Search

Kommerell's diverticulum is a rare congenital anomaly of the aortic arch system in which there is a left- or right-sided aortic arch with an aberrant subclavian artery on the contralateral side. Patients with this anomaly can be asymptomatic or have features of tracheal or oesophageal compression. However, there is a rising suspicion that it may be a rare cause of unilateral vocal cord palsy through its compression of the recurrent laryngeal nerve. We describe a patient who had a long history of hoarse voice and left vocal cord palsy with no other obvious cause, who was found to have a Kommerell's diverticulum on a contrast-enhanced computed tomography scan.

Background: Few studies describe outcomes after complete vascular ring surgery in a comprehensive manner., Objectives: This study sought to describe the clinical presentation, diagnostic work-up, operative approach, and outcomes in children undergoing surgery for complete vascular rings., Methods: This single-center retrospective cohort study includes consecutive patients (January 1990 through September 2023) undergoing primary surgery for complete vascular rings, or rerepair after primary surgery elsewhere. The primary outcome of interest was complete (as distinct from partial) symptom resolution at latest clinic follow-up. Our current preference is to pursue a comprehensive initial operation including adjunctive vascular and airway procedures targeting common causes of residual aerodigestive symptoms, such as Kommerell diverticulum resection and tracheobronchopexy. Preoperative work-up routinely involved computed tomographic angiography, dynamic bronchoscopy, and laryngoscopy., Results: Of 515 patients (including 39 rerepairs), the most common diagnoses were right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum (n = 323, 62.7%) and double aortic arch (n = 174, 33.8%). There was no perioperative mortality. Chylothorax occurred in 28 patients (5.4%), vocal cord dysfunction in 22 patients (4.3%), and diaphragm paralysis in 2 patients (0.4%). Follow-up was available on 453 patients (88.0%) with a median duration of 3.0 years (Q1-Q3: 0.6-9.2 years). At latest clinic follow-up, 429 patients (94.7%) reported complete symptom resolution. The risk of reoperation for residual or recurrent aerodigestive symptoms was 9.6% (95% CI: 5.7%-13.5%) at 10 years and 12.4% at 20 years (95% CI: 6.9%-17.8%)., Conclusions: Surgery for complete vascular rings provides good symptomatic relief with low risk of complications, whereas reoperations for aerodigestive symptoms are infrequent., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Objective: To analyze the value of prenatal diagnostic genetic testing in cases with isolated aberrant right subclavian artery (ARSA)., Methods: This is a retrospective cohort study, conducted between January 2015-January 2022 in a fetal medicine center. Women who had an ultrasound scan and diagnosed with fetal ARSA were included. Ultrasonographic characteristics, genetic, obstetric, and neonatal outcomes were collected and analyzed., Results: A total of 240 fetuses with ARSA were identified and included to the analysis. Eighty-two of the group had isolated ARSA (34.2%, 82/240), 57 had additional soft markers (23.8%, 57/240) and 101 had additional major ultrasonographic abnormalities (42.1%, 101/240). Genetic results were available in 196 cases (81.7%, 196/240). Seventy-four of isolated ARSA cases underwent genetic testing (90.2%, 74/82). A chromosomal abnormality was present in 60 cases; 54 (22.5%, 54/240) aneuploidies and 6 (2.5%, 6/240) copy number variants. Five (6.1%) of the isolated ARSA cases had chromosomal abnormalities. All of these five cases had prenatal genetic testing due to high-risk aneuploidy screening fetuses who had ARSA with at least one additional anomaly had the highest chromosomal abnormality rate (38.6%, 39/101). Seventy-seven of isolated ARSA cases were liveborn (93.9%, 77/82)., Conclusion: Our results supports the evidence from the literature that isolated ARSA confers a very low-risk for aneuploidy, if the aneuploidy screening tests are low-risk. Also, chromosomal microarray analysis did not yield any extra information in isolated ARSA., (© 2024 Wiley Periodicals LLC.)

Competing Interests: None.

