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Right-sided aortic arch, first documented by Fioratti and Aglietti in 1763, is a rare variant of the thoracic vascular anatomy present in about 0.1% of the adult population. In half of these cases the left subclavian artery is also aberrant. The aberrant left subclavian artery usually originates from a conical dilatation near its origin from the aorta also known as “Kommerell's diverticulum.” Fewer than 80 of these cases have been reported in the literature as far as our web search is concerned. It is usually asymptomatic and diagnosed incidentally during adulthood. We are presenting a 56 years old male patient presented with right side chest and shoulder pain of 1 week duration. The pain exacerbated with motion of the right upper extremity and radiates to his lower back. However, he had no history of cough, shortness of breath, syncope, and dysphagia. The vital signs were in normal range. Pulmonary and cardiovascular exam were unremarkable. The complete blood count (CBC), electrocardiogram (EKG), and echocardiography showed no abnormality. In the adult population a right-sided aortic arch with an aberrant left subclavian artery arising from Kommerell's diverticulum is a rare occurrence often asymptomatic unless aneurysmal disease or compression of mediastinal structures ensues. Even though it is rare and at times an incidental finding, the condition is clinically relevant because of the morbidity caused by the complications. We report a case of Kommerell's Diverticulum of an aberrant left subclavian artery in an adult patient with a right-sided aortic arch. Right-sided aortic arch with aberrant left subclavian artery arising from Kommerell's Diverticulum is quite rare and may remain asymptomatic. On times it may cause symptoms in adulthood often as a result of early atherosclerotic changes of the anomalous vessels, dissection, or aneurysmal dilatation with compression of adjacent structures causing dysphagia, dyspnea, cough, or chest pain. Even though there are no general guidelines for the management of this condition patients need to be informed about the nature and possible outcomes of their condition. Close follow up of asymptomatic patients is one option of management until there are situations which require consideration of surgical intervention.

Abstract Background A right-sided aortic arch is a rare congenital vascular structure variation. Right lobectomy is not commonly performed on patients with such a condition. Further, there are no reports on lobectomy under uniportal video-assisted thoracoscopic surgery (VATS) in this patient group. Case presentation A 67-year-old man with a right-sided aortic arch and Kommerell diverticulum underwent right upper lobectomy with mediastinal lymph node dissection under uniportal VATS for primary lung cancer. Due to the right descending aorta, which narrows the space of the dorsal hilum, handling of the stapler for stapling the right upper lobe bronchus from the uniport in the 6th intercostal space at the medial axillary line can be challenging. This issue was resolved by manipulating the staple over the azygos vein toward the inferior margin of the aortic arch. Via mediastinal lymphadenectomy, we found that the right recurrent laryngeal nerve branched from the right vagus nerve and hooked around the right-sided aortic arch. Conclusions Right lobectomy with mediastinal lymph node dissection under uniportal VATS can be performed for lung cancer in patients with a right-sided aortic arch.

Background: A right-sided aortic arch is a rare congenital vascular structure variation. Right lobectomy is not commonly performed on patients with such a condition. Further, there are no reports on lobectomy under uniportal video-assisted thoracoscopic surgery (VATS) in this patient group. Case presentation: A 67-year-old man with a right-sided aortic arch and Kommerell diverticulum underwent right upper lobectomy with mediastinal lymph node dissection under uniportal VATS for primary lung cancer. Due to the right descending aorta, which narrows the space of the dorsal hilum, handling of the stapler for stapling the right upper lobe bronchus from the uniport in the 6th intercostal space at the medial axillary line can be challenging. This issue was resolved by manipulating the staple over the azygos vein toward the inferior margin of the aortic arch. Via mediastinal lymphadenectomy, we found that the right recurrent laryngeal nerve branched from the right vagus nerve and hooked around the right-sided aortic arch. Conclusions: Right lobectomy with mediastinal lymph node dissection under uniportal VATS can be performed for lung cancer in patients with a right-sided aortic arch. [ABSTRACT FROM AUTHOR]

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

This report describes a patient with a right-sided aortic arch, aberrant left subclavian artery and Kommerell diverticulum, who presented with aneurysmal degeneration of the aortic root to the descending aorta, in addition to an acute type B2-10 aortic dissection. He underwent hybrid treatment with a valve-sparing aortic root replacement, transverse arch replacement with reattachment of the right subclavian artery, bilateral common carotid arteries, and thoracic endovascular aneurysm repair with left subclavian artery embolization and a left common carotid to subclavian artery bypass.

