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Vascular compression of the esophagus by an aberrant right subclavian artery (aRSA) leading to dysphagia is a rare occurrence. There has been a significant advancement in the diagnostic and surgical treatment modalities available for this disorder. Anesthetic management has evolved too and this case report highlights the anesthetic management of a 41-year-old woman presenting with symptoms of dysphagia because of compression of esophagus by an aRSA, who subsequently underwent re-implantation of aRSA into ascending aorta.

Resumo A artéria subclávia direita aberrante, também conhecida como artéria lusória, é a anomalia do arco aórtico mais comum, ocorrendo entre 0,5 e 1% da população. Possui prevalência em mulheres e normalmente está associada a outras variações anatômicas, como o nervo laríngeo não recorrente, presente em 86,7% dos casos. Em sua maioria, a artéria subclávia direita aberrante não apresenta sintomas. Descrevemos essa alteração em uma paciente de 82 anos, hipertensa e assintomática, que havia sido submetida a uma angiotomografia toracoabdominal para a avaliação de uma dissecção crônica tipo III (DeBakey) associada à dilatação de aorta descendente. No achado, a artéria subclávia direita aberrante apresentava percurso retroesofágico associado a um divertículo de Kommerell. Devido à raridade, realizamos revisão bibliográfica integrativa das bases de dados MEDLINE, UpToDate, LILACS, SciELO e Portal CAPES dos últimos 6 anos e discutimos as alterações anatômicas mais frequentes, a sintomatologia e as condutas terapêuticas adotadas. Abstract The aberrant right subclavian artery, also known as the arteria lusoria, is the most common aortic arch anomaly, occurring in 0.5 to 1% of the population. There is a higher prevalence in women and it is usually associated with other anatomical variations, such as the non-recurrent laryngeal nerve, present in 86.7% of cases. In the majority of cases, the aberrant right subclavian artery causes no symptoms. We describe this anomaly in an 82-year-old, hypertensive, and asymptomatic patient who had undergone a thoracoabdominal angiography to investigate a chronic DeBakey type III aortic dissection with dilation of the descending aorta. The aberrant right subclavian artery followed a retroesophageal course and was associated with a Kommerell diverticulum. In view of its rarity, we conducted an integrative bibliographic review of literature from the last 6 years indexed on the Medline, UpToDate, Lilacs, Scielo, and Portal Capes databases and discuss the most frequent anatomical changes, symptomatology, and therapeutic management adopted.

The right vertebral artery (VA) rarely arises from the right common carotid artery (CCA); however, the majority of anomalous right VAs are seen in combination with an aberrant right subclavian artery (SA). Independent (or isolated) right VA of the right CCA origin is extremely rare, with only a few cases reported previously. We herein report an additional case diagnosed incidentally using computed tomography angiography. This patient had a left VA arising from the aortic arch between the origins of the left CCA and left SA. The bilateral VAs took an anterior course and entered the C3 transverse foramina, at an extremely high level.

Introduction The right subclavian artery normally arises as the first vessel from the brachiocephalic trunk. An aberrant right subclavian artery (ARSA) arises directly from the aortic arch and crosses behind the trachea towards the right arm. This variant occurs in approximately 1–2% of the population; however, the frequency increases in individuals with chromosomal abnormalities such as trisomy 21 and 22q11.2 microdeletion. Prenatal identification of ARSA therefore has a role in screening for such conditions. Methods Databases were searched for studies reporting the prenatal ultrasound evaluation of ARSA and its frequency in normal fetuses and in those with chromosomal abnormalities. Results A total of 23 studies were evaluated. Feasibility for the ultrasound evaluation of ARSA was 85–95%. The sonographic detection of ARSA is best in the three-vessel trachea view; however, sagittal and coronal imaging of the aortic arch may be useful. ARSA in isolation was not found to be associated with chromosomal abnormalities. The prevalence of ARSA in chromosomally abnormal fetuses was up to 24-fold higher than in normal fetuses, but the majority of chromosomally abnormal fetuses with ARSA had additional abnormal ultrasound findings, particularly cardiac abnormalities. Conclusions The prenatal detection of ARSA is a clinically useful prenatal marker for chromosomal abnormalities. In isolation, it is unlikely to be associated with pathogenic genetic variants. The ultrasound diagnosis of ARSA should prompt meticulous assessment of associated abnormalities. Invasive diagnostic testing should be offered to patients with non-isolated ARSA or in the presence of non-reassuring screening results or other risk factors.

