A case of right aortic arch type II combined with Kommerell's diverticulum and left innominate vein beneath the aortic arch.

The combination of the right aortic arch and aberrant left subclavian artery (ALSA) with Kommerell's diverticulum (KD) is rare to coexist with the left innominate vein (LINV) beneath the aortic arch. It escalates the surgical risk undoubtedly and increases the difficulty of clinical procedures. We report one case diagnosed by Ultrasound and Computed Tomography Angiography (CTA).

Postnatal outcome of fetal aberrant right subclavian artery: a single center study.

PURPOSE: This study aims to explore the correlation between fetal aberrant right subclavian artery (ARSA) and chromosomal disorders, with a specific focus on Down syndrome and DiGeorge syndrome. METHODS: From November 2017 to February 2020, we conducted fetal anomaly screening and assessed the fetal heart in 8494 at our institution. The right subclavian artery tracing was assessed using Doppler ultrasonography following the 3-vessel and tracheal views (3VTV) in the fetal heart scan. RESULTS: ARSA was found in 31 fetuses, which accounts for 0.36% of the total of 8494 fetuses. 96.8% of fetuses with ARSA were found to have normal chromosomal analysis. We identified only one case of trisomy 21 as the chromosomal condition present. In 80% of the identified ARSA, there were no additional associated findings. CONCLUSION: ARSA is a rare condition that often does not manifest any concomitant abnormalities. The majority of ARSA instances identified in the second trimester are euploid. If ARSA is the only sonographic finding during fetal anomaly screening and there are no maternal or laboratory risk factors, further evaluation with non-invasive diagnostics may be recommended. Non-invasive genetic testing may be used for additional investigation.

Right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery.

PURPOSE: Here, we report a case of the right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery. METHODS: A 42-year-old male patient underwent a whole-body computed tomography angiography (CTA) examination upon the clinical suspicion of vasculitis. RESULTS: CTA revealed a right-sided aortic arch with the isolation of the left innominate artery and hypoplasia of the left internal carotid artery. CONCLUSION: The right-sided aortic arch, with the isolation of the left innominate artery, is a scarce vascular variation that may occur with other cardiovascular anomalies such as ventricular septal defect. It can be asymptomatic or can present with symptoms of subclavian steal syndrome. Although its association with the agenesis of the left internal carotid artery has been reported, its association with the hypoplasia of the left internal carotid artery has not been reported previously to the best of our knowledge.

Dysplastic aberrant left subclavian artery originating from a thoracic intersegmental artery associated with a right aortic arch.

PURPOSE: A right aortic arch (RAA) is a rare vascular anomaly that often coexists with an aberrant left subclavian artery (ALSA). Due to the rarity of RAA, the development of an ALSA is not well understood. METHOD: We describe a case in which a 58-year-old man who was scheduled to undergo posterior decompression and fusion surgery for thoracic ossification of the posterior longitudinal ligament from Th1 to Th3 was found to have a RAA and an ALSA. RESULTS: Preoperative computed tomography angiography demonstrated a RAA and an ALSA. The ALSA was extremely tortuous and ran in the paraspinal muscles behind the thoracic laminae, which meant it was in the surgical field. The ALSA arose from the descending aorta and bifurcated into the left segmental arteries of Th1 and Th2, and also bifurcated into the left vertebral artery, which had a normal subsequent course. The dysplastic ALSA was considered to have developed from the thoracic intersegmental artery. Based on preoperative examination findings, we performed spinal surgery without vessel injury. CONCLUSION: We report a rare case of a dysplastic ALSA that developed from the thoracic intersegmental artery with a RAA. The knowledge of this anomaly provides safety in spinal surgery of the cervicothoracic junction.

[Aortic Arch:Embryology and Remodeling].

A right aortic arch and aberrant subclavian artery result from an interruption in the remodeling of the pharyngeal arch arteries. We occasionally encounter this anatomical variation during angiography. Patients with disorders such as Down syndrome and congenital heart disease show a high incidence of an aberrant right subclavian artery, and this anomaly can cause symptomatic esophageal or tracheal compression. The root of the aberrant artery may show dilatation(referred to as a Kommerell diverticulum), dissection, intramural hematoma, or rupture necessitating cardiac intervention using a surgical or endovascular approach. Neurointerventionalists should have working knowledge of the anatomy to rapidly understand the anatomy and ensure a safe procedure. A left transradial approach should be considered if prior knowledge of the aberrant subclavian anatomy is available.

[Right aortic arch with mirror image branching : a rare cause of dysphagia]. / L'image du mois. Arc aortique droit avec image en miroir : une cause rare de dysphagie.