Objectives: Kommerell's diverticulum (KD) is associated with a high incidence of right-sided aortic arch (RAA). Hybrid thoracic endovascular aortic repair (TEVAR) is an effective and less invasive alternative to open repair. However, the long-term results regarding KD diameter regression or symptom improvement remain inadequately described., Methods: Nine patients underwent TEVAR for KD associated with RAA between January 2016 and September 2023 at our university hospital and affiliated institutions. A hybrid procedure was performed to exclude KD by blocking the proximal blood flow with TEVAR and distal blood flow with embolization of the aberrant subclavian artery. Simultaneously, extra-anatomical bypass surgery was performed to revascularize the covered supra-arch vessels., Results: The patients' mean age was 65.2 years, and six patients were men. Two patients presented with dysphagia, whereas the rest were asymptomatic. The mean diameter and distance to the opposite aortic wall of KD were 32.1 mm and 56.2 mm, respectively. For revascularization of the covered supra-arch vessels, six and two patients underwent total debranching with sternotomy and extra-thoracic bypass (bilateral common carotid artery-axial artery bypass), respectively. The 30-day and in-hospital mortality rates were 0%, with no instances of cerebral infarction or spinal cord ischemia. The mean follow-up period was 3.2 years. The survival and avoidance rates of aortic events were 100% at 1 and 3 years. Follow-up computed tomography scans showed no endoleaks; however, one (11.1%) type 2 endoleak from the aberrant left subclavian artery occurred 1 week postoperatively, necessitating additional coiling. Seven patients were followed up for more than 1 year, with five experiencing reductions of more than 3 mm in KD diameter, distance to the opposite aortic wall, or both., Conclusion: Although further follow-up and investigations are needed, TEVAR may be a safe and effective surgical treatment for KD associated with RAA., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Our case presents a unique occurrence marking the first documentation of a connection between a typical carcinoid in the context of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and vascular anomalies, including a right-sided aortic arch with Kommerell's diverticulum. Kommerell's diverticulum is a rare congenital anomaly. The lusoria subclavian artery, another developmental anomaly, arises from the right aortic arch instead of the typical left side. Neuroendocrine cells may contribute to lung disease pathogenesis by altering their physiology before clinical symptoms appear. A 56-year-old woman with an unyielding chronic cough underwent diagnostic evaluation, unveiling rare vascular anomalies alongside a pulmonary nodule. Radiological investigations disclosed a solid nodule in the middle lobe, accompanied by proximal right-sided aortic arch ectasia and an aberrant left subclavian artery. Following multidisciplinary deliberation, thoracic and vascular surgeons elected for surgical nodule resection. Utilizing uniportal video-assisted thoracoscopic surgery, the procedure revealed the anomaly of the right-sided aortic arch. Preliminary histological examination indicated a low-grade pulmonary carcinoid, obviating the need for further lymphadenectomy due to its low malignancy potential. Subsequent histological analysis confirmed a well-differentiated neuroendocrine tumor G1 consistent with typical carcinoid within a DIPNECH framework. Currently, the patient is in follow-up. This case underscores the importance of multidisciplinary evaluation and tailored surgical approaches for managing patients with rare vascular anomalies and pulmonary nodules, emphasizing the requisite comprehensive preoperative assessment and collaborative efforts among diverse medical specialties to optimize outcomes., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nocera et al.)

