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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.

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.

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.

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.

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.

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.

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.

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.

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.

Managing type B aortic dissection (TBAD) involving Kommerell’s diverticulum (KD), aberrant right subclavian artery (ARSA), and isolated left vertebral artery (ILVA), is extremely challenging. As treatment, we described a one-stage hybrid technique combined with thoracic endovascular aortic repair (TEVAR) with open surgery through a supraclavicular incision. A 57-year-old man with TBAD and the three anomalies successfully underwent hybrid TEVAR. A side-to-side artificial bypass between the ARSA and the right common carotid artery was established through a right supraclavicular incision before TEVAR. The release of the stent-graft was designed from the distal aortic ostium to the left common carotid artery (LCCA) to cover the ILVA, ARSA, and left subclavian artery (LSA). Then, the ILVA and LSA were transposed to the LCCA through a left supraclavicular incision. Intraoperative angiography confirmed complete false lumen exclusion and KD, with all branches patent and without endoleaks. Computed tomography angiography 7 days and 1 year postoperatively demonstrated well-perfused ARSA, LSA, and ILVA, and a fully expanded stent-graft with no endoleaks, migration, disconnection, or stenosis. TBAD involving KD, ARSA, and ILVA in one case is rare. This is the first report to treat this pathology with a one-stage supraclavicular hybrid procedure.

Objectives We present a case in which a 44-year-old female smoker with a complex medical and surgical history presented with acute upper limb ischemia. Methods The patient was diagnosed with thoracic outlet syndrome causing symptomatic thrombosis of an aberrant right subclavian artery associated with diverticulum of Kommerell. The patient had history of extensive surgeries and radiation in the right chest with fibrotic changes, so surgical dissection in the area was associated with significant risk of nerve injury. Therefore, revascularization of the arm was performed via carotid to brachial artery bypass with reversed saphenous vein and the thoracic outlet was decompressed with segmental clavicular resection. Results Patient has been symptom free for 3 years with patent bypass. Conclusions This case demonstrates a unique presentation of distal thrombosis of a symptomatic aberrant right subclavian artery related to thoracic outlet syndrome. The combined claviculectomy–vein bypass is a safe and efficacious surgical approach that provides durable relief.

Aberrant right subclavian is a rare anomaly presenting in 0.3–3.0% of the population. Kommerell’s diverticulum is an aneurysm associated with this phenomen; data relating to its incidence is sparse. Currently there are no well-established guidelines for the treatment of Kommerell’s diverticulum, limitation being the rare occurrence of disease and heterogenous population with disease presentation. This case report illustrates a novel approach to the repair of a symptomatic Kommerrel’s diverticulum with severe coronary disease. Management should be tailored on a case by case basis to the individual patient.

Purpose: Aberrant right subclavian artery (ARSA) associated with Kommerell’s diverticulum (KD) is a common congenital arch anomaly. It can be complicated by type B aortic dissection (TBAD) or aneurysmal formation at its ostium. Recently, hybrid repair with thoracic endovascular aortic repair (TEVAR) has appeared to be more favorable. Due to the normal anatomic proximity of the ARSA to the left subclavian artery (LSA) orifice in KD, coverage of the bilateral subclavian arteries (SCAs) to obtain an adequate proximal landing zone (PLZ) is usually required, and double cervicotomy for SCA revascularization potentially increases the risk of complications. Technique: This technique was demonstrated on a 50-year-old man presenting with progressive aneurysmal formation of KD with ARSA after chronic TBAD. A 3-step technique, namely left cervical debranching with a left common carotid artery to LSA bypass graft, TEVAR, and an LSA-to-ARSA endovascular debranching with a self-expanding covered stent by a through-and-through wire from the right brachial artery to the bypass graft, was performed in a 1-stage repair to cover the primary tear of TBAD and preserve the bilateral SCAs. The postoperative course was uneventful. Conclusion: This technique can prevent complications from double cervicotomy and achieve an adequate PLZ with preservation of the bilateral SCAs for TEVAR.

Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortic arch. The presence of ARSA has been previously associated with an increased risk of Down syndrome. ARSA at birth may be associated with dysphagia, respiratory distress and stridor and there is no clear evidence‐based management. The aim of this study was to describe the associations with chromosomal abnormalities and the postnatal outcome of fetuses diagnosed with ARSA. We analysed fetuses diagnosed antenatally with ARSA between January 2013 and September 2019 in the fetal echocardiography unit of the Hospital Monaldi, University ‘Vanvitelli’ of Naples, Italy. The results showed fifty fetuses diagnosed with ARSA, all confirmed after birth. The ARSA was an isolated finding in 46 fetuses (92%), while in 4 fetuses the ARSA was associated with other cardiac and/or extra-cardiac anomalies. Only one fetus was diagnosed with trisomy 21 (2%). In this fetus the ARSA was the only ultrasound anomaly identified. There were no cases necessitating referral due to the presence of compression symptoms at birth. The presence of ARSA was associated with trisomy 21 in the 2% of cases in our series and there were no neonatal complications due to airway compression at birth.IMPACT STATEMENTWhat is already known on this subject? Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortich arch. ARSA at birth could be associated with dysphagia, respiratory distress and stridor and no evidence-based management of these fetuses has been described yet. The presence of ARSA has been previously associated with an increased risk of Down syndrome. What do the results of this study add? This study confirms known data on association with chromosomal defects and provides some original data on the absence of symptomatology due to tracheal compression with a postnatal follow-up up to three years of age. What are the implications of these findings for clinical practice and/or further research? Our findings suggest that in cases with adequate prenatal assessment performed by experienced clinicians, delivery can safely take place at local hospitals, with no need of referral soon after birth. The use of transthoracic echocardiography to confirm the diagnoses of ARSA after birth and to plan the next follow-up appointments can be supported.

Abnormal right subclavian artery (ARSA) is the most common anomaly of the aortic arch with a rate of 0.5-1.4%. Normally, three vessels arises from the aortic arch, while four vessels arise in ARSA. ARSA leaves the distal of the aortic arch and passes behind the esophagus and trachea. It is also called the abnormal retroesophageal right subclavian artery. It is generally an asymptomatic benign finding, but it can cause esophageal compression causing dysphagia. In this article, an isolated ARSA and trisomy 21 case referred to our clinic in the second trimester of pregnancy and detected ultrasonographically is presented.

Koolen de Vries syndrome is a rare genetic disorder with an estimated prevalence 1:16000 in the general population but it is considered to be an underdiagnosed syndrome. We report a 17q21.31 microdeletion which was diagnosed prenatally in a fetus with aberrant right subclavian artery (ARSA). Koolen de Vries syndrome is a rare chromosomal abnormality and according to the literature this is the first case in which the detection of ARSA led to this syndrome.

La arteria subclavia derecha aberrante (ARSA) es la anomalía del arco aórtico más común. La probabilidad de hallar un ARSA durante un estudio ecográfico es de alrededor de 1%. En aquellos fetos con ARSA, la probabilidad de tener otros hallazgos cardiacos y/o extracardiacos o una anomalía cromosómica es elevada. La prevalencia de la relación del ARSA con el síndrome de Down es de aproximadamente 20%, por lo que este marcador puede contribuir al asesoramiento del síndrome de Down en el segundo trimestre y probablemente en el primer trimestre. La recomendación ante el hallazgo de ARSA es realizar un estudio detallado de la anatomía fetal en busca de otros marcadores de aneuploidías y realizar ecocardiografía fetal. La realización de estudios invasivos quedará limitado a aquellas situaciones en donde además del ARSA se encuentren otros marcadores u otras condiciones que aumenten el riesgo de síndrome de Down. Sin embargo, ante el hallazgo de ARSA aislado, el incremento en el riesgo es igual a cero, pudiendo considerarse una variante de la normalidad.

An aberrant right subclavian artery is a congenital aortic arch anomaly in which the right subclavian artery originates from the proximal descending aorta. The presence of an aberrant right subclavian artery can make right transradial coronary interventions more difficult and even lead to complications. Iatrogenic intramural hematomas and dissection of aberrant right subclavian arteries during transradial coronary angiography have been previously reported. We herein report a case of iatrogenic aortic dissection following attempts to perform right transradial coronary angiography in a patient with an aberrant right subclavian artery. Clinicians should be vigilant for the presence of an aberrant right subclavian artery during right transradial coronary angiography and ensure gentle manipulation of wires and catheters to avoid complications.

