Mirror-image Branching Research Articles

Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies as Aberrant Right Subclavian Artery, Right Aortic Arch and Double Aortic Arch

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.

Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography

Introduction: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery. The first branch of the mRAA is the left brachiocephalic artery, which appears to be the same as that of an incomplete DAA due to blood flow interruption. The present retrospective study aimed to evaluate the differences between DAA and mRAA by fetal echocardiography. Methods: This single retrospective cohort study included all patients diagnosed with complete DAA, incomplete DAA, or mRAA at our facility between 2010 and 2022. The patients were diagnosed with complete DAA, incomplete DAA, or mRAA after birth and remaining fetal echocardiograms. The patients were divided into the DAA (complete DAA: n = 4, incomplete DAA: n = 3) and mRAA (n = 4) groups. The following three outcomes were compared: (1) angle between the right aortic arch and first branch (RF angle), (2) ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta, and (3) maximum tracheal diameter on a three-vessel trachea view. Results: The incomplete DAA cases were difficult to diagnose via fetal echocardiography. On fetal echocardiography, the RF angle was significantly steeper in the DAA group than in the mRAA group (median 57° [36°–69°] vs. 75° [62°–94°]; p < 0.05). The DAA and RAA groups showed no significant differences in the ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta (median 0.57 [0.17–0.68] vs. 0.73 [0.56–1.0]) and maximum tracheal diameter (median 2.5 [1.4–3.3] vs. 3.2 [2.8–3.5] mm). The cut-off value for the presence of DAA was an RF angle <71°. Conclusion: The DAA group (complete and incomplete DAA) had a significantly steeper RF angle than the mRAA group. Therefore, RF angle measurement could improve the fetal diagnosis and postnatal prognosis of DAA.

Prenatally detected aortic arch anomalies and their consequences after birth

Aortic arch anomalies are frequently associated with cardiac or extracardiac malformations, chromosomal aberrations and postpartum esophagus/trachea compression. We aimed to establish the prevalence of associated cardiac and extracardiac malformations, the frequency of chromosomal aberrations in fetuses with the diagnosis of aortic arch anomalies and to assess the pregnancy and the postnatal outcome. Retrospective cohort study of all fetuses with aortic arch anomalies and genetic diagnosis in a tertiary referral obstetric and fetal cardiology centre between 2016 and 2020. Postpartum data were collected within 24 months after birth. In a cohort of 11.380 pregnant women, the prevalence of aortic arch anomalies was 0.25%. Among 28 cases of right aortic arch anomalies, in 27 fetuses prenatal genetic diagnosis was available. We diagnosed 4 fetuses with mirror-image branching (right sided V-sign) and 23 fetuses with U-sign (4 fetuses with complete double aortic arch). 18 cases (66%) were isolated. Associated anomalies were cardiac in 3 cases and extracardiac in 7 cases (33%). The most frequent cardiac anomaly was tetralogy of Fallot (2/27), the extracardiac anomalies were thymus hypoplasia, single umbilical artery and subclavian artery malformations. In 1 case (3.7%), fluorescent in situ hybridization diagnosed 22q11.2 microdeletion. 75% of fetuses with right sided V-sign were associated with conotruncal malformations. Pregnancy and postpartum outcome were known in 24 pregnancies. Postnatal diagnosis was different from prenatal in 2 cases, the concordance rate was 93%. Isolated cases resulted in live birth in 17/18 pregnancies (93%). The frequency of postpartum trachea/esophagus compression was 42,9% (9 cases) due to vascular ring, in 6 children (28,6%) operation was necessary. Fetal aortic arch anomalies are multidisciplinary diseases to be diagnosed by proper prenatal ultrasound examination. Associated fetal anomalies necessitate extended obstetric and cardiac sonography, invasive prenatal testing should be offered, and thorough postnatal long-term follow-up is recommended. Orv Hetil. 2023; 164(28): 1111-1120.

The prenatal diagnosis and prognosis of fetal right aortic arch and double aortic arch malformation: A single-center study.

