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Aortic arch anomalies encompass a diverse spectrum of conditions. Elucidating the prevalence of these anomalies, their impact on patient wellbeing, and the most effective diagnostic tools are crucial steps in ensuring optimal patient care. This paper aims to explore the various presentations of aortic arch anomalies, emphasizing the remarkable utility of computed tomography (CT) angiography in their definitive diagnosis and characterization. We conducted a retrospective study on patients who were submitted to the CT angiography of the thoracic aorta or supra-aortic trunks, or the contrast-enhanced CT scans of the thorax and/or cervical region between January 2021 and February 2024 in our Hospital. Out of the total of 2350 patients, 18 were diagnosed with aortic arch anomalies, with an average age of approximately 55 years. The aortic arch anomalies identified in the study were as follows: left aortic arch with the aberrant origin of the right subclavian artery, right aortic arch (types I and II), double aortic arch, aortic coarctation, aortic pseudocoarctation, and ductus diverticulum. Although often asymptomatic, aortic arch anomalies require recognition and CT using advanced post-processing techniques is the optimal diagnostic method with the ability to also identify other associated cardiac or vascular malformations. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to develop and validate a convolutional neural network (CNN) that automatically detects an aberrant right subclavian artery (ARSA) on preoperative computed tomography (CT) for thyroid cancer evaluation. Materials and Methods: A total of 556 CT with ARSA and 312 CT with normal aortic arch from one institution were used as the training set for model development. A deep learning model for the classification of patch images for ARSA was developed using two-dimension CNN from EfficientNet. The diagnostic performance of our model was evaluated using external test sets (112 and 126 CT) from two institutions. The performance of the model was compared with that of radiologists for detecting ARSA using an independent dataset of 1683 consecutive neck CT. Results: The performance of the model was achieved using two external datasets with an area under the curve of 0.97 and 0.99, and accuracy of 97% and 99%, respectively. In the temporal validation set, which included a total of 20 patients with ARSA and 1663 patients without ARSA, radiologists overlooked 13 ARSA cases. In contrast, the CNN model successfully detected all the 20 patients with ARSA. Conclusion: We developed a CNN-based deep learning model that detects ARSA using CT. Our model showed high performance in the multicenter validation. [ABSTRACT FROM AUTHOR]

Aberrant right subclavian artery (ARSA) causing dysphagia, the so-called "dysphagia lusoria", is a frequent embryologic anomaly of the aortic arch. In symptomatic patients, studies report several management options including surgical, hybrid, and totally endovascular strategies. Hybrid techniques have the advantage of no chest opening with reduced morbidity, but the problem of the ARSA stump causing recurrent or persistent dysphagia remains challenging in some cases. We conducted a literature review on the management strategies of ARSA and presented the case of a 72-year-old female patient with ARSA and dysphagia managed with thoracic endovascular repair of the aorta (TEVAR) and bilateral carotid–subclavian artery bypass. This technique was chosen because of the severe calcifications at the level of ARSA origin that would make surgical ligation difficult, or if an occluder device not suitable. We think that a patient-tailored approach should be considered in cases of dysphagia lusoria, considering that a multitude of strategies are reported. [ABSTRACT FROM AUTHOR]

OBJECTIVES Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention. METHODS All surgical patients with IAA (2001–2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention. RESULTS Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5–9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n  = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68–5.80), IAA-B: 4.60 (3.92–5.50), P  = 0.007], AAb [AAb: 4.00 (3.70–5.04) versus none: 5.15 (4.30–5.68), P  = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70–5.02) versus other: 5.00 (4.30–5.68), P  = 0.002]. The likelihood of LVOTO was higher among AAb [ N  = 6 (25%) vs N  = 1 (2%), P  = 0.004] and IAA-B + AAbS [ N  = 1 (2%) vs N  = 6 (30%), P  = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P  = 0.11). CONCLUSIONS IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair. [ABSTRACT FROM AUTHOR]

Dysphagia lusoria occurs due to compression of the esophagus as an aberrant right subclavian artery (ARSA) crosses the mediastinum. Surgical management includes open, hybrid, and endovascular techniques, with no consensus gold standard. There are few reports of robotic-assisted ARSA resection. We describe the innovative technique and outcomes for two patients who successfully underwent robotic-assisted transthoracic resection of an ARSA after right carotid–subclavian bypass for dysphagia lusoria. Both patients experienced improvement or resolution of their dysphagia and no major complications. In select patients with a noncalcified origin of the ARSA without aneurysmal degeneration, the robotic-assisted approach represents a viable option.

Background: Endovascular repair of the thoracic aorta (TEVAR) is the preferred option for the treatment of the distal arch and descending thoracic aorta. Fenestrated and branched TEVAR have become an option to treat pathologies of the aortic arch, avoiding sternotomy and cardiopulmonary arrest as well as total surgical debranching. We describe here the case of a symptomatic patient with an arteria lusoria aneurysm associated with Kommerel diverticulum who underwent total endovascular repair with a triple-branched TEVAR. Case Report: A 66-year-old male patient was treated for a symptomatic arteria lusoria artery associated with a Kommerel diverticulum, resulting in difficulty swallowing and choking. We used a custom-made triple inner-branch endograft (Cook Medical, Bloomington, Indiana) following implantation of a right-sided carotid-subclavian (C-S) bypass. The C-S bypass occluded in the interval time between the 2 procedures and required recanalization and stent-graft placement during the aortic arch procedure. The arteria lusoria was embolized with a vascular plug. No complications occurred and postoperative tomography showed exclusion and thrombosis of the Kommerel diverticulum and perfusion of the supra-aortic vessels. Conclusions: Treatment of arteria lusoria aneurysms can be performed with total endovascular arch inner-branch repair, avoiding increased risk of morbidity and mortality caused by open or hybrid procedures. [ABSTRACT FROM AUTHOR]

