Value of combination of three-vessel views in ultrasound diagnosis of fetal aortic arch and pulmonary arterial branch anomalies

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

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 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

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.

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

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and review other malformations of the aortic arch, including interrupted aortic arch (IAA), hypoplastic aortic arch, and coarctation of aorta. I have also described few anomalies of pulmonary artery (PA) origin and bifurcation like PA sling which has clinical presentation similar to vascular ring and anomalous origin of PA from aorta which present as large aorto-pulmonary communication with congestive heart failure. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression.

Strategies for Accurate Diagnosis of Fetal Aortic Arch Anomalies: Benefits of Three-Dimensional Sonography With Spatiotemporal Image Correlation and a Novel Algorithm for Volume Analysis

Objective To investigate the ultrasonic imaging features of and differential diagnosis methods for the right aortic arch, to analyze the causes of missed diagnosis and misdiagnosis, and to further improve the accuracy of prenatal ultrasound diagnosis. Methods Cardiac screening examination of the fetus was carried out with views of gray-scale and color Doppler or power Doppler imaging (PDI). When the three-vessel tracheal (3VT) view found that the aortic arch was located in the right of the trachea, the coronary views of the trachea and its branches were obtained to further clarify the relationship among the aortic arch, arterial ductus, and trachea. The sonographic features of 31 cases of fetal right aortic arch were summarized, and the reasons for misdiagnosis were analyzed. Results Of the 31 cases of fetal right aortic arch diagnosed by prenatal ultrasound, 20 were alive, 15 were induced, and 6 were lost. As for the results of echocardiography for postpartum children, operation or autopsy were obtained in 31 cases, of which 6 had misdiagnosis or missed diagnosis by prenatal ultrasonography. The coronal views of the trachea and its branches were not obtained in two cases of fetal left aortic arch, and they were misdiagnosed as right aortic arch because the transversely sectioned bronchus were mistaken as the transversely sectioned trachea in the 3VT view. In two cases of right aortic arch with aberrant left subclavian artery, the left common carotid artery was misdiagnosed as a stunted left arch, which resulted in a misdiagnosis as a double aortic arch of the advantage type. In two cases of right aortic arch combined with cardiac defects, right aortic arch was missed in prenatal ultrasound diagnosis. Of 20 children who were followed, 13 were diagnosed with isolated right aortic arch, 2 with combined persistent left superior cavity, 1 with combined 2-3 lumbar vertebral body fusion, and 2 with combined right aortic arch inner or outer non-complex structure abnormality. Follow-up was conducted to the maximum age up to 3 years and 6 months, and 18 of these cases were in good condition and showed no symptoms of respiratory tract compression. One case suffered from complicated intracardiac malformation combined with IDD type congenitally corrected transposition of the great arteries. The child is currently in good condition 11 months after the delivery. One case suffered from combined esophageal atresia and died 14 days after delivery. Chromosome karyotype determination was performed in 6 cases, of whom 5 were normal and 1 was confirmed to have 18-trisomy with severe intracardiac multiple malformation. Conclusions The 3VT view is the most important view for diagnosis of right aortic arch of fetus. The accuracy of prenatal ultrasound diagnosis can be improved through the combination of the 3VT view and the coronal view of the trachea and its branches. Simple right aortic arch offers a good prognosis, and the prognosis of right aortic arch with severe ectopic and extracardial malformations depends on the severity of the combined malformation. Key words: Fetal right aortic arch; Ultrasonography, prenatal; Pregnancy outcome