This study aimed to characterize the pathological types, diagnosis, chromosomal abnormalities, and postnatal clinical manifestations of right and double aortic arch malformations in fetuses. In this retrospective study, all fetuses diagnosed with right or double aortic arch anomalies for whom conventional two-dimensional echocardiography combined with spatio-temporal image correlation was performed at our tertiary referral center between December 2012 and December 2021 were included. In total, 234 fetuses with aortic arch abnormalities were identified. Forty-one cases lost to follow-up. One hundred ninety-three cases were included in this study. One hundred eighty-seven cases with right aortic arch. Six cases with double aortic arch. Most cases of right aortic arch with aberrant left subclavian artery (77/101, 76.2%) were isolated lesions, whereas most of those with mirror-image branching (45/75, 60%) were associated with intracardiac or extracardiac anomalies. Chromosomal abnormalities were screened prenatally in 113 fetuses with right aortic arch, among whom three with aberrant left subclavian artery (3/63, 4.8%) and eight with mirror-image branching (8/50, 16%) had chromosome anomalies (p < 0.05). Furthermore, three cases had microdeletion 22q11.2 and these were significantly associated with intracardiac malformations. Most cases of isolated right aortic arch do not present with clinical symptoms except isolated left subclavian artery and isolated left brachiocephalic trunk. In addition, the risk of chromosomal abnormalities in patients with isolated right aortic arch is very low. We recommend that pregnant women should be informed of the risks and benefits of undergoing invasive prenatal chromosomal detection.

Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch.

There has been a rise in the prenatal detection of right sided aortic arch (RAA). When associated with a left-sided arterial duct (LD), this forms a vascular ring encircling the trachea. Infants may have symptoms or signs suggestive of tracheoesophageal compression but many are asymptomatic. The objective of this study was to investigate the relationship between symptoms and severity of tracheobronchial compression assessed by bronchoscopy. Retrospective review of all cases of prenatally diagnosed RAA-LD in the absence of associated congenital heart disease at Evelina London Children's Hospital and Kings College Hospital over a 4-year period between April 2015-2019. Clinical records, fetal echocardiograms, and free-breathing flexible bronchoscopy (FB) data were reviewed. One hundred and twelve cases of isolated RAA-LD were identified of whom 82 cases (73%) underwent FB. FB was performed median age of 11 months (range 1-36 months), no complications occurred. Aberrant left subclavian artery (ALSA) was present in 86% (96/112) and mirror image branching (MIB) in 13% (15/112). 34/112 (30%) reported symptoms during follow-up. 36/77 (47%) with ALSA who underwent FB showed moderate-severe compression mostly at distal tracheal and carinal level of whom 38% had parent-reported symptoms. Moderate-severe compression was seen in 3/5 (60%) with MIB mostly at mid tracheal level; 3 were symptomatic but only 2 had tracheal compression. In total 36% (18/50) of investigated asymptomatic patients showed moderate-severe compression. Respiratory symptoms were poorly predictive of moderate-severe tracheal compression (positive predictive value 66%, negative predictive value 64%). The absence of symptoms did not exclude significant tracheal compression. The anatomical effect of the vascular ring is under appreciated when symptoms alone are used as a marker of tracheal compression.

Utility of fetal cardiovascular magnetic resonance imaging in assessing the fetuses with complete vascular ring.