Kommerell diverticulum refers to the bulbous configuration of the origin of an aberrant left subclavian artery. It was originally described as a diverticular out - pouching at the origin of an aberrant right subclavian artery with a left-sided aortic arch. This is alsoassociated with a right - sided aortic arch. We report a case of a 66 year old female who presented with chronic cough. Clinico-radiological evaluation demonstrated anaberrant right subclavian artery with Kommerelldiverticulum. There was focal compressionof the esophagus and proximal dilatation. This probably caused regurgitation of fluid leading to persistent cough. Symptoms improved with medical management and she is under regular follow-up. [ABSTRACT FROM AUTHOR]

Aortic arch anomalies encompass a diverse spectrum of conditions. Elucidating the prevalence of these anomalies, their impact on patient wellbeing, and the most effective diagnostic tools are crucial steps in ensuring optimal patient care. This paper aims to explore the various presentations of aortic arch anomalies, emphasizing the remarkable utility of computed tomography (CT) angiography in their definitive diagnosis and characterization. We conducted a retrospective study on patients who were submitted to the CT angiography of the thoracic aorta or supra-aortic trunks, or the contrast-enhanced CT scans of the thorax and/or cervical region between January 2021 and February 2024 in our Hospital. Out of the total of 2350 patients, 18 were diagnosed with aortic arch anomalies, with an average age of approximately 55 years. The aortic arch anomalies identified in the study were as follows: left aortic arch with the aberrant origin of the right subclavian artery, right aortic arch (types I and II), double aortic arch, aortic coarctation, aortic pseudocoarctation, and ductus diverticulum. Although often asymptomatic, aortic arch anomalies require recognition and CT using advanced post-processing techniques is the optimal diagnostic method with the ability to also identify other associated cardiac or vascular malformations.

Background: Kommerell's diverticulum (KD) with a right aortic arch (RAA) and aberrant left subclavian artery (aLSCA) is a rare congenital anomaly of the aortic arch. Treatment is not well defined due to its uncommon presentation, with rupture and dissection risk rates of up to 53%. Case summary: A 54-year-old male with a history of chronic obstructive pulmonary disease (COPD) and hypertension presented with difficulty breathing during exercise without dysphagia. Follow-up computerized tomography angiogram (CTA) revealed the presence of a RAA and aLSCA arising from the descending thoracic aorta with an adjacent 58 × 41-mm KD and tracheal and esophageal displacement. Due to the size of the KD, risk of rupture, unsuitable anatomy for total endovascular aortic repair (EVAR), and high COPD burden, the patient was planned to undergo a hybrid surgical repair. Left common carotid (LCCA) artery to LSCA bypass, full aortic debranching, LSCA embolization and percutaneous thoracic endovascular aortic repair (TEVAR) were performed. Successful device position and exclusion of the diverticulum and aneurysmal aorta were observed after completion thoracic aortogram. 18-month follow-up CTA demonstrated patency of the LSCA to LCCA bypass graft and arch vessel branches, as well as stable exclusion of the KD. Persistence of a type II endoleak originated at the right first posterior intercostal artery has been noted and is being followed conservatively since no sac growth has occurred. Conclusion: We highlight the presence of a KD with RAA and aberrant subclavian artery, a rare congenital anatomic variation of the aortic arch with complex anatomy. Surgical planning must be individualized according to comorbidities and anatomical variations identified on imaging and 3D reconstructions. [ABSTRACT FROM AUTHOR]

Kommerell's diverticulum in association with left or right aberrant subclavian arteries is a rare finding and is challenging to treat. Contemporary surgical and endovascular techniques provide a broad arsenal of possible treatments. Imaging techniques and modeling technology allow a more personalized strategy for each patient. In this case, we present a symptomatic patient with a Kommerell's diverticulum and a left aberrant subclavian artery complicated by proximal stenosis and poststenotic aneurysm. A hybrid technique using a single-branched thoracic stent-graft (Castor, MicroPort Medical, Shanghai, China) in combination with a surgical left subclavian-carotid bypass and endovascular occlusion of the poststenotic aneurysm using a vascular plug device (Amplatzer Vascular Plug, Abbott, Chicago, United States) was performed. This approach was planned and facilitated by the use of a 3D model. Alternative treatment options and the strengths of this approach are briefly reviewed and discussed.

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Background: Many anatomical variations of the branching pattern of the aortic arch have been documented in the literature. These find their origin in alterations to the embryological development of the arch and have significant implications for surgical and radiological interventions. Methods: Embase and Medline database searches were carried out in June 2021 and identified 1197 articles, of which 24 were considered eligible. Results: Twenty-eight variations were found. The prevalence of the six main variations found is as follows: normal configuration (61.2–92.59%); bovine arch type 1 (4.95–31.2%); bovine arch type 2 (0.04–24%); origin of left vertebral artery (0.17–15.3%); aberrant right subclavian artery (0.08–3.33%); thyroid ima artery (0.08–2%). Concomitant variations present in conjunction with these variations are also documented, as were other variations which could not be classified into these six groups. Conclusions: Anatomical variations in the branching pattern of the aortic arch are present in over one-third of individuals in some populations. These are important pre- and intra-operatively during thoracic, neck and thyroid surgery. A greater effort should be employed to construct an official classification to facilitate greater understanding among clinicians. [ABSTRACT FROM AUTHOR]