Purpose: This study aimed to develop and validate a convolutional neural network (CNN) that automatically detects an aberrant right subclavian artery (ARSA) on preoperative computed tomography (CT) for thyroid cancer evaluation., Materials and Methods: A total of 556 CT with ARSA and 312 CT with normal aortic arch from one institution were used as the training set for model development. A deep learning model for the classification of patch images for ARSA was developed using two-dimension CNN from EfficientNet. The diagnostic performance of our model was evaluated using external test sets (112 and 126 CT) from two institutions. The performance of the model was compared with that of radiologists for detecting ARSA using an independent dataset of 1683 consecutive neck CT., Results: The performance of the model was achieved using two external datasets with an area under the curve of 0.97 and 0.99, and accuracy of 97% and 99%, respectively. In the temporal validation set, which included a total of 20 patients with ARSA and 1663 patients without ARSA, radiologists overlooked 13 ARSA cases. In contrast, the CNN model successfully detected all the 20 patients with ARSA., Conclusion: We developed a CNN-based deep learning model that detects ARSA using CT. Our model showed high performance in the multicenter validation., Competing Interests: The authors have no potential conflicts of interest to disclose., (© Copyright: Yonsei University College of Medicine 2024.)

We report in-vivo imaging of a constellation of arterial variants found incidentally on CT-angiography in a 23-year-old woman presenting with an ischaemic stroke. This extremely rare combination includes a common origin of both common carotid arteries, an abnormal origin of the right vertebral artery (VA) from the right common carotid artery and of the left VA from the aortic arch, associated with an aberrant right subclavian artery. This constellation, previously described in a female cadaver, has not been reported in-vivo. Awareness of this configuration is crucial for radiological diagnosis and when performing angiography and endovascular or surgical procedures in thorax, head and neck, to avoid complications., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Cardiovascular complications represent a significant health concern for individuals with diabetes mellitus. The relationship between diabetes and cardiovascular diseases is complex and multifaceted, involving a variety of pathophysiological mechanisms. This study aimed to investigate the potential role of the cardiothoracic ratio as a prognostic tool for cardiovascular disorders in patients with diabetes. A retrospective case-control study of 530 adult patients referred to a tertiary care hospital in Saudi Arabia was conducted. Medical records, including chest X-rays, were analyzed to determine the cardiothoracic ratio. Patients diagnosed with diabetes who experienced cardiac disorders had significantly higher cardiothoracic ratios compared to patients with diabetes alone and controls. HbA1c was significantly elevated among patients with diabetes and cardiovascular disorders (mean = 71.5 ± 25.43 mmol/mol) compared to the other patients. There was a significant positive correlation between the duration of diabetes and the cardiothoracic ratio (r = 0.64, P < 0.001). Furthermore, the cardiothoracic ratio above 0.51 was a good discriminator of cardiovascular disorders in patients with diabetes, with an area under the curve of 0.737, sensitivity of 97.1%, and specificity of 87.2%. This study provided comprehensive evidence supporting the association between cardiothoracic ratio and subsequent cardiovascular adverse outcomes in patients with diabetes. We recommend adopting the cardiothoracic ratio as a valuable prognostic tool for risk stratification among people with diabetes., Competing Interests: The authors declare no conflict of interest., (© 2024 The Author(s).)

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: Funding Support and Author Disclosures The work was supported by Sichuan Science and Technology Program (Grant Number 2022ZDZX0030). The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Arterial tortuosity syndrome is an extremely rare hereditary connective tissue disorder. We present a case of an incidentally diagnosed aneurysm of the aortic root and the ascending aorta caused by arterial tortuosity syndrome, which was confirmed genetically. The aneurysm was repaired surgically. One year after the procedure, there was no further dilation of the aorta or formation of new aneurysms.

Objective: To assess the effectiveness and safety of neurological interventions using the right transradial approach (R-TRA) in patients with aberrant right subclavian artery (ARSA)., Methods: We retrospectively analyzed cases that underwent cerebral angiography and interventions at Huangpi District People's Hospital from January 2023 to July 2023. Out of 335 cases, 5 patients with ARSA were identified., Results: All 5 cases underwent diagnostic cerebral angiography via R-TRA. Two of the patients received interventions via R-TRA: 1 underwent right internal carotid artery balloon dilation angioplasty, while another underwent left vertebral artery stenting. No surgery-related complications were observed during these procedures., Conclusions: R-TRA proves to be a safe and effective option for neuro-interventional surgery in patients with ARSA., (Copyright © 2024 Elsevier Inc. All rights reserved.)

