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Background: Symptoms, imaging characteristics, and early and midterm surgical outcomes for aberrant subclavian arteries (ASCA) are not well defined in the adult population., Methods: A single-institution retrospective review was conducted of adults undergoing surgical repair of ASCA and descending aorta origin/Kommerell diverticulum (KD) from January 1, 2002, to December 31, 2021. Symptom improvement and differences in imaging characteristics between anatomic groups and the number of symptoms were assessed., Results: Mean age was 46 ± 17 years. There were 23 of 37 left aortic arches with right ASCA (62%) and 14 of 37 right aortic arches with left ASCA (38%). Of these, 31 of 37 (84%) were symptomatic, and 19 of 37 (51%) had KD size/growth meeting criteria for surgical repair. KD aortic origin diameter was larger in more symptomatic patients: 20.60 mm (interquartile range [IQR], 16.42-30.68 mm) in patients with ≥3 symptoms vs 22.05 mm (IQR, 17.52-24.21 mm) for 2 symptoms vs 13.72 mm (IQR, 12.70-15.95 mm) for 1 symptom (P = .018). Aortic replacement was required in 22 of 37 (59%). There were no early deaths. Complications occurred in 11 of 37 (30%): vocal cord dysfunction (4 of 37 [11%]), chylothorax (3 of 37 [8%]), Horner syndrome (2 of 37 [5%]), spinal deficit (2 of 37 [5%]), stroke (1 of 37 [3%]), and temporary dialysis requirement (1 of 37 [3%]). Over a median follow-up of 2.3 years (IQR, 0.8-3.9 years), there was 1 endovascular reintervention and no reoperations. Dysphagia and shortness of breath resolved in 92% and 89%, respectively, whereas gastroesophageal reflux persisted in 47%., Conclusions: The KD aortic origin diameter correlates with the number of symptoms, and surgical repair of ASCA and descending aorta origin/KD effectively relieves symptoms, with low rates of reintervention. Given the operative complexity, surgical repair should be performed in patients meeting size criteria or with significant dysphagia or shortness of breath symptoms., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The aberrant right subclavian artery (i.e., arteria lusoria) arising from the left part of the aortic arch is a rare congenital anomaly. In some patients, esophageal compression may cause symptoms of dysphagia, also called dysphagia lusoria. It can cause serious feeding disorders and poor weight gain in young children. We present the case of an early onset of dysphagia lusoria in a 1-month-old girl whose clinical diagnosis was confirmed by esophagography and magnetic resonance imaging. This kind of vascular anomaly can present a diagnostic challenge and should be considered in diagnosing dysphagia in childhood., (Published by Elsevier Inc.)

While unusual, aberrant right subclavian artery (ARSCA) can occasionally be a source of significant dysphagia in children. We present a case of a 13-year-old female who reported a three-year history of dysphagia to solid foods and was found to have ARSCA on a barium swallow study and computed tomography scan of the chest. We reimplanted the ARSCA into the right carotid artery in end-to-side fashion using a partial median sternotomy approach. At six months follow-up, her symptoms had completely resolved, and her postoperative echocardiogram showed an unobstructed reimplanted ARSCA. Meeting presentation: AATS 102nd Annual Meeting; May 14, 2022; Boston, MA.

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., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