Dysphagia is a common condition in clinical practice; however, an unusual type of dysphagia due to compression of the esophagus by an abnormal right subclavian artery may be discovered in a rare subset of patients. The prognosis and treatment will depend on the severity of the symptoms and the compromise of surrounding structures. We present the case of an 18-year-old female who presented with gradually progressive dysphagia. At first, it was treated as gastroesophageal reflux disease; nonetheless, the dysphagia became severe, and after a thorough evaluation, an aberrant right subclavian artery that compressed the esophagus was discovered along with a truncus bicaroticus. She was successfully treated with surgery without any complications. On follow-ups, she's doing well. [ABSTRACT FROM AUTHOR]

Introduction: The management of Kommerell's Diverticulum (KD) has evolved from open surgical resection and graft replacement of the aorta, to endovascular repair in asymptomatic patients due to its recognized possible sequelae – aortic rupture and dissection. Despite these technical advances, standard indications for intervention and treatment algorithms remain unclear. We will present our single-center experience in the treatment of KD, supporting a multidisciplinary endovascular-first approach. Methods: All patients who underwent thoracic endovascular aortic repair (TEVAR) for KD between 2017 and 2020 were retrospectively identified from a prospectively maintained institutional surgery database. Chart review was used to characterize presenting symptoms, interventions, technical results, and complications. Revascularization was performed using carotid-axillary bypass. Routine endovascular subclavian artery occlusion was employed to eliminate retrograde diverticulum perfusion and avoid open ligation. Results: 8 patients were identified, including 6 females and 2 males between the ages of 44-76. Patients presented with dysphagia (n = 3), acute embolic stroke (n = 1), transient ischemic attack (TIA) (n = 1), upper extremity embolization (n = 1), and acute type B aortic dissection (n = 1). One patient had a prior incomplete open repair that was successfully treated endovascularly. Another patient had a mediastinal neoplasm infiltrating an incidental aberrant subclavian artery and KD. All cases had symptomatic improvement and successful endovascular repair as demonstrated on post-operative imaging. Perioperative complications included percutaneous access site pseudoaneurysm (n = 2), stroke (n = 1), and subclavian artery rupture immediately recognized and treated (n = 1). There was no perioperative mortality. Conclusion: Endovascular techniques have resulted in technical success and symptomatic improvement for KD without open thoracotomy or sternotomy. Significant rates of endovascular complications and paucity of long-term durability data should be considered. Until formal criteria for repair are established, early application of TEVAR using a consistent multi-specialty approach may mitigate the risk of unpredictable aortic complications in these patients while avoiding the accepted morbidity and mortality of open surgery. [ABSTRACT FROM AUTHOR]

Abstract Anomalous aortic origin of the right coronary artery is a rare disease. Although there are various reports on its treatment, the method of the surgical approach is still controversial. Here, we present a rare case of a 17 year-old man who had an anomalous aortic origin of the right coronary artery with an aberrant right subclavian artery. As a treatment, he underwent reimplantation of the right coronary artery. The surgical approach for the anomalous aortic origin of the right coronary artery should be selected by considering the age of the patient and size of the right coronary artery.

Aortic abnormalities account for 15 to 20% of all congenital cardiovascular diseases. The purpose of this pictorial essay is to illustrate various congenital anomalies of the aortic arch, which influence patient management and outcome.

Intramural Hematoma (IMH) forms part of the acute aortic syndrome, aortic dissection, and penetrating aortic ulcer. It is a life-threatening aortic disease that warrants prompt diagnosis and management. Like aortic dissections, it is classified using the Stanford classification system as type A (proximal to the origin of the left subclavian artery) and type B (distal to the origin of the left subclavian artery). Patients with type A IMH is generally managed surgically, and uncomplicated type B IMH is managed medically. The right subclavian artery arises typically from the brachiocephalic trunk. Aberrant right subclavian arteries (ARSA) are rare and derive directly from the aortic arch distal to the left subclavian artery. In this case report, a 73-year-old female presented with right-sided chest pain and shortness of breath. On examination, her heart rate was 100 bpm and blood Pressure was 185/85 and her ECG showed sinus rhythm. Following a CT scan, she was found to have a type B Aortic IMH with an ARSA. She was medically managed with vigorous blood pressure control. After a period of intravenous blood pressure treatment, she was treated with oral medication. Her subsequent CT scan showed that the hematoma was stable. She was followed up with MRI scanning 1 year later, which showed complete healing of the aorta with no changes in diameter. This case illustrates the importance of strict blood pressure management and follow-up imaging in patients presenting with type B IMH. It is important to monitor these patients regularly and where blood pressure control alone is not sufficient, further intervention may be required. Even though the complete resolution may be achieved as in this case, these patients will need to be kept under surveillance with repeated scans to monitor for any changes.