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

To evaluate the value of MDCT on diagnosis of congenital vascular rings on children. Retrospective analysis on 43 cases of congenital vascular rings, which underwent MDCT during Oct 2008 to Dec 2014 in Beijing Anzhen hospital affiliated to capital medical university. 21 males, 22 females; age from 29 days to 8 years, mean age 1.46 years, 33 cases are not beyond 1 year. All the results were compared with that of the echocardiogram or record of the surgery. The CT data were read and reconstructed with multiplanar reconstruction (MPR), maximum intensity projection (MPR), minimum intensity projection (MinIP), volume rendering (VR). The image quality was evaluated and the diagnostic value and the standard diagnostic program were discussed. Of 43 cases of vascular rings:there were 6 cases of pulmonary artery sling (13.95%), 9 cases of right aortic arch /aberrant left subclavian artery(20.93%), 18 cases of left aortic arch/aberrant right subclavian artery (41.86%), 10 cases of double aortic arch (23.26%). Forty cases (93.02%) were combined with other cardiovascular or pulmonary malformations. Every malformation was revealed clearly and proved by echocardiogram. Of 3 cases (6.98%) without any other malformation, 2 cases were combined tracheal stenosis. A pulmonary artery sling was proved by surgery; the other 2 cases were double aortic arch. All the images of 43 cases could be reconstructed well. MPR and VR showed the origin, shape, and whole course of vascular rings directly; MinIP and VR could display the shape, width and development of trachea, revealed the relationship between vascular rings, trachea and esophagus. It was important to show and measure the component vascular of the ring. Attention should be paid to the whole course of trachea and esophagus, especially those segments which were close to the ring vascular. The tracheal stenosis as well as intra-cardio anatomy malformations should be measured on MPR images if existed. According to the segmental analysis method, comes the overall final diagnosis. A standard diagnostic program on vascular ring was proposed. MDCT axis images with various 3D post processing methods could reveal the compose of vascular rings and the relationship between vascular rings, trachea and esophagus.

Objective To explore the application values of prenatal ultrasound, vascular cast in the diagnosis of fetal aortic arch and its branches anomalies and to analyze the genetic characteristics by gene detection. Methods Twenty-two cases of the vascular cast specimens of the fetal aortic arch and its branches anomalies were analyzed and studied by comparing with their prenatal ultrasonography. Then the characteristics of each type of fetal aortic arch and its branches anomalies, the missed diagnosis and misdiagnosis were summarized and the results of their gene detection were also analyzed. Results The 22 cases of fetal aortic arch and its branches anomalies were as follows: 2 cases of double aortic arch showed the ascending aorta was divided into two branches after converging as the descending aorta. Three cases were left aortic arch with aberrant right subclavian artery. Twelve cases were right aortic arch: 8 cases were right aortic arch with mirror-image branching, 3 cases were right aortic arch with aberrant left subclavian artery, 1 case was right aortic arch with isolated left subclavian artery. Of the 8 right aortic arch with mirror-image branching, 3 cases of left arterial duct showed the vertical walking between the fusion site of the left innominate artery and the pulmonary artery. Right aortic arch with aberrant left subclavian artery with arterial duct showed U shaped vascular ring. Five cases were other types, including 2 cases of the coarctation of aortic arch, 1 case of interrupted aortic arch, 1 case of pulmonary artery sling, and 1 case of abnormal origin of right pulmonary artery. The ultrasonic missed diagnosis were the 6 deformities: 3 cases of arterial duct and 3 cases of aberrant subclavian artery. The ultrasonic misdiagnosis were the 5 deformities: 2 cases of arterial duct location, 1 case of aberrant subclavian artery, 1 case of isolated left subclavian artery, and 1 case of the coarctation of aortic arch. Genetic test results: In the 18 cases of the genetic detection, 2 cases were positive, 1 case was 22q11.2 microdeletion syndrome and 1 case was carrying KMT2D gene variant. Conclusions There are various kinds of fetal aortic arch and its branches anomalies, which are often associated with intracardiac malformations and venous branches variation. And prenatal ultrasound is of great value in diagnosing them.Vascular cast can visually display their characteristic changes, which is helpful to improve the differential diagnosis of the different aortic arch and its branches anomalies. The detailed genetic detection can improve the further understanding of its etiology. Key words: Ultrasonography, prenatal; Fetus; Aortic arch anomalies; Vascular cast; Gene detection