Los anillos vasculares son anomalías anatómicas que ocurren durante el desarrollo embrionario del arco aórtico, sus ramas y de la arteria pulmonar. Estas estructuras vasculares pueden conducir a grados variables de sintomatología respiratoria y/o digestiva debido a la formación de un anillo completo o parcial que comprime la tráquea y/o el esófago. La arteria subclavia derecha aberrante es la anomalía más común del arco aórtico, con una incidencia reportada entre 0.5 a 2.5%. Generalmente es asintomática, sin embargo, la disfagia es el síntoma más frecuente porque en la mayoría de los casos transcurre detrás del esófago. El diagnóstico puede ser incidental en casos asintomáticos o como resultado del estudio de síntomas digestivos y/o respiratorios persistentes o recurrentes. Los pacientes sintomáticos requieren intervención quirúrgica. Describimos el caso de una paciente de 3 meses de edad con arteria subclavia derecha aberrante. El esofagograma mostró compresión extrínseca del esófago, y la angiotomografía computada confirmó el diagnóstico. La corrección quirúrgica se realizó exitosamente.

Aberrant right subclavian artery (ARSA) is a relatively rare congenital anomaly of the aortic arch. A 74-year-old woman was referred to our Cardiac Surgery Department for chest pain. Angio-CT scan showed an acute aortic dissection and revealed an ARSA routed behind the trachea. We performed supra-coronary ascending aorta and total arch replacement with Frozen Elephant Trunk (FET) technique. The ARSA was then termino-terminal anastomosed to one branch of the Thoraflex arch graft. Postoperative CT-scan indicated a successful reconstruction of the aortic arch, proving the open stent-grafting technique as a useful and effective approach for aortic disease with ARSA.

Background: The current study aims to determine the prevalence of variations of the aortic arch using computed tomography angiography (CTA), as well as morphometries and gender correlations. Materials and methods: A retrospective, transverse, observational and descriptive study of 220 CTA was performed. The branching pattern, most cranial vertebral level of the aortic arch, area of the proximal, middle and distal segments of the arch, area of each branch, and the path of atypical arteries were recorded. Results were analysed and stratified by gender. Results: The typical aortic arch branching pattern was present in 77.7% without statistical significance between genders. The most common variant was a two-branch pattern with a common trunk and a left subclavian (13.6%), followed by a typical branching pattern with an added left vertebral artery (7.3%). T3 was the most frequent cranial level (32.3%), followed by T2–T3 (26.8%), and T3–T4 (23.2%). The mean areas of the aortic arch were 685.5 ± 183.9, 476.1 ± 124.1, and 445.0 ± 145.1 mm2 for the proximal, middle and distal segments, with statistical difference between men and women in the middle and distal segments. Three paths of atypical arteries were identified: bifurcated vertebral artery (0.5%), aberrant right subclavian artery (0.5%), and left subclavian ostium obstruction (0.5%). Conclusions: Mexican population has one of the highest prevalence of variations in the aortic arch branching pattern. The high probability of finding these should be taken into consideration when assessing patients. A standardised classification method would contemplate future un-reported findings, without causing confusion by the different numbers assigned by each author.

The most common anomaly of the aortic arch and its branches is the aberrant right subclavian artery – arteria lusoria. Usually, it produces dysphagia or dyspnea and chronic coughing.Our purposeis to underline that it is necessary to exclude the anomalies of the branches of the thoracic aorta, including arteria lusoria, in the patients with cardialgia of unknown origin.Clinical case.An 18-year-old female patient without a previously diagnosed chronic pathology was admitted to a hospital with chest pain after emotional stress for about an hour. The ECG revealed a sinus rhythm with a heart rate of 50 per minute, the normal direction of the electrical axis of the heart, the incomplete right bundle branch block, the negative T wave in the lead III. After excluding ischemic heart disease, acute coronary syndrome, pulmonary embolism, contrast-enhanced chest computed tomography revealed an aortic arch anomaly – a. lusoria.Conclusion.A. lusoria may manifest by cardiac pain. In patients with chest pain of unknown origin, it is advisable to include anomalies of the aorta and its branches, including the presence of the lusoria artery, in the range of differential diagnostics.