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Right sided aortic arches with concomitant aberrant left subclavian arteries are exceedingly rare anatomical variants. We present a case of a 45 year old male that presented with symptoms consistent with dysphagia and known right sided aortic arch with an aberrant left subclavian artery. Though previous reports of repair have indeed been reported, we confirm that a hybrid approach to these anatomic variants remain feasible and with symptom resolution for patients., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: An aberrant right subclavian artery (ARSA) is one of the most common anatomic variants of the aortic arch. The combination of an ARSA and a transection is naturally rare., Methods: This case report describes the treatment of a transection in the presence of an ARSA and the follow-up of two years., Results: We successfully treated the contained rupture with a stentgraft. Both subclavian arteries had to be covered in the emergency setting. At the two-year follow-up, the patient did not suffer from any neurological impairment., Conclusions: In emergency settings, primary cover of both subclavian arteries with a stentgraft can be performed in individual cases after risk assessment. Severe complications such as development of upper limb ischaemia, ASAS or reduced perfusion of the posterior cerebral circulation should be considered in treatment planning., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Symptoms, imaging characteristics, and early and midterm surgical outcomes for aberrant subclavian arteries (ASCA) are not well defined in the adult population., Methods: A single-institution retrospective review was conducted of adults undergoing surgical repair of ASCA and descending aorta origin/Kommerell diverticulum (KD) from January 1, 2002, to December 31, 2021. Symptom improvement and differences in imaging characteristics between anatomic groups and the number of symptoms were assessed., Results: Mean age was 46 ± 17 years. There were 23 of 37 left aortic arches with right ASCA (62%) and 14 of 37 right aortic arches with left ASCA (38%). Of these, 31 of 37 (84%) were symptomatic, and 19 of 37 (51%) had KD size/growth meeting criteria for surgical repair. KD aortic origin diameter was larger in more symptomatic patients: 20.60 mm (interquartile range [IQR], 16.42-30.68 mm) in patients with ≥3 symptoms vs 22.05 mm (IQR, 17.52-24.21 mm) for 2 symptoms vs 13.72 mm (IQR, 12.70-15.95 mm) for 1 symptom (P = .018). Aortic replacement was required in 22 of 37 (59%). There were no early deaths. Complications occurred in 11 of 37 (30%): vocal cord dysfunction (4 of 37 [11%]), chylothorax (3 of 37 [8%]), Horner syndrome (2 of 37 [5%]), spinal deficit (2 of 37 [5%]), stroke (1 of 37 [3%]), and temporary dialysis requirement (1 of 37 [3%]). Over a median follow-up of 2.3 years (IQR, 0.8-3.9 years), there was 1 endovascular reintervention and no reoperations. Dysphagia and shortness of breath resolved in 92% and 89%, respectively, whereas gastroesophageal reflux persisted in 47%., Conclusions: The KD aortic origin diameter correlates with the number of symptoms, and surgical repair of ASCA and descending aorta origin/KD effectively relieves symptoms, with low rates of reintervention. Given the operative complexity, surgical repair should be performed in patients meeting size criteria or with significant dysphagia or shortness of breath symptoms., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: Aberrant right subclavian artery (ARSA) is the most common of the aortic arch anomalies, occurring in .5% to 1% of the population. There is no standardized follow up protocol, especially in the asymptomatic cases. The purpose of the present study was to evaluate the natural history of ARSA and the role of serial CT scans. Methods: This is a single-center retrospective study of patients with ARSA depicted on chest computed tomography (CT) scans between February 2013 and July 2022. Data were collected from their medical records. Measurements of the aorta at different segments including the aortic diameter at the orifice of ARSA, and ARSA at ostium followed by 1 cm intervals were collected, as well as for follow-up CT scans. Results: 65 patients were diagnosed with ARSA, 70.8% of whom were women. The average age for the cohort was 58.569 ± 16.99 years. The median follow up time was 4 years (range 0-10 years), KM estimated survival after ARSA diagnosis at 1 and 5 years as 97% and 93%, respectively. Nineteen patients had a second CT scan and were included in the morphological CT dynamic analysis, on average of 29 ± 27 months apart (range 7-108). The mean ARSA diameter at origin was larger in the second scan 16.91 ± 4.31 mm compared to the initial scan 16.31 ± 4.96 mm, ( P = .04).The mean aortic arch diameter in the first and second CT were 28.54 ± 4.24 and 29.64 ± 5.14 ( P = .10), respectively. All other measurements did not disclose any significant enlargement over time. Conclusions: Our cohort demonstrate a benign natural history of ARSA with slow growth rates. However, due to our small sample size we can't draw a clinically sound recommendation on the need for imaging follow up, and further larger cohort with longer follow up interval are required., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Kommerell's diverticulum (KD) with a right aortic arch (RAA) and aberrant left subclavian artery (aLSCA) is a rare congenital anomaly of the aortic arch. Treatment is not well defined due to its uncommon presentation, with rupture and dissection risk rates of up to 53%., Case Summary: A 54-year-old male with a history of chronic obstructive pulmonary disease (COPD) and hypertension presented with difficulty breathing during exercise without dysphagia. Follow-up computerized tomography angiogram (CTA) revealed the presence of a RAA and aLSCA arising from the descending thoracic aorta with an adjacent 58 × 41-mm KD and tracheal and esophageal displacement. Due to the size of the KD, risk of rupture, unsuitable anatomy for total endovascular aortic repair (EVAR), and high COPD burden, the patient was planned to undergo a hybrid surgical repair. Left common carotid (LCCA) artery to LSCA bypass, full aortic debranching, LSCA embolization and percutaneous thoracic endovascular aortic repair (TEVAR) were performed. Successful device position and exclusion of the diverticulum and aneurysmal aorta were observed after completion thoracic aortogram. 18-month follow-up CTA demonstrated patency of the LSCA to LCCA bypass graft and arch vessel branches, as well as stable exclusion of the KD. Persistence of a type II endoleak originated at the right first posterior intercostal artery has been noted and is being followed conservatively since no sac growth has occurred., Conclusion: We highlight the presence of a KD with RAA and aberrant subclavian artery, a rare congenital anatomic variation of the aortic arch with complex anatomy. Surgical planning must be individualized according to comorbidities and anatomical variations identified on imaging and 3D reconstructions.