Background Diagnostic cerebral angiography (DSA) and neurointerventions have traditionally been performed via transfemoral access (TFA). The perception of a steep learning curve associated with transradial access (TRA) has limited adoption in neurointervention. This study compares the learning curves of transradial vs. transfemoral DSA in a cohort of neurointerventional fellows. Methods The first 150 consecutive radial and femoral DSA were identified for each fellow from a prospective neurointerventional registry from July 2017 to March 2020. Total fluoroscopy time and number of intracranial arteries injected were recorded. Mean fluoroscopy time per intracranial artery injected (termed angiographic efficiency) was calculated and was used as a surrogate measure of technical proficiency. Mean angiographic efficiencies were compared across partitions of 25 consecutive DSAs (e.g. 1–25, 26–50, 51–75, etc.). Results There were 607 radial DSA and 635 femoral DSA identified among 5 fellows. The overall angiographic efficiencies were not significantly different based on access site (radial mean 3.2 min, femoral mean 3.7 min, p>0.05). For 3 fellows without prior endovascular experience, technical proficiency was obtained between 25–50 femoral DSA procedures. Among these same fellows, one fellow achieved technical proficiency after 25–50 radial DSA procedures, while the other 2 fellows had flattened learning curves. There were 2 fellows that had no significant learning curve for either access type, but both had extensive experience with endovascular procedures prior to starting fellowship. Two patients (2/1342 = 0.1%) experienced transient neurologic symptoms post-procedure. Among 635 femoral DSA, there were 22 (3.5%) minor adverse events (14 small groin hematomas not requiring transfusion, 1 pseudoaneurysm, 7 non-flow-limiting dissections). Among 607 radial DSA, there were 3 (0.5%) minor adverse events (2 small forearm hematomas, 1 intraluminal wire removed with radial cut-down), which was significantly less than femoral DSA minor adverse events (p=0.0001). Radial to femoral conversion rate was 1.2% (7/607, 2 radial spasm, 2 unsuccessful radial artery access, 3 aberrant right subclavian artery). Femoral to radial conversion rate was 0.3% (2/635). From March 2019 to February 2020, the proportion of DSA performed via transradial access increased from 36% to 78% at our institution. Conclusion This study demonstrates the learning curves for both TRA and TFA diagnostic cerebral angiograms. Proficiency improves significantly with either type of access, typically requiring between 25–50 procedures. TRA DSA have significantly less access site complications than TFA DSA. Disclosures M. Tso: None. G. Rajah: None. R. Dossani: None. M. Meyer: None. M. McPheeters: None. K. Vakharia: None. M. Waqas: None. K. Snyder: None. E. Levy: None. A. Siddiqui: None. J. Davies: None.

We hereby present a rare variant course of left brachiocephalic vein coexisting with an aberrant right subclavian artery in a patient with a ventricular septal defect with pulmonary atresia. This case highlights the importance of CT angiography in diagnosing associated aortic arch and systemic venous anomalies in complex congenital heart diseases.

A 69-year-old female suffering from severe dysphagia due to an aberrant right subclavian artery was treated with a two-step approach. A right carotid-subclavian bypass was followed 2 weeks later by a robotic-assisted thoracoscopic resection of the aberrant right subclavian artery. The postoperative course was uneventful, and the patient immediately recovered from her dysphagia. In our case, the robotic-assisted technology offered major advantages and, based on our experience, may be useful in the treatment of this rare disease.

Objectives: We hoped to reveal the frequency of Aberrant Right Subclavian Artery (ARSA) and to find the relationship of isolated/non-isolated ARSA with chromosomal defects and other fetal congenital heart diseases (FCHD) in a heterogeneous population. Material and methods: This was a retrospective cohort study conducted between December 2015 to September 2018. Women admitted for routine ultrasound examination or referred to our hospital for a suspected fetal anomaly were underwent detailed fetal anomaly ultrasonography scan and tested for the presence of ARSA. Results: ARSA was detected in 27 patients and an isolated finding in 13 (48%) cases. Among 13 cases with isolated ARSA, trisomy 21 was diagnosed in 1 case. In the non-isolated group (n: 14, 52%), five cases presented with trisomy 21. There was no significant difference of trisomy 21 frequency between isolated and non-isolated groups (7.6% vs 35.7%, p = 0.08). In 3 patients, FCHD was diagnosed and 2 of them had trisomy 21. Conclusions: Our study shows that ARSA can be the only marker in trisomy 21. The examination of the subclavian artery must be a part of the fetal anomaly ultrasonography. Detecting an ARSA should increase the attentiveness of the sonographer to investigate for the other markers of trisomy 21. In the existence of other findings, invasive diagnostic procedures should be offered to the patients, whereas in cases that arsa is the only finding, other risk factors should be investigated to offer karyotyping or cell-free DNA analysis.