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 69-year-old female suffering from severe dysphagia due to an aberrant right subclavian artery was treated with a two-step approach. A right carotid-subclavian bypass was followed 2 weeks later by a robotic-assisted thoracoscopic resection of the aberrant right subclavian artery. The postoperative course was uneventful, and the patient immediately recovered from her dysphagia. In our case, the robotic-assisted technology offered major advantages and, based on our experience, may be useful in the treatment of this rare disease., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: There is no consensus on the optimal approach to treatment of dysphagia lusoria (DL), especially in the absence of a Kommerell diverticulum (KD). We leveraged our institutional experience to clarify the safety and efficacy of aberrant subclavian artery (aSA) division with revascularization by a supraclavicular approach alone in patients with DL., Methods: We identified 10 consecutive patients who underwent surgery for DL at our institution between January 2007 and March 2019. Clinical and radiographic characteristics for all patients were collected. The primary outcome was improvement in dysphagia symptoms. Secondary outcomes included need for second-stage operation, postoperative complications, and long-term radiologic changes in the aSA remnant., Results: Seven patients underwent initial division of the aSA followed by revascularization through a supraclavicular exposure, and three had simultaneous supraclavicular and transthoracic intervention. Median follow-up was 52 (range, 1-143) months. In the four patients without a KD, a supraclavicular approach alone resulted in improvement in dysphagia symptoms. Conversely, the three patients who had a KD did not have durable relief of dysphagia and required a second-stage transthoracic procedure. Postoperatively, one developed a pulmonary embolism after supraclavicular approach alone and two patients developed complications after transthoracic intervention: left recurrent laryngeal nerve neurapraxia and pleural effusion requiring thoracentesis in one, and thoracic duct injury requiring reoperation in the second. Among those who underwent a single-stage procedure by a supraclavicular approach alone, no patients had aneurysm progression, rupture, or dissection after median follow-up of 60 (range, 1-100) months., Conclusions: Division and revascularization of the aSA through a supraclavicular exposure in patients with DL result in durable symptomatic improvement in the majority of patients, particularly those without a concomitant KD. Patients with a KD may require a second-stage procedure to address the KD, but it appears safe to trial staged intervention rather than simultaneous operations to divide the aSA and to address the KD., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Dysphagia lusoria is an uncommon cause of mechanical dysphagia due to extrinsic compression. It is a congenital aortic root abnormality that occurs as a consequence of an aberrant right subclavian artery, in most cases. We report an illustrative case of dysphagia lusoria in a young female, secondary to the presence of the abnormal mediastinal vessel.

Dysphagia lusoria is a rare disease due to an aberrant right subclavian artery that passes posteriorly between the esophagus and the spine. David Bayford coined the term itself meaning "freak or jest of nature" in 1761 describing a case in which the patient had long term dysphagia that eventually led to death. Most cases of dysphagia are due to an aberrant right subclavian artery running posterior to and causing esophageal compression, but only 20-40% of aberrant arteries actually lead to trachea-esophageal symptoms, including dysphagia. The majority of patients with an aberrant right subclavian artery are asymptomatic. Treatment for dysphagia lusoria varies depending on the severity of the symptoms. Dietary modifications are recommended in patients with mild to moderate symptoms while vascular reconstruction is necessary for patients with severe symptoms. We present a 44-year-old female who was diagnosed with dysphagia lusoria due to a right-sided aortic arch and aberrant left subclavian artery (ALSA) with aneurysmal dilation. Right aortic arch with ALSA is an uncommon arch anomaly, and only occurs in about 0.05% of the population. Prior case reports of dysphagia lusoria differ in that they did not report patients with an uncommon presentation of dysphagia lusoria with aneurysmal dilatation nor was the patient diagnosed in the emergency department., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Aim: To investigate the behavior of pulsatile pressure zones (PPZ's) as noted on high resolution esophageal impedance manometry (HREIM), and determine their association with dysphagia., Methods: Retrospective, single center case control design screening HREIM studies for cases (dysphagia) and controls (no dysphagia). Thoracic radiology studies were reviewed further in cases for (thoracic cardiovascular) thoracic cardiovascular (TCV) structures in esophageal proximity to compare with HREIM findings. Manometric data was collected for number, location, axial length, PPZ pressure and esophageal clearance function (impedance)., Results: Among 317 screened patients, 56% cases and 64% controls had PPZ's. Fifty cases had an available thoracic radiology comparison. The distribution of PPZ's in these 50 cases and 59 controls was similar (average 1.4 PPZ/patient). Controls (mean 31.2 ± SD 12 years) were a significantly younger population than cases (mean 67.3 ± SD 14.9 years) with P < 0.0001. The upright posture PPZ pressure was higher in controls (15.7 ± 10.0 mmHg) than cases (10.8 ± 9.7 mmHg). Although statistically significant ( P = 0.005), it was a weak predictor using logistic regression and ROC model (AUC = 0.65). Three dysphagia patients had partial compression from external TCV on radiology (1 aberrant subclavian artery, 2 dilated left atrium). The posture (supine vs upright) with more prominent PPZ's impaired bolus clearance in 9 additional cases by > 20%., Conclusion: Transmitted TCV pulsations observed in HREIM bear no significant impact on swallowing. However, in older adults with dysphagia, evidence of impaired bolus clearance on impedance should be evaluated for external TCV compression. These associations have never been explored previously in literature, and are novel., Competing Interests: Conflict-of-interest statement: No potential conflicts of interest relevant to this article were reported.