Objective: To investigate the frequency and types of chromosomal abnormalities in fetuses with the aberrant right subclavian artery (ARSA) and to evaluate its association with other ultrasonographic findings. Materials and Methods: In all, 11,666 fetal anatomic surveys were performed between March 2014 and March 2020. The cases diagnosed as ARSA were examined. Accompanying ultrasound findings and chromosomal abnormalities were collected. Results: ARSA was detected in 140 fetuses (1.2%). The ARSA appeared isolated in 47.1% (66/140) of cases and the remaining 52.9% (74/140) of cases were associated with cardiac or extracardiac malformations and soft markers. Chromosomal abnormalities were detected in 17.8% (25/140) of all cases. Trisomy 21 was the most common chromosomal anomaly with a prevalence of 11.4% (16/140). The corresponding rate was 3% (2/66) and 18.9% (14/74) for isolated and non-isolated ARSA, respectively. DiGeorge syndrome was detected in 3% (n=2) and Turner syndrome was in 3% (n=2) of the isolated group. ARSA was not an isolated finding in any of the 4 fetuses with trisomy 18. Conclusion: Isolated ARSA may be the only antenatal predictor of trisomy 21 or other chromosomal anomalies, including DiGeorge or Turner syndrome. Hence, visualization of the right subclavian artery should be a part of the fetal anatomic survey and genetic analysis should be recommended even in the absence of associated findings., Amaç: Aberran sağ subklavyen arteri (ASSA) olan fetüslerde kromozomal anomalilerin sıklığını ve tiplerini belirlemek ve diğer sonografik bulgularla ilişkisini değerlendirmektir. Gereç ve Yöntemler: Mart 2014 ile Mart 2020 tarihleri arasında toplam 11,666 fetal anatomik inceleme yapıldı. ASSA tanısı konulan olgular incelendi. Eşlik edilen ultrason bulguları ve kromozom anormallikleri toplandı. Bulgular: Yüz kırk fetüste (%1,2) ASSA tespit edildi. ASSA, olguların %47,1’inde (66/140) izole olarak göründü ve olguların geri kalan %52,9’u (74/140) kardiyak anomaliler, ekstrakardiyak malformasyonlar veya minör belirteçler ile ilişkili bulundu. Tüm olguların %17,8’inde (25/140) kromozom anormallikleri tespit edildi. Trizomi 21, %11,4 (16/140) prevalansı ile en sık görülen kromozomal anomaliydi. İzole ve izole olmayan ASSA için karşılık gelen oranlar sırasıyla %3 (2/66) ve %18,9 (14/74) idi. İzole grupta %3 (n=2) DiGeorge sendromu ve %3 (n=2) Turner sedromu da saptandı. ASSA, trizomi 18’i olan 4 olgunun hiçbirinde izole bir bulgu değildi. Sonuç: İzole ASSA, trizomi 21’in veya DiGeorge veya Turner sendromu dahil diğer kromozomal anomalilerin tek antenatal prediktörü olabilir. Bu nedenle, sağ subklavyen arterin görselleştirilmesi fetal anatomik incelemenin bir parçası olmalı ve ilişkili bulguların yokluğunda bile genetik analiz önerilmelidir

Highlights • Blunt thoracic aortic injuries are life threatening and require urgent intervention. • Rare presentation of a blunt thoracic aortic injury in a patient with an aberrant right subclavian artery off the aortic arch distal to the takeoff of the left subclavian artery. • An aberrant right subclavian artery originating from the aortic arch, distal to the left subclavian artery is an anatomic variant that adds significant complexity to TEVAR., Introduction Blunt thoracic aortic injuries (BTAIs) are an uncommon traumatic injury that if not treated promptly, can result in death. We present the case of a BTAI with aberrant aortic anatomy. Presentation of case A 60-year-old female was involved in a motor vehicle crash where she suffered significant polytrauma including a BTAI. She was also found to have an aberrant right subclavian artery originating from the aortic arch. Thoracic Endovascular Aortic Repair (TEVAR) with a right common carotid artery to right subclavian artery bypass was accomplished. She required three more vascular surgical interventions, two for persistent type II endoleak and the third for left upper extremity acute limb ischemia. She had a 2-month hospital course for her devastating injuries and was eventually discharged home. A follow-up CT angiogram showed a stable thoracic aortic arch stent. Discussion BTAIs are uncommon in the trauma population. In our patient who had an aberrant right subclavian artery, further procedures were required in the form of a right common carotid artery to right subclavian artery bypass and embolizations to resolve endoleaks. Conclusion Blunt thoracic aortic injuries are life threatening and require urgent intervention. TEVAR is associated with better outcomes. An aberrant right subclavian artery originating from the aortic arch, distal to the left subclavian artery is an anatomic variant that adds significant complexity to TEVAR. TEVAR is still an option for repair of blunt thoracic aortic injuries despite anatomic variations as open repair still carries an increased risk of morbidity and mortality.

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

Background: The most common aortic arch abnormality is an aberrant right subclavian artery (ARSA). ARSA-esophageal fistula is a rare sequela that can present with a life-threatening upper gastrointestinal (GI) bleed. Case Report: We report the case of an 88-year-old male who presented with signs of upper GI bleeding. Esophagogastroduodenoscopy demonstrated extrinsic compression of the upper third of the esophagus with ulceration. Imaging studies revealed ARSA posterior to the esophagus with pseudoaneurysm formation. These findings confirmed an upper GI bleed secondary to ARSA-esophageal fistula. The patient underwent prompt embolization of the ARSA pseudoaneurysm, followed a few days later by coil embolization of the ARSA pseudoaneurysm. Despite these interventions, the patient continued to have bleeding with anemia. He and his family opted to avoid any further interventions and instead pursued comfort care. The patient was discharged to hospice and died 3 months later. Conclusion: ARSA-esophageal fistula is a rare but potentially lethal cause of upper GI bleeding. Initial signs and symptoms can be subtle, but the presence of a GI bleed requires immediate stabilization. Surgical interventions have been shown to have longer-lasting success, but endovascular repair may be an option for patients who are deemed unfit for surgery but still require prompt stabilization. Regardless of the intervention, mortality rates for ARSA-esophageal fistula are high.