The objective of this study was to evaluate the association between aberrant right subclavian artery (ARSA) and chromosomal abnormalities. The study included 5211 women having attended our unit for fetal anatomic screening and fetal echocardiography from August 2016 until February 2019. After diagnosing ARSA, prenatal invasive testing was discussed with the patients. ARSA affected fetus was determined in 57 cases; of these, there were 38 cases of isolated ARSA and 19 cases of non-isolated ARSA but associated with soft markers and fetal anomalies. Nineteen patients underwent amniocentesis; Down syndrome was determined in two women, both of them from the non-isolated ARSA group, with fetal hydrops, atrioventricular septal defect and esophageal atresia. Fifteen of 38 patients who declined prenatal diagnostic testing, accepted karyotype analysis after delivery and none of these 15 cases had chromosomal abnormalities. Identification of ARSA should be followed by detailed ultrasound examination to ensure that there are no accompanying soft markers and/or structural defects. Isolated ARSA may not be an indication for karyotype analysis or 22q11.2 microdeletions. Non-ARSA implies a strong predictor of aneuploidy, and when additional findings are detected, invasive testing should be offered to the parents. The association between isolated ARSA and genetic disease should be evaluated in large powered prospective studies., Cilj ovoga istraživanja bio je procijeniti udruženost aberantne desne potključne arterije (ADPA) i poremećaja kromosoma. Istraživanje je obuhvatilo 5211 žena koje su posjetile našu jedinicu za anatomski probir i fetalnu ehokardiografiju od kolovoza 2016. do veljače 2019. godine. Nakon što je dijagnosticirana ADPA sa ženama se razgovaralo o prenatalnom invazivnom testiranju. Fetalna ADPA utvrđena je u 57 slučajeva, uključujući 38 slučajeva izolirane ADPA i 19 slučajeva ne-izolirane ADPA, ali udružene s „mekim“ biljezima i fetalnim anomalijama. Devetnaest žena podvrgnuto je amniocentezi. Downov sindrom utvrđen je kod dvije žene, obje iz skupine s ne-izoliranom ADPA, s fetalnim hidropsom, atrioventrikulskim septalnim defektom i atrezijom jednjaka. Petnaest od 38 žena koje su odbile prenatalno dijagnostičko testiranje prihvatilo je analizu kariotipa nakon porođaja i nijedna od njih nije imala kromosomne poremećaje. Nakon identificiranja ADPA treba uslijediti podroban ultrazvučni pregled kako bismo bili sigurni da ne postoje prateći „meki“ biljezi i/ili strukturni defekti. Izolirana ADPA ne mora biti indikacija za analizu kariotipa ili mikrodelecije 22q11.2. Ne-ADPA snažno upućuje na aneuploidiju, a kad se dobiju dodatni nalazi tada treba roditeljima ponuditi invazivno testiranje. Udruženost izolirane ADPA i genetske bolesti treba procijeniti u velikim i valjanim prospektivnim studijama.

An aberrant right subclavian artery aneurysm ruptured in an 83-year-old male who presented with chest pain, shortness of breath, with associated hemothorax. Patient underwent thoracic endovascular aortic repair (TEVAR) and coil embolization of subclavian artery without carotid-subclavian bypass. Right hand ischemia gradually improved during the follow-up period of 10 months with no residual symptoms in the right hand. There was significant improvement in the right wrist brachial index.

Deep Hypothermic Circulatory Arrest (DHCA) is a technique used to obtain optimal operating conditions while providing cerebral protection. The case report presented a DHCA on an infant that was basically done as an emergency in an attempt to correct a previously unrecognized anomaly. We report a case of a 1-year-old that had surgery for Ventricular Septal Defect (VSD) and Patent Ductus Arteriosus (PDA) ligation but following closure was noted to have no palpable peripheral pulses, only carotid pulsation. This necessitated an emergency reopening of the chest. Close inspection revealed an interrupted aortic arch with aberrant right subclavian artery and pre-surgery PDA supplying both upper and lower limb. DHCA was immediately commenced and the patient cooled to 16°C. The surgeon promptly set out to attach the subclavian artery to the ascending aorta and descending aorta. At the completion of the surgery, the patient was taken to Intensive Care Unit (ICU) for critical care support. She was subsequently discharged after spending a little more than a week in ICU. This procedure is rarely done as an emergency but was instituted in our case in effort to immediately achieve perfusion to the limbs. As the expertise to carry out the procedure is limited, it might be better to develop ways to efficiently ensure the skill set is continually updated.