Aberrant subclavian arteries are congenital vascular anomalies that usually do not cause any symptoms. When symptomatic they are considered as a rare cause of dysphagia. This presentation is known as dysphagia lusoria. They are diagnosed by barium swallow or contrast-enhanced computed tomography, although it may be an incidental finding. Management varies from life modifications and drug therapy to surgical intervention. We report two cases of the unusual form of late onset symptomatic presentation because of the presence of aberrant right subclavian artery. Main symptom was chest pain without dysphagia. Due to age and medical comorbidities both patients where managed conservatively.

Kommerell diverticulum is uncommon, and it carries risks of dissection or rupture. Hybrid aortic arch repair is being used increasingly for this pathology. We report the hybrid arch repair of Kommerell diverticulum in a 72-year-old woman known for muscular dystrophy and right aortic arch with aberrant left subclavian artery. Head-vessel debranching was performed through median sternotomy using a handmade, bifurcated, Dacron graft. Stent-grafting was performed from the ascending aorta to the proximal descending aorta. To our knowledge, this report is the first description of debranching using a custom-made graft for hybrid repair of Kommerell diverticulum., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 48-year-old woman was seen in a surgical outpatient clinic with a 2 year history of progressive dysphagia with occasional regurgitation, partially controlled with a proton pump inhibitor. Primary investigations of pH testing and gastroscopy were normal, although a barium swallow study revealed significant hold-up at the aortic arch impression and a posterior right-sided oesophageal impression suggestive of a right-sided aortic arch. A follow-up computed tomography angiogram discovered a vascular ring encircling the trachea and oesophagus, formed by a right-sided aortic arch with aberrant aortic branches, and a Kommerell's diverticulum. It was deemed that the patient's symptoms were related to this vascular ring. The patient underwent stage-one surgery - an extra-anatomic bypass of the double aortic arch and right subclavian artery - and 4 months later a stent graft insertion over the origin of the diverticulum with the aim of complete symptomatic relief. This case presents a common symptom familiar to any clinician (dysphagia), which has been caused by a rare pathology. It is even more unusual that this should present itself in adulthood.

Endovascular treatment offers a great advantage in the management of main arteries stenoses. However, simultaneous presence of a group of anomalies may complicate the situation. Here we present a case of 21-year-old man with aortic coarctation. Radiographic imaging and angiography demonstrated aortic coarctation of the left-circumferential aortic arch, right-sided descending aorta, and Kommerell's diverticulum at the origin of right subclavian artery. These anomalies have rarely been reported to concurrently exist in the same case and the treatment is challenging. Percutaneous treatment for repair of aortic coarctation was successfully performed with deployment of self-expanding nitinol stents. Follow-up demonstrated the correction of blood pressure and improvement of the symptoms. It appears that deployment of self-expandable nitinol stents present a viable option for the management of coarcted aorta in patients having all or some of these anomalies together., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The non-recurrent laryngeal nerve (NRLN) is a rare but potentially serious anomaly that is commonly associated with the aberrant right subclavian artery (ARSA). It is easy to damage during surgical resection of esophageal cancer, leading to severe complications., Methods: Preoperative enhanced thoracic computed tomography (CT) scans of 2697 patients with esophageal carcinoma treated in our hospital between January 2010 and December 2013 were examined. We classified the positional relationship between the right subclavian artery and the membranous wall of the trachea into two types and used this method to predicate NRLN by identifying ARSA., Results: Twenty-six patients (0.96%) were identified with ARSA, all of which were cases of NRLN by CT. NRLN was identified during surgery in the 26 patients, and a normal right recurrent laryngeal nerve was observed in 2671 patients. The ARSA was detected on the dorsal side of the membranous wall of the trachea in all 26 NRLN cases, while it was detected on the ventral side in all 2671 recurrent laryngeal nerve cases., Conclusion: Enhanced CT scanning is a reliable method for predicting NRLN by identifying ARSA. Preoperative recognition of this nerve anomaly allows surgeons to avoid damaging the nerve and abnormal vessels during esophagectomy., (© 2016 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd.)

Right-side aortic arch is a rare congenital aortic anomaly occurring in 0.05% to 0.1% of the general population. Approximately, half of these cases may be associated with an aberrant left subclavian artery and occasionally with aneurysmatic change at its origin known as Kommerell diverticulum or aneurysm (KA). Herein we report a challenging case of a right-side aortic arch associated with KA incidentally observed in a 73-year-old male with metastatic lung cancer. After careful multidisciplinary discussion, a conservative strategy of care was successfully adopted., (© The Author(s) 2016.)