This study aimed to report our experience in qualitative and quantitative evaluation of fetal complete vascular ring (CVR) using fetal cardiovascular magnetic resonance imaging (MRI) to improve prenatal diagnosis and make early postnatal management possible. A retrospective case-control study was performed on cases of CVR diagnosed using fetal cardiovascular MRI, and confirmed by postnatal imaging diagnosis. Associated abnormalities were recorded. The diameters of aortic arch isthmus (AoI) and ductus arteriosus (DA), and tracheal diameters in fetuses with tracheal compression were measured and compared with those of the control group. All fetal CVR cases in this study included right aortic arch (RAA) with aberrant left subclavian artery (ALSA) and left DA (n = 93), double aortic arch (DAA) (n = 29), RAA with mirror-image branching and retroesophageal left ductus arteriosus (RLDA) (n = 8). Compared with the control group, the diameters of AoI in fetuses with DAA were decreased (p < 0.001), and the diameters of DA in fetuses with RAA with ALSA and left DA were increased (p < 0.001). The diameters of AoI and DA were positively correlated with gestational age (GA) in the normal control group (both p < 0.001); The diameters of AoI and DA were also positively correlated with GA in RAA with ALSA and left DA subgroup (both p < 0.001) and RAA with mirror-image branching and RLDA subgroup (AoI: p = 0.003; DA: p = 0.002); The diameters of DA were positively associated with GA in DAA subgroup (p < 0.001), however, there was no linear tendency between the diameters of AoI and GA in the DAA subgroup (p = 0.074). There were CVR fetuses with associated intracardiac malformation (n = 13), especially ventricular septal defect rather than complex heart disease, and extracardiac malformation (n = 14). Sixteen fetuses were shown the airway compression whose tracheal diameters were smaller than the normal (p < 0.001). The altered diameters of AoI and DA can be detected and measured in CVR fetuses using fetal cardiovascular MRI. Fetal CVR can occur alone or with intracardiac and extracardiac malformation. Fetal CVR can be associated with prenatal airway compression.

The influence of prenatal screening on the contemporary operative outcomes and functional alleviation after vascular ring surgery in children

Objective: The impact of prenatal screening on contemporary operative outcomes and functional alleviation during pediatric Vascular Ring Surgery (VRS) is analysed in this research. Methods:All patients who had surgical treatment of an isolated VR at the Texas Children’s Hospitalbetween 1996 and 2018were included in the study. Patients who simultaneously underwent intracardiac repair were disqualified. Other congenital cardiac conditions, clinical symptomatology, contemporary surgical intervention, perioperative outcome, reoperation rates, functional alleviation, and complications were all examined in the data analysis.To examine the data for this investigation, we employed SPSS 13.0.Results: 148 participants were chosen for analysis. Of the 148, 72(48.6%)have a Double Aortic Arch (DAA), 69(47.2%) have a ligamentum and a normal left subclavian artery or Right Aortic Arch (RAA) with a left-sided ductus, 5 (2.7%)have an RAA, Left Descending Aorta (LDA) and 2(1.3%) had RAA, Left Ligamentum Arteriosum (LLA), mirror-image branching. Right aortic arch vascular ring(RAAVR) aregeneral than Double Aortic Arch Vascular Ring (DAAVR): 31 patients had the earlier, with a frequency of 5.7 per 10,000 live births, whereas 6 patients had the latter, with an incidence of 1.1 per 10,000 live births (p = 0.0001).Conclusion: VRs are a prevalent form of congenital cardiovascular abnormality, especially when they affect the right aortic arch. Additionally, prenatal screening reveals a significant percentage of VRs.

Analysis of prognostic risk factors and development of a predictive model for fetuses with right aortic arch with mirror-image branching: a prognostic evaluation method based on ultrasound parameter features.