Right-sided aortic arch, first documented by Fioratti and Aglietti in 1763, is a rare variant of the thoracic vascular anatomy present in about 0.1% of the adult population. In half of these cases the left subclavian artery is also aberrant. The aberrant left subclavian artery usually originates from a conical dilatation near its origin from the aorta also known as "Kommerell's diverticulum." Fewer than 80 of these cases have been reported in the literature as far as our web search is concerned. It is usually asymptomatic and diagnosed incidentally during adulthood. We are presenting a 56 years old male patient presented with right side chest and shoulder pain of 1 week duration. The pain exacerbated with motion of the right upper extremity and radiates to his lower back. However, he had no history of cough, shortness of breath, syncope, and dysphagia. The vital signs were in normal range. Pulmonary and cardiovascular exam were unremarkable. The complete blood count (CBC), electrocardiogram (EKG), and echocardiography showed no abnormality. In the adult population a right-sided aortic arch with an aberrant left subclavian artery arising from Kommerell's diverticulum is a rare occurrence often asymptomatic unless aneurysmal disease or compression of mediastinal structures ensues. Even though it is rare and at times an incidental finding, the condition is clinically relevant because of the morbidity caused by the complications. We report a case of Kommerell's Diverticulum of an aberrant left subclavian artery in an adult patient with a right-sided aortic arch. Right-sided aortic arch with aberrant left subclavian artery arising from Kommerell's Diverticulum is quite rare and may remain asymptomatic. On times it may cause symptoms in adulthood often as a result of early atherosclerotic changes of the anomalous vessels, dissection, or aneurysmal dilatation with compression of adjacent structures causing dysphagia, dyspnea, cough, or chest pain. Even though there are no general guidelines for the management of this condition patients need to be informed about the nature and possible outcomes of their condition. Close follow up of asymptomatic patients is one option of management until there are situations which require consideration of surgical intervention., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Kommerell’s diverticulum (KD) is a rare aneurysm of the origin of an aberrant subclavian artery. Hybrid aortic arch repair for KD is being performed more often. We report hybrid arch repair for KD in a 63-year-old man with a right aortic arch and aberrant right vertebral artery, an extremely rare variant. We performed total arch replacement to completely reconstruct the five cervical arteries with elephant trunk to create an adequate landing zone, followed by second-stage endovascular stent-grafting from the ascending aorta to the proximal descending aorta.

Background A right-sided aortic arch (RAArch) is present in approximately 0.1% of the population. A Kommerell's diverticulum (KD), a remnant of the dorsal aortic arch usually refers to an aneurysmal aortic enlargement at the origin of an aberrant left subclavian artery (ALSA) and is associated with an increased risk of aortic dissection. Case summary A 59-year-old female smoker with a history of hypertension and hypercholesterolaemia presented with a 24-hour history of sudden-onset and severe stabbing chest pain radiating to the interscapular region. Physical examination was normal except for bilateral basal crepitations. Computed tomography angiography (CTA) showed a type B aortic dissection in a RAArch with an ALSA arising from KD with a peri-aortic haematoma and haemothorax without any active contrast extravasation. After medical stabilization, a semi-urgent hybrid repair was performed with a right carotid-subclavian bypass, thoracic endovascular aortic repair (TEVAR), a plug in the left subclavian artery, and left carotid-subclavian bypass due to severe ischaemia of the left arm. The postoperative CTA showed patent bypasses, aortic remodelling, and a minimal type IIa endoleak at the level of the ALSA. Discussion In patients with a type B dissection and KD, hybrid repair including TEVAR is feasible after careful pre-operative assessment of the patient's unique anatomy and may reduce post-surgical morbidity and mortality compared to open surgery. Prophylactic repair may be considered in patients with an asymptomatic RAArch and KD. [ABSTRACT FROM AUTHOR]

Background The coexistence of a right-sided aortic arch (RAA), an aberrant left subclavian artery (ALSA), and a patent ductus arteriosus (PDA) is a rarely seen vascular ring anomaly. There is currently no general guideline consensus on the management and follow-up of this congenital defect, posing a challenge to the clinicians. At this point, the heart team plays a critical role in the management of the disease. Case summary In the present case, a 25-year-old male patient was presented to the outpatient clinic with dyspnoea and fatigue. A transthoracic echocardiography revealed PDA with a left-to-right shunt. To evaluate the anatomy thoroughly, a thoracic computed tomographic angiography was performed and showed PDA accompanying ALSA and RAA. The patient was evaluated by the Heart Team, and a percutaneous closure of PDA was recommended due to signs of left ventricular volume overload. The closure was successfully performed with Amplatzer vascular plug II. At follow-up, the patient was free of symptoms. Discussion Clinicians should be aware of the potential concomitant lesions during the diagnostic work-up. In selected patients, percutaneous closure of PDA may be the first-line therapy in experienced centres. [ABSTRACT FROM AUTHOR]

