Clues and techniques of prenatal ultrasonographic diagnosis for fetal aortic arch anomalies

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

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 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

BackgroundThe purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and diagnostic accuracy.Methods140 children with abnormal aortic arch diagnosed by ultrasound in Children’s Hospital of Hebei Province from January 2014 to December 2019 were selected for retrospective analysis. All were confirmed by surgery or/and computerized tomography angiography. Series of views for aortic arch (the three-vessel and tracheal view, aortic arch short axis view, left aortic arch long axis view, aortic arch long axis continuous scan views) were performed in all cases on the basis of the routine views of echocardiography. The screening sensitivity and diagnostic coincidence rate of different echocardiographic views for aortic arch anomalies were analyzed.ResultsAmong the 140 infants, right aortic arch were 21 cases (6/21 were accompanied by mirror branch and 15/21 were with aberrant left subclavian artery). Left aortic arch with aberrant right subclavian artery were 2 cases, and double aortic arch with both arches open were 20 cases. Double aortic arch with left arch atresia were 2 cases, and atresia of the proximal aorta with aortic arch dysplasia was 1 case. Coarctation of the aorta were 67 cases, and interruption of aortic arch were 27 cases. All the patients were correctly diagnosed except that 2 infants with interruption of aortic arch were incorrectly diagnosed as coarctation of the aorta, and 1 infant with coarctation of the aorta was misdiagnosed as interruption of aortic arch by echocardiography. The screening sensitivities of four views and four-view combination for abnormal aortic arch were 99.3, 73.6, 87.1, 99.3, and 100%; the diagnostic coincidence rates were 85.7, 27.1,66.4, 95.0%, and 97.9% respectively. On the basis of traditional left aortic long axis view, other three views had their own advantages. The screening sensitivity and diagnostic coincidence rate of four-view combination were significantly improved.ConclusionsThe three-vessel trachea view is simple and feasible, which is suitable for screening abnormal aortic arch. The combination of four views conduces to improving screening sensitivity and diagnostic accuracy of aortic arch abnormalities.

To study diagnostic value of 64 multislice CT in typing of congenital aortic anomaly in neonates and infants. 120 pediatric patients (under one year of age) with congenital heart disease (CHD) underwent 64 contrast-enhanced MSCT before a corrective operations. The diagnostic sensitivity, specificity and accuracy of 64 MSCT were evaluated and also compared with those of echocardiography with Doppler. The patients were randomly assigned to two groups (72 and 48 persons) respectively according to tube tension of 80 and 100 kV. The differences of the image qualities were compared between them. 36 congenital aortic anomalies were found (36/120, 30%) by 64 MSCT, which were furtherly distinguished into 2 cases in double aortic arch, 2 cases in left-sided aortic arch with aberrant right subclavian artery, 22 cases in right aortic arch (6 cases in right-sided aortic arch with aberrant left subclavian artery, 12 cases in right aortic arch with mirror image branching), 10 cases in coarctation of aorta. Diagnostic sensitivity, specificity and accuracy of 64 MSCT were all 100%. By contrast, those of echocardiography with Doppler were 27.8%, 97.6%, 76.7%, respectively. The quality scores were 4.69 ± 0.52, 4.58 ± 0.58 at 80 kV and 100 kV, respectively. No significant statistical difference was found between them (t = 1.08, P = 0.28). MSCT allows a detailed assessment of the anatomy of congenital aortic anomaly, which can be used as an important supplementary method in diagnosing CHD and offer important information for operation. 80 kV should be selected in CHD patients less than one year old for CT examination to reduce radiation exposure.

Hybrid Endovascular Treatment of an Anomalous Right Subclavian Artery Dissection in a Patient With Marfan Syndrome

Repair of Kommerell's Diverticulum and Aberrant Subclavian Arteries: Classification of the Five Groups in Declining Frequency and Their Operative Approaches

To discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors. The clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case. In addition to 4 cases the remaining 38 patients were still combined with other cardiovascular malformations. Thirty-six cases of children underwent spiral CT airway remodeling, 23 children underwent fiber- bronchoscopy. In addition to 2 cases of airway abnormalities not seen, the rest of the children were present in varying degrees in different parts of tracheal stenosis or tracheomalacia. All patients underwent surgical correction of congenital vascular ring, concomitant heart deformity correction surgery according to the situation (7 cases of atrial septal defect repair, 3 of ventricular septal defect repair, 1 of mitral valvuloplasty, 1 of bi-Glenn, 1 of coarctation of the aorta correction with ventricular septal defect repair, 1 of trilogy of Fallot correction). One case suffered resection of tracheal stenosis and Slide tracheoplasty simultaneously, another case was implanted tracheal stenting postoperatively after pulmonary artery sling correction. Three patients died in hospital (7.1%), the cause of death were recurrent granuloma formation postoperatively. Remaining 39 patients were successfully discharged. The median time of overall survival of children with mechanical ventilation was 14 h (22 h) (M (Q(R))), median ICU residence time was 5 d (8 d), and the median in-hospital time was 19 d (9 d). Tracheal intubation time and postoperative ICU duration time in children with pulmonary artery sling children were much more than in children with double aortic arch (23 h (123 h) vs. 9 h(9 h), 7 d (13 d) vs. 4 d (2 d)), but the difference were not statistically significant. There was significant difference in the duration of hospitalization between the patient with the pulmonary artery sling and double aortic arch (23 d (9 d) vs. 16 d(6 d)) (χ(2) = 10.157, P = 0.006). The recent results of surgical treatment of congenital vascular ring is safe and effective. The extent and scope of tracheal stenosis and tracheomalacia is a critical influence prognosis.

Evaluate the prevalence of aortic arch anatomy and branching pattern variants and anomalies in 1359 patients by Multi-Detector Computed Tomography Angiography. Retrospective multi-centric study including exams performed for various clinical issues with the same acquisition and injection protocols on 64-slices scanners. Multi-Planar reformations and Volume Rendering Images were available in all cases. A total of 965 patients (71%) had normal aortic arch anatomy and branching pattern. Anatomical variations and anomalies were present in the remaining 394 patients (29%). The most common variation was the common origin of the brachiocephalic and the left common carotid arteries (302/1359 cases, about 22%). The most common anomaly were aberrant right subclavian artery (4/1359, about 0.5%) and right aortic arch (4/1359 cases, about 0.5%). Our multicentric series is, as far as we know, the largest reported to date for the definition of the prevalence of variation and anomalies of aortic arch anatomy and branching pattern. Knowledge of such findings may avoid both in the immediate and in the long term an injury to the patient, decreasing the rate of complications during surgery and interventional radiology procedures in the head and neck district.

We aimed to evaluate retrospectively associated anomalies and outcome in prenatal aortic arch anomalies (AAAs). We included ninety patients with aberrant right subclavian artery (ARSA), right aortic arch (RAA) with mirror image branching (RAA-mirror) or aberrant left subclavian artery (RAA-ALSA) and double aortic arch (DAA) between 2011 and 2020. In total, 19/90 (21.1%) had chromosomal anomalies, the highest rate being within the ARSA subgroup (17/46, 37%). All (13/13) of the RAA-mirror subgroup, 10/27 (37.0%) of RAA-ALSA, 13/46 (28.3%) of ARSA and 0/4 within the DAA subgroup had additional intracardiac anomaly. The rate of extracardiac anomalies was 30.7% in RAA-mirror, 28.3% in ARSA, 25.0% in DAA and 22.2% in the RAA-ALSA subgroup. A total of 42/90 (46.7%) had isolated AAAs: three (7.1%) with chromosomal anomalies, all trisomy 21 (3/26, 11.5%) within the ARSA subgroup. Out of 90, 19 (21.1%) were lost to follow-up (FU). Two (2.2%) intrauterine deaths occurred, and six (6.7%) with chromosomal anomalies terminated their pregnancy. In total, 63 (70.0%) were liveborn, 3/63 (4.8%) with severe comorbidity had compassionate care and 3/60 (5.0%) were lost to FU. The survival rate in the intention-to-treat cohort was 53/57 (93%). Forty-one (77.4%) presented with vascular ring/sling, two (4.9%) with RAA-ALSA developed symptoms and one (2.4%) needed an operation. We conclude that intervention due to vascular ring is rarely necessary. NIPT could be useful in isolated ARSA cases without higher a priori risk for trisomy 21 and after exclusion of other anomalies.