Aberrant right subclavian artery (ARSA) is associated with chromosomal abnormalities, mainly Down syndrome, and congenital heart disease1-5. ARSA is caused by abnormal regression of the primitive right aortic arch between the right subclavian artery and the right common carotid artery. As a result, the aortic arch branches into four arteries instead of three, and the ARSA originates distal to the left subclavian artery at the level of the aortic isthmus. ARSA follows an oblique course behind the trachea and esophagus to reach the right arm. In 2005, Chaoui et al.2 described the methodology for assessment of fetal ARSA in the transverse three vessels and trachea view, in which the anomalous origin of ARSA, close to the ductus arteriosus, and its retrotracheal course can be visualized (Figures 1a and c). This group subsequently showed how to visualize fetal ARSA in a longitudinal view6, in which the artery arises as a fourth and distal vessel from the aortic arch; however, in this view the retrotracheal course is not identifiable. As Quarello and Carvalho7 remarked, although several anatomical variations can manifest as an aortic arch with four vessels in the longitudinal view, in cases in which ARSA originates anteriorly from the aortic arch, visualization of four supra-aortic vessels is not possible. (a,b) Color Doppler ultrasound images of fetal aberrant right subclavian artery (ARSA) at 20 weeks' gestation. (a) Transverse view showing ARSA in the three vessels and trachea plane with its origin at the level of the aortic isthmus and with a retrotracheal course (arrows). (b) Coronal view showing ARSA arising from the aorta with an oblique course towards the right shoulder. (c) Schematic representation of the transverse (A) and coronal (B) planes in a fetus at the level of the ARSA. Ao, aorta; DA, ductus arteriosus; DAo, descending aorta; P, pulmonary artery; S, spine; T, trachea. We illustrate how this vessel can be visualized in the coronal plane. In order to assess ARSA, we obtain a coronal view of the fetal thorax, posterior to the trachea and anterior to the spine, until we are able to see the thoracic descending aorta. Highly sensitive color Doppler with a low velocity range (10–15 cm/s) shows ARSA as a vessel arising from the descending aorta at the level of the aortic isthmus and following an S-shaped course towards the right clavicle and shoulder (Figures 1b and c). This view has the advantage of providing visualization of the origin and course of the anomalous artery in the same plane. This view can also facilitate evaluation in cases in which the origin of ARSA is not in its most common position, such as ARSA originating anteriorly from the aortic arch or very distal to the left subclavian artery. It is important not to confuse ARSA with the azygos vein and its anastomosis with the superior vena cava. In the coronal view, the azygos vein courses parallel to the right side of the aorta, while ARSA arises from the aorta and follows an oblique course to reach the right arm. Pulsed Doppler interrogation is recommended to distinguish ARSA from the azygos vein in this region. In summary, fetal ARSA can be viewed in the coronal plane using ultrasonography. This approach shows both the anomalous origin of the artery and its S-shaped course to the right shoulder. Although the detection of ARSA is possible in a coronal view, it should be confirmed in the three vessels and trachea view. This work was supported by Fondo de Investigaciones Sanitarias (FIS# PI081712 and BA#09/90011). The authors are grateful to Mr David De León for assistance with graphics and to Mr Thomas O'Boyle for editorial assistance. J. De León-Luis*, C. Bravo*, F. Gámez*, L. Ortiz-Quintana*, * Fetal Medicine Unit, Department of Obstetrics and Gynecology, Hospital General Universitario Gregorio Marañón, Calle O'Donnell, 48. 28009 Madrid, Spain

Morphologic features of the aortic arch and its branches in the adult Chinese population

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. ©RSNA, 2016.

Background: The main aim of this study is to increase the recognizability of aortic arch anomalies during fetal echocardiographic examination. To evaluate intervessel distances in the three-vessel view in fetuses with isolated right or double aortic arch (DAA) anomalies during fetal echocardiography. Methods: This retrospective study included six fetuses examined between 19+0 and 21+0 weeks of gestation, with isolated right or DAA anomalies confirmed postnatally. Five fetuses had an isolated right aortic arch (RAA) anomaly, while one had an isolated DAA anomaly. Diameters of the main pulmonary artery (PA) and ascending aorta (Ao) were measured from the three-vessel view. Additionally, intervessel distances between the main PA–Ao, the Ao and superior vena cava (Ao–SVC), and the main PA–SVC were assessed. The standard deviation (SD) values were assessed using nomograms appropriate for gestational age. Results: The diameters of the PA and Ao were not significantly different from those in the normal population in any of the fetuses. In five of the six fetuses, the PA–Ao distance exceeded +3 SD, indicating an increase compared with fetuses without congenital heart defects. Additionally, the PA–SVC distance exceeded +3 SD in four fetuses, indicating a marked increase compared to fetuses without congenital heart anomalies. In contrast, the Ao–SVC distance was below –3 SD in only two fetuses, indicating a decrease, while no significant deviation was observed in the remaining four cases. Conclusions: In fetal echocardiography, isolated right or DAA anomalies can lead to altered intervessel distances on the three-vessel view. Notably, increases in the PA–Ao and PA–SVC distances were observed.