Background: Upper extremity intermittent ischemia due to non-aneurysmal, not occluded aberrant right subclavian artery (ARSA) is rare. Case presentation: We describe a 30-year-old male who suffered from PFO and non-aneurysmal, not occluded ARSA, and presented by intermittent right upper extremity ischemia. He was treated by right carotid subclavian transposition for ARSA and antiplatelet medication for PFO. Conclusion: Authors assume that intermittent limb ischemia can occur secondary to anatomical changes in a patient without aneurysmal degeneration or occluded ARSA and the existence of pure PFO without any evidence of venous thrombosis is not enough to prove the paradoxical emboli scenario.

An aberrant right subclavian artery usually arises from the aortic arch just distal to the left subclavian artery and crosses behind the esophagus on its way to the right axillary artery. Several reconstructive techniques of this artery in aortic surgery have been reported but mostly resulted in troublesome procedures. Here, we describe an alternative strategy presenting the occlusion of the aberrant right subclavian artery through the right pleural approach followed via extraanatomical axillary artery bypass. This surgical approach might be a simple and safe option for the aberrant right subclavian artery.

Congenital aortic arch malformations manifest a broad-spectrum of differences and abnormalities that come from disturbed embryogenesis of branchial arches. Current case was a 10 months old baby girl with length of 69 cm (less than –3 SD) and weight of 5.5 kg (less than –3 SD). The patient had history of recurrent lower respiratory tract infections since the time of birth and failure to gain adequate weight since the time of birth. The patient has been having multiple check-ups with registered medical practitioners in the nearby locality and multiple courses of antibiotics with only partial resolution of symptoms. The 2-D echocardiogram showed her to be a case of supracardiac type of “Total Anomalous Pulmonary Venous Return (TAPVR)”. All pulmonary veins making a confluence and draining into the right atrium. Significant turbulence observed at the level of superior vena cava to right atrium junction. A level of obstruction was recorded at the junction of the confluence of pulmonary veins and the vertical vein. There was aberrant right subclavian artery from the aortic arch as its third branch with no obstruction or aneurysm formation, having retrotracheal and esophageal course.

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

An aberrant right subclavian artery (ARSA) is one of the famous anatomical abnormalities with a prevalence of 0.16-4.4%. In esophagectomy, anatomical abnormalities of the ARSA could cause complications with some surgical procedures. An 85-year old man was referred to our department for esophageal adenocarcinoma that was at a slightly high position for esophagectomy with the abdominal approach. However, he had a significant past medical history. This risk factor made it difficult to perform thoracoscopic esophagectomy. He underwent mediastinoscopic esophagectomy (ME) with the left cervical and laparoscopic approach. The ARSA presented no difficulties with the ME surgical technique including the dissection of the left recurrent laryngeal nerve lymph node. Although this patient had a respiratory dysfunction and some difficulties in a prone position, ME enabled a safe esophagectomy with lymph node dissection.

Dysphagia Lusoria is a condition when aberrant right subclavian artery (ARSA) causes esophageal compression. We report 2 cases of Dysphagia Lusoria treated by hybrid endovascular technique which included subclavian to carotid transposition and embolization of origin of ARSA. By using this technique, we avoided the need for thoracotomy. Both patients had complete symptom relief without any surgery related complication.

Graphical abstract, Highlights • A TEE probe can compress an ARSA. • ARSA compression can be unsafe. • The authors offer guidelines for safe use of TEE probes.

A 34-year-old woman who demonstrated an aberrant right subclavian artery aneurysm was referred to our hospital. Single-stage total endovascular therapy with preservation of the right vertebral artery using a surgeon-modified fenestrated stent graft was performed. Contrast-enhanced computed tomography on postoperative day 4 revealed the patency of the right vertebral artery with no evidence of endoleaks. The patient was discharged on postoperative day 6. Three years later, the patient had no adverse events. When an aberrant right subclavian artery aneurysm is anatomically suitable, single-stage total endovascular therapy for such an aneurysm is considered to be a feasible and appropriate minimally invasive treatment.

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

Highlights • Aberrant right subclavian artery (ARSA) is a rare cause of dysphagia. • Surgical intervention is the mainstem of therapy. • Endoscopic dilation is a suitable treatment alternative in non-surgical candidates., Introduction The aberrant right subclavian artery (ARSA) is a rare cause of dysphagia. Surgical intervention has remained the mainstem of therapy, accompanied with certain morbidities and mortalities. Although rarely reported in literature, endoscopic dilation may be considered a suitable treatment alternative in patients who are not a surgical candidate or do not consent for surgery. We report a case suffering from dysphagia and diagnosed with ARSA treated by endoscopic dilation. Presentation of case A 52-year-old male presented to our clinic in 2015 with dysphagia. Chest Computed Tomography scan confirmed the diagnosis of ARSA. He first underwent esophagogastroduodenoscopy (EGD) with staged dilation of the stricture, making him free of his symptoms for an approximate 2.5 years. Upon recurrence of symptoms in 2018, he underwent repeat endoscopic dilation, which again completely resolved the symptom with an excellent peri-operative and post-operative course. Conclusion Endoscopic dilation of the esophageal stricture in patients with ARSA is a safe alternative to surgery in patients who are unable or unwilling to undergo surgery. It provides relief for a relatively long time and can be safely repeated multiple times upon recurrence.