We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This is a rare embryonic developmental anomaly where the aorta wraps around the right bronchus and the supra-aortic trunks emerge from the arch in the opposite order to normal. Most of the patients are asymptomatic unless there is a significant compression of mediastinal structures. Major compression of the esophagus or trachea, aneurysmal disease, dissection of the thoracic aorta, or the presence of a Kommerell diverticulum larger than 2 cm may require a surgical repair. There is no standard treatment and it must be adapted to the clinical presentation and the anatomic configuration of each patient. Our patient did not receive any treatment for her condition.

Nous rapportons le cas d'une patiente de 36 ans dont le tableau de dysphagie a permis de mettre en évidence une anomalie congénitale de l'aorte thoracique : l'arc aortique droit avec image en miroir. Il s'agit d'une anomalie de développement embryonnaire rare où l'aorte s'enroule autour de la bronche souche droite et où les troncs supra-aortiques émergent de la crosse dans l'ordre inverse et opposé à la normale. La grande majorité des patients est asymptomatique, à moins qu'il existe une compression des structures médiastinales. Une compression majeure de l'oesophage ou de la trachée, une maladie anévrismale, une dissection de l'aorte thoracique ou la présence d'un diverticule de Kommerell de plus de 2 cm peuvent justifier une sanction chirurgicale. Il n'y a pas de traitement standard et celui-ci doit être adapté à la présentation clinique et à la configuration anatomique du patient. Notre patiente n'a bénéficié d'aucun traitement pour son affection.

A systematic review of open, hybrid, and endovascular repair of aberrant subclavian artery and Kommerell's diverticulum treatment.

BACKGROUND: Aberrant subclavian artery (AScA) with or without associated Kommerell's diverticulum (KD) is the most frequently encountered anomaly of the aortic arch, it may be life threatening, especially when associated with aneurysmal degeneration. The best management is still debated and depends on many clinical and anatomical factors. A systematic review was conducted to assess the current evidence on the treatment options and their efficacy and safety for AscA and KD repairs. METHODS: A literature search in PubMed and Cochrane Library was performed, and articles that were published from January 1947 to August 2021 reporting on AscA and KD management were identified. Multicenter studies, single-center series, and case series with three or more patients were considered eligible in the present review. A comparison of outcomes of patients who underwent open surgery (OS), a hybrid approach (HA), and total endovascular repair (ER) (ie, 30-day mortality and stroke were analyzed when available and compared among the three groups (P < .05; Benjamini and Hochberg-adjusted P < .05; Bonferroni-adjusted P < .05). Titles, abstracts, and full texts were evaluated by two authors independently. Primary outcomes included survival rate, perioperative stroke, arm ischemia, and spinal cord ischemia. Endoleak, in the case of HA and ER, and reintervention rates were considered outcomes. RESULTS: Three hundred thirty-one articles were initially evaluated and 30 studies, totaling 426 patients treated for AScA with 324 KD, were included. Of the 426 patients, 241 were male (56.5%), and the mean patient age was 57.9 ± 12.0 years. The approach was OS in 228 patients, HA in 147 patients, and ER in 51. Dysphagia was the most common presentation in 133 cases. Aortic rupture was observed in 16 of the 426 patients (3.8%), including 14 AScA/KD (3.3%) aneurysm rupture. KD maximum diameter varied widely from 12.6 to 63.6 mm. The overall 30-day mortality was 20 (4.7%) (OS group 8/228 [3.5%]; HA group 10/147 [6.8%]; ER 2/51 [3.9%]; P = NS). The overall stroke rate was 4.9% (21/426) (OS group 10/228 [4.4%]; HA group 6/147 [4.1%]; ER group 5/51 [9.8%]; P = NS), including nine transient and nondisabling neurological deficits. Overall secondary procedures for complications were reported in 36 of the 426 cases (8.4%), mostly owing to endoleak. Follow-up varied from 13 to 74 months. CONCLUSIONS: This systematic review assessed the current outcomes of the three types of therapeutic management for AScA and KD and demonstrated that they are all relatively safe and effective, providing satisfactory early and midterm outcomes. Long-term outcomes are warranted, especially for total ERs since the long-term durability of stent grafts remains unknown.

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

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

International Multi-Institutional Experience with Presentation and Management of Aortic Arch Laterality in Aberrant Subclavian Artery and Kommerell's Diverticulum.

