The "full rectangle" sign: a novel method for ultrasonographic diagnosis of fetal aberrant right subclavian artery.

OBJECTIVE: To evaluate the feasibility and accuracy of a novel ultrasonographic screening method for an aberrant right subclavian artery (ARSA) using the novel "full rectangle" method. METHODS: This prospective study was conducted at a tertiary care center, September 2022 to February 2023. The study included unselected pregnant women at 14-38 weeks of gestation referred for routine or targeted anomaly scans. All participants underwent scanning by two experienced sonographers to ascertain the presence or absence of aberrant right subclavian artery (ARSA) using both conventional and novel "full rectangle sign" methods for validation purposes. This is a novel screening method for ARSA that demonstrates the retro-tracheal course at the level of the supra-aortic vessels, forming what we term the "full rectangle sign". RESULTS: A cohort of 138 patients was enrolled. The "full rectangle" sign was discerned in 6 fetuses with ARSA (4.3%), while the typical three-sided figure of the right subclavian artery was demonstrated in the remaining 132 fetuses (95.7%). The novel method demonstrated 100% feasibility and complete concordance with the conventional method. CONCLUSION: The study results indicate that the full rectangle sign serves as an effective and dependable screening tool for identifying ARSA. It offers the advantage of a clear, unobstructed view at a level unaffected by sternum shadowing and eliminates confusion with the azygous vein.

Left aortic arch and aberrant right subclavian artery in children: spectrum of symptoms and response to surgical intervention.

BACKGROUND: Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery. MATERIALS AND METHODS: This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022. Primary outcome was symptom improvement. Pre- and post-operative questionnaires were used to assess dysphagia (PEDI-EAT-10) and respiratory symptoms (PEDI-TBM-7). Paired t-test and Fisher's exact test were used to analyse symptom resolution. Secondary outcomes included perioperative outcomes, complications, and length of stay. RESULTS: Twenty children, median age 2 years (IQR 1-11), were included. All presented with swallowing symptoms, and 14 (70%) also experienced respiratory symptoms. Statistically significant improvements in symptoms were reported for both respiratory and swallowing symptoms. Paired (pre- and post-op) PEDI-EAT-10 and PEDI-TBM-7 scores were obtained for nine patients, resulting in mean (± SD) scores decreasing (improvement in symptoms) from 19.9 (± 9.3) to 2.4 (± 2.5) p = 0.001, and 8.7 (± 4.7) to 2.8 (± 4.0) p = 0.006, respectively. Reoperation was required in one patient due to persistent dysphagia from an oesophageal stricture. Other complications included lymphatic drainage (n = 4) and transient left vocal cord hypomobility (n = 1). CONCLUSION: Children with a left aortic arch with aberrant right subclavian artery can present with oesophageal and respiratory symptoms beyond solid food dysphagia. A thorough multidisciplinary evaluation is imperative to identify patients who can benefit from surgical repair, which appears to be safe and effective.

Fenestrated Physician-Modified Endovascular Grafts for Aberrant Right Subclavian Artery and/or Kommerell's diverticulum.

OBJECTIVE: The aim of this case series is to report feasibility, efficiency, and safety of fenestrated physician-modified endografts (PMEGs) in aortic arch pathologies with aberrant right subclavian artery (ARSA) and/or Kommerell's diverticulum (KD). METHODS: All consecutive patients with ARSA and/or KD who underwent hybrid aortic arch repair combined with homemade fenestrated stent-graft from 2018 to 2022 were reviewed. RESULTS: Six patients with ARSA and/or KD underwent hybrid surgery for aortic repair, 4 of whom were men, with a mean age of 49 years. Furthermore, 2 of them were symptomatic with dysphagia, 1 was taken in emergency, 1 had a bovine arch and a KD, and 2 had right descending thoracic aortas. The mean operation time was 138 (111-216) minutes. In addition, 83% of the homemade grafts were double fenestrated. All the proximal landings were in zone 0; the mean proximal aortic diameter was 29 (23-34) mm. The range of diameters for the endografts were 24 to 38 mm. There was a 100% technical success, with 0% 30 days mortality, no stroke, and no endoleak. During the follow-up, no aortic-related death or secondary intervention was required and all supra-aortic vessels remain patent. CONCLUSION: Hybrid aortic arch repair, with fenestrated PMEGs for ARSA and/or KD, is associated with acceptable early and midterm major morbidity and mortality. CLINICAL IMPACT: This retrospective case series analyzed outcomes in 6 patients with an aberrant right subclavian artery and/or Kommerell's diverticulum treated with fenestrated PMEGs during an average 16 month follow-up. The case series suggests that the use of these fenestrated PMEGs for the management of patients with an aberrant right subclavian artery is a safe, effective and durable method in the medium-term.