During the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF), cardiovascular malformations occasionally create a technical challenge. We report a novel case of a 4-day-old girl with EA/TEF and multiple cardiovascular malformations, including right-sided aortic arch (RAA), right ductus arteriosus (RDA), single ventricle with single atrium, common atrioventricular valve, pulmonary atresia, bilateral superior vena cava, and interruption of the inferior vena cava (IVC) with hemiazygos continuation. In this case, a right-sided approach would require the mobilization of the RAA and RDA, which were supplying the pulmonary blood flow due to pulmonary atresia. Alternatively, the left-sided approach would require the mobilization of the hemiazygos vein, which was essential for venous return from the lower body due to IVC interruption. We performed the less intrusive left-sided approach, and the postoperative course was uneventful. Right-sided EA/TEF repair should be avoided because RDA spasm or injury caused by RAA mobilization would be fatal. In cases of interrupted IVC with azygos or hemiazygos vein continuation, care must be taken not to ligate these vessels or block the venous return. Preoperative evaluation is important to prevent complications in such complicated cases. If sufficient information is not available, the left-sided approach may be preferred.

Background: The right-sided aortic arch (RAA) is an uncommon anatomical anomaly found in <0.1% of the adult population. In this article, we report a case of RAA anomaly with an aberrant left subclavian artery (ALSA) and Kommerell's diverticulum associated with aneurysmal dilation of the ascending aorta, left carotid artery (CCA) stenosis, and pancake kidney presented with a transient ischemic attack (TIA). To the best of our knowledge, this is the first case in the literature that discusses such associations, especially in a symptomatic patient with neurological rather than tracheaesophageal symptoms and in the absence of the steal phenomenon. Case Description: A 52-year-old male, with a history of recurrent multiple TIAs, presented immediately after the onset of blurred vision and left-sided weakness. The initial diagnostic cerebral angiogram revealed a left CCA stenosis of <30%, with normal posterior circulation vasculature. The diagnosis of RAA was made with computed tomography angiography (CTA) of the thoracic and abdominal aorta, which revealed Type 2 RAA, with ALSA, which had a bullous dilatation at its origin that suggests Kommerell's diverticulum. Another two findings on CTA were a persistent left-sided superior vena cava that ended in the coronary sinus and a single pelvic fused renal mass (Pancake kidney). Conclusion: We presented an extremely rare case of RAA with ALSA associated with a group of extra rare anomalies. Understanding the anatomical variants of RAA and its characteristics is critical to improving the management and follow-up of patients with such anomalies. [ABSTRACT FROM AUTHOR]

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

The right aortic arch with aberrant left subclavian artery is the most common cause of vascular ring and can be either asymptomatic or symptomatic owing to mass effect. Removal of Kommerell diverticulum and division of the ligamentum arteriosum through a left thoracotomy is currently advocated in symptomatic patients and patients with large diverticulum (>5 cm) to avoid inherent complications.

Transcatheter aortic valve implantation (TAVI) represents the standard of care for relieving aortic stenosis in high-risk patients for surgery. The transfemoral approach is preferable with respect to invasiveness, but is often difficult in patients with complex vascular structures. Recently, the clinical application of advanced visualization and guidance technology with three-dimensional computed tomography (3D-CT) during TAVI has received considerable attention. Herein we report successful transfemoral TAVI in a patient with a right-sided aortic arch and chronic aortic dissection without vascular complications by 3D-CT/fluoroscopy fusion imaging guidance. [ABSTRACT FROM AUTHOR]

A right-sided aortic arch (RAA) is a rare variant of the aortic arch found. We present a case of endovascular treatment (EVT) for acute basilar artery (BA) occlusion with an RAA. A 70-year-old man developed acute ischemic stroke due to BA occlusion. During urgent EVT for BA occlusion with an RAA, which was observed unexpectedly, navigation of the catheter to the target vessel was achieved as a result of an early change in the access route. Flexible treatment strategies are warranted for cases of unexpected anatomical variant in the fight against time for emergent cases, such as acute ischemic stroke. [ABSTRACT FROM AUTHOR]

We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process.