BACKGROUND: Aberrant subclavian artery (ASA) with or without Kommerell's diverticulum (KD) is a rare anatomic aortic arch anomaly that can cause dysphagia and/or life-threatening rupture. The objective of this study is to compare outcomes of ASA/KD repair in patients with a left versus right aortic arch. METHODS: Using the Vascular Low Frequency Disease Consortium methodology, a retrospective review was performed of patients ≥18 years old with surgical treatment of ASA/KD from 2000 to 2020 at 20 institutions. RESULTS: 288 patients with ASA with or without KD were identified; 222 left-sided aortic arch (LAA), and 66 right-sided aortic arch (RAA). Mean age at repair was younger in LAA 54 vs. 58 years (P = 0.06). Patients in RAA were more likely to undergo repair due to symptoms (72.7% vs. 55.9%, P = 0.01), and more likely to present with dysphagia (57.6% vs. 39.1%, P < 0.01). The hybrid open/endovascular approach was the most common repair type in both groups. Rates of intraoperative complications, death within 30 days, return to the operating room, symptom relief and endoleaks were not significantly different. For patients with symptom status follow-up data, in LAA, 61.7% had complete relief, 34.0% had partial relief and 4.3% had no change. In RAA, 60.7% had complete relief, 34.4% had partial relief and 4.9% had no change. CONCLUSIONS: In patients with ASA/KD, RAA patients were less common than LAA, presented more frequently with dysphagia, had symptoms as an indication for intervention, and underwent treatment at a younger age. Open, endovascular and hybrid repair approaches appear equally effective, regardless of arch laterality.

Prenatal ultrasound findings, genetic testing, and literature review of Isolated left subclavian artery.

BACKGROUND: The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency. CASE PRESENTATION: We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES-Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future. CONCLUSIONS: Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling.

Recognising ligamentous atresia in double aortic arch.

Ligamentous atresia of the left side of a double arch distal to the left subclavian artery is a rare form of vascular ring, which can easily be confused, on transthoracic echocardiography, with the right-sided aortic arch when there is mirror-imaged branching. Because of its rapid acquisition, computed tomographic angiography with three-dimensional reconstruction has now become the modality of choice for accurate diagnosis of the various forms of double aortic arch. It can be performed without sedation in any age group, including neonates. It provides excellent visualisation of the aortic arch and its branching pattern, thus permitting accurate diagnosis and surgical planning. We present a case series of six children with this rare vascular ring assessed using CT, highlighting their outcomes.

A rare adult case of asymptomatic double aortic arch accompanied by the right vertebral artery directly originating from the aortic arch.

INTRODUCTION: A double aortic arch (DAA) is a rare congenital vascular anomaly. No case of DAA with a direct aortic origin of the right vertebral artery (VA) has been reported in adults. Here, we report a rare case of an asymptomatic DAA accompanied by the right VA directly originating from the right aortic arch in an adult. CASE PRESENTATION: A DAA and right VA directly originating from the right aortic arch were identified in a 63-year-old man using digital subtraction angiography and computed tomography angiography. The patient underwent digital subtraction angiography for evaluation of an unruptured cerebral aneurysm. Intraprocedural selection of vessels branching from the aorta with the catheter was difficult. To confirm the bifurcation of the aorta, aortography was performed, which revealed a DAA. Following digital subtraction angiography, computed tomography angiography was performed, which showed that the right VA originated directly from the right aortic arch. The trachea and esophagus were located in the vascular ring of the DAA; however, they were not compressed by the aorta. This was consistent with the lack of symptoms related to the DAA. CONCLUSIONS: This is the first adult case of an asymptomatic DAA with an unusual origin of the VA. A rare asymptomatic vascular anomaly, such as a DAA, can be incidentally identified using angiography.

[Dysphagia lusoria treated by surgery]. / «Dysphagia lusoria¼ traitée chirurgicalement.

Aberrant right subclavian artery, also called arteria lusoria, is the most common congenital anomaly of the aortic arch (prevalence 0,5-1.8 %). Patients with trisomy 21 have a higher prevalence (35 %). It takes its origin from the aortic arch after the three others supra-aortic trunks. It may be associated with other cardiovascular anomalies. In more than 80 % of cases, the aberrant artery is positioned behind the oesophagus. It is often asymptomatic. Sometimes it is associated with symptoms after the age of 40 years old, more often among women: dysphagia (dysphagia lusoria), dyspnoea or cough. Diagnosis is made by computed tomography or magnetic resonance imaging. When it is symptomatic, a barium contrast radiography of the oesophagus is indicated. Surgical treatment by transposition of the aberrant vessel is performed only for symptomatic patients. We report the case of a 50-year-old woman with dysphagia lusoria treated by transposition of the aberrant right subclavian artery into the right common carotid through a cervical approach.