Highlights • Aberrant Right Subclavian artery is also known as Arteria Lusoria. • Rarer congenital malformations of the aortic arch. • Causes compression of the trachea or esophagus causing dysphagia. • May occur simultaneously with an ostium secundum atrial septal defect. • Both conditions repaired simultaneously via Median Sternotomy., Introduction Aberrant right subclavian artery, also known as arteria lusoria, is one of the rarer congenital vascular malformations of the left sided aortic arch which arises distal to the left subclavian artery, following a retroesophageal course to the right side causing compression of the trachea or esophagus and resulting in a variety of symptoms most commonly dysphagia. Presentation of case We report a case of a two and a half year old child who was diagnosed as having as having an aberrant right subclavian artery with an ostium secundum atrial septal defect and underwent simultaneous repair of both conditions via a median sternotomy. Discussion Aberrant right subclavian artery (ARSA) has a prevalence of 1.8 % and most commonly presents in fourth or fifth decade of life. Various surgical approaches have been described to repair this anomaly over the past years, each with its own limitations and complications. However, the median sternotomy approach provides adequate exposure during division and re-implantation of the vessel and simultaneously allows repair of any intra-cardiac defect. Conclusion We believe that the best exposure for the correction of aberrant right subclavian artery is via a median sternotomy, especially in pediatric patients associated with other cardiac anomalies.

Aim: Congenital vascular malformations of the thorax are not very common. These conditions are mostly asymptomatic and detected incidentally on imaging performed for various complaints. In this study, it is aimed to examine the incidence of congenital thoracic vascular anomalies in our region.Methods: The features of the patients with non-specific complaints who underwent a thorax Computed Tomography (CT) for over 2 years period were retrospectively reviewed on the hospital database and demographical data (Gender and age) were recorded. All the Computed Tomography CT images were interpreted regarding thoracic vascular anomalies including persistent left superior vena cava, azygos lobe, aberrant right subclavian artery, dilated left superior intercostal vein, right-sided aortic arch, situs inversus, and partial anomalous pulmonary venous return. The incidences of these thoracic vascular anomalies were calculated and compared with previous studies. Results: A total of 2000 patients with a mean age of 56.13±14.62 (12-93 years) were included in this study. Among them 776 (38.8%) ones were female (mean age 56.93±15.19 (13-93 years)) and 1224 (61.2%) were male (mean age 55.63±14.23 (12-88 years)). Mediastinal vascular anomaly was detected in 62 (3.1%) patients. A mediastinal vascular anomaly was observed in 27 (3.5%) female patients and 35 (2.6%) male patients. The most common mediastinal vascular anomaly inthis study was the right aberrant subclavian artery (n:17, 0.9%) and the rarest was partial anomalous pulmonary venous return (n:1, 0.1%). Persistent left superior vena cava incidence was 0.3% (n:6), azygos lobe 0.7% (n:14), right-sided aortic arch 0.3% (n:5), situs inversus totalis 0.2% (n:3), and dilated left superior intercostal vein 0.8% (n:16).Conclusion: Mediastinal vascular anomalies are rare and usually asymptomatic. But the imaging findings of these conditions must be well-known to accurately planning the interventions and also to prevent iatrogenic injuries., Amaç: Toraksın konjenital vasküler anomalileri çok sık olmayıp sıklıkla asemptomatiktirler ve görüntülemelerde tesadüfen saptanırlar. Bu çalışmamızda bölgemizde konjenital mediastinal vasküler anomalilerin sıklığını araştırmayı planladık.Yöntem: Yaklaşık 2 yıllık bir süreçte non-spesifik semptomlar ile kontrastlı toraks Bilgisayarlı Tomografi (BT) çekilen hastaların özellikleri hastane veri tabanından retrospektif olarak tarandı. Yaş ve cinsiyet gibi demografik özellikler kaydedildi. Tüm Bilgisayarlı Tomografi (BT) BT görüntüleri, persistan sol superior vena kava, azygos lobu, aberran sağ subklavien arter, dilate sol superior interkostal ven, sağ taraflı arkus aorta, situs inversus ve parsiyel anormal pulmoner venöz dönüş anomalileri açısından yorumlandı. Çalışmamızda bulunan insidans değerleri ile önceki çalışmaların sonuçları karşılaştırıldı. Bulgular: Nihai olarak ortalama yaşı 56.13±14.62 (12-93 yıl) olan 2000 hasta çalışmaya dahil edildi. Bunların 776 (%38.8) tanesi kadın (ortalama yaş 56.93±15.19 (13-93 yıl)) ve 1224 (% 61.2) tanesi erkekti (ortalama yaş 55.63±14.23 (12-88 yıl)). Mediastinal vasküler anomali 62 (%3.1) hastada saptandı. Kadın hastaların 27 (%3.5)’inde ve erkek hastaların 35 (%2.6) ’inde bir mediastinal vasküler anomali gözlemlendi. Çalışmamızdaki en sık saptanan mediastinal vasküler anomali, sağ aberran subklavien arter (n:17, %0.9) ve en nadir görüleni ise parsiyel anormal pulmoner venöz dönüş anomalisi idi (n:1, %0.1). Persistan sol superior vena kava insidansı %0.3 (n:6), azygos lobu %0.7 (n:14), sağ taraflı arkus aorta %0.3 (n:5), situs inversus totalis %0.2 (n:3) ve genişlemiş sol superior interkostal ven ise %0.8 (n:16) olarak bulundu.Sonuç: Mediastinal vasküler anomaliler nadir olup sıklıkla asemptomatiktir. Ancak bu durumların görüntüleme bulgularının iyi bilinmesi, girişimsel işlemleri doğu biçimde planlamada ve aynı zamanda iatrojenik yaralanmaları önlemede gereklidir.