Non-recurrent inferior laryngeal nerve (NRILN) is rare but one of the important anatomical variations in thyroid and parathyroid surgery. Almost all cases were observed on the right side with aberrant right subclavian artery and left NRILN have been reported in only five cases so far. Here, we reported a 38 year-old Japanese male with left NRILN accompanying adenomatous goiter. He was referred to our hospital for the surgical treatment of left thyroid goiter. Preoperative computed tomography revealed right-sided aortic arch and aberrant left subclavian artery with no signs of complete situs inversus viscerum, suggesting possible left NRLN. Left hemithyroidectomy was performed using nerve monitoring system. Intraoperatively, left recurrent laryngeal nerve was not identified along tracheoesophageal groove, but directly originated from vagal nerve and was running horizontally to larynx. Mobility of vocal cords were not impaired and postoperative course was uneventful. During thyroid surgery for the patients with right-sided aortic arch, meticulous care should be taken using nerve monitoring system to avoid nerve injury. [ABSTRACT FROM AUTHOR]

Spontaneous ruptured aneurysm involving an aberrant subclavian artery with a right-sided aortic arch and Kommerell's diverticulum is a rare life-threatening condition that can be treated successfully if promptly identified. Multidetector Computed Tomography angiography is the first line imaging modality of thoracic vascular anomalies diagnosis. We report the case of a 74- year-old man suffering from this emergency condition with mediastinal hematoma mostly extending to the left-side extrapleural cavity. The patient underwent successful emergency thoracic endovascular aortic repair and an Amplatzer vascular plug was placed into the first segment of the ALSA. Post-procedural imaging showed complete exclusion of the aneurysm. Emergency endovascular repair can be effective in such cases. [ABSTRACT FROM AUTHOR]

Spontaneous ruptured aneurysm involving an aberrant subclavian artery with a right-sided aortic arch and Kommerell’s diverticulumis a rare life-threatening condition that can be treated successfully if promptly identified. Multidetector Computed Tomography angiography is the first line imaging modality of thoracic vascular anomalies diagnosis. We report the case of a 74-year-old man suffering from this emergency ondition with mediastinal hematoma mostly extending to the left-side extrapleural cavity. The patient underwent successful emergency thoracic endovascular aortic repair and an Amplatzer vascular plug was placed into the first segment of the ALSA. Post-procedural imaging showed complete exclusion of the aneurysm. Emergency endovascular repair can be effective in such cases.

Abstract Background It is a very rare condition for a patient to have right lung cancer and a right-sided aortic arch simultaneously. Right lobectomy under video-assisted thoracoscopic surgery (VATS) in such a patient is a challenging procedure that is seldom reported. We successfully performed a VATS right upper lobectomy in a 77-year-old female with a right-sided aortic arch and Kommerell diverticulum. Case presentation A 77-year-old woman was referred to our division for a mixed ground-glass opacity lesion in the right upper lung. A right-sided aortic arch with Kommerell diverticulum was identified by preoperative 3D CT reconstruction. A VATS right upper lobectomy with radical mediastinal lymph node dissection was performed, and the final histological staging was Ia3 (pT1cN0M0). The patient was discharged without any complications. Conclusions We conclude that the video-assisted thoracic surgery can be safely performed in such conditions. It is difficult to determine the extent of upper mediastinal lymph node dissection in such cases.

Right aortic arch is easily diagnosed at echocardiography and its presence must prompt to identify aortic arch vessels by computed tomography angiography in case of respiratory or digestive symptoms raising the possibility of the presence of a vascular ring. Anterior view of chest computed tomography angiography image showing the right-sided aortic arch (RAA) with the origin of the right subclavian artery (RSCA) and the Kommerell diverticulum (KD) with the aberrant retro-oesophageal left subclavian artery (LSCA). A chest computed tomography angiography was performed that incidentally showed right-sided aortic arch with a Kommerell diverticulum and aberrant retro-oesophageal left subclavian artery (Figures 1 and 2). [Extracted from the article]