Aberrant subclavian arteries (aSCAs), with or without aortic pathology, are uncommon. The purpose of the present study was to review our experience with the surgical management of aSCA. We performed a retrospective review of patients who had undergone surgery for an aSCA between 1996 and 2020. Symptomatic and asymptomatic patients were included. The primary end points were ≤30-day and late mortality. The secondary end points were ≤30-day complications, graft patency, and reinterventions. A total of 46 symptomatic and 3 asymptomatic patients with aSCA had undergone surgery (31 females [62%]; median age, 45 years). An aberrant right subclavian artery was present in 38 (78%) and an aberrant left subclavian artery in 11 patients (22%). Of the 49 patients, 41 (84%) had had a Kommerell diverticulum (KD) and 11 (22%) had had a concomitant distal arch or proximal descending thoracic aortic aneurysm. Symptoms included dysphagia (56%), dyspnea (27%), odynophagia (20%), and upper extremity exertional fatigue (16%). Five patients (10%) had required emergency surgery. The aSCA had been treated by transposition in 32, a carotid to subclavian bypass in 11, and an ascending aorta to subclavian bypass in 6. The KD was treated by resection and oversewing in 19 patients (39%). Fifteen patients (31%) had required distal arch or proximal descending thoracic aortic replacement for concomitant aortic disease and/or KD treatment. Thoracic endovascular aortic repair was used to exclude the KD in six patients (12%). Seven patients (14%) had undergone only bypass or transposition. The 30-day complications included one death from pulseless electrical activity arrest secondary to massive pulmonary embolism. The 30-day major complications (14%) included acute respiratory failure in three, early mortality in one, stroke in one, non–ST-elevation myocardial infarction in one, and temporary dialysis in one patient. The other complications included chylothorax/lymphocele (n = 5; 10%), acute kidney injury (n = 2; 4%), pneumonia (n = 2; 4%), wound infection (n = 2; 4%), atrial fibrillation (n = 2; 4%), Horner syndrome (n = 2; 4%), lower extremity acute limb ischemia (n = 1; 2%), and left recurrent laryngeal nerve injury (n = 1; 2%). At a median follow-up of 53 months (range, 1-230 months), 40 patients (82%) had had complete symptom relief and 9 (18%) had experienced improvement. Six patients had died at a median of 157 months; the deaths were not procedure or aortic related. The primary patency was 98%. Reintervention at ≤30 days had been required for two patients (4%) for ligation of lymphatic vessels and bilateral lower extremity fasciotomy after proximal descending thoracic aorta replacement. One patient had required late explantation of an infected and occluded carotid to subclavian bypass graft, which was treated by cryopreserved allograft replacement. Surgical treatment of the aSCA can be accomplished with low major morbidity and mortality with excellent primary patency and symptom relief. [ABSTRACT FROM AUTHOR]

An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become aneurysmal and result in dissection, involving Kommerell's diverticulum when present and the aorta. Data of its significance in genetic arteriopathies are not available. The purpose of this study was to assess the prevalence and complications of ASA in gene-positive and -negative nonatherosclerotic arteriopathies. The series includes 1,418 consecutive patients with gene-positive (n = 854) and gene-negative arteriopathies (n = 564) diagnosed as part of institutional work-up for nonatherosclerotic syndromic and nonsyndromic arteriopathies. Comprehensive evaluation includes genetic counseling, next-generation sequencing multigene testing, cardiovascular and multidisciplinary assessment, and whole-body computed tomography angiography. ASA was found in 34 of 1,418 cases (2.4%), with a similar prevalence in gene-positive (n = 21 of 854, 2.5%) and gene-negative (n = 13 of 564, 2.3%) arteriopathies. Of the former 21 patients, 14 had Marfan syndrome, 5 had Loeys-Dietz syndrome, 1 had type-IV Ehlers-Danlos syndrome, and 1 had periventricular heterotopia type 1. ASA did not segregate with genetic defects. Dissection occurred in 5 of 21 patients with genetic arteriopathies (23.8%; 2 Marfan syndrome and 3 Loeys-Dietz syndrome), all with associated Kommerell's diverticulum. No dissections occurred in gene-negative patients. At baseline, none of the 5 patients with ASA dissection fulfilled criteria for elective repair according to guidelines. The risk of complications of ASA is higher in patients with genetic arteriopathies and is difficult to predict. In these diseases, imaging of the supra-aortic trunks should enter baseline investigations. Determination of precise indications for repair can prevent unexpected acute events such as those described. [Display omitted] [ABSTRACT FROM AUTHOR]

Parathyroid adenomas are responsible for 80% to 85% of cases of primary hyperparathyroidism. Giant parathyroid adenomas are a rare type of parathyroid adenoma defined as weighing >3.5 grams. Although giant parathyroid adenomas are rare entities whose clinical presentation may not be atypical, their surgical management can be challenging, especially in the setting of anatomical variants. We present here a case of a 29-year-old female with a 37-gram giant adenoma which was diagnosed after presentation with recurrent severe headaches. The presentation was also unique in that it was associated with a right aberrant subclavian artery and nonrecurrent laryngeal nerve. [ABSTRACT FROM AUTHOR]

Background: Kommerell's diverticulum with Stanford type A aortic dissection is a relatively rare condition. There is currently no standard treatment strategy for Kommerell's diverticulum. We herein report our surgical strategy with a review of literature for a case of Kommerell's diverticulum that developed acute aortic dissection. Case presentation: A 54-year-old woman was referred to our hospital after being diagnosed with Stanford type A acute aortic dissection at the hospital where she was admitted for chest and back pain. Contrast-enhanced computed tomography showed a right and left common carotid and left subclavian artery branching from the aortic arch; Kommerell's diverticulum and an aberrant right subclavian artery (RSCA) arising from it were noted in the distal arch. The contralateral aortic wall where Kommerell's diverticulum was located was the entry, resulting in aortic dissection, with retrograde dissection extending into the ascending aorta. Partial thrombosis was detected in the false lumen of the ascending aorta. The dissection extended from the ascending aorta to the common iliac artery. In addition, the thrombosed false lumen in the left common iliac artery was considered to have caused dynamic obstruction due to compression and narrowing of the true lumen. Based on these findings, total arch replacement with reconstruction of the 4-branched vessels in the arch, including an aberrant RSCA, the frozen elephant trunk technique, and femoro-femoral bypass for malperfusion of the left lower extremity were performed with deep hypothermic circulatory arrest. An artificial graft was anastomosed to the right axillary artery, which was used as the blood supply route for cardiopulmonary bypass. After cooling to hypothermia, the central portion of the aberrant right subclavian artery was clipped on the right side of the trachea. Thus, cerebral blood flow was maintained via the right vertebral artery, the aortic arch was opened, and cannulas for selective cerebral perfusion were inserted into the right common carotid artery, the left common carotid artery, and the left subclavian artery. After each vessel was reconstructed, the graft that had been anastomosed to the right axillary artery was anastomosed to the graft used for reconstruction of the right common carotid artery. The patient's postoperative course was generally good. Conclusion: The strategy used by us effectively created transient occlusion of the origin of Kommerell's diverticulum and the primary entry. Our results indicate that this method is feasible and effective for cases in which in situ reconstruction of the branch may be challenging.