Medial-type persistent trigeminal artery and ipsilateral posterior communicating artery supplying bilateral posterior cerebral arteries combined with an aberrant right subclavian artery and bicarotid trunk.

PURPOSE: To describe a case of a medial-type persistent trigeminal artery (PTA) associated with multiple arterial variations. METHODS: A 34-year-old woman with multiple sclerosis underwent cranial magnetic resonance (MR) angiography from the aortic arch to the neck region and intracranial region for the evaluation of an unruptured cerebral aneurysm that was previously detected on MR imaging. The MR machine was a 3-T scanner. RESULTS: There was an aberrant right subclavian artery and bicarotid trunk, medial-type left PTA and ipsilateral posterior communicating artery (PCoA) supplying bilateral posterior cerebral arteries (PCAs). The unruptured aneurysm was located at the paraclinoid segment of the left internal carotid artery and was treated successfully by coil embolization via a transfemoral approach. CONCLUSION: Only 10% of PTAs are classified as the medial type. The association with extremely large ipsilateral PCoA, which supplies the bilateral PCAs, has not been previously reported. Aberrant right subclavian arteries are common and are frequently associated with a bicarotid trunk. Before catheterization of the cerebral arteries, the aortic arch and its branches must be evaluated by MR angiography or computed tomography angiography to prevent catheterization failure via the right transradial approach.

Arteria lusoria with patent foramen ovale: clinical and embryological significance with literature review.

A 73-year-old female donated cadaver had an unusual origin of the right subclavian artery (RSA), which is usually known as 'Arteria lusoria' (AL) or "Aberrant Right Subclavian Artery" (ARSA). This artery originated as the fourth and most extreme left branch from the arch of the aorta (AOA), distal to the origin of the left subclavian artery (LSA), and traversed obliquely upwards, towards the right side posterior to the oesophagus, heading for the thoracic inlet. The brachiocephalic trunk (BCT) was absent. The right common carotid (RCCA), left common carotid (LCCA), LSA and ARSA were four branches that originated from the aortic arch and ran from right to left. The course and distribution of these branches were normal. On opening the right atrium, a patent foramen ovale (PFO) was observed in the upper part of the interatrial septum. As far as we know, this is the first cadaveric case report of arteria lusoria with the presence of an atrial septal defect in the form of a PFO. Early diagnosis of aortic arch abnormalities using diagnostic interventions is beneficial for identifying risk factors after invasive procedures.

Aberrant right subclavian artery: case report and literature review.

The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

Contemporary management and natural history of aberrant right subclavian artery.