The aberrant right subclavian artery (i.e., arteria lusoria) arising from the left part of the aortic arch is a rare congenital anomaly. In some patients, esophageal compression may cause symptoms of dysphagia, also called dysphagia lusoria. It can cause serious feeding disorders and poor weight gain in young children. We present the case of an early onset of dysphagia lusoria in a 1-month-old girl whose clinical diagnosis was confirmed by esophagography and magnetic resonance imaging. This kind of vascular anomaly can present a diagnostic challenge and should be considered in diagnosing dysphagia in childhood., (Published by Elsevier Inc.)

Transradial access for diagnostic and therapeutic neurointerventional procedures has gained popularity due to a decreased incidence of access site complications and improved patient comfort compared with transfemoral access.1-4 An aberrant right subclavian artery is an aortic arch variant characterized by a right subclavian artery that arises directly from the arch as the most distal great vessel. Transradial access with an aberrant right subclavian artery is anatomically challenging due to the predilection of the catheter system to collapse into the descending aorta. In this (video 1), we describe a step-by-step technique for transradial access in a patient with an aberrant right subclavian artery undergoing endovascular flow diversion for a left superior hypophyseal artery aneurysm. Particular emphasis is placed on the technique for accessing the proximal arch and aortic valve as well as distal catheter navigation while avoiding prolapse into the descending aorta. neurintsurg;15/11/1164/V1F1V1Video 1 ., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Aberrant right subclavian artery (ARSA) is a rare condition, but the most common anomaly of the aortic arch. Although neurointerventions via transradial access (TRA) are becoming increasingly popular worldwide, transradial carotid cannulation has been extremely challenging in patients with an ARSA. Herein, we present a case of ARSA-associated anterior communicating artery (ACoA) aneurysm that was successfully treated with a radial-specific 6F Simmons guiding sheath via left TRA. We also review the relevant literature., Methods: A 68-year-old-woman who was diagnosed as having an ARSA-associated ACoA aneurysm underwent simple coiling via left TRA. After the 6F Simmons guiding sheath was engaged into the right common carotid artery using the pull-back-technique, transradial quadraxial system (6F Simmons guiding sheath/6F intermediate catheter/3.2F intermediate catheter/coil-delivery microcatheter) was implemented., Results: Simple coiling of the aneurysm was successfully achieved without catheter kinking or system instability. The postprocedural course was uneventful. A follow-up magnetic resonance angiography showed no evidence of recanalization 1 years 9 months after the procedure., Conclusions: Transradial anterior circulation intervention has been rarely used for patients with an ARSA due to unfavorable catheter trajectory. Left TRA using the 6F Simmons guiding sheath is a useful treatment option to address anterior circulation interventions for patients with an ARSA. Preoperative diagnosis of ARSA is necessary for the application of our method., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: This meta-analysis aimed to investigate the diagnostic value of color ultrasound screening for fetal cardiovascular abnormalities during the second trimester in China., Methods: A literature search was conducted to evaluate the clinical value of color ultrasound screening for fetal cardiovascular abnormalities during the second trimester using English and Chinese databases. Numerical values for sensitivity and specificity were obtained from false-negative, false-positive, true-negative, and true-positive rates, presented alongside graphical representations with boxes marking the values and horizontal lines showing the confidence intervals. Summary receiver operating characteristic (SROC) curves were applied to assess the performance of the diagnostic tests. Data were processed using Review Manager 5.3., Results: Four studies (151 patients with fetal cardiovascular abnormalities and 3397 undiagnosed controls) met our evaluation criteria. The sensitivity was 0.91 and 0.96, respectively, and the specificity was 1.00. The Area Under the Curve (AUC) from the SROC curves was >90%; therefore, it was classified as excellent. Furthermore, there were 6 types of fetal cardiovascular abnormalities, and the pooled screening rate of atrioventricular septal defects was the highest., Conclusion: Our meta-analysis showed that the use of color ultrasound during the second trimester can be an excellent diagnostic tool for fetal cardiovascular abnormalities., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