Arterioenteric or arteriotracheal fistula is a known complication of an aberrant right subclavian artery (ARSA) and is often associated with prolonged nasogastric or endotracheal intubation or oesophageal stenting. Fistula formation from the ARSA can present unexpectedly with rapid exsanguination with massive haemoptysis or haematemesis, and unless promptly recognised and treated is rapidly fatal. We present a novel endovascular method for treating a fistula between the oesophagus, trachea and an ARSA in an unstable patient following oesophageal stent removal, utilising a covered iliac limb stent, eliminating the need for an open surgical approach.

Background Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic disease. KD patients have a high risk of rupture, dissection, and compression of adjacent structures. Although several treatment options have been proposed (traditional surgery, hybrid operation, and endovascular intervention), a consensus regarding optimal surgical management has not yet been established. Case presentation A case of successful hybrid repair of distal aortic arch dissection aneurysm by dissecting KD and ARSA with debranching of right and left common carotid arteries, left subclavian artery, and stent grafting was presented. Conclusions The hybrid operation is suitable for elderly patients or those with high risks. Along with intervention, the hybrid operation needs to be developed as a minimally invasive method.

Background: Coarctation of the aorta (CoA) can either present alone as an isolated condition or in association with other aortic arch or cardiac anomalies. One percent of patients with CoA have concomitant an aberrant right subclavian artery (ARSA). Purpose: We report the case of a 35-year-old woman with uncontrolled hypertension who was found to have CoA and ARSA. Results: The patient was treated successfully using a hybrid procedure comprising ARSA ligation and subclavian to carotid transposition, followed by thoracic endovascular aortic repair. Conclusions: Patients with CoA should be carefully studied, considering the possible coexistence of other congenital aortic arch defects, such as ARSA. Hybrid repair is a safe and effective approach for this condition.

Diverticulum of Kommerell is a congenital anomaly in the development of the primitive embryonic aortic vasculature resulting in either an aberrant left subclavian artery (ALSA) from a right-sided aortic arch (RSAA) or an aberrant right subclavian artery (ARSA) from a left-sided aortic arch (LSAA). Kommerell’s diverticulum (KD), is extremely rare in the general population having prevalence of 0.7–2.0 %. KD with true aneurysmal dilatation like any vascular lesion of the thoracic aorta weakens the vasculature and increases the risk of aortic rupture. Timely surgical intervention is of paramount importance in most of these cases. Variable clinical presentations in patients with KD are either a result of compression of some mediastinal organs or strangulation by the vascular ring from the aberrant subclavian artery (ASA). We report a case of a 39 years old Chinese female with a rare co-existence of a huge KD with a microaneurysm, an aberrant right subclavian artery (ARSA), and ventricular septal defect (VSD). She underwent a successful operation and all her pre-surgical symptoms disappeared.

Objective The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). Methods This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. Results An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed amalformation: one case of hypospadias and 1 case of cleft palate. Conclusion The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers. Resumo Objetivo O objetivo do presente estudo foi determinar a frequência demalformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). Métodos Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico prénatal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Resultados Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. Conclusão A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed a malformation: one case of hypospadias and 1 case of cleft palate. The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers. O objetivo do presente estudo foi determinar a frequência de malformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). MéTODOS: Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico pré-natal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. CONCLUSãO: A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

Arteria lusoria is a rare cause of dysphagia in which dysphagia due to esophageal compression. The upper GI endoscopy does not bring significant element that can orient the diagnosis. The injected thoracic CT scan remains the key examination for the diagnosis of dysphagia lusoria and to characterize the defective artery., Arteria lusoria is a rare cause of dysphagia, in which dysphagia due to esophageal compression. The upper GI endoscopy does not bring significant element that can orient the diagnosis. The injected thoracic CT scan remains the key examination for the diagnosis of dysphagia lusoria and to characterize the defective artery.