Introduction Aberrant right subclavian artery (ARSA), or arteria lusoria is the most common embrologic anomaly of the aortic arch. It is generally asymptomatic and incidentally diagnosed during a radiological exam or procedure. Presentation of case Here, we report a case of ARSA incidentally diagnosed and injured (dissection with mediastinal hematoma) during a right transradial coronary angiography in a 83 years old female patient. The patient underwent prompt hybrid procedure with the isolation of the right humeral artery from where we positioned a GORE® VIABAHN® 9 × 100 mm endoprosthesis. The procedure was successful with optimal results at early and long term follow up. Discussion Sometimes, a dissection of the ARSA may occur, especially for excessive manipulation during endovascular procedures, and when such complication happens it should be promptly treated as it can be life-threatening. Conclusion If transradial catheterization during coronary angiography becomes particularly difficult, requires longer time, or the guide wire enters in the descending aorta, particularly attention should be paid, as dreadful complications such as dissection or lesion may happen and prompt treatment is required.

espanolIntroduccion: la arteria subclavia aberrante es la anomalia mas comun del arco aortico, aunque la clinica de disfagia lusoria aparece tan solo en el 0,5-2 % de los pacientes. No existe ningun consenso sobre su tratamiento. Caso clinico: se presenta el caso de una paciente de 86 anos con clinica de disfonia y disfagia de 1,5-2 anos de evolucion que asocia una perdida de peso de 10 kg en los ultimos 6 meses. En el escaner realizado durante el estudio se encuentra una arteria subclavia aberrante no dilatada que comprime el esofago a su paso retrocardial. Ademas, la paciente presentaba una estenosis del 60 % de la arteria carotida ipsilateral que le habia ocasionado un ictus 5 meses antes. Se realiza un bypass carotido-subclavio derecho con protesis PTFE de 6 mm y en la misma intervencion, tras el procedimiento anterior, se realiza una endarterectomia carotidea derecha y angioplastia quirurgica. El posoperatorio transcurrio sin complicaciones. La paciente se mantuvo asintomatica, sin eventos neurologicos; tampoco disfagia ni disfonia. Pudo retomar la ingesta normal de alimentos. En el CT de control a los 10 dias se observo la arteria subclavia derecha trombosada sin compresion residual, por lo que no se considero necesaria la exclusion de su origen con una endoprotesis toracica. Discusion: aunque en un primer momento se considero la posibilidad de realizar un tratamiento en dos tiempos (cirugia abierta y, posteriormente, endovascular toracico), el tratamiento quirurgico combinado permitio tratar la disfagia lusoria, asi como la estenosis carotidea sintomatica ipsilateral, durante la misma intervencion para no tener que someter a una paciente mayor a una cirugia excepcionalmente larga, ademas de omitir el uso de una endoprotesis toracica complementaria que finalmente no fue necesaria. EnglishIntroduction: the aberrant subclavian artery is the most common variation of the aortic arch but symptoms of dysphagia lusoria appear only in 0,5-2 % of the patients. There is no consensus on its treatment. Case report: we hereby present the case of an 86 year-old woman who had suffered dysphonia and dysphagia for 1.5-2 years associating a loss of 10 kg of weight in the last 6 months. An angioCT scan revealed a non-dilated aberrant right subclavian artery which compressed of the retrocardial esophagus, and a 60 % stenosis of the right carotid artery, which had caused an ipsilateral minor stroke 5 months before. We performed a right carotid-subclavian bypass using a 6 mm ePTFE graft, followed by a right carotid endarterectomy and Dacron patch angioplasty in the same procedure. The postoperative period was uneventful, the patient remained asymptomatic, with no neurological symptoms, dysphagia or dysphonia, and resumed normal oral intake. Ten days after the procedure the control CT revealed a thrombosed right subclavian artery with no residual esophageal compression, so an additional endovascular occlusion of the subclavian origin was deemed unnecessary. Discussion: although we first considered the possibility of performing a sequential hybrid treatment, with initial surgical bypass and secondary thoracic endovascular grafting, the combined open surgical technique allowed us to solve both the dysphagia lusoria and the symptomatic right carotid stenosis in one single procedure, and the complementary thoracic endografting was ultimately unnecessary.