Objectives: The ideal treatment for aneuryms of aberrant left subclavian arteries with Kommerell's diverticulum arising from right aortic arches remains open., Methods: Between January 2015 and December 2020, 5 patients with aneurysms from a right-sided aortic arch with aberrant left subclavian artery and Kommerell's diverticulum underwent repair by using the frozen elephant trunk technique in 3 aortic centres. Patients' characteristics were retrospectively reviewed and the surgical procedure and outcomes are presented., Results: The median age of the 2 male and 3 female patients was 59 (range from 49 to 63) years. The median operative times were as follows: surgery 405 min (range from 335 to 534), cardiopulmonary bypass time 244 min (range from 208 to 280) and aortic clamp time 120 min (from 71 to 184). The mean core temperature was 25.94°C (from 24 to 28). The intensive care unit stay was 4 days (range from 1 to 8) and the in-hospital stay 21 days (from 16 to 34). All patients were discharged and we observed no stroke or spinal cord ischaemia postoperatively. During the median follow-up time of 1003 days (range from 450 to 2306), 3 patients required subsequent thoracic endovascular distal stent graft extension., Conclusions: The frozen elephant trunk technique is a good treatment option for patients with aneuryms of an aberrant left subclavian artery with Kommerell's diverticulum arising from right aortic arches. Secondary stent graft extension is a frequently needed component of the treatment concept., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Transposition of the great vessels (TGV) is a common congenital heart defect that is difficult to diagnose before birth. Antenatal diagnosis is associated with increased survival. Unusual features such as anomalous pulmonary artery origin may delay cyanosis, decreasing clinical suspicion. A three‐week old female infant who had never been cyanotic presented for forensic autopsy due to onset of unresponsiveness at home. History included risk factors for TGV and signs of heart enlargement that were not recognized during life. Cardiac pathology consultation identified D‐type TGV with additional rare anomalies. TGV may present as sudden unexplained infant death (SUID) for forensic autopsy if variant features prevent development of cyanosis. Cardiac pathology consultation is helpful in clarifying these features. [ABSTRACT FROM AUTHOR]

We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process. [ABSTRACT FROM AUTHOR]

Abstract Objective Congenital aortic arch variations are more common in patients with thoracic aortic disease for reasons unknown. Additionally, little is understood about their relation to type B aortic dissections (TBAD) specifically. We investigated the prevalence of variant aortic arch anatomy in patients with TBAD compared with controls. To understand the implications of how variant aortic arch anatomy may contribute to degenerative aortic disease, we compared flow hemodynamics of three variations of aortic arches using four-dimensional flow magnetic resonance imaging (4D flow MRI). Methods Arch anatomy on computed tomography imaging was reviewed and compared between patients with TBAD and age/sex-matched controls free of aortic pathology. Arch variants were defined as follows: common origin of innominate and left common carotid artery (bovine arch), aberrant right subclavian artery, and right-sided aortic arch. Demographics, TBAD characteristics, and follow-up data were abstracted. Patients with TBAD with variant and conventional aortic arches were compared. Additionally, three matched healthy controls with conventional, bovine, and aberrant right subclavian artery arches underwent 4D flow MRI evaluation to assess if there were differences in flow patterns by arch type. Indices of regional hemodynamic wall sheer stress were compared. Results Computed tomography scans of 185 patients with TBAD (mean age, 58.1 ± 12.4 years; 72.4% males; 71.4% Caucasian) and 367 controls (mean age, 62.5 ± 13.4 years; 67% males; 77.9% Caucasian) were reviewed. Variant arch anatomy was more prevalent in patients with TBAD (40.5% vs 24.5%; P <.001). In patients with TBAD, there were no differences in the mean age of presentation and descending thoracic aorta diameter among those with variant or conventional arch anatomy. Patients with TBAD with variant arch anatomy had a higher percentage of dissection related thoracic aortic repairs (54.7% vs 33.6%; P =.004) with repairs occurring predominantly in the acute phase. 4D flow MRI demonstrated a higher systolic wall shear stress along the inner curve of the bovine arch compared with the conventional aberrant right subclavian artery arches. Conclusions Variant aortic arch anatomy is significantly more prevalent in patients with TBAD. patients with TBAD with variant arch anatomy had a higher percentage of dissection-related aortic repair. Preliminary 4D flow MRI data show differences in hemodynamic flow patterns between variant and conventional arches. Studies of long-term outcomes based on arch anatomy may offer additional insight to TBAD genesis and possibly influence management decisions. [ABSTRACT FROM AUTHOR]

The occurrence of aortic dissection involving a right-sided aortic arch (RAA) is extremely rare. Majority of the symptomatic cases have been managed with open surgical treatment. However, middle-aged and elderly patients with coexistent comorbidities have higher postoperative risks if managed by surgery. The purpose of this case study is to review the literature for occurrence and treatment modalities for RAA with dissection, to review the various literature studies which have helped establish a consensus and guidelines in the management of aortic dissections, to review previous reported cases of successful endovascular management of RAA dissections, and to report our experience of treating a case of Stanford Type B right-sided aortic dissection managed by complete endovascular approach, the first of its kind in India. Our search in the databases revealed around 32 cases of RAA with aortic dissection published in the literature, most of which have been treated with open surgical approach; however, very few cases are managed with endovascular approach only like in our case.

