Incidental Finding of Arteria Lusoria.

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.

Development and Multicenter, Multiprotocol Validation of Neural Network for Aberrant Right Subclavian Artery Detection.

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.

Vascular Ring Surgical Repair: Re-Implantation of the Left Subclavian Artery into the Left Carotid Artery in a Pediatric Patient.

Vascular rings are a rare congenital anomaly of the aortic arch, in which a ring-shaped structure forms, surrounding the trachea and/or esophagus, potentially causing compression. We describe the case of a 14-month-old female patient with failure to thrive secondary to dysphagia, and a vascular ring formed by a right aortic arch, an aberrant left subclavian artery, and a left ligamentum arteriosum. Surgical repair involved ligamentum arteriosum division, Kommerell's diverticulum obliteration, and left subclavian artery re-implantation into the left carotid artery. Endoscopy and EndoFLIPTM evaluated the intraoperative improvement in esophageal narrowing and impedance, respectively. The postoperative period was uneventful, and follow-up visits demonstrated dysphagia resolution and a patent re-implanted left subclavian artery.

Technical considerations in robotic aberrant right subclavian artery resection for dysphagia lusoria.

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.

Repair of thoracic aortic aneurysm with bilateral aberrant subclavian artery.

We present a rare anatomical configuration of a 19-year-old woman, characterized by descending thoracic aortic aneurysm with right aberrant subclavian arteries with a Kommerell's diverticulum in a left aortic arch. The complexity of this vascular anomaly was accompanied by an anomalous origin of left subclavian artery. The patient underwent a single-stage open surgical repair via left thoracotomy under deep hypothermic circulatory arrest. The bilateral aberrant subclavian arteries were separately reconstructed in situ using hand-sewn branched grafts.

Hybrid Management of Dysphagia Lusoria with Tevar Implantation and Bilateral Subclavian Arteries Debranching: A Review of the Literature and a Case Report.

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.

Utilizing Single-Branched Stent in Combination With Fenestration or Chimney for Endovascular Repair of Aortic Arch Lesions With Aberrant Subclavian Artery.

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 (padj=0.031); KD diameter was significantly reduced at the third (padj=0.001) and sixth (padj<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.

Association of left ventricular outflow tract size with arch morphology in interrupted aortic arch.

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.

Lusory dysphagia: Unusual cause of intermittent dysphagia.

INTRODUCTION AND IMPORTANCE: 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. CASE PRESENTATION: 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. DISCUSSION: 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. CONCLUSION: 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.

Case Report: Kommerell's diverticulum and left aberrant subclavian artery stenosis hybrid treatment with branched aortic stent-graft.

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.

[Surgical treatment of aberrant subclavian artery]. / Khirurgicheskoe lechenie aberrantnoi podklyuchichnoi arterii.

OBJECTIVE: To determine the indications and optimal surgical treatment of aberrant subclavian artery. MATERIAL AND METHODS: There were 3 patients with aberrant subclavian artery with clinical manifestations such as dysphagia, shortness of breath, weight loss, cough and chest pain between 2005 and 2020. Right-and left-sided aberrant artery was observed in 2 and 1 case, respectively. Supraclavicular unilateral or bilateral access depended on the side of aberrant artery. Carotid-subclavian anastomosis was performed. RESULTS: All patients were discharged without any symptoms. CONCLUSION: Close location of aberrant subclavian artery to common carotid artery can disturb circulation in brain and upper limb. Abnormal discharge of subclavian artery can cause compression of nearby organs (esophagus, trachea). Abnormal vascular discharge does not always have clinical manifestations that do not require surgical correction. Only symptomatic patients are subjects to surgical correction of aberrant subclavian artery. Knowledge of variant anatomy of supra-aortic vessels can minimize the risk of complications.

Transhiatal esophagectomy on a patient with aberrant right subclavian artery: A case report.

INTRODUCTION: Esophageal dissection is generally safe and easy during transhiatal esophagectomy (THE). The right subclavian artery crosses between the esophagus and spine in about 1 % to 2 % of cases. This condition is called aberrant right subclavian artery and is the most common congenital aortic arch anomaly. Pre-operative recognition of this anomaly is important in esophageal surgeries. In unprepared situations injury to this vessel may result in life threatening bleeding. CASE PRESENTATION: A 45 year old female patient presented with progressive dysphagia and weight loss. Esophageal mass was found during upper gastrointestinal endoscopy. Additionally, a CT scan of the thorax and abdomen revealed an aberrant right subclavian artery and a distal esophageal mass. Biopsy revealed squamous cell carcinoma. A transhiatal esophagectomy was performed successfully with no complications. DISCUSSION: The vast majority of patients with an aberrant right subclavian artery do not experience any symptoms. In rare cases, patients may present with dysphagia (dysphagia lusoria) and obstructive respiratory symptoms in their fourth or fifth decade. When patients present with dysphagia from esophageal cancer, careful review of imaging is needed to identify the aberrant artery. CONCLUSION: The presence of aberrant right subclavian artery causes difficulty in performing esophagectomy. If it is diagnosed preoperatively, cautious retroesophageal dissection prevents injury to this vessel and complications following it.

Hybrid Repair of Complicated Type B Aortic Dissection Aneurysm With Dissected Kommerell Diverticulum Without Thoracotomy.