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., (© 2023 Wiley Periodicals LLC.)

A 45-year-old man complaining of chest and back pain due to acute aortic dissection was referred to our department. A contrast enhanced computed tomography( CT) scan showed Stanford type B aortic dissection with Kommerell diverticulum and aberrant right subclavian artery. The patient underwent antihypertensive treatment for one month. Despite the successful treatment, CT scan revealed a 5 mm false lumen dilatation in this period. We decided to close the primary entry. The operation was performed through median sternotomy;after establishing cardio-pulmonary bypass, the ostium of the aberrant right subclavian artery( ARSA) was sutured closed and anastomosed the ARSA and right common carotid artery. Total arch replacement was performed using frozen elephant trunk technique. His postoperative course was uneventful.

Objective: Aberrant subclavian arteries (aSCAs), with or without aortic pathology, are uncommon. The purpose of the present study was to review our experience with the surgical management of aSCA., Methods: We performed a retrospective review of patients who had undergone surgery for an aSCA between 1996 and 2020. Symptomatic and asymptomatic patients were included. The primary end points were ≤30-day and late mortality. The secondary end points were ≤30-day complications, graft patency, and reinterventions., Results: A total of 46 symptomatic and 3 asymptomatic patients with aSCA had undergone surgery (31 females [62%]; median age, 45 years). An aberrant right subclavian artery was present in 38 (78%) and an aberrant left subclavian artery in 11 patients (22%). Of the 49 patients, 41 (84%) had had a Kommerell diverticulum (KD) and 11 (22%) had had a concomitant distal arch or proximal descending thoracic aortic aneurysm. Symptoms included dysphagia (56%), dyspnea (27%), odynophagia (20%), and upper extremity exertional fatigue (16%). Five patients (10%) had required emergency surgery. The aSCA had been treated by transposition in 32, a carotid to subclavian bypass in 11, and an ascending aorta to subclavian bypass in 6. The KD was treated by resection and oversewing in 19 patients (39%). Fifteen patients (31%) had required distal arch or proximal descending thoracic aortic replacement for concomitant aortic disease and/or KD treatment. Thoracic endovascular aortic repair was used to exclude the KD in six patients (12%). Seven patients (14%) had undergone only bypass or transposition. The 30-day complications included one death from pulseless electrical activity arrest secondary to massive pulmonary embolism. The 30-day major complications (14%) included acute respiratory failure in three, early mortality in one, stroke in one, non-ST-elevation myocardial infarction in one, and temporary dialysis in one patient. The other complications included chylothorax/lymphocele (n = 5; 10%), acute kidney injury (n = 2; 4%), pneumonia (n = 2; 4%), wound infection (n = 2; 4%), atrial fibrillation (n = 2; 4%), Horner syndrome (n = 2; 4%), lower extremity acute limb ischemia (n = 1; 2%), and left recurrent laryngeal nerve injury (n = 1; 2%). At a median follow-up of 53 months (range, 1-230 months), 40 patients (82%) had had complete symptom relief and 9 (18%) had experienced improvement. Six patients had died at a median of 157 months; the deaths were not procedure or aortic related. The primary patency was 98%. Reintervention at ≤30 days had been required for two patients (4%) for ligation of lymphatic vessels and bilateral lower extremity fasciotomy after proximal descending thoracic aorta replacement. One patient had required late explantation of an infected and occluded carotid to subclavian bypass graft, which was treated by cryopreserved allograft replacement., Conclusions: Surgical treatment of the aSCA can be accomplished with low major morbidity and mortality with excellent primary patency and symptom relief., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