Unlabelled: Objection: To evaluate of Kommerell diverticulum (KD) in adults during routine CT examinations., Material and Methods: Eighty-seven cases of left aortic arch with aberrant right subclavian artery (LAARS) and 28 cases of right aortic arch with aberrant left subclavian artery (RAALS) were found on routine CT examinations using 64-128 multidetector CT. We assessed the incidence of KD and measured the size of KD, and compared the results between both groups. We evaluated associated congenital and acquired cardiovascular diseases on both groups., Results: The incidence of KD in each group was as follows: RAALS 100 % and LAARS 48.3 % and incidence of KD in RAALS group was significant higher than in LAARS group. The mean KD size was as follows: RAALS 32.8 mm and LAARS 17.8 mm and, the size of KD in RAALS group was larger than that in LAARS group. Two cases were associated with congenital heart disease. Several cases were associated with acquired aortic diseases including aortic aneurysm, dissection, severe atherosclerosis and aortitis., Conclusion: KD was common among adults with an ASA and some adults were associated with aortic diseases.

Background: A 59-year old man being investigated for back pain was found to have aneurysmal dilatation of a right-sided aortic arch and descending thoracic aorta together with an aberrant left subclavian artery., Case Presentation: He underwent repair of this utilising the frozen elephant trunk technique, which dealt with all three pathologies in one-stage. He made an unremarkable recovery and was discharged home on the 8th post-operative day., Conclusions: This case report further demonstrates the flexibility and safety of the frozen elephant trunk in dealing with complex aortic pathology as a single-stage procedure.

A 57-year-old man was incidentally found to have a dissected diverticulum of Kommerell originating from aberrant origin of right subclavian artery during follow-up for treated colon cancer. A right carotid-axillary bypass was followed by embolization of aberrant artery and the diverticulum by deployment of multiple detachable coils using the Penumbra Ruby System (Penumbra, Alameda, CA). Angiography after embolization showed exclusion of flow to the aberrant artery and a patent right carotid-axillary bypass. The patient recovered uneventfully and remained well for 12 weeks after the operation, without any complications. We believe this targeted endovascular approach can avoid complications related to the coverage of thoracic aorta and reduce the risk of access vessel trauma., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The objective of this study was to determine the frequency and the nature of associated anomalies, especially malformations and chromosome abnormalities, in a population of fetuses with an aberrant right subclavian artery (ARSA)., Materials and Methods: This is a 7-year descriptive study. All patients whose fetus had an ARSA diagnosed by ultrasound performed during the 1st, 2nd, or 3rd trimester of pregnancy were included, regardless of their risk of chromosomal abnormalities., Results: Between May 2007 and April 2014, an ARSA was diagnosed in 120 fetuses. The outcome was found in 108 cases (90%). ARSA was an isolated finding in 54/108 cases (50%). In 20% (22/108) of the fetuses, chromosomal abnormalities were detected. No chromosomal abnormalities were found in fetuses with an isolated ARSA. 82% (18/22) of chromosomal abnormalities were usual, such as trisomies 21 and 18, monosomy X, and 22q11.2 deletion. 21% (23/108) of the fetuses presenting an ARSA were associated with having a congenital heart disease., Conclusion: The presence of an isolated ARSA is a condition rarely associated with a chromosomal abnormality. The decision to perform an invasive karyotyping procedure under such circumstances or not may be made according to the principle of parental autonomy after extensive counselling and mostly a thorough assessment of the fetus., (© 2016 S. Karger AG, Basel.)

A 50-year-old man with a history of systemic lupus erythematosus and hemodialysis developed acute type A aortic dissection. Computed tomography demonstrated acute type A aortic dissection with chronic distal arch aneurysm and aberrant right subclavian artery that arose from the proximal descending aorta and ran in a retro-esophageal track. Emergent total arch replacement was performed using antegrade cerebral perfusion with circulatory arrest. Both common carotid arteries and the left subclavian artery were chosen as selective cerebral perfusion sites. The right subclavian artery was snared during cerebral perfusion. The right subclavian artery was reconstructed with the right common carotid artery in an end-to-side fashion in the anterior mediastinum. The patient's postoperative course was uneventful, and computed tomography showed excellent blood flow to all four branches. The case description is followed by a discussion of cerebral protection, reconstruction route of the right aberrant subclavian artery and steroids for systemic lupus erythematosus.