OBJECTIVE: An aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the aortic arch. A paucity of reported studies is available regarding the treatment of these patients. The purpose of the present study was to evaluate the contemporary management strategies and natural history of ARSA in these patients. METHODS: A single-center retrospective review of patients with a diagnosis of ARSA from 2009 to 2019 was performed. Computed tomography scans were analyzed, and the aortic and ARSA diameters were measured at 10 different segments. The demographic data, comorbidities, and operative interventions were collected. The patients were categorized into those who had undergone intervention and those who had undergone expectant management. Linear mixed effect models were used to estimate the annual ARSA diameter changes. RESULTS: A total of 30 patients with ARSA were identified, 17 (57%) of whom were women. The average age for the cohort was 54.5 ± 14.6 years. Of the 30 patients, 20 (67%) had undergone operative repair at presentation and 10 (33%) were initially observed. The most common presenting symptom was dysphagia (30%). Of the 10 patients who had been initially treated expectantly, 4 had subsequently required intervention. Of the 24 operative interventions, 13 (54%) were hybrid procedures involving right carotid-subclavian bypass or transposition and thoracic endovascular aortic repair. The mean diameter of ARSA at its origin was 20.4 ± 5.7 mm, and the mean cross-sectional aortic diameter at the level of the ARSA was 31.8 ± 8.5 mm for the entire cohort. For the patients who had initially been observed and had subsequently required intervention, the largest change in the ARSA cross-sectional diameter was observed 1 cm distally to the vessel ostium at a rate of 3.05 mm annually (95% confidence interval, 1.54-4.56; P < .001). No statistically significant changes in the annual growth rate of the aortic segments were observed in the entire cohort or for those patients who had undergone intervention (P > .05). CONCLUSIONS: The decision to intervene on an ARSA should be individualized by the presence of symptoms (eg, dysphagia lusoria) or complications (eg, dissection, concomitant aortic aneurysmal disease, enlarging Kommerell diverticulum). Asymptomatic patients with nonaneurysmal ARSA might not require any intervention and can be safely observed. Measurement of the cross-sectional ARSA diameter 1 cm distally to the ostium of the vessel might aid in the surveillance of vessel diameter changes. Additional studies are required to determine the specific size criteria as an indication for operative repair of asymptomatic Kommerell diverticulum.

Two-year follow-up after treatment of an aortic transection in the presence of an aberrant right subclavian artery.

OBJECTIVES: An aberrant right subclavian artery (ARSA) is one of the most common anatomic variants of the aortic arch. The combination of an ARSA and a transection is naturally rare. METHODS: This case report describes the treatment of a transection in the presence of an ARSA and the follow-up of two years. RESULTS: We successfully treated the contained rupture with a stentgraft. Both subclavian arteries had to be covered in the emergency setting. At the two-year follow-up, the patient did not suffer from any neurological impairment. CONCLUSIONS: In emergency settings, primary cover of both subclavian arteries with a stentgraft can be performed in individual cases after risk assessment. Severe complications such as development of upper limb ischaemia, ASAS or reduced perfusion of the posterior cerebral circulation should be considered in treatment planning.

Aberrant right subclavian artery as soft marker in the diagnosis of trisomy 21 during the first trimester of pregnancy.

PURPOSE: Aberrant right subclavian artery is an anatomical variation with a prevalence of around 0.5-1.5% of the general population, being more frequently found among people with chromosomopathies, especially, trisomy 21. Despite being an anatomical finding, and thus, constant through the whole pregnancy, its value in the diagnosis of aneuploidies during the first trimester of pregnancy has been little studied. The aim of this study is to evaluate the reliability of the first-trimester ultrasound in the diagnosis of ARSA and its utility in the early diagnosis of aneuploidies. METHODS: This was a descriptive, observational, cross-sectional study that included all fetuses with sonographic diagnosis of ARSA between 2011 and 2018. RESULTS: There were 257 cases of ARSA diagnosed. The first-trimester ultrasound showed the following results in the detection of ARSA: sensitivity of 68% (CI 95% 60.8%-74.5%), specificity of 99.9% (CI 95% 99.9%-100%), positive predictive value of 93.7% (CI 95% 88.1%-96.8%), and negative predictive value of 99.6% (CI 95% 99.5%-99.7%). Due to the presence of ARSA, two cases of trisomy 21, that would have been missed in the first trimester, were diagnosed, using ARSA as a soft marker and modifying the risk obtained by the combined screening as part of the genetic sonogram of the first trimester. CONCLUSIONS: ARSA visualization during the first-trimester ultrasound is trustworthy and it can improve the detection of trisomy 21 in some cases of aneuploidy missed during the combined screening of the first trimester.

Aberrant right subclavian artery: the association with chromosomal defects and the related post-natal outcomes in a third level referral centre.

Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortic arch. The presence of ARSA has been previously associated with an increased risk of Down syndrome. ARSA at birth may be associated with dysphagia, respiratory distress and stridor and there is no clear evidence-based management. The aim of this study was to describe the associations with chromosomal abnormalities and the postnatal outcome of fetuses diagnosed with ARSA. We analysed fetuses diagnosed antenatally with ARSA between January 2013 and September 2019 in the fetal echocardiography unit of the Hospital Monaldi, University 'Vanvitelli' of Naples, Italy. The results showed fifty fetuses diagnosed with ARSA, all confirmed after birth. The ARSA was an isolated finding in 46 fetuses (92%), while in 4 fetuses the ARSA was associated with other cardiac and/or extra-cardiac anomalies. Only one fetus was diagnosed with trisomy 21 (2%). In this fetus the ARSA was the only ultrasound anomaly identified. There were no cases necessitating referral due to the presence of compression symptoms at birth. The presence of ARSA was associated with trisomy 21 in the 2% of cases in our series and there were no neonatal complications due to airway compression at birth.IMPACT STATEMENTWhat is already known on this subject? Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortich arch. ARSA at birth could be associated with dysphagia, respiratory distress and stridor and no evidence-based management of these fetuses has been described yet. The presence of ARSA has been previously associated with an increased risk of Down syndrome.What do the results of this study add? This study confirms known data on association with chromosomal defects and provides some original data on the absence of symptomatology due to tracheal compression with a postnatal follow-up up to three years of age.What are the implications of these findings for clinical practice and/or further research? Our findings suggest that in cases with adequate prenatal assessment performed by experienced clinicians, delivery can safely take place at local hospitals, with no need of referral soon after birth. The use of transthoracic echocardiography to confirm the diagnoses of ARSA after birth and to plan the next follow-up appointments can be supported.

Aberrant right subclavian artery with right type 1 proatlantal artery and segmental dysplasia of the right internal carotid artery: a case report.

PURPOSE: We present a case of an aberrant right subclavian artery (ARSA) with extremely rare vascular anomalies. CASE REPORT: A 69-year-old woman was suspected to have right internal carotid artery (ICA) stenosis. Computed tomography angiography demonstrated an ARSA and hypoplasia of the right ICA. The proximal segment of the right vertebral artery (VA) was aplasia, and a right type 1 proatlantal artery (PA) arose from the right common carotid artery. Cerebral angiography demonstrated segmental dysplasia of the right ICA. The ascending intrapetrous segment and the ascending foramen lacerum-horizontal intracavernous segment of the right ICA demonstrated hypoplasia. The collateral pathways promoted reconstitution of each of the distal segments. Left internal carotid angiography demonstrated anterior communicating artery aneurysm and sufficient cross flow to the contralateral middle cerebral artery via the AcomA. DISCUSSION: A type 1 PA with an ARSA may result in the regression of the right dorsal aorta with persistence of the first cervical intersegmental artery. Although there are few findings of a relationship between an ARSA and intracranial artery anomalies, a developmental error of the right dorsal aorta may cause such complex vascular anomalies. CONCLUSION: Knowledge of anatomical variations in patients with ARSA is useful when performing angiography or endovascular therapy, as well as during clinical follow-up.

Type 2 left proatlantal artery with normal left vertebral artery and association with an aberrant right subclavian artery and a bi-carotid trunk.

Type 2 proatlantal artery (PA) is a rare type of the carotid-vertebrobasilar anastomosis that arises from the external carotid artery and enters the posterior fossa via the foramen magnum (FM). The type 2 PA is usually large and takes a similar course to the occipital artery (OA). The peripheral branch of the OA arises from the distal segment, just proximal to the FM. The ipsilateral vertebral artery (VA) is usually aplastic or hypo-plastic. We diagnosed a case of relatively small type 2 left PA in a patient with a normally developed ipsilateral VA. Furthermore, the patient had an aberrant right subclavian artery associated with a bi-carotid trunk. The combination of these extracranial arterial variations has not been reported in the relevant English language literature.

Prenatal chromosomal microarray analysis in 2466 fetuses with ultrasonographic soft markers: a prospective cohort study.