L'artère sous-clavière droite aberrante, appelée aussi arteria lusoria, est l'anomalie congénitale de l'arc aortique la plus fréquente (prévalence 0,5-1,8 %). Les patients atteints de trisomie 21 ont une prévalence plus élevée (35 %). Elle prend son origine sur la crosse aortique après les trois autres troncs supra-aortiques. Elle peut être associée à d'autres anomalies cardiovasculaires. Dans plus de 80 % des cas, l'artère aberrante est positionnée derrière l'œsophage. Elle est souvent asymptomatique. Elle est parfois associée à des symptômes après l'âge de 40 ans, plus souvent chez les femmes : dysphagie (dysphagia lusoria), dyspnée ou toux. Le diagnostic se fait par tomodensitométrie ou résonance magnétique nucléaire. Lorsqu'elle est symptomatique, un examen radiographique avec contraste baryté de l'œsophage est indiqué. Le traitement chirurgical par transposition du vaisseau aberrant n'est réalisé que pour les patients symptomatiques. Nous rapportons le cas d'une patiente de 50 ans souffrant de dysphagia lusoria traitée par transposition de l'artère sous-clavière droite aberrante dans la carotide primitive droite par abord cervical.

Alterations of arterial morphology in patients with aberrant subclavian artery and type B dissection and its association with dissection.

OBJECTIVE: The arterial morphology in patients with aberrant subclavian artery (ASA) and its association with type B aortic dissection are important for treatment and prevention. In the present study, we examined the arterial morphology of ASA patients with type B dissection and evaluated its association with type B dissection in vivo. METHODS: Patients with aortic dissection who had undergone computed tomography angiography were screened for the presence of ASA and type B dissection from January 2011 to May 2021. The angles of ascending aorta, aortic arch, and aortic deviation and the diameters of the ascending aorta, aortic arch, ASA ostium, and middle ASA segment were measured on the computed tomography angiography scans of the ASA patients with type B dissection (group 1; n = 16), clinically matched counterparts without type B dissection (group 2; n = 32), and patients with clinically matched type B dissection without ASA (group 3, n = 32). The correlation between ASA morphology and type B dissection was analyzed using variance analysis or the Wallis H test. RESULTS: Compared with group 2, group 1 had a sharper ascending aortic angle (131.5° ± 13.7° vs 148.1° ± 7.8°; P = .001), a larger aortic deviation angle in plane 2 (28.2° ± 6.0° vs 22.1° ±7.2°; P = .005) and plane 3 (26.4° ±7.3° vs 21.8° ± 6.3°; P = .028). Similarly, group 1 had a greater diameter in the ascending aorta and aortic arch and the ostium and middle of the ASA (38.3 ± 4.1 mm vs 33.6 ± 4.5 mm [P = .001]; 34.0 ± 9.3 mm vs 26.2 ± 2.9 mm [P = .004]; 20.3 ± 9.3 mm vs 14.0 ± 3.2 mm [P = .018]; 10.8 ± 2.3 mm vs 9.0 ± 1.5 mm [P = .002], respectively), without a significant difference in the aortic arch angle. Compared with group 3, group 1 had a sharper ascending aortic angle (131.5° ± 13.7° vs 142.5° ± 11.7°; P = .026) and smaller aortic deviation angle in plane 1 (21.7° ± 6.2° vs 28.9° ± 6.2°; P = .04) and plane 3 (26.4° ± 7.3° vs 21.8° ± 6.3°; P = .007), although with no significant differences in the aortic arch angle, aortic deviation angle in plane 2, and ascending aortic diameter. CONCLUSIONS: The diameters of the ostium and middle segment of the ASA and ascending aorta and the angles of the ascending aorta and aortic deviation are potential risk factors for type B dissection in patients with ASA, which could provide new insights into the mechanism of type B dissection in patients with ASA.

Contemporary management and natural history of aberrant right subclavian artery.