While unusual, aberrant right subclavian artery (ARSCA) can occasionally be a source of significant dysphagia in children. We present a case of a 13-year-old female who reported a three-year history of dysphagia to solid foods and was found to have ARSCA on a barium swallow study and computed tomography scan of the chest. We reimplanted the ARSCA into the right carotid artery in end-to-side fashion using a partial median sternotomy approach. At six months follow-up, her symptoms had completely resolved, and her postoperative echocardiogram showed an unobstructed reimplanted ARSCA. Meeting presentation: AATS 102nd Annual Meeting; May 14, 2022; Boston, MA.

To evaluate the correlation between chromosomal abnormalities and fetal aberrant right subclavian artery (ARSA) with or without additional ultrasound anomalies (UAs). A total of 340 fetuses diagnosed with ARSA by ultrasound between December, 2015, and July, 2021, were included. All cases were subdivided into three groups: (A) 121 (35.6%) cases with isolated ARSA, (B) 91 (26.8%) cases with soft markers, and (C) 128 (37.6%) cases complicated with other UAs. Invasive testing was performed via amniotic fluid or cord blood karyotyping and chromosomal microarray analysis (CMA) in parallel, and pregnancy outcomes were followed. Karyotype abnormalities were identified in 18/340 (5.3%) patients. Karyotype abnormalities in Groups A, B, and C were 0/121 (0.0%), 7/91 (7.7%), and 11/128 (8.6%), respectively. CMA abnormalities with clinically significant variants were detected in 37/340 (10.9%) cases, of which 22q11.2 deletion syndrome and trisomy 21 accounted for 48.6% (18/37). The overall abnormal CMA with clinically significant variant detection rates in Groups A, B, and C were 3/121(2.5%), 13/91 (14.3%), and 21/128 (16.4%), respectively. There were significant difference in clinically significant CMA anomalies detection rate between Groups A and C (p < 0.05), as well as Groups A and B (p < 0.05). Comparing CMA to karyotyping showed a clinically significant incremental yield in Group C (7.8%, 10/128) compared to Groups A (2.5%, 3/121) and B (6.6%, 6/91) (p > 0.05). Fetal ARSA with additional UAs, concurred with cardiac and extra-cardiac anomalies, constitutes a high-risk factor for chromosomal aberrations, especially for pathogenic or likely pathogenic copy number variants., (© 2023. The Author(s).)

The primary aim of this review is to estimate the prevalence of ARSA both in euploid fetuses as well as in fetuses with Down Syndrome. Secondary objectives were to estimate the association of ARSA with cardiac anomalies and chromosomal defects, especially trisomy 21 and 22q11 deletion (DiGeorge Syndrome). The incidence of ARSA in normal population varies from 0.35% to 3.5%, based on different studies. Since the first reported association between ARSA and trisomy 21 in 2015 until today, several studies have emerged to confirm different degrees of this correlation. Indeed, ARSA appears to be a clinically useful prenatal ultrasound marker for trisomy 21. Particularly, most recent studies concluded that ARSA as a non-isolated finding can be used as screening for Down syndrome. However, when ARSA is an isolated finding, various studies proved that there is no significant correlation with Down syndrome. Apart from these, ARSA appears to be associated with other chromosomal abnormalities, such as 22q11 deletion, cardiac defects and other morphological anomalies. As a conclusion ARSA should be characterized as isolated or non-isolated, as the non - isolated ARSA appears to be a clinically useful marker of Down syndrome and thus, additional testing is required when diagnosed.