Objectives: The aberrant subclavian artery is among the congenital vascular anomalies of the aortic arch. Aberrant subclavian arteries can be seen in different types and on the right or left side. Aneurysmal dilatation, named Kommerell's diverticulum, may be seen in the descending thoracic aorta at the origin of the right aberrant subclavian artery. The aim of this study aimed to present our experience with aberrant subclavian artery pathology. Materials and Methods: This was a retrospective review of our experience from two instutitions with 14 patients who had an aberrant subclavian artery between 2015-2021. Seven patients (62.5%) were male, and the median age at the time of surgery was 38.4 months (range: 6 months to 75 years). Patients were either asymptomatic, incidentally diagnosed, or presented with dyspnea and/or dysphagia lusoria. Results: Asymptomatic patients were not interfered. There was no early or late mortality in 6 patients who underwent surgery. Three (50%) patients underwent aberrant right subclavian artery repair through right thoracotomy, two received a hybrid approach due to Kommerell's diverticulum, and one patient underwent aberrant left subclavian artery repair from left thoracotomy. The median postoperative duration of the intensive care unit and hospital stays were 1 and 6 days, respectively. Patients were followed for a median of 3.6 years (range, 1.2-6.8 years) after intervention. No endoleak was observed in the follow-up of patients who underwent endovascular repair. Conclusion: The data suggest that symptomatic aberrant subclavian artery pathology can be treated safely in selected patients with good early and mid-term results. The right or left side of the aberrant subclavian artery may change the surgical approach. Patients with Kommerell's diverticulum with a right aberrant subclavian artery can be treated with a hybrid approach using endovascular techniques. [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of the right coronary artery is a rare disease. Although there are various reports on its treatment, the method of the surgical approach is still controversial. Here, we present a rare case of a 17 year-old man who had an anomalous aortic origin of the right coronary artery with an aberrant right subclavian artery. As a treatment, he underwent reimplantation of the right coronary artery. The surgical approach for the anomalous aortic origin of the right coronary artery should be selected by considering the age of the patient and size of the right coronary artery. [ABSTRACT FROM AUTHOR]

A female cadaver fixated with 10% formalin solution was dissected during a routine undergraduate anatomy class. It was found that both the right and left carotid arteries arose from a bi-carotid trunk as the first branch of the aortic arch. The bi-carotid trunk was followed by the left subclavian artery. The aberrant right subclavian artery (the last branch) had a retro-oesophageal course. These variations were associated with the linguofacial trunk bilaterally. The group of variant anatomy of vessels encompassing the bi-carotid trunk, aberrant right subclavian artery, and the linguofacial trunk is extremely rare. A similar case has not been reported yet in the literature. The anatomic and morphologic variations of the aortic arch and its branches are important for diagnostic and surgical procedures in the thorax and neck region. Thoracovascular surgeons and interventional radiologists should be aware of these anomalies during head and neck surgery, aortic instrumentation, and four-vessel angiography. [ABSTRACT FROM AUTHOR]

Purpose of Review: Vascular rings are congenital malformations resulting from abnormal development of the great vessels, with the consequent encircling and compression of the trachea, esophagus, or both. We conducted a review of the current literature to identify the different management strategies that can be implemented based on the prognosis of each of these anomalies. Recent Findings: Although most vascular rings occur in isolation, they can also be associated with other congenital cardiac and/or respiratory diseases; therefore, thorough investigation is necessary before definitive surgical repair. Clinical presentation varies from asymptomatic to severe, with both respiratory and digestive symptoms. Although early surgical results are acceptable, the long-term outcome is variable; therefore, there is still controversy regarding the appropriate timing of treatment. This is especially true with regard to the Kommerell diverticulum (KD) and in patients without symptoms at the time of initial surgical evaluation. Summary: As more sophisticated diagnostic tools have become available and more studies on adults affected by this condition have been published, understanding of this condition and its additional clinical implications has grown and appears to be tilting management toward earlier intervention. [ABSTRACT FROM AUTHOR]

Aortic abnormalities account for 15 to 20% of all congenital cardiovascular diseases. The purpose of this pictorial essay is to illustrate various congenital anomalies of the aortic arch, which influence patient management and outcome. [ABSTRACT FROM AUTHOR]

Background: Congenital aortic arch anomalies are commonly encountered during neurointerventional procedures. While some anomalies are identified at an early age, many are incidentally discovered later in adulthood during endovascular evaluations or interventions. Proper understanding of the normal arch anatomy and its variants is pivotal to safely navigate normal aortic arch branches and to negotiate the catheter through anomalies during neurointerventional procedures. This is particularly relevant in the increasingly "transradial first" culture of neurointerventional surgery. Moreover, some of these anomalies have a peculiar predilection for complications including aneurysm formation, dissection, and rupture during the procedure. Therefore, an understanding of these anomalies, their underlying embryological basis and associations, and pattern of circulation will help endovascular neurosurgeons and interventional radiologists navigate with confidence and consider relevant pathologic associations that may inform risk of cerebrovascular disease. Methods: Here, we present a brief review of the basic embryology of the common anomalies of the aortic arch along with their neurological significances and discuss, through illustrative cases, the association of aortic arch anomalies with cerebral vascular pathology. Conclusions: Understanding the aortic arch anomalies and its embryological basis is essential to safely navigate the cerebral vascular system during neurointerventional surgeries. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to develop and validate a convolutional neural network (CNN) that automatically detects an aberrant right subclavian artery (ARSA) on preoperative computed tomography (CT) for thyroid cancer evaluation., Materials and Methods: A total of 556 CT with ARSA and 312 CT with normal aortic arch from one institution were used as the training set for model development. A deep learning model for the classification of patch images for ARSA was developed using two-dimension CNN from EfficientNet. The diagnostic performance of our model was evaluated using external test sets (112 and 126 CT) from two institutions. The performance of the model was compared with that of radiologists for detecting ARSA using an independent dataset of 1683 consecutive neck CT., Results: The performance of the model was achieved using two external datasets with an area under the curve of 0.97 and 0.99, and accuracy of 97% and 99%, respectively. In the temporal validation set, which included a total of 20 patients with ARSA and 1663 patients without ARSA, radiologists overlooked 13 ARSA cases. In contrast, the CNN model successfully detected all the 20 patients with ARSA., Conclusion: We developed a CNN-based deep learning model that detects ARSA using CT. Our model showed high performance in the multicenter validation., Competing Interests: The authors have no potential conflicts of interest to disclose., (© Copyright: Yonsei University College of Medicine 2024.)