We report a case of an accidental finding of an aberrant right subclavian artery diagnosed in an adult man during a 4-French coronary angiography performed by right transradial access, then confirmed by multi-slice computed tomography. Tips and tricks have been suggested to complete the 4-French procedure avoiding changing the vascular access., (Copyright © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objectives: The primary objective was to estimate the prevalence of aberrant right subclavian artery (ARSA) in fetuses with Down syndrome. Secondary objectives were to assess the prevalence of ARSA in euploid fetuses, the feasibility of ultrasound evaluation of the right subclavian artery (RSA) in the first and second trimesters of pregnancy, the performance of ARSA in screening for trisomy 21 and its association with other abnormalities., Methods: Web-based databases (PubMed, EMBASE and MEDLINE) were searched up to July 2014. The STROBE, PRISMA and QUIPS instruments were used to assess all included studies and for reporting of methodology, results and conclusions. Original studies that reported prenatal ultrasound evaluation of ARSA, assessment of its prevalence in Down-syndrome and euploid fetuses, feasibility of ultrasound evaluation of the RSA in the first and second trimesters of pregnancy and correlation of ARSA with other abnormalities were included, excluding duplications and case reports. Collected data were summarized to estimate prevalence and feasibility. A meta-analysis was performed pooling the study-specific positive and negative likelihood ratios (LR+ and LR-), detection rates and false-positive rates for trisomy 21., Results: Prevalence of ARSA in Down-syndrome fetuses was 23.6% (95% CI, 19.4-27.9%), whereas in euploid fetuses it was 1.02% (95% CI, 0.86-1.10%). Ultrasound evaluation of the RSA course and origin in the first and second trimesters of pregnancy was feasible in 85% and 98% of cases (first and second trimester, respectively) and it was directly related to sonographic experience and fetal crown-rump length and inversely related to maternal body mass index. In more than 20% of fetuses with ARSA there was an association with other abnormalities but ARSA seemed to be an independent marker of trisomy 21. The meta-analysis showed that ARSA is a significant risk factor for Down syndrome (pooled LR+ = 26.93, 95% CI, 19.36-37.47, P for effect < 0.001, P for Q = 0.3, I(2) = 17.3%), whereas normal RSA is a significant protective marker (pooled LR- = 0.71, 95% CI, 0.51-0.99, P for effect = 0.043, P for Q = 0.9, I(2) = 0%)., Conclusions: ARSA appears to be a clinically useful prenatal ultrasound marker of Down syndrome. Additional testing when ARSA is diagnosed should involve evaluation of all risk factors by applying a mathematical model. There is insufficient evidence to recommend fetal karyotyping in cases with isolated ARSA. If the background risk is higher or additional markers are present, full fetal karyotyping is advisable, including analysis for 22q11 microdeletion., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: The objective of this multicenter registry was to review current treatments and late results of repair of aneurysm of aberrant right subclavian artery (AARSA)., Methods: All consecutive AARSA repairs from 2006 to 2013 in seven centers were reviewed. End points were 30-day and late mortality, reintervention rate, and AARSA-related death., Results: Twenty-one AARSA repairs were included (57% men; mean age, 67 years); 3 ruptures (14%) required emergent treatment; 12 (57%) were symptomatic for dysphagia (33%), dysphonia (24%), or pain (19%). Eight cases (38%) presented with thoracic aortic aneurysm, two with intramural hematoma, and one with acute type B aortic dissection. Mean AARSA diameter was 4.2 cm; a single bicarotid common trunk was present in 38% of cases. The majority of patients underwent hybrid intervention (n = 15; 71%) consisting of single (n = 2) or bilateral (n = 12) subclavian to carotid transposition or bypass or ascending aorta to subclavian bypass (n = 1) plus thoracic endovascular aortic repair (TEVAR); 19% of cases underwent open repair and 9% simple TEVAR with AARSA overstenting. Perioperative death occurred in two patients (9%): in one case after TEVAR in ruptured AARSA, requiring secondary sternotomy and aortic banding; and in an elective case due to multiorgan failure after a hybrid procedure. Median follow-up was 30 (interquartile range, 15-46) months. The Kaplan-Meier estimate of survival at 36 months was 90% (standard error, 0.64). Late AARSA-related death in one case was due to AARSA-esophageal fistula presenting with continuing backflow from distal AARSA and previous TEVAR. At computed tomography controls, one type I endoleak and one type II endoleak were detected; the latter required reintervention by aneurysm wrapping and ligature of collaterals. AARSA-related death was more frequent after TEVAR, a procedure reserved for ruptures, compared with elective open or hybrid repair., Conclusions: Hybrid repair is the preferred therapeutic option for patients presenting with AARSA. Midterm results show high rates of clinical success with low risk of reintervention. Simple endografting presents high risk of related death; these findings underline the importance of achieving complete sealing to avoid treatment failures., (Copyright © 2015 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Congenital aortic arch and vertebral artery anomalies are a relatively rare finding discovered on imaging either incidentally or for evaluation of entities like dysphagia or subclavian steal. Right aortic arch is an uncommon anatomical anomaly that occurs in less than 0.1% of the population, and in half of these cases the left subclavian artery is also aberrant.(1) Unilateral vertebral artery (VA) duplication is rare with an observed prevalence of 0.72% in cadavers.(2) Fenestration of the VA is more common than duplication, with a prevalence of approximately 0.23%-1.95%.(3,4) We describe the case of a 25-year-old female who was found to have a right aortic arch with aberrant left subclavian artery, duplicated left vertebral artery and a fenestrated right vertebral artery on CT angiography performed for evaluation of dysphagia. This combination of findings has not been reported before, to the best of our knowledge. We review the embryologic mechanism for the development of the normal aortic arch, right aortic arch, vertebral artery duplication and vertebral artery fenestration. The incidence of these entities, resultant symptoms and clinical implications are also reviewed. The increased associated incidence of aneurysm formation, dissection, arteriovenous malformations and thromboembolic events with fenestration is also discussed., (© The Author(s) 2015.)