An aberrant right subclavian artery (ARSA) is a rare arterial anomaly. Although a few cases of total endovascular repair for the ARSA aneurysm have been previously reported, anatomical limitations and the possibility of endoleaks remained. In this case, we created 4 holes on the stent graft for each cervical branch, with reference to the preoperative computed tomography findings. This approach might enable us to repair all types of thoracic aortic aneurysms with ARSA with each anatomical feature.

We report an emergent complex hybrid repair of a type A intramural hematoma with a tear of the aortic arch at the site of Kommerell's diverticulum and an aberrant right subclavian artery. We identified a type IA endoleak intraoperatively, which was managed immediately with proximal extension. Performing this operation in the hybrid operating room facilitated optimal surgical management.

Se presenta una paciente femenina de 54 años con hallazgo incidental de arteria subclavia derecha aberrante, también conocida en la literatura como arteria lusoria. Se puede considerar a la arteria lusoria como la anomalía embriológica del arco aórtico más común; descrita desde 1735 por Hunauld. La incidencia se estima entre 0.5-2% en la población general.1 La anatomía se describe como 4 arterias originandose del cayado aórtico: arteria carótida común derecha, carótida común izquierda, subclavia izquierda y subclavia derecha (la cual cruza la línea media en su recorrido). La prevalencia de esta anomalía varía según la región geográfica, en Estados Unidos se reporta una prevalencia de 0.5%.1 La falta de protocolos estandarizados de tratamiento hacen que el mismo sea basado en criterio médico, la sintomatología del paciente y su capacidad para ser sometido a procedimientos invasivos. La paciente descrita no presentaba sintomatología por lo que no recibió tratamiento.

Background An anomalous origin of the right vertebral artery (VA) from the right internal carotid artery (ICA) occurs only rarely. Case Description A 70-year-old man underwent carotid artery stenting for right internal carotid artery stenosis. In this patient, the right VA arose from the right ICA associated with an aberrant right subclavian artery. Conclusion Embryologically, failure of involution of 1 of the first 6 intersegmental arteries causes various abnormal origins of the VA. The embryonic development of this anomaly is also reviewed.

Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic arch anomaly. Patients with KD have a high risk of rupture, dissection, compression of adjacent structures, as well as distal embolization symptoms. Several treatment options have been proposed (surgical, hybrid, endovascular), however, a consensus regarding optimal surgical management has not been established yet. We present a successful single-stage hybrid repair of distal aortic arch dissection aneurysm with dissecting KD and ARSA with debranching of innominate and left common carotid artery, bilateral carotid-subclavian bypass, and stent grafting.

Purpose of review To address the incidental versus pathogenic nature of dysphagia lusoria and to provide a review of the cause, clinical presentation, diagnosis, and treatment of this condition with respect to recent literature. Recent findings Case reports comprise the majority of recent literature concerning dysphagia lusoria. Many patients with an aberrant right subclavian artery (ARSA) have additional coexisting vascular anomalies. While most individuals present around age 50, some present as children and neonates. Of note, this population may present with dysphagia as opposed to respiratory findings alone, as previously described. In the diagnostic workup, most patients receive a battery of radiologic tests, which may not be necessary. Significantly, dietary modifications and medical management alone may resolve symptoms. Nonetheless, a wide range of operative techniques are available for the treatment of dysphagia lusoria. Summary Clinicians should have a higher suspicion for dysphagia lusoria in patients with known vascular, heart, or chromosomal anomalies. Diagnosis should begin with a barium esophagram followed by a computed tomography angiogram or magnetic resonance angiogram. Avoid unnecessary studies. In many cases, an ARSA may be an incidental finding with comorbid gastroesophageal reflux disease or another medical condition responsible for the symptoms. Medical versus surgical management should be considered on a case-by-case basis.

BACKGROUND We report a rare case of numbness in the right hand, finally diagnosed as bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome and explain the cause of these diseases. CASE SUMMARY The patient was a 65-year-old woman. She complained of dizziness, numbness and weakness of the right hand for 6 mo. She was diagnosed with bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome by ultrasound, enhanced computed tomography, computed tomography angiography and other examinations. Considering the surgical risks, the patient refused the aberrant right subclavian artery stent implantation and was discharged. We hypothesize that these two kinds of deformity and right subclavian steal syndrome may not occur by accident and result from multiple malformations. CONCLUSION Bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome is rare. This case reminds interventional radiologists of the possibility of these abnormalities before surgery.

p.p1 {margin: 0.0px 0.0px 2.9px 0.0px; text-align: justify; font: 9.0px 'Helvetica Neue Light'} Sag aberrant subclavian arter (ARSA), nadir gorulen ve genellikle asemptomatik seyreden konjenital bir anomalidir (populasyonun %0.5-1.8’i). Bu calismada supraklavikular insizyon ile basarili bir sekilde tedavi edilen uc kiz hastanin sunulmasi amaclandi. Ameliyat sirasinda ortanca yas 15’di (aralik: 10-18 yas). Ameliyat sirasinda ve sonrasinda komplikasyon izlenmedi. Bilateral ulnar ve radyal pulsasyonlari ameliyat sonrasi normaldi. Ameliyat sonrasinda beslenme ile ilgili problem gozlenmedi. Supraklavikuler insizyon ile ARSA’ nin uc yan anastomozu, disfaji lusoriasi olan cocuklar icin uygulanabilir ve minimal invaziv bir yontem olarak dusunulmelidir.