BACKGROUND: Soft markers are nonspecific findings detected by ultrasonography during the second trimester that are often transient and nonpathologic but may imply an increased risk of underlying fetal aneuploidy. However, large-scale prospectively stratified studies focusing on the prevalence of chromosomal aberrations, including copy number variants, in fetuses with different types of isolated soft markers have rarely been published in the literature. OBJECTIVE: This study aimed to investigate clinical outcomes in fetuses with isolated soft markers by single nucleotide polymorphism array with long-term follow-up and to propose a diagnostic algorithm based on specific types of soft markers. STUDY DESIGN: The prevalence of fetal isolated soft markers was 13.2% (7869 of 59,503). A total of 2466 fetuses with ultrasonographic soft markers during the second trimester, which were subjected to single nucleotide polymorphism array with long-term follow-up, were selected in this prospective study over a 5-year period. Soft markers were categorized into 12 groups. The demographic profile and chromosomal microarray analysis detection results were analyzed and compared among different groups. RESULTS: The overall prevalence of chromosomal aberrations in fetuses with soft markers was 4.3% (107 of 2466), which comprised 40.2% with numeric chromosomal abnormalities, 48.6% with pathogenic copy number variants, and 11.2% with likely pathogenic copy number variants. The incidence of numeric chromosomal abnormalities was significantly higher in multiple soft markers (5.5% vs 1.5%; P=.001) and the thickened nuchal fold group (8.3% vs 1.7%; P=.024). Meanwhile, the incidence of pathogenic copy number variants was significantly higher in multiple soft markers (5.5% vs 2.4%; P=.046) and the short femur length group (6.6% vs 2.2%; P<.0001). The incidences of pathogenic copy number variants in fetuses with isolated echogenic intracardiac focus, enlarged cisterna magna, choroid plexus cysts, echogenic bowel, or single umbilical artery were lower than 1.5%. The normal infant rate in fetuses without chromosomal aberrations was 91.7%; however, it was significantly lower in the mild ventriculomegaly (86.2% vs 93.0%; P<.0001) and short femur length groups (71.4% vs 93.6%; P<.0001). CONCLUSION: The potential chromosomal aberrations and clinical prognoses varied widely among different types of isolated soft markers. Pathogenic copy number variants are more often present in specific soft markers, especially when multiple soft markers are found. Thus, a specific soft marker type-based prenatal genetic testing algorithm was proposed.

Predictive value of aberrant right subclavian artery for fetal chromosome aneuploidy in women of advanced maternal age.

BACKGROUND: In the entire population, an aberrant right subclavian artery (ARSA) is closely associated with chromosomal abnormalities. ARSA with additional ultrasonic findings would increase risk of chromosomal abnormalities. The risk of fetal chromosomal abnormalities increased exponentially with the maternal age. These risks in the advanced maternal age (AMA) group are uncertain. This study aimed to determine the incidence of ARSA in Chinese AMA and non-AMA women and the frequency of aneuploidy among AMA and non-AMA women with ARSA. METHODS: This retrospective study included 13,690 singleton pregnancies, were divided into AMA and non-AMA groups. Integrated obstetric ultrasonic screening, biochemical screening, noninvasive prenatal screening, and fetal karyotype analysis were analyzed. RESULTS: The overall incidence of ARSA was 0.69%, with no difference between age groups. The incidence of chromosomal abnormalities in the AMA group (37 / 2860) was much higher than that of the non-AMA group. The risk of chromosomal abnormalities significantly increased with both ARSA detected and additional ultrasound findings. With combined ARSA and AMA, the likelihood of the incidence of chromosomal abnormalities increased. Chimerism (45X / 46XX) was found with isolated ARSA in AMA pregnancies. CONCLUSION: There is a high prevalence of chromosomal abnormalities in fetuses of AMA women. ARSA increases the risk of chromosomal abnormalities in both age groups, especially combined with ARSA. When ARSA occurs in AMA women, it confers a high likelihood of chromosomal abnormalities.

Is it useful to evaluate the presence of aberrant right subclavian artery in prenatal diagnosis ultrasounds?