PURPOSE: The coincidence of aberrant right subclavian artery (ARSA) and Kommerell diverticulum (KD) with type B aortic dissection (TBAD) is a rare but dangerous disease. Currently, there are no well-established guidelines for treatment. Most authors seem to agree that surgical treatment is warranted. However, a hybrid repair technique as we performed is flexible, and a promising approach should be considered. CASE REPORT: Here, we summarized a case report of successful single-stage hybrid repair of a complicated TBAD combined with ARSA and KD without thoracotomy. CONCLUSION: Hybrid repair is a flexible and promising technique that has the potential to replace most open operation procedures in the future with a developed technique and more evidence-based medicine. CLINICAL IMPACT: As for ARSA and KD with TBAD patients, open surgical repair has been historically the treatment of choice; however, hybrid repair without thoracotomy means less invasion, simpler operation and faster recovery, which provides a flexible and promising technique that has the potential to replace most open operation procedures in the future with more evidence-based medicine.

Kommerell's Diverticulum in a Right-Sided Aortic Arch With an Aberrant Left Subclavian Artery Hybrid Repair.

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.

Prevalence and Complications of Aberrant Subclavian Artery in Patients With Heritable and Nonheritable Arteriopathies.

BACKGROUND: 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. OBJECTIVES: The purpose of this study was to assess the prevalence and complications of ASA in gene-positive and -negative nonatherosclerotic arteriopathies. MATERIALS: 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. RESULTS: 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. CONCLUSIONS: 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.

A natural systemic to pulmonary artery shunt.

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.

Mid-Term Results of Treating Kommerell's Diverticulum and Aberrant Subclavian Artery Anomalies Using a Patient-Centered Team Approach.

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.

Contemporary outcomes after treatment of aberrant subclavian artery and Kommerell's diverticulum.

OBJECTIVE: Aberrant subclavian artery (ASA) and Kommerell's diverticulum (KD) are rare vascular anomalies that may be associated with lifestyle-limiting and life-threatening complications. The aim of this study is to report contemporary outcomes after invasive treatment of ASA/KD using a large international dataset. METHODS: Patients who underwent treatment for ASA/KD (2000-2020) were identified through the Vascular Low Frequency Disease Consortium, a multi-institutional collaboration to investigate uncommon vascular disorders. We report the early and mid-term clinical outcomes including stroke and mortality, technical success, and other operative outcomes including reintervention rates, patency, and endoleak. RESULTS: Overall, 285 patients were identified during the study period. The mean patient age was 57 years; 47% were female and 68% presented with symptoms. A right-sided arch was present in 23%. The mean KD diameter was 47.4 mm (range, 13.0-108.0 mm). The most common indication for treatment was symptoms (59%), followed by aneurysm size (38%). The most common symptom reported was dysphagia (44%). A ruptured KD was treated in 4.2% of cases, with a mean diameter of 43.9 mm (range, 18.0-100.0 mm). An open procedure was performed in 101 cases (36%); the most common approach was ASA ligation with subclavian transposition. An endovascular or hybrid approach was performed in 184 patients (64%); the most common approach was thoracic endograft and carotid-subclavian bypass. A staged operative strategy was employed more often than single setting repair (55% vs 45%). Compared with endovascular or hybrid approach, those in the open procedure group were more likely to be younger (49 years vs 61 years; P < .0001), female (64% vs 36%; P < .0001), and symptomatic (85% vs 59%; P < .0001). Complete or partial symptomatic relief at 1 year after intervention was 82.6%. There was no association between modality of treatment and symptom relief (open 87.2% vs endovascular or hybrid approach 78.9%; P = .13). After the intervention, 11 subclavian occlusions (4.5%) occurred; 3 were successfully thrombectomized resulting in a primary and secondary patency of 95% and 96%, respectively, at a median follow-up of 39 months. Among the 33 reinterventions (12%), the majority were performed for endoleak (36%), and more reinterventions occurred in the endovascular or hybrid approach than open procedure group (15% vs 6%; P = .02). The overall survival rate was 87.3% at a median follow-up of 41 months. The 30-day stroke and death rates were 4.2% and 4.9%, respectively. Urgent or emergent presentation was independently associated with increased risk of 30-day mortality (odds ratio [OR], 19.8; 95% confidence interval [CI], 3.3-116.6), overall mortality (OR, 3.6; 95% CI, 1.2-11.2) and intraoperative complications (OR, 8.3; 95% CI, 2.8-25.1). Females had a higher risk of reintervention (OR, 2.6; 95% CI, 1.0-6.5). At an aneurysm size of 44.4 mm, receiver operator characteristic curve analysis suggested that 60% of patients would have symptoms. CONCLUSIONS: Treatment of ASA/KD can be performed safely with low rates of mortality, stroke and reintervention and high rates of symptomatic relief, regardless of the repair strategy. Symptomatic and urgent operations were associated with worse outcomes in general, and female gender was associated with a higher likelihood of reintervention. Given the worse overall outcomes when symptomatic and the inherent risk of rupture, consideration of repair at 40 mm is reasonable in most patients. ASA/KD can be repaired in asymptomatic patients with excellent outcomes and young healthy patients may be considered better candidates for open approaches versus endovascular or hybrid modalities, given the lower likelihood of reintervention and lower early mortality rate.