内頚動脈低形成と異所性右鎖骨下動脈に合併した未破裂内頚動脈瘤に対し，コイル塞栓術を施行した症例を経験したので報告する．症例は56歳女性．めまいの精査目的に他院で施行したmagnetic resonance (MR) imagingで左内頚動脈瘤を指摘されたため当院へ紹介となった．初診時，神経学的な異常は認めなかった．脳血管撮影とCT angiographyで右内頚動脈低形成と異所性右鎖骨下動脈を認めた．左内頚動脈瘤の大きさは7.5×5.8 mm，neckは3.6 mmであり，ブレブを伴っていた．破裂予防目的に左内頚動脈瘤に対してバルーン併用下コイル塞栓術を施行した．術中，左内頚動脈の血流遮断を数回要した．左内頚動脈瘤は完全に塞栓され，周術期に有害事象は認めなかった．内頚動脈低形成と異所性右鎖骨下動脈の合併は稀であり，これらの破格を有する症例では血管内手術を行う際，アクセスルートの制限や脳虚血耐性に注意が必要である．

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

DiGeorge’s syndrome is a 22q11.2 deletion leading to abnormal embryogenesis of pharyngeal arches and it is manifesting in a variety of clinical signs and symptoms. The spectrum of anomalies varies from minor facial dysmorphism and cleft palate to a broad spectrum of cardiovascular anomalies, thymic disfunction and immune deficiencies, hypocalcemia due tohypoparathyroidism,growth and developmental delay and speech disturbances. Cardiovascular anomalies might include right sided aortic arch, aberrant vesiclesand vascular ring. Here we present an atypical case of partial DiGeorge’s syndrome with feeding and swallowing difficulties and laryngeal stridor in the neonatal period. Early presentation in this period is usually due to severe hypocalcemia and cardiac disease. Feeding difficulties in a preterm baby needed clinical assessment skills in order to establish the diagnosis and delineate it from feeding difficulties usually seen in preterm babies. Esophagogram (barium X Ray) showed antero-posterior oblique impression towards the right side, the latero- lateral view showed impression on the rare side, suspected to be esophageal sub stenosis due to vascular anomaly, aberrant right subclavian arteryand suspectedthymic hypoplasia. We report a 9-year follow up periodbya team of subspecialists. The child had two surgeries due to aberrant vessel and velopharyngeal deficiency. Optimal management of patients with DiGeorge’s syndrome requires a multidisciplinary teamwhichshould include a cardiologist, immunologist, geneticist, speech/language therapist, endocrinologist and other subspecialists depending on patient`'s phenotype., DiGeorge Синдромот е резултат на делеција на 22q11.2 која води до пореметена ембриогенеза на фарингеалните лаци и се манифестира со различни знаци и симптоми. Спектарот на аномалии во овој синдром е варијабилен и може да се презентира само со минорна лицева дизморфија и расцеп на непцето но и со широк спектар на кардиоваскуларни аномалии, дисфункција на тимус и имун дефицит, хипокалцемија поради хипопаратироидизам, пореметување во растот и развојот и проблеми во говорот. Кардиоваскуларните аномалии може да се од типот на десен аортен лак, аберантни крвни садови и васкуларен ринг. Во овој приказ на случај прикажуваме атипичен случај на парцијален синдром на DiGeorge кој се презентираше со потешкотии во голтање и ларингеален стридор во неонаталниот период. Раната презентација на овој синдром во неонаталната возраст најчесто се дијагностицира со хипокалцемија и конгенитална кардиопатија. Потребна е добра клиничка проценка за да се разликува проблем при хранење на прематурно новородено од другите проблеми при голтање. Езофагограмот покажа коса импресија на антеро-постериорниот правец со ориентација кон десно суспектна за субстеноза на езофагусот на опишаното ниво од васкуларна етиологија, аберантна десна артерија субклавија и суспектна хипоплазија на тимус. Пациентот беше следен во период од 9 години од тим на субспецијалисти. Во тој период имаше две хируршки интервенции (корекција на аберантен крвен сад и велофарингеален дефицит). Пациентите со синдром на DiGeorge треба да бидат следени од мултидисциплинарен тим кој вклучува кардиолог, имунолог, генетичар, ендокринолог, логопед и други субспецијалисти според фенотипот на пациентот.