AIM: Analyze if the evaluation of aberrant right subclavian artery in the prenatal echography has improved the detection of chromosomal, genetic and/or morphological abnormalities in our population. METHODS: Descriptive, observational, cross-sectional study of the cases of aberrant right subclavian artery diagnosed in our Prenatal Diagnosis Unit between January of 2011 and December of 2018. RESULTS: Two hundred and fifty-seven cases of aberrant right subclavian artery were diagnosed and among them, 179 were considered isolated cases and thus were confirmed after birth. The detection of aberrant right subclavian artery did not improve itself neither the diagnosis of trisomy 21 in the second trimester of pregnancy nor other chromosomal or genetic abnormalities, including the not isolated cases. There were two cases of trisomy 21 diagnosed in the second trimester that presented major sonographic disorders and an inadequate examination during the first trimester. When aberrant right subclavian artery was associated with soft markers of aneuploidy in the second trimester, any case was a trisomy 21. Aberrant right subclavian artery seems to be associated with some minor and major heart defects, especially ventriculoseptal defect and aneurismatic ductus, and in some cases, also with clubfeet. CONCLUSION: When an adequate screening of aneuploidies and a thorough ultrasound have been performed during the first trimester, aberrant right subclavian artery hardly helps to perform other diagnosis in the second trimester.

Aberrant right subclavian artery - a rare congenital anatomical variation causing dysphagia lusoria.

An aberrant right subclavian artery (ARSA) is a rare anatomical variation of the aortic arch. Although an incidental finding and asymptomatic in the majority of individuals, an ARSA can cause troubling symptoms during both childhood and in later life. In adulthood, the most common symptom is dysphagia, where the condition is named dysphagia lusoria. In other rare cases it can cause shortness of breath, chronic cough and hoarseness of voice amongst others. We present a case of a 65-year-old female patient who was diagnosed with dysphagia lusoria following a barium swallow examination to investigate a 10-year history of dysphagia. She was further investigated with other imaging modalities to establish her diagnosis. The dysphagia was not progressive, nor did it result in malnutrition, and hence the patient was managed conservatively. There is currently no established guideline to classify the severity of symptoms or radiological findings of this anatomical anomaly. Our case reiterates the importance of such protocols, in order to be able to avoid the risks of an unnecessary surgical procedure, whilst being sure to prevent the undertreatment of affected individuals.

A systematic classification of the left-sided aortic arch variants based on cadaveric studies' prevalence.

INTRODUCTION: Typical branching pattern of the left-sided aortic arch consists of the brachiocephalic trunk (BCT), the left common carotid artery (LCCA) and the left subclavian artery (LSA). Variant patterns have been associated with a broad spectrum of pathologies. The meticulous knowledge of potential aortic arch variants is of utmost importance to radiologists, interventional cardiologists, vascular and thoracic surgeons. The current systematic review collects all aortic arch branching patterns and their frequency as published by various cadaveric studies, calculates prevalence taking into account the gender and the different people background, as well. All extracted variant patterns are classified into types and subtypes according to the number of emerging (major and minor) branches (1, 2, 3, 4 and 5) and to the prevalence they appear. In cases of similar prevalence, total cases were taken into consideration; otherwise the variants were classified under the title "other rare variants". METHODS: A systematic online search of PubMed and Google books databases was performed only in cadaveric studies. RESULTS: Twenty studies with typical (78% prevalence) and variable (22%) branching patterns were included. Types 3b, 2b, 4b, 1b and 5b had a prevalence of 81%, of 13%, of 5%, 0% and of 0%, respectively. Common variants were the brachiocephalico-carotid trunk (BCCT, 49% prevalence), the aberrant left vertebral artery (LVA, 41%) and the aberrant right subclavian artery (ARSA, 8%). LVA of aortic origin was detected in 32%, the bicarotid trunk (biCT) in 5% and the bi-BCT trunk in 3%. Thyroidea ima artery, a minor branch emerging from the aortic arch was found in 2%. Coexisted variants were detected in 4% (ARSA with a distinct RCCA and LCCA origin), in 3% (BCCT with a LVA of aortic origin), in 2% (ARSA with a biCT and a vertebrosubclavian trunk). CONCLUSION: No significant gender or ethnic differences exist among the 5 branching types. The proposed classification scheme aims to become a valuable and easy to use tool in the hands of all physicians involved in diagnosis and treatment of aortic arch pathology. It could be also useful in anatomical education, as well.