The arteria lusoria or retroesophageal right subclavian artery is the most common malformation of the aortic arch. It may be discovered with some symptoms of airway and/or esophageal compression, such as dyspnea or dysphagia, but in most cases it is an asymptomatic pathology. We report a case of a 3 months old patient diagnosed with Down syndrome who was admitted for pulmonary infection with incidental finding of retrooesophageal right subclavian artery., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

To synthesise and present all available evidence regarding the surgical treatment of patients with aberrant subclavian artery (aSA) pathologies during the last 30 years. The study also aimed to create a checklist of suggested reporting items to help increase reporting homogeneity. A systematic search of medical databases was performed to identify all studies published between 1990 and 2020 reporting on the outcomes of patients undergoing surgery for an aSA pathology. Patients were divided into two groups; Group A included patients undergoing open or hybrid surgery through sternotomy/thoracotomy and Group B included patients undergoing endovascular or hybrid treatment without chest access. The % crude rates (CR) of all variables of interest were calculated. A proportion meta-analysis was performed reporting pooled rates with 95% confidence intervals (CIs). Three hundred and fourteen studies were identified reporting on the surgical outcomes of 732 patients (60.1% males; 440/732). The quality of evidence was generally low, with 286 studies including fewer than four patients and 28 studies with five or more cases. Aberrant right subclavian artery (aRSA) in a left sided aortic arch was present in 71.4% of the patients and a Kommerell's diverticulum was present in 50.1% of the cases. A total of 68% of the patients were symptomatic on presentation, with the majority complaining of dysphagia (49.6%). Group A included 453 and Group B 279 patients. Studies reporting on five or more patients were included in the main meta-analysis. The pooled early mortality rate was 1.62% (95% CI 0.05% – 4.53%) in Group A and 1.96% (95% CI 0 – 6.34%) in Group B. Pooled rates of symptom relief were 99.52% (95% CI 92.05% – 100.00%) in Group A and 95.79% (95% CI 83.96% – 100.00%) in Group B. The surgical techniques used to treat aSA and aortic pathologies involving an aSA had remarkably low mortality rates and high clinical success, regardless of the technique used. [ABSTRACT FROM AUTHOR]

Isolation of the left subclavian artery is a rare anomaly associated with right aortic arch. We report a case in which this subclavian artery was supplying the pulmonary circulation in a patient with tetralogy of Fallot (TOF) which effectively acted as a natural systemic to pulmonary artery shunt. The patient had good room air saturation and no features of vertebrobasilar insufficiency or left upper limb ischemia. She underwent a total correction of TOF and ligation of the aberrant left subclavian artery. [ABSTRACT FROM AUTHOR]

We report a rare case of vascular ring due to a left cervical circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum. This is a very rare case of vascular ring in a newborn with stridor and stenosis of the left pulmonary artery. A cardiac catheterization and an angio-computed tomography scan were helpful to clarify the diagnosis. The surgical correction was performed with division of the arterial duct, reimplantation of the right subclavian artery, aortic and tracheal suspension, and pulmonary arterial plasty. The patient was asymptomatic at discharge and 1-year follow-up.

Lusory dysphagia (LD) is a rare disorder resulting from extrinsic vascular compression of the esophagus, is often associated with an aberrant right subclavian artery (ARSA). LD is characterized by abnormal development of the right fourth aortic arch during embryonic stages, leading to posterior coursing of the artery in 80 % of cases. Predominantly presenting as dysphagia, LD can occasionally manifest with laryngeal stridor or dyspnea, yet it often remains asymptomatic and incidentally discovered. We present a case of LD in a 24-year-old male with Down syndrome, experiencing prolonged dysphagia and recurrent aspiration episodes. Emergency upper gastrointestinal endoscopy revealed food impactation beyond the upper esophageal sphincter, and subsequent imaging studies confirmed an aberrant right subclavian artery causing extrinsic compression. LD is a rare condition. It is a challenging diagnosis often necessitating a combination of endoscopy, contrasted imaging and angiography. Treatment strategies vary, with conservative approaches for mild symptoms involving dietary modifications, and surgical intervention considered for persistent or severe cases. Recognizing LD in the context of long-standing dysphagia is crucial for accurate diagnosis and appropriate management. Further research is needed to establish standardized diagnostic and therapeutic guidelines for this uncommon condition. • Lusory dysphagia is a rare disorder resulting from extrinsic vascular compression of the esophagus. • It is commonly associated with the presence of an aberrant right subclavian artery (ARSA). • Dysphagia is the most common symptom. • Management options vary based on symptom severity. [ABSTRACT FROM AUTHOR]

The aberrant right subclavian artery (ARSA, arteria lusoria) is the most common intrathoracic vascular anomaly, affecting up to 2% of the population. However, aneurysms of congenital anomalies are extremely unusual and often present with dysphagia, dysphonia, or dyspnea due to compression of the surrounding structures. We report a case of an asymptomatic 57-year-old male with chronic kidney disease who was incidentally found to have a large aneurysm of the ARSA on preoperative computed tomography for laparoscopic nephrectomy. Surgery is unequivocally warranted as these aneurysms are associated with a high risk of complications, including thrombosis, embolism, and rupture. We debranched the ARSA, followed by anastomosis to the right carotid artery through a right neck incision. Subsequently, aneurysmal resection was performed through left thoracotomy. The patient had an uneventful postoperative recovery and was asymptomatic during the follow-up.