Objective: To examine the potential of aortic arch variants, specifically bovine aortic arch, isolated left vertebral artery, and aberrant right subclavian artery, as markers for thoracic aortic disease (TAD)., Methods: We screened imaging data of 556 patients undergoing surgery due to TAD for presence of aortic arch variations. Demographic data were collected during chart review and compared with a historical control group of 4617 patients., Results: Out of 556 patients with TAD, 33.5% (186 patients) demonstrated anomalies of the aortic arch, compared with 18.2% in the control group (P < .001). Three hundred seventy (66.5%) had no anomaly of the aortic arch. Bovine aortic arch emerged as the most common anomalous branch pattern with a prevalence of 24.6% (n = 137). Thirty-five patients (6.3%) had an isolated left vertebral artery, and 10 patients (1.8%) had an aberrant right subclavian artery. When compared with the control group, all 3 arch variations showed significant higher prevalence in patients with TAD (P < .001). Patients with aortic aneurysms and anomalous branch patterns had hypertension less frequently (73.5% vs 81.8%; P = .048), but had a higher rate of bicuspid aortic valve (40.8% vs 30.6%; P = .042) when compared with patients with aneurysms but normal aortic arch anatomy. Patients with aortic branch variations were significantly younger (58.6 ± 13.7 years vs 62.4 ± 12.9 years; P = .002) and needed intervention for the aortic arch more frequently than patients with normal arch anatomy (46% vs 34.6%; P = .023)., Conclusions: Aortic arch variations are significantly more common in patients with TAD than in the general population. Atypical branching variants may warrant consideration as potential anatomic markers for future development of TAD., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

An aberrant right subclavian artery can be diagnosed by PET/CT, as demonstrated in this case of a 70-y-old man undergoing PET/CT for staging of squamous cell carcinoma of the right lung. It is important to report this finding during the evaluation of oncologic patients, to prevent severe complications that may arise from various oncologic interventions., (© 2015 by the Society of Nuclear Medicine and Molecular Imaging, Inc.)

Unlabelled: The development of the fetal aorta ends with the formation of the aortic arch which normally branches into three blood vessels: 1) a. brachiocephalica (a. innominata), which divides into the right subclavian artery (RSA) and the right carotid artery; 2) the left carotid artery; and 3) the left subclavian artery. Occasionally, RSA originates as a separate fourth branch of the aortic arch, passing behind the trachea with an oblique course to the right shoulder. This rare variant is called an aberrant right subclavian artery (ARSA) and is observed in approximately 2% of normal individuals. On the other hand, the reported incidence of ARSA varies between 25 and 37% in cases with Down syndrome and other chromosomal abnormalities., Objective: To evaluate the success rate of ultrasound visualization of the fetal RSA between 18 and 23 weeks of gestation and to establish the importance of the prenatal diagnosis of ARSA in the risk assessment for fetal chromosomal abnormalities in the second trimester., Results: Three experienced sonographers scanned 992 fetuses in MC "Markovs", Sofia between 01.09.2013-01.06.2014 with Voluson 730 Expert (GE Healthcare) ultrasound equipment. Visualization of RSA was successful in 92.7% of cases. Overall, 17 cases with ARSA were diagnosed in the study period. ARSA was an isolated sonographic finding in 13 of them. The remaining 4 cases had additional pathology. In the first case ARSA was associated with a short femurand humerus, short nasal bone and borderline nuchal thickness without any other soft markers or structural abnormalities. Trisomy 21 was diagnosed after amniocentesis and the pregnancy was terminated at patient's request. In the second case ARSA was associated with severe polymalformation syndrome. Trisomy 18 was diagnosed by DNA analysis post abortem. In the third case ARSA was associated with an unilateral cleft lip and cleft palate. Abnormalities of the fetal karyotype and Di George syndrome were excluded by amniocentesis. The fourth case was associated with a single umbilical artery without any structural and chromosomal abnormalities. The pregnancy had a favourable perinatal outcome at term., Conclusion: Visualization of RSA and prenatal diagnosis of ARSA in the second trimester is relatively easy in experienced hands. The examination slightly prolongs the fetal morphology scan. Since there is an obvious association between ARSA and chromosomal fetal abnormalities, implementation of its sonographic evaluation in the protocol of fetal echocardiography in the second trimester is strongly recommended.