Prenatal ultrasound plays a crucial role in the diagnosis and classification of right aortic arch (AO) with mirror-image branching (RAA-MB). The recent research in this area has primarily focused on qualitative diagnosis, neglecting the quantitative analysis of ultrasound factors that impact RAA-MB outcomes. This study used echocardiography to measure prenatal ultrasound parameters for vascular ring and trachea in fetuses with RAA-MB, employing a nomogram model to evaluate factors influencing their prognosis, thereby providing a comprehensive characterization of potential outcomes. A retrospective case-control study was conducted from March 2019 to March 2023. A systematic gathering of prenatal echocardiograms and clinical data was completed for a cohort comprising 92 cases of fetal RAA-MB at the Ultrasound Medicine Center of Gansu Provincial Maternity and Child Care Hospital. Participant recruitment was executed through random selection from among those receiving outpatient medical care. Within the cohort, 42 cases were categorized as fetuses with isolated RAA-MB, while the remaining 50 cases were characterized as fetuses with RAA-MB and associated anomalies. Measurements were taken of the angle between the right AO and the ductus arteriosus (DA) (AO-DA), the distance between the AO and DA, the diameter of AO and DA, and the distance growth rate (DGR) of the AO-DA distance. Additionally, measurements were taken of the tracheal anterior-posterior diameter, tracheal left-right diameter, and tracheal circumference in the three-vessel tracheal view. In the AO view, measurements were taken of the tracheal cross-sectional area (TA) and the vessel ring cross-sectional area (VRA). The relationship between these parameters and the prognosis of fetuses with RAA-MB was assessed using logistic regression analysis. A receiver operating characteristic (ROC) curve was constructed to evaluate the diagnostic performance of the predictive model based on these factors. The multivariate logistic regression analysis revealed that the independent predictive factors for the prognosis of fetuses with RAA-MB were the AO-DA distance [odds ratio (OR) =0.012], TA (OR =0.401), and VRA (OR =1.103) (all P values <0.001). The area under the ROC curve was 0.891 [95% confidence interval (CI): 0.789-0.914; P<0.001], indicating a high accuracy of the model's predictions. The AO-DA distance, TA, and VRA are factors that influence the prognosis of fetuses with RAA-MB. The column chart model constructed based on these parameters can effectively provide a reference for predicting the risk of adverse outcomes in fetuses with RAA-MB.

Detection of Coronary Artery and Aortic Arch Anomalies in Patients with Tetralogy of Fallot Using CT Angiography.

Background: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD). Furthermore, the prevalence of anomalous origin of a coronary artery is higher in patients with TOF than in the general population (6% vs. ≤1%). Preoperative assessment of cardiovascular anatomy using computed tomography (CT) angiography enables the adaptation of the surgical approach to avoid potentially overlooked anomalies. Our purpose was to determine the prevalence of coronary artery and aortic arch anomalies in a cohort of TOF patients. Methods: In this retrospective analysis, data were collected from CT reports (2015–2021) of 105 TOF patients. All images were acquired using a 64-slice multi-detector CT (MDCT) scanner. Results: The median age of the patients was 38.7 months, with a male-to-female ratio of 1.39. The overall prevalence of coronary artery anomalies (CAAs) was 7.61% (8 of 105 cases). The anomalous origin and course of coronary arteries across the right ventricular outflow tract (RVOT; prepulmonic course) were defined in 5.71% of cases (six patients). In four of these, the left anterior descending artery (LAD) originated from the right coronary artery (RCA), while in two cases, the RCA arose from the LAD. In the remaining two patients, the coronary arteries followed an interarterial course. The most frequent anomalous aortic arch pattern in the overall TOF population was the right aortic arch (RAA) with mirror image branching, seen in 20% of patients (21 cases). The most frequent anomaly of the supra-aortic trunks was bovine configuration, found in 17.14% (18 cases). Conclusions: The prevalence of CAAs and aortic arch anomalies detected by CT angiography was in line with the data reported in anatomical specimens. Therefore, this technique represents a powerful tool for the evaluation of congenital cardiovascular anomalies.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with isometric atrial appendages on multidetector CT.

The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial appendages as detected on multidetector computed tomography (CT) angiography MATERIALS AND METHODS: The CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 were retrospectively evaluated to identify patients with left atrioventricular valve atresia and patent aortic root with isometric atrial chambers. The morphology of the atrial appendage and extent of pectinate muscles was used to identify the type of isomeric atrial chambers. A comprehensive assessment of discordant arrangement of organs and the variations in venoatrial connections was done. Left atrioventricular valve atresia in the presence of patent aortic root and isomeric atrial appendages was identified in eight patients (four males). The mean age at diagnosis was 7 years. Right isomerism of the atrial appendages was seen in five patients while three patients had left isomerism. Disharmonious patterns of abdominal visceral arrangement were seen in two patients. Atrial and ventricular septal defects were seen in all patients. The most common pattern of ventricular arterial connection was DORV (double outlet right ventricle) (n = 4). While fivepatients had normally related great arteries, three had aorta anterior to the pulmonary trunk. Right ventricular outflow obstruction was seen in seven patients. Right aortic arch with mirror image branching was seen in four patients. Coronary artery anomalies were seen in four patients. The presence of left atrioventricular valve atresia and patent aortic root with isomeric atrial appendages is a rare congenital anomaly frequently associated with anomalous systemic and pulmonary drainage, conotruncal anomalies, and right ventricular outflow obstruction. Hence, comprehensive CT-based evaluation is indispensable in providing detailed anatomy and aids in presurgical planning.