ObjectivesTo evaluate the in-hospital and later outcomes of thoracic endovascular aortic repair (TEVAR) for type B intramural hematoma (TBIMH) combined with an aberrant subclavian artery (aSCA).MethodsIn the period from January 2014 to December 2020, 12 patients diagnosed with TBIMH combined with aSCA and treated by TEVAR were enrolled in this retrospective cohort study, including 11 patients with the aberrant right subclavian artery (ARSA) and 1 with an aberrant left subclavian artery (ALSA). A handmade fenestrated stent-graft or chimney stent or hybrid repair was performed when the proximal landing zone was not enough.ResultsThe mean age of all the patients was 59.2 ± 7.6 years, and 66.7% of patients were men. There were 4 patients with Kommerell's diverticulum (KD). The procedures in all 12 patients were technically successful. There was one case each of postoperative delirium, renal impairment, and type IV endoleak after TEVAR. During follow-up, 1 patient died of acute pancreatitis 7 months after TEVAR. The overall survival at 1, 3, and 5 years for the patients was 90.9, 90.9, and 90.9%, respectively. KD was excluded in 2 patients, and the handmade fenestrated stent-graft was applied in the other 2 patients to preserve the blood flow of the aSCA. No neurological complications developed and no progression of KD was observed during the follow-up.ConclusionThoracic endovascular aortic repair for patients with aSCA and TBIMH is promising. When KD was combined, we could exclude KD or preserve the blood flow of aSCA with regular follow-up for the diverticulum according to the size of the KD.

Congenital Aortic arch malformations are rare in adults. Often they present with hypertension or tracheoesophageal compression. The involved anatomy is dependent on the sidedness of the aortic arch and the variable development of the primitive pharyngeal arches. Sternotomy and thoracotomy are usually required for surgical repair, while need for circulatory arrest is not uncommon. With caution and adequate planning, surgery can be carried out with satisfactory results. [ABSTRACT FROM AUTHOR]

Objective: The study was to figure out the feasibility, efficacy, and safety of a single-branched stent graft, namely Castor, in combination with fenestration or chimney in the context of aortic arch lesions presenting with aberrant subclavian artery (ASA) and/or Kommerell's diverticulum (KD)., Methods: All consecutive patients with aortic arch lesions and ASA and/or KD receiving Castor from June 2018 to June 2023 were investigated., Results: Incorporating 18 patients, the study encompassed 11 cases with KD, 3 cases with dysphagia; 2 cases of right-sided aortic arch with left-sided aberrant left subclavian artery (ALSA), and 16 cases of left-sided aortic arch with right-sided aberrant right subclavian artery (ARSA). The mean operation time was 132±23 minutes. The mean measured proximal aortic diameter was 30.9±1.6 mm, and proximal diameter of Castor stent was 34 (32, 34.5) mm, with oversize of 9.1±1.6%; the mean measured branch diameter was 8.8±0.97 mm, and branch diameter of Castor stent was 10 (8, 10) mm, with oversize of 0.86±0.57 mm. Technical success rate was 100%, and no in-hospital mortality, no stroke, and no endoleak were identified. One (5.6%) case with spinal cord ischemia and one (5.6%) case with poor healing of operative site were identified. During the follow-up period, no aortic-related death or secondary intervention was recorded. The maximal aortic diameter was significantly reduced at the sixth postoperative month (p adj =0.031); KD diameter was significantly reduced at the third (p adj =0.001) and sixth (p adj <0.001) postoperative month., Conclusion: Totally endovascular repair of aortic arch lesions with ASA and KD via Castor stent in combination with fenestration or chimney is feasible, effective, and safe, which can achieve an encouraging medium-term outcome and provide excellent remodeling at the lesions., Clinical Impact: Single branched stent in combination with fenestration or chimney achieved a sufficient proximal landing zone and provided an encouraging medium-term outcome in this retrospective review of 18 patients receiving endovascular treatment of pathological aortic arch with aberrant subclavian artery and/or Kommerell's diverticulum. The authors suggest this time-saving and efficient technique to establish systematic experience for the treatment in this kind of patients., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Dysphagia lusoria occurs due to compression of the esophagus as an aberrant right subclavian artery (ARSA) crosses the mediastinum. Surgical management includes open, hybrid, and endovascular techniques, with no consensus gold standard. There are few reports of robotic-assisted ARSA resection. We describe the innovative technique and outcomes for two patients who successfully underwent robotic-assisted transthoracic resection of an ARSA after right carotid-subclavian bypass for dysphagia lusoria. Both patients experienced improvement or resolution of their dysphagia and no major complications. In select patients with a noncalcified origin of the ARSA without aneurysmal degeneration, the robotic-assisted approach represents a viable option., Competing Interests: O.T.O. is a consultant for Intuitive Surgical and Johnson & Johnson. The remaining authors have no conflicts of interest to disclose., (© 2024 The Authors.)

Kommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA) translocation or aortopexy. These procedures remain controversial. We compared the outcomes of both procedures. This retrospective study included 48 patients who underwent LSCA translocation (n = 26) or aortopexy (n = 22). Lateral thoracotomy and sternotomy were performed in 38 and 10 patients with severe intracardiac malformations, respectively. The median age of the aortopexy group was 10 months, median follow-up was 14 months, and mean length of hospitalization was 11.41 ± 6.8 days. The median age of the LSCA translocation group was 12 months, median follow-up was 22 months, and mean length of hospitalization was 14.96 ± 7.74 days. Two patients (LSCA translocation group) required secondary intubations and none suffered from recurrent laryngeal nerve injury or complications related to LSCA translocation. Breathing and swallowing symptoms were relieved in all patients. No additional thoracotomies due to respiratory symptoms were required. These two procedures relieve the pressure on the trachea and esophagus with similar short-term outcomes. The long-term outcomes are unknown. LSCA translocation may be the best strategy to prevent symptom recurrence and late complications in these patients. Level III [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Kommerell diverticulum (KD) is a rare congenital vascular anomaly often associated with an aberrant subclavian artery (ASCA). Definitive indications for intervention remain unclear. We present open and endovascular (EV) operative outcomes in a large contemporary series and propose a management algorithm. METHODS Between 2004 and 2020, 224 patients presented with ASCA and associated KD to our institution. Of the 43 (19.2%) patients who underwent operative repair, 31 (72.1%) had open surgical (OS) repair via thoracotomy and 12 (27.9%) had EV repair. Univariable and bivariable statistical analyses were conducted stratified by approach. The median follow-up time was 5.4 years (IQR, 2.9–9.7). RESULTS Patients in EV group were older (68 years vs 47 years, P  < 0.001) and had larger aneurysms (base diameter 3.2 cm vs 21.5 cm, P  = 0.007). All patients with dysphagia lusoria were treated with open surgery (n  = 20). Asymptomatic patients with incidentally detected KD (50% vs 16.1%), those with chest or back pain (50% vs 19.4%) and patients who presented with an aortic emergency (25% vs 6.5%) were more likely to be treated endovascularly (P  = 0.001). Carotid-to-subclavian bypass was used in 38 (88.4%) patients. There were no operative mortalities. In-hospital mortality was similar between groups (3.2% vs 16.7%, P  = 0.121). Mid-term mortality was higher in the EV group [4 (33.8%) vs 0, P  < 0.001]. There were 2 (15.4%) postoperative strokes in the EV group. There were no statistically significant differences in other postoperative complications or hospital length of stay between groups. CONCLUSIONS KD can be managed using open or EV approaches with low morbidity and mortality. Treatment strategy should depend on clinical presentation and patient factors. [ABSTRACT FROM AUTHOR]

Non-recurrent Laryngeal nerve is constantly associated with Arteria Lusoria. Knowing this association is the basis of predicting this condition preoperatively. Ultrasonography assessment before thyroid and parathyroid surgery should include identification of brachiocephalic trunk division. Absence of its visualization indicates Arteria Lusoria and then non-recurrent laryngeal nerve. [ABSTRACT FROM AUTHOR]

Abstract Non‐recurrent Laryngeal nerve is constantly associated with Arteria Lusoria. Knowing this association is the basis of predicting this condition preoperatively. Ultrasonography assessment before thyroid and parathyroid surgery should include identification of brachiocephalic trunk division. Absence of its visualization indicates Arteria Lusoria and then non‐recurrent laryngeal nerve.

Background: Retroesophageal right subclavian artery, aberrant right subclavian artery, or lusoria artery constitutes an anatomical variation with a very low prevalence in the general population. Its presence in a patient does not add difficulty for cerebral arteries catheterization when a femoral approach is used, but may make the catheterization of cerebral vessels difficult when a right transradial approach is used. Unfortunately, this type of configuration of the artery is discovered after the decision to use the type of approach. Case Report: A 60-year-old woman with a diagnosed subarachnoid hemorrhage was studied with angiography through a transradial approach. A left carotid bifurcation aneurysm was diagnosed after three-vessel angiography was performed with a Simmons type 2 catheter, making a complete loop between the right subclavian artery, aortic arc, and left carotid artery. The ruptured cerebral aneurysm was completely occluded with 8 coils. The microcatheter used for the procedure emerged from the tip of an intermediate catheter located at the union of the vertical and horizontal segment of the petrous segment of the left carotid artery. Conclusions: The presence of an aberrant right subclavian artery during cerebral angiography performed through a right transradial approach renders the treatment of a left carotid cerebral aneurysms more difficult but not impossible. Utilizing an appropriate intermediate catheter allows for stable navigation of the microcatheter and complete treatment of the aneurysm. This is the first report of cerebral aneurysm embolization through a right transradial approach in a patient with an aberrant right subclavian artery. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We report the case of a fetus presenting aortic arch anomalies associated with a ventricular septal defect (VSD). This fetus, which was referred at 25 weeks of gestation, was suspected to have coarctation of aorta (CoA) evidenced by enlarged right chambers at the four‐chamber view during a routine obstetric ultrasonographic scan. The prenatal diagnosis of CoA remains a challenge. Here, we review the ultrasonographic findings that could contribute to this diagnosis. [ABSTRACT FROM AUTHOR]

We report a rare case of vascular ring due to a left cervical circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum. This is a very rare case of vascular ring in a newborn with stridor and stenosis of the left pulmonary artery. A cardiac catheterization and an angio-computed tomography scan were helpful to clarify the diagnosis. The surgical correction was performed with division of the arterial duct, reimplantation of the right subclavian artery, aortic and tracheal suspension, and pulmonary arterial plasty. The patient was asymptomatic at discharge and 1-year follow-up. [ABSTRACT FROM AUTHOR]