Surgical treatment of esophageal cancer with anomaly of the aortic arch and its branches.

BACKGROUND: Anomalies of the aortic arch and its branches rarely develop. The surgery for esophageal cancer may be challenging with the presence of these anomalies. This study is aimed to analyze the influence of these variations during the esophagectomy. METHODS: A total of 21 patients with aortic arch and brachiocephalic vessel variations were retrospectively identified from 2013 to 2019. Anomalies were distributed: 15 patients with left-sided aortic arch combined with aberrant right subclavian artery (LAA + ARSA), 2 right-sided aortic arch with mirror-image arch branches (RAA + MIAB), and 4 right-sided aortic arch combined with aberrant left subclavian artery (RAA + ALSA). Perioperative characteristics and long-term survival were analyzed. RESULTS: Tumors were mostly located in the upper and middle thorax (42.9% and 47.6%, respectively). Of the 15 patients with LAA + ARSA, 13 underwent McKeown esophagectomy and 2 via transhiatal approach. Left thoracotomy was performed on all 6 patients with RAA. The R0 resection rate was 90.5% (19/21). Recurrent laryngeal nerve (RLN) injury occurred in 2 patients. Two patients died within 30 days postoperatively. Lymph node yield was 23.6 ± 1.2, with a metastasis rate of 38.1% (8/21). The median follow-up time was 18 months. Recurrence occurred in 6 patients (11-35 months) and 4 patients died after recurrence with a median time to death of 21 months (8-47 months). CONCLUSION: For resectable esophageal cancer combined with aortic arch and its branches anomalies, satisfactory surgical results can be obtained under careful preoperative evaluation and reasonable surgical approach selection.

Endovascular repair of type A aortic intramural hematoma accompanied by aberrant right subclavian artery and Kommerell's diverticulum: a case report.

BACKGROUND: The lesions of aberrant right subclavian artery, Kommerell's diverticulum and type A aortic intramural hematoma are rare, and we usually treat them with open surgery. In some cases patients have increased risk to undergo surgery, the experiences of endovascular or medical treatment are limited. CASE PRESENTATION: Here we reported a case of a 53-year-old man with these three entities present with chest and back ache and attempted a novel approach, thoracic endovascular aortic repair, in the absence of surgical treatment. The patient lived over 5 years and this case provides initial experience and lesson about the endovascular and medical management of the uncommon and dangerous disease- type A aortic intramural hematoma with aortic congenital malformation. CONCLUSION: Thoracic endovascular aortic repair with medical treatment may be a potential alternative approach for type A aortic intramural hematoma.

Dysphagia Lusoria: Is the Dysmotility Connection Illusory or Real?

Dysphagia lusoria is rare embryologic defect of the aortic arch vasculature characterized by an aberrant retro-esophageal course of the right subclavian artery (RSA), comprising a vascular sling. This may manifest clinically with symptoms of dysphagia or reflux. Given the rarity of this pathology, there remains uncertainty regarding the most effective treatment algorithm. Vascular decompression procedure surgery is often used to rectify the aberrant vascular morphology. However, a low percentage of aberrant arteries result in trachea-esophageal symptoms. As such, we suggest (1) one must exclude co-morbid esophageal motility disorders in patients with dysphagia; and (2) aberrant RSA may be a cause of dysphagia, so-called dysphagia lusoria, but represents an unlikely mechanism, since this morphological arrangement typically does not result in a highly constrictive, circumferential vascular ring. It follows, therefore, that directly addressing the esophageal motility issue should take precedence over complex and potentially noncurative vascular procedures directed to the aberrant RSA. However, the presence of marked aneursymal dilation of the aberrant RSA, and/or the presence of a bicarotid (socalled bovine) trunk, would favor vascular repair in the clinical decision-making due to the elevated risk of rupture and more severe esophageal compression in these specific situations.