2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome.

Congenital cystic adenomatoid malformation associated with right aortic arch with mirror image branching and absence of left pulmonary artery in an adult

BackgroundCongenital cystic adenomatoid malformation is an uncommon developmental anomaly that is diagnosed prenatally or during early childhood and is rare to present in adulthood. Type 2 congenital cystic adenomatoid malformation is often associated with other anomalies such as congenital cardiac anomalies. We present a case of type 2 congenital cystic adenomatoid malformation in an adult male associated with absence of left pulmonary artery and right aortic arch with mirror image branching. To our knowledge, this is the first case report with the combination of all three anomalies.Case presentationA 33-year-old male presented to the Department of Pulmonary Medicine with high-grade fever, chills and rigor, breathlessness, and productive cough for a duration of one week. He had multiple similar self-limiting episodes in the past which did not require hospitalization. The plain radiograph showed right aortic arch and a small left hilum. CT thorax showed multiple cystic lesions of size 0.5–2.0 cm in the apico-posterior segment of the left upper lobe; anterior, posterior, and lateral segments of the left lower lobe; and the apical segment of the right upper lobe with a maximal cyst wall thickness of 2 mm. Volume of left lung was reduced with trans-thoracic herniation of the medial segment of the right middle lobe; bronchiectatic changes were seen in the apical, anterior, and posterior segments of the right upper lobe; and fibrotic strands were seen in the apical segment of the right lower lobe and the apico-posterior segment of the right upper lobe. Mediastinal window showed right-sided aortic arch with mirror image branching. The pulmonary trunk was seen to continue as the right pulmonary artery with absent left pulmonary artery.ConclusionsThough congenital cystic adenomatoid malformation is a disease of infancy and childhood, it should also be considered in adults presenting with recurrent lower respiratory tract infections and/or pneumothorax. Radiologists must be aware of the types of congenital cystic adenomatous malformation (CCAM) and their associations, as type 2 CCAM has a high association with congenital cardiac disease. Proximal interruption of pulmonary artery should be considered if the pulmonary trunk continues as either right or left pulmonary artery.

Vascular ring surgery – can we do better?

Abstract Background Vascular anomalies of the Aortic arch can cause respiratory symptoms in children due to tracheal compression. Treatment consists of division of the vascular rings, nevertheless data regarding mid- term results is scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods Between2007–2014, 85 children underwent vascular ring surgery. 51 had Double Aortic Arch (DAA, 60%), 31 Right Arch with Aberrant Subclavian Artery (RAA &amp; ALSA, 36.5%) and 3 had RAA, mirror image branching &amp; left ductus arteriosus (3.5%). Mean age and weight at operation were 12.4±13months and 8.6±4.1 kg respectively. Mid-term follow-up included clinical follow up by a pulmonologist (38 patients, 44.7%) and a telephone questionnaire (71 patients, 83%) performed 57±25.7 months after surgery. Results In most patients, symptomatic relief occurred in less than 6 months (table 1). Mid –term follow up revealed that although most parents described a significant improvement in their child's respiratory symptoms (95%), a significant number of patients described some residual respiratory symptoms (table 2). We did not find any significant association between age at surgery (under 6 month), or vascular ring anatomy (DAA vs. RAA&amp;ASA) and the presence of residual symptoms at follow up Conclusions Surgical division of vascular rings results in a significant clinical improvement within one year, nevertheless many patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms in mid- term follow up. Further evaluation whether a more aggressive surgical approach is warranted in order to decrease the incidence long-term symptoms. Funding Acknowledgement Type of funding source: None

Innominate Artery Cannulation during Redo Sternotomy for Patients with a Right Aortic Arch with Mirror Image Branching