The purpose of this study was to evaluate the value of multi-detector computed tomography (MDCT) angiography for the diagnosis of congenital aortic arch anomalies and present the radiological images of congenital aortic arch anomalies in Chinese children. MDCT angiography and transthoracic echocardiography (TTE) were applied for the diagnosis of congenital aortic arch anomalies in 362 Chinese children between May 2006 and December 2011 (age ranges from 5 days to 12 years; mean age, 3.3 years). Surgery and/or catheter angiography (CA) were conducted in all patients to confirm the final diagnosis. In the 362 Chinese children with congenital heart anomalies, congenital aortic arch anomalies were definitely diagnosed in 198 children and 164 children ruled out by operation and/or (CA). Among the 198 children with anomalies, coarctation of aorta (CoA), interruption of aortic arch (IAA), right aortic arch, aberrant right subclavian artery and double aortic arch were diagnosed in 134, 32, 20, 10 and 2 children respectively, and there were 6 cases with uncommon congenital aortic arch anomalies: 2 had double aortic arch including 1 with five branches of the aortic arch, 2 had isolation of the right subclavian artery with two patent ductus arteriosus (PDA), 1 had an isolation of the common carotid artery with a PDA, and 1 had double PDA with a single ventricle and pulmonary artery atresia. Among the 32 children with IAA, 28 were of type A, and 4 were of type B. The diagnostic sensitivity, specificity and accuracy of MDCT angiography for congenital aortic arch anomalies were 100% (198/198), 98% (161/164) and 99% (359/362), respectively. The diagnostic sensitivity, specificity and accuracy of TTE were 92% (182/198), 81% (133/164) and 87% (315/362), respectively. In conclusion, MDCT angiography is a reliable, noninvasive imaging technique for the diagnosis of congenital aortic arch anomalies in children. Sometimes, even more information can be obtained from this technique than from conventional angiography.

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies. Foetal echocardiograms, clinic and genetic histories of 33 patients who had been diagnosed with aortic arch anomaly from 2007 to 2015 were reviewed. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. In a patient with isolated right aortic arch, 22q11 microdeletion had been revealed. Given this data, we strongly suggest foetal karyotype analysis when aortic arch anomalies are identified.Impact StatementWhat is already known on this subject: The data about the prenatal diagnosis of aortic arch anomalies are limited.What the results of this study add: In our study, 653 patients were examined by foetal echocardiography during the study period. Thirty three patients who had been diagnosed with aortic arch anomaly prenatally and confirmed after delivery were enrolled in the study. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. Trisomy 18 was detected in the patient with bilateral ductus arteriosus and Di George syndrome (22q11 microdeletion) was determined in two patients with right aortic arch. While in the first patient, there were no other intracardiac anomalies; in the second patient with 22q11 microdeletion, Fallot tetralogy accompanied the right aortic arch.What the implications are of these findings for clinical practice and/or further research: This results showed that aortic arch anomalies can be associated with genetic anomalies even when they are found without other congenital heart disease. Given these data, we strongly suggest foetal karyotype analysis and genetic testing when aortic arch anomalies are identified.

Objective To explore the value and availability of echocardiography in the diagnosis of interrupted aortic arch (IAA) with aberrant right subclavian artery (ARSCA) in children. Methods The echocardiographic images and clinical data of 6 patient s with IAA and ARSCA confirmed by operation were analyzed retrospectively. Results six cases of IAA (all were type B) was associated with ARSCA. Among them, 4 cases were misdiagnosed as IAA-C. In IAA-B associated with ARSCA right and left common carotid artery were originated from the aortic arch before interruption, and they appeared as a symmetrical ″Y″ shape with ascending aorta; left and right subclavian artery were originated from the aortic arch after interruption, and the blood vessel lines of ARSCA were variable but always extending to the upper right; the ratio of the diameter of pulmonary artery and aorta were increased obviously. Conclusions It is significant to carefully examine aberrant right subclavian artery, and it was help to the cardiac surgery. Key words: Echocardiography; Interrupted aortic arch; Right subclavian artery/aberration

Objective: To analyze the methods of prenatal sonographic diagnosis of all kinds of aortic arch anomalies and to improve prenatal sonographic detection rate of aortic arch anomalies. Methods: 73 fetuses with congenital aortic arch anomalies were studied. The results from prenatal ultrasound examination, postnatal examination were compared. Results: 73 cases were with congenital aortic arch anomalies, 33 cases with malposition of aortic arch, 9 cases with normal left aortic arch with aberrant left subclavian artery, 5 cases with interruption of aortic arch, 26 cases with coarctation of aortic arch. The accuracy rate of prenatal sonographic diagnosis was 87.7%. Conclusions: Aortic arch anomalies have typical sonographic image. If we grasp typical sonographic images, we could make accurate diagnosis.

Objective: To analyze the methods of prenatal sonographic diagnosis of all kinds of aortic arch anomalies and to improve prenatal sonographic detection rate of aortic arch anomalies. Methods: 73 fetuses with congenital aortic arch anomalies were studied. The results from prenatal ultrasound examination, postnatal examination were compared. Results: 73 cases were with congenital aortic arch anomalies, 33 cases with malposition of aortic arch, 9 cases with normal left aortic arch with aberrant left subclavian artery, 5 cases with interruption of aortic arch, 26 cases with coarctation of aortic arch. The accuracy rate of prenatal sonographic diagnosis was 87.7%. Conclusions: Aortic arch anomalies have typical sonographic image. If we grasp typical sonographic images, we could make accurate diagnosis.