Objective: The aberrant right subclavian artery is a malformation of the aortic arch present at less than 2 % of the individuals in the general population. This incidence is higher in trisomy 21, making it possible use the aberrant right subclavian artery as a prenatal marker of trisomy 21., Material and Methods: This work, which relates to a series of 11,479 consecutive fetal autopsies aims to measure the force of association between the aberrant right subclavian artery and trisomy 21, to confront our results with the sonographic series previously published and to contribute to assess the place that can have this sign in the echographic screening and the fetopathologic diagnosis of trisomy 21., Results: The isolated presence of an aberrant right subclavian artery does not represent an argument sufficient for the indication of a karyotype. But the detection of this anomaly must make pay a special attention in search of other associated signs., Conclusion: On the results of this study, the aberrant right subclavian artery has to be considered as a part of the spectrum not only of trisomy 21, but also of many other congenital syndromes., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

A 68-year-old man, having right-sided aortic arch (RAA), underwent thoracic endovascular aortic repair (TEVAR) to treat Stanford B type dissection with the chimney technique to extend the proximal landing zone between his right subclavian artery and left subclavian artery (LSA). Six-month follow-up showed that there was sufficient cerebral infusion and positive aortic remodeling, and the chimney stents were patent. The results show the safety and effectiveness of chimney TEVAR for Stanford type B dissection in patients having RAA with an aberrant LSA who have no sufficient proximal fixation zone., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: Presentation of our own, preliminary experiences in the assessment of the right subclavian artery's (RSA) position during the second trimester scan., Material and Methods: Since January 2012 our center has started to conduct the assessment of the position of the right subclavian artery in the second trimester scan. Patients who were diagnosed with an aberrant right subclavian artery (ARSA) were referred to invasive method of prenatal diagnosis. Abnormal karyotype and microdeletion 22q11 were analyzed. Detailed echocardiography was conducted in each case., Results: Between January 2012 and September 2013 we diagnosed 19 cases of ARSA. There were three cases of congenital heart defect (15.8%; 3/19) (ventricular septal defect--VSD, n=2, atrioventricular septal defect--AVSD, n=1). Two out of 17 cases showed an abnormal karyotype (11.8%; 2/17)--46,XY del(5) (q15q31) and 47,XX+18. No 22q11.2 deletions were observed. Two patients did not consent to invasive methods of prenatal diagnosis., Conclusions: The position of the right subclavian artery (RSA) should be routinely assessed during the second trimester of ultrasound screening. The presence of ARSA increases the risk for abnormal karyotype in the fetus and therefore, all patients who are diagnosed with ARSA should be referred to the reference center.