Innominate Artery Cannulation during Redo Sternotomy for Patients with a Right Aortic Arch with Mirror Image Branching

VP14.10: Prenatal diagnosis of vascular rings and its clinical significance in postnatal life

Prenatal detection of vascular rings has increased over the past two decades. These lesions may be asymptomatic or cause respiratory and/or gastrointestinal (GI) symptoms from tracheal or esophageal compression. Symptomatology dictates surgical correction. We present 12 cases of prenatally diagnosed vascular rings with their postnatal course. We reviewed all fetal aortic arch anomalies diagnosed from 2016–2019 at our institution. We collected prenatal and postnatal diagnosis, cardiac and non-cardiac anomalies, genetics, postnatal clinical course, symptoms and surgical outcomes. 32 fetal echocardiograms revealed arch sidedness and branching anomalies; 25 isolated right aortic arch (RAA) with mirror image branching, 6 isolated aberrant right subclavian artery, 1 isolated aberrant left subclavian, 11 RAA with aberrant left subclavian and 1 double aortic arch. Out of the 12 vascular ring patients, no genetic syndromes were identified prenatally or postnatally despite the risk for 22q11 deletion with RAA. Postnatal imaging confirmed the prenatal diagnosis in all 12 patients. Associated congenital heart disease included ventricular septal defect in one patient. Associated extracardiac disease included pelvic kidney and tracheobronchomalacia. 7 (58%) remained asymptomatic and 5 (42%) reported respiratory and/or GI symptoms. Average age of symptom onset was 67 days. 3 (25%) patients required surgery for respiratory and GI symptoms at mean age of 7.3 months. Mean age of clinical follow-up was 6.4 months. We found that the majority of prenatally diagnosed vascular rings remain asymptomatic and do not have concomitant intracardiac or extracardiac disease or genetic syndromes. Presently, there are no prenatal markers to predict postnatal surgery. Further research may identify prenatal markers of respiratory or gastrointestinal compromise.

Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

Key Features on the 3-Vessel View and 3-Vessel Tracheal View of Isolated Right Aortic Arch Anomalies.

Right aortic arch anomalies are a spectrum of malformations that include right aortic arch with mirror image branching, right aortic arch with an aberrant left subclavian artery, and double aortic arch. Although these are rare anomalies, they are of importance as they form vascular rings, which can cause symptoms in the newborn period. These anomalies are not detected with routine cardiac views, and it is only with the 3-vessel, and the 3-vessel tracheal view that they can be identified and characterized. We describe specific sonographic findings of these anomalies on the 3-vessel and the 3-vessel tracheal view.

Right Aortic Arch with Mirror Image Branching and Situs Solitus: A case report on anatomic variation

During a routine cadaver dissection performed by first year medical students at Oakland University William Beaumont School of Medicine, a 60‐year‐old female cadaver was found to have right aortic arch with mirror image branching (RAMI). The descending aorta was observed to pass to the right of the midline and subsequently to cross left, anterior to the esophagus, returning to its typical orientation at the aortic hiatus. This defect involves a mirror image branching in which a left brachiocephalic trunk, right common carotid artery, and right subclavian artery emerging directly from the aortic arch proximal to distal. The right recurrent laryngeal nerve passes under the aortic arch while the left recurrent laryngeal nerve was located more superiorly, passing underneath the left subclavian artery. The heart presented in its typical orientation with the apex located to the left of midline with no noted abnormalities of the pulmonary trunk or vascular ring surrounding the trachea. The aortic arch was studied in 24 formalin fixed cadavers (13 female, 11 male, average age 80) which had been dissected by first year medical students. Only 1 of the 24 specimens (4.17%) had a congenital aortic arch and branching defect while the remaining 23 did not display any aortic arch or branching abnormalities. After review of the published clinical literature, this variation appears consistent with RAMI and situs solitus which has been found in &lt;0.1% of the general population. Studies have shown that 75–85% of cases with this defect are associated with cyanotic congenital heart disease, which makes early detection and follow‐up with pediatric cardiology essential. Other considerations for cardiac patients presenting with RAMI and situs solitus include accurate procedural imaging to provide safe and effective interventional care.

