right aortic arch without a vascular ring usually exhibits mirror image branching of the arteries with the left innominate (brachiocephalic) artery arising first followed by the right common carotid and right subclavian artery6. By contrast, the right aortic arch with vascular ring very often has an association with an aberrant left subclavian artery. The left common carotid arises first from the aortic arch, followed by the right common carotid, right subclavian artery, and finally a retroesophageal vessel segment from which the left subclavian artery arises and the ductus arteriosus connects. The retroesophageal (and retrotracheal) vessel segment is known as the diverticulum of Kommerell. In other words, the left subclavian artery is connected ventrally to the ductus arteriosus arising from the left pulmonary artery, and dorsally through the Kommerell’s diverticulum to the descending aorta. In postnatal life, after closure of the ductus arteriosus, blood enters the left subclavian artery via the descending aorta and Kommerell’s diverticulum4. Prenatal assessment of a right-sided aortic arch and its branching pattern requires scanning in such planes as a transverse 3VT view (Figure 1), oblique cephalad Left

Objective This study aimed to summarize the method of diagnosis and surgical treatment for congenital vascular ring in infants.Methods A retrospective analysis of clinical data of 16 cases (male =11; female =5) with congenital vascular rings from March 2008 to July 2011were conducted.Patients’ agesranged from 4 months to 3.2 years old (mean 2.2 years 1.7).Preoperative 64 slice CT chest with three-dimensional reconstruction of the great vessels and trachea were performed,The diagnosis included double aortic arch in 3 cases; right aortic arch with left ligament and aberrant left subclavian artery in 7 cases; right aortic arch with aberrant left innominate artery in 1 case,left aortic arch with aberrant right subclavian artery in 2 cases; pulmonary artery sling in 3 cases.Clinical manifestations of childrenincluded recurrent respiratory tract infection,chronic cough,wheezing,respiratory distress,dysphagia,and vomiting.13 patients underwent surgical repair; 3 cases of pulmonary artery sling had surgery under cardiopulmonary bypass; while the others had off-pump surgery,including l case of aortopexy surgery.Results No operative mortality was seen in any patient.Postoperative complication included 1 case of chylothorax and 1 case of left vocal cord palsy.The mean follow-up period was 6 months.Gastrointestinal symptoms disappeared 2 weeks after the operation.6 patients still had wheezing,recurrent respiratory tract infection 3 months after the operation.By 6 months,2 patients had wheezing,and chronic cough.Only 1 patient still had wheezel year after operation.Significant reduction of seizure frequency was obtained comparing with the previous status and normal growth and development were achieved.Conclusions In infants and young children with recurrent pneumonia,breathing difficulties unexplained wheezing,dysphagia,the possibility of vascular ring should be borne in mind.Early detection,and surgical treatment should be offered. Key words: Vascular ring,congenital; Arch of the aorta; Infants

Objective To explore the value of MRI in the diagnosis of fetal aortic arch anomalies. Methods We retrospectively collected 10 fetuses with aortic arch anomalies indicated by prenatal ultrasound and underwent MR examination and were subsequently proven by autopsy or post-birth follow-up from 320 pregnant women. We focused on the observations of the location of the aortic arch and brachiocephalic artery anomalies, the locations of the liver and stomach in the abdominal cavity, and the large vessels in abdomen. The above-mentioned finding were compared with prenatal ultrasound and follow-up findings. Results Of 10 cases, right aortic arch with aberrant left subclavian artery was seen in 7 cases, right aortic arch with the mirror branch, left aortic arch with aberrant right subclavian artery, right aortic arch with aberrant left subclavian artery combined with cervical aortic arch and double aortic arch was seen in 1 case, respectively. All aortic arch anomalies detected by MRI were consistent with post-birth or autopsy findings. Ultrasound misdiagnosed aortic branch malformation in 5 places, which included right aortic arch but misdiagnosed aberrant left subclavian artery in 2 cases, right aortic arch never diagnosed mirror branch anomaly in 1 case, right aortic arch with left subclavian artery misdiagnosed cervical aortic arch in 1 case, left atrial isomerism but misdiagnosed left aortic arch with aberrant right subclavian artery in 1 case; One double aortic arch was misdiagnosed as right aortic arch with aberrant left subclavian artery in ultrasound. Conclusion Fetal cardiovascular MRI is an effective and supplementary examination to complement ultrasound in diagnosis of fetal aortic arch anomalies. Key words: Fetus; Aorta,thoracic; Vascular malformations; Magnetic resonance imaging