Purpose: Right-sided aortic arch is a rare vessel anomaly with an incidence of 0.1% worldwide. Supra-aortic branches form a mirror image of the left-sided aortic arch or an aberrant left subclavian artery associated with Kommerell diverticulum. Most patients are diagnosed by a difference in blood pressure in each upper extremity or by the presence of left subclavian steal syndrome in their younger age. The diagnosis of onset of ischemic stroke in middle age is rare. Methods: We present the case of a female patient who presented with an ischemic stroke in the left posterior circulation area. She had no history of congenital heart malformation. We performed head magnetic resonance imaging, cerebral angiography, and enhanced computed tomography of the aortic arch and major branches. Results: The patient had a right-sided aortic arch and an aberrant left subclavian artery. The left subclavian artery was occluded at the proximal portion with a fibrous string. Collateral flow in the anterior cervical subcutaneous area supported left limb perfusion. Conclusion: An atheromatous change reduced shunt flow via collateral networks at the anterior cervical region. Congenital subclavian steal supported the ischemic stroke. [ABSTRACT FROM AUTHOR]

Right-sided aortic arch with an isolated left subclavian artery is a very rare congenital anatomical variant that can lead to subclavian steal syndrome. We present a case of an asymptomatic patient who was incidentally found to have this variant on CT angiography with QMRA evidence of retrograde flow in the left vertebral artery consistent with subclavian steal phenomena. Since patients often remain asymptomatic until their compensatory mechanisms become compromised later in life, serial monitoring using non-invasive hemodynamic studies such as QMRA may guide treatment. [ABSTRACT FROM AUTHOR]

Background Left aberrant subclavian artery (LASA), is a type of right aortic arch (RAA) branching, which takes-off distally to the right subclavian artery and usually crosses behind the esophagus to the left upper limb. Taking into account the rarity of RAA, LASA is much more rarely seen than the right aberrant subclavian artery (RASA) originating from the left aortic arch. However, RAA may be associated with much more frequent presence of LASA, than left aortic arch with RASA. Anatomical LASA characteristics were not described up to date. Methods Individual patient records filed in the electronic database from a single high-volume tertiary cardiac center were retrospectively screened for the presence of RAA in the consecutive patients who underwent chest computed tomography from 2008 to 2016. Results 14 patients with LASA were identified. Only 3 were free of coexisting intra- or extra-cardiac anomalies. The most common defect was tetralogy of Fallot (3 patients). One patient had five congenital defects. Kommerell's diverticulum (KD) was present in 9 patients. In all patients the LASA course was retrotracheal and retroesophageal. In all but one patients esophageal modelling or compression by KD or LASA was present. Conclusions Knowledge of Kommerell's diverticulum presence and morphology as well as the LASA course during preinterventional work-up of patients with congenital heart defects is critical, since it may have surgical implications during corrective procedures. [ABSTRACT FROM AUTHOR]

We describe a rare case of the right-sided aortic arch, the unusual origin of the main arterial vessels and the unusual courses of bilateral recurrent laryngeal nerves in a Japanese cadaver. Chiefly, the right-sided aortic arch turned to the left side from the dorsal part of the trachea and esophagus, and Kommerell's diverticulum was found at the end of the arch. The right common carotid artery arose from the end part of the ascending aorta, but the left common carotid artery arose from the proximal portion of the ascending aorta. The right subclavian artery arose from the upper edge of the aortic arch, but the left one arose from the front wall at the upper side of the ligamentum arteriosum. The right recurrent laryngeal nerve hooked around the aortic arch (but not the right subclavian artery) dorsoventrally, and the left recurrent laryngeal nerve hooked around the ligamentum arteriosum and arose from the ventral side (but not dorsal) of the aortic arch. These variations are very rare, and understanding them is useful and important for clinical research. [ABSTRACT FROM AUTHOR]