Anatomical Variations of the Left Subclavian Artery and Their Significance in Clinical Practice.

We study variations of the aortic arch (AA) and its first branches, the brachiocephalic, common carotid, and subclavian arteries. Specifically, we describe anatomical variations of the left subclavian artery (LSA) and their significance in clinical practice. Such variations are commonly associated with congenital anomalies of the right-sided AA (RAA) and/or great vessels. A systematic search of the PubMed online database for studies of LSA variations and RAA, published between 2000 and 2020, produced a total of 73 articles for our study (n = 258 cases). Three of the most common variants were aberrant LSA (32.9%), RAA with mirror-image branching (49.6%), and isolated LSA (17.4%). Although RAA and LSA variations are rare, they may give rise to symptoms during the first stages of life or adulthood and may require surgical repair. Patients can remain asymptomatic, but incidental findings on imaging studies may lead to surgical interventions in areas of the neck or thorax. In such cases, planning the surgical procedure can be challenging, and possible implications must be considered.

Right Aortic Arch: 10-Years Experience of Surgical Treatment

Introduction - The right aortic arch is rare congenital anomaly and is present in 0.05% to 0.1% of radiology series and in 0.04%>0.1% of autopsy series. Clinical or surgical studies in the adult have been limited to isolated cases or small series and have essentially focused on the diagnosis and approach to these lesions. The purpose of this study is to report our 10 years’ experience in surgical treatment of right aortic arch in patients with vascular ring, aortic coarcrtaion and thoracic aortic aneurysm. Methods - The clinical records of 16 patients who underwent operation for right aortic arch and its association with other congenital or acquired aortic disease between 2006 and 2017 were reviewed. The 7 male and 9 female patients ranged in age from 8 to 75 years (mean 30,2 ±20,3 years). Right aortic arch with mirror-image branching was diagnosed in 5 patients (31%); right aortic arch with aberrant left subclavian artery - in 11 cases (69%). Four patients (25%) had isolated vascular rings and symptoms attributable to tracheal or esophageal compression. Six patients (38%) had symptoms and signs of an associated anomaly, coarctation of the aorta, and in 5 cases (31%) thoracic aortic aneurysm was revealed (in 1 cases type B aortic dissection). One patient (6%) had signs of left subclavian artery aneurysm. A right thoracotomy was used when right aortic arch was associated with right sided descending thoracic aorta: in 5 patients with aortic coarctation and in 2 patients with aortic aneurysm. In 4 patients access to the vascular ring was obtained through a left thoracotomy, in all cases thoracic segment of left subclavian artery was translocated into left common carotid artery and ligamentum arteriosum was divided. The management of Kommerell’s aneurysms (in 3 patients) with and to end anastomosis were carried out. In 1 case of type B aortic dissection and in 1 case of postoperative false aneurysm of aortic isthmus aortic graft interposition was necessary. In aneurysm associated cases we advocate the use of distal circulatory support to reduce the incidence of paraplegia. A left subclavian-to-carotid transposition completed before the thoracic approach in 3 patients allowed to avoid the complexity of the intrathoracic procedure and to prevent spinal cord ischemia and subclavian steal syndrome. Results - There was no in-hospital mortality. The mean postoperative hospital stay was 10 days. No operations were necessary for recurrent or persistent compression of the trachea and esophagus. Two postoperative complications occurred: one patient had residual respiratory symptoms and one patient had asymptomatic thrombosis of translocated left subclavian artery without subclavian steal syndrome. At mean long term follow up time 3,75 years in 14 patients ( range 12 month to 10 years) no patient died and all patients were free of symptoms. Conclusion - Careful preoperative imaging and consideration of the individual anatomy in surgical planning are essential to achieve a successful outcome. Aortic coarcrtation or/and aneurysm associated with right sided aortic arch may be repaired through a right thoracotomy and distal circulatory support. A left subclavian-to-carotid transposition before thoracic repair allows decrease the risk of ischemic cerebral and spinal cord complications.