OBJECTIVE: An aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the aortic arch. A paucity of reported studies is available regarding the treatment of these patients. The purpose of the present study was to evaluate the contemporary management strategies and natural history of ARSA in these patients. METHODS: A single-center retrospective review of patients with a diagnosis of ARSA from 2009 to 2019 was performed. Computed tomography scans were analyzed, and the aortic and ARSA diameters were measured at 10 different segments. The demographic data, comorbidities, and operative interventions were collected. The patients were categorized into those who had undergone intervention and those who had undergone expectant management. Linear mixed effect models were used to estimate the annual ARSA diameter changes. RESULTS: A total of 30 patients with ARSA were identified, 17 (57%) of whom were women. The average age for the cohort was 54.5 ± 14.6 years. Of the 30 patients, 20 (67%) had undergone operative repair at presentation and 10 (33%) were initially observed. The most common presenting symptom was dysphagia (30%). Of the 10 patients who had been initially treated expectantly, 4 had subsequently required intervention. Of the 24 operative interventions, 13 (54%) were hybrid procedures involving right carotid-subclavian bypass or transposition and thoracic endovascular aortic repair. The mean diameter of ARSA at its origin was 20.4 ± 5.7 mm, and the mean cross-sectional aortic diameter at the level of the ARSA was 31.8 ± 8.5 mm for the entire cohort. For the patients who had initially been observed and had subsequently required intervention, the largest change in the ARSA cross-sectional diameter was observed 1 cm distally to the vessel ostium at a rate of 3.05 mm annually (95% confidence interval, 1.54-4.56; P < .001). No statistically significant changes in the annual growth rate of the aortic segments were observed in the entire cohort or for those patients who had undergone intervention (P > .05). CONCLUSIONS: The decision to intervene on an ARSA should be individualized by the presence of symptoms (eg, dysphagia lusoria) or complications (eg, dissection, concomitant aortic aneurysmal disease, enlarging Kommerell diverticulum). Asymptomatic patients with nonaneurysmal ARSA might not require any intervention and can be safely observed. Measurement of the cross-sectional ARSA diameter 1 cm distally to the ostium of the vessel might aid in the surveillance of vessel diameter changes. Additional studies are required to determine the specific size criteria as an indication for operative repair of asymptomatic Kommerell diverticulum.

Systematic Review and Meta-Analysis of Outcomes After Operative Treatment of Aberrant Subclavian Artery Pathologies and Suggested Reporting Items.

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

Computed tomography and magnetic resonance imaging of vascular rings and other things: a pictorial review.

Compression of the airway, esophagus or both by aortic and pulmonary vessels can be caused by a variety of anatomical situations. Vascular rings are the most commonly encountered entity; however, compression can also occur from less common anomalies such as a left pulmonary artery sling or innominate artery compression. Vascular rings and other vascular compression abnormalities can be challenging to visualize and image and often require advanced imaging by CT or MRI to better understand the cause and extent of compression. Atretic vascular structures, such as the ligamentum arteriosum or atretic arch, play a key role in creating a vascular ring and do not enhance with contrast agent in a typical fashion. Despite these imaging challenges, classic and useful signs can indicate the presence or absence of a vascular ring or compression.

A rare presentation and management of thrombosis of an aberrant right subclavian artery related to thoracic outlet syndrome.

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.

Novel interrupted aortic arch: A case report.

BACKGROUND AND AIM OF THE STUDY: Interrupted aortic arch (IAA) is a rare and fatal malformation. Most patients with IAA are diagnosed in early childhood because of the severity of their symptoms. IAA is classified into three morphologic types (A, B, or C), depending on the site of the interruption. In our case, this patient did not have a common brachiocephalic trunk, left carotid artery, or left subclavian artery, IAA classification of this case cannot be judged based on the existing interruption method. METHODS: We present a 6-year-old Chinese boy with a history of neck masses since birth, and an echocardiogram from a local county hospital revealing an IAA without any cardiac anomalies, was referred to our hospital. RESULTS: The patient was feeling good and was nearly asymptomatic. Computed tomography angiography was performed, which indicated an absent aortic arch, likely due to disruption during development, and aortic discontinuity. The ascending aorta gave rise to both carotid arteries, and the descending aorta was supplied by large subclavian arteries. The right vertebral artery was supplied by right large collateral vessels that connected the right carotid artery. The left side was similar in structure to the right side. The descending aorta was supplied by large subclavian arteries. The subclavian arteries and carotid arteries were connected by large collateral vessels. Due to the large collateral vessels, the child's lower body had sufficient blood supplied, so that the typical differential cyanosis did not occur, and the child without symptomatic can survive to now. CONCLUSIONS: This patient did not have a common brachiocephalic trunk, left carotid artery, or left subclavian artery. Maybe, this patient belonged to a new type of IAA.

Surgical treatment of Kommerell's diverticulum.

Kommerell's diverticulum (KD) is a rare congenital vascular abnormality due to the formation of an abnormal aortic arch, most commonly associated with dilatation at the proximal part of the aberrant subclavian artery. This article describes an option of surgical treatment in a patient with KD. The proposed new technique of aortic arch surgical reconstruction with a stent-graft placement in the descending aorta reduces CPB time, aortic clamp and circulatory arrest time.