To describe a novel, sonographic approach for in-utero evaluation of normal and abnormal aortic arch. Aortic arch was evaluated by imaging of the axial view of the upper fetal mediastinum. The normal left aortic arch was defined by the V-shaped appearance of the junction between the ductus arteriosus and aortic arch, with the trachea situated posteriorly. Right and double aortic arches were diagnosed when the great vessels appeared U-shaped, with intermediate location of the trachea. Between 1997 and 1999, 18 347 women were scanned in three prenatal centers, and pathological findings were prospectively recorded. In a retrospective analysis of the records, we identified 19 fetuses (0.1%) with atypical, U-shaped appearance, and no other structural abnormalities present. With the exception of one fetus with a ventricular septal defect, no congenital cardiac defects were present. Right aortic arch was found in 18 cases, while color Doppler made it possible to diagnose one case with double aortic arch, and one fetus was demonstrated as having Kommerell's diverticulum. In all 18 cases, a left descending aorta and left ductus arteriosus were present, the latter coursing to the left of the trachea, forming a loose partial vascular ring. All were asymptomatic at birth and early infancy. The fetus with double aortic arch that had a true vascular ring underwent early infantile correction. It is possible to diagnose right and double fetal aortic arch using prenatal ultrasound. The use of color Doppler facilitated in-utero evaluation of possible complications, such as true vascular ring.

Objective To evaluate the role of combination of three-vessel views in ultrasound diagnosis of fetal aortic arch and pulmonary arterial branch anomalies. Methods The data of 66 cases of fetal aortic arch and pulmonary arterial branch anomalies were retrospectively analysed, including echocardiographic data, autopsy and operation records and postnatal follow-up results. Echocardiogaphic features and display frequencies on three vessels and trachea view(3VT), three vessels view(3VV) and three vessels and pulmonary arterial branches view(3VP) were summarized. Results There were 52 cases of aortic arch abnormalities, including 32 cases of right aortic arch with left ductus arteriosus, 4 cases of double aortic arch, 7 cases of aberrant right subclavian artery, 6 cases of coarctation of aorta and 3 cases of interruption of aortic arch. Fourteen cases were diagnosed pulmonary aterial branch abnormalities, including 10 cases of crossed pulmonary arteries, 2 cases of anomalous origin of left pulmonary artery from aortic arch and 2 cases of pulmonary artery sling. The display frequencies of fetal aortic arch and pulmonary arterial branch anomalies on 3VT, 3VV and 3VP were 80.3%, 19.7% and 39.4%. Conclusions The combination of three-vessel views is of great value in prenatal diagnosis of fetal aortic arch and pulmonary arterial branch anomalies. Key words: Ultrasonography, prenatal; Heart defects, congenital; Three-vessel view; Aortic arch; Pulmonary arterial branch

Objective To investigate the value of clinical follow-up in prenatal diagnosis of isolated double aortic arch (DAA). Methods The clinical follow-up materials were retrospectively reviewed in 17 fetuses. Of all the isolated DAA fetuses, the accuracy rate of prenatal diagnosis was confirmed by CT, MRI, autopsy or echocardiography, and pregnant outcomes were summarized. Results A total of 17 fetuses had a sonographic diagnosis of isolated DAA in our centers at a mean gestational age of 23-32(27±3)weeks, with mother mean age 19-44 (28±6) years old. One case of DAA type-A was misdiagnosed, 15 cases were delivered with 2 cases occurred respiratory distress or mild dysphagia, who received surgical treatment, and 13 cases clinical findings were unremarkable at the fellow-up of 24 months, the silent-rate of clinical symptoms was 86%. Termination of pregnancy happened in 2 cases, with 1 (6%) had additional ventricular septal defects, another (6%) had additional anomalies of congenital high airway obstruction syndrome. In 17 cases of fetuses with isolated DAA, there were 15(88%) cases with dominant right-sided arch, 1 case (6%) with dominant left arch, and 1 case (6%) with equal arches in size. Karyotyping prenatal testing was offered to 15 parents with normal results. Conclusions Prenatal ultrasound can accurately diagnose isolated DAA by multiple sections scan, expanding diagnostic ideas by combination with other medical imaging data to prevent apparent life-threatening event, or to guide for ex-utero intrapartum treatment. Isolated DAA clinical follow-up results in good outcome. Key words: Ultrasonography, prenatal; Aortic arch syndromes; Vascular ring