Objectives: First, to estimate the prevalence of fetal aberrant right subclavian artery (ARSA) in our population and its association with Down syndrome. Second, to determine the feasibility of ultrasound to visualize ARSA in the three planes. Finally, to carry out a systematic review of the literature on the performance of second-trimester ARSA to identify fetuses with Down syndrome., Methods: ARSA was assessed by ultrasound in the axial plane and confirmed in the longitudinal and coronal planes during the second half of pregnancy in women attending our unit (from February 2011 to December 2012). A search of diagnostic tests for the assessment of ARSA was carried out in international databases. Relevant studies were subjected to a critical reading, and meta-analysis was performed with Meta-DiSc., Results: Of the 8781 fetuses in our population (mean gestational age: 24 ± 5.4 weeks), 22 had Down syndrome. ARSA was detected in the axial view in 60 cases (0.7%) and confirmed in the coronal view in 96.7% and in the longitudinal view in 6.7% (P < 0.001). Seven cases with ARSA had Down syndrome and all were in the non-isolated-ARSA group. The estimates of positive likelihood ratio (LR) were 0 for isolated ARSA and 199 (95% CI, 88.9-445.2) for non-isolated ARSA. In the systematic review, six studies were selected for quantitative synthesis. The pooled estimates of positive and negative LRs for global ARSA were, respectively, 35.3 (95% CI, 24.4-51.1) and 0.75 (95% CI, 0.64-0.87). For isolated ARSA, the positive and negative LRs were 0 (95% CI, 0.0-14.7) and 0.98 (95% CI, 0.94-1.02), respectively., Conclusions: The prevalence of ARSA seems close to 1%. The coronal plane is the most suitable for its confirmation after detection in the axial plane. Detection of isolated or non-isolated ARSA should guide decisions about karyotyping given that isolated ARSA shows a weak association with Down syndrome., (Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.)

We describe the case of a 74-year-old man who underwent a hybrid open and endovascular approach for repair of dissecting thoracic aortic aneurysm of a right aortic arch with aberrant left subclavian artery arising from a Kommerell's diverticulum. Total debranching using a tailored quadrifurcated graft and thoracic endovascular aneurysm repair for the transverse aortic lesion were performed. The procedures were successfully accomplished with complete exclusion of the aneurysm. This hybrid procedure for complex aortic arch disease may reduce perioperative complications compared to challenging conventional open approaches., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

We report an endovascular approach that used to treat a symptomatic extracranial vertebral artery aneurysm associated to an asymptomatic aberrant right subclavian artery aneurysm. A 54-year-old man presented with neck pain, vertigo and loss of balance. The computed tomography (CT) scan demonstrated a left extracranial vertebral artery aneurysm that compressed and eroded the C5 vertebra associated to an aberrant right subclavian artery aneurysm. Endovascular exclusion of the vertebral aneurysm using a covered stent and a hybrid treatment of the aberrant subclavian artery aneurysm were performed. The 13th month follow-up CT scan confirmed the stent-grafts and supra-aortic vessels patency. The endovascular treatment represents a good option for these complex pathologies with excellent immediate results, reduces the complication rate and the hospital stay if compared to open repair. Long-term follow-up is necessary. To our knowledge this is a unique case in the literature.

We report a case involving a ruptured acute type B aortic dissection originating from an aberrant right subclavian artery (ARSA). A thoracic stent-graft was deployed in the distal arch close to the origin of the ARSA; the entry site at the origin of the ARSA was embolized with metallic coils. Perfusion of the left subclavian artery was preserved without a surgical bypass by using a chimney graft. This procedure is a feasible and less invasive treatment for high-risk sternotomy patients and is an effective strategy for acute aortic dissections involving an ARSA., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We herein report the case of a 22-year-old Caucasian man with known vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, cardiac defects, renal and limb anomalies (VACTERL) association who presented with a headache and vertigo following the sudden and temporary loss of consciousness while attending a concert four days before admission to the hospital. On a physical examination, the following findings were found: a low body height, low-set ears, thoracic scoliosis and a mild holosystolic heart murmur. A neurosonological examination revealed a partial subclavian steal phenomenon. CT angiography of the neck vessels and aortic arch confirmed an anomalous right subclavian artery -known as the lusorian artery. Further studies are warranted in patients with VACTERL in order to identify possible links between the prevalence of an aberrant right subclavian artery (lusorian artery) and possible congenital subclavian steal syndrome or dysphagia lusoria. In addition, duplex ultrasound of the carotid and vertebral arteries may be performed as part of screening examinations in patients with congenital syndromes.

Acute traumatic aortic injury is a potentially lethal condition with most patients die at the scene of the accidents. Rapid deceleration due to motor vehicle accidents is the commonest mechanism of injury. These injuries can be successfully repaired in the few patients who survive the initial trauma if proper diagnosis and rapid treatment are provided. The occurrence of acute traumatic aortic injury in patients with congenital abnormality of the aortic arch has been rarely reported; however, it renders the diagnosis and treatment more difficult. In this paper, we describe an extremely rare case of aortic injury in a young patient who had a right sided aortic arch with rupture of an aberrant left subclavian artery. The patient was suspected to have a Kommerell's diverticulum in the aberrant subclavian artery origin. This injury resulted in an unusually huge pseudoaneurysm involving part of the mediastinum and extending into the neck. Unfortunately; patient succumbed in spite of surgical intervention.