No guidelines for the treatment of Kommerell diverticulum (KD) have been established. Endovascular treatment with arch vessel reconstruction of 4 patients with a right-sided aortic arch and aberrant left subclavian artery are presented herein. A 39-year-old woman and 47-year-old man experienced dyspnea and retrosternal pain. The first patient concomitantly underwent left subclavian transposition and thoracic endovascular aortic repair (TEVAR). The second patient underwent bilateral carotid-subclavian bypass grafting, followed by TEVAR and KD embolization. The 2 other patients were 72- and 75-year old men who presented with KD and type B dissection, in which these conditions were characterized by acute thoracic pain in the former patient and were noted on incidental body scan for prostatic adenocarcinoma in the latter. The third patient underwent carotid-subclavian bypass grafting, followed by TEVAR and subclavian plugs, and in the fourth patient, regular follow-up with antihypertensive therapy was scheduled. After a mean follow-up period of 12 months, all patients were alive., (Copyright © 2022 SIR. Published by Elsevier Inc. All rights reserved.)

Anomalous aortic origin of the right coronary artery is a rare disease. Although there are various reports on its treatment, the method of the surgical approach is still controversial. Here, we present a rare case of a 17 year-old man who had an anomalous aortic origin of the right coronary artery with an aberrant right subclavian artery. As a treatment, he underwent reimplantation of the right coronary artery. The surgical approach for the anomalous aortic origin of the right coronary artery should be selected by considering the age of the patient and size of the right coronary artery., (© 2022. The Author(s).)

Background: Kommerell diverticulum (KD) is a dilated proximal aberrant right or left subclavian artery associated with either right or left aortic arches (RAA-ARSA or LAA-ALSA). Although case series suggest that KD may be a liability for vascular complications, the risk, pattern of dilation throughout the life span, and differences between arch sides are not known., Methods: This study was a single-center retrospective review of patients of all ages with KD on cross-sectional imaging. Maximal short-axis diameter of KD (KDmax), absolute and indexed to descending aortic diameter (DAo), was correlated with age. Comparisons were made between arch sides. Patients with vascular complications were described., Results: A total of 104 patients with KD were included: 68 (65%) with RAA-ALSA, 36 (35%) with LAA-ARSA, 43 (41%) asymptomatic. Although KDmax was correlated with age (RAA-ALSA r = 0.84; [P< .0001]; LAA-ARSA r = 0.51 [P = .001]), KDmax indexed to DAo was not (RAA-ALSA r = 0.14 [P = .27]; LAA-ARSA r = -0.22 [P = .21]). Patients with RAA-ALSA had larger KDmax indexed to DAo (1.02 ± 0.20 mm/mm vs 0.89 ± 0.18 mm/mm; P = .002) and more symptoms (75% vs 28%; P < .0001), and they were younger (median, 9.5 years vs 61.7 years; P < .0001). Six patients (58 to 80 years of age) had vascular complications, and all 6 had LAA-ARSA and risk factors for acquired aneurysms., Conclusions: In older patients, KDmax indexed to DAo was not larger, thus arguing against isolated KD dilation with age. Diverticula from RAA-ALSA and LAA-ARSA demonstrated different phenotypes, a finding suggesting different disease processes and likely different risk. The incidence of vascular complications was lower than in previous reports, and these complications occurred exclusively in patients with LAA-ARSA and aneurysm risk factors. This finding suggests that conservative management of asymptomatic KD is often reasonable, especially in patients with RAA-ALSA., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Fetal magnetic resonance imaging (MRI) has become a valuable adjunct to ultrasound in the prenatal diagnosis of congenital pathologies of the central nervous system, thorax, and abdomen. Fetal cardiovascular magnetic resonance (CMR) was limited, mainly by the lack of cardiac gating, and has only recently evolved due to technical developments., Method: A literature search was performed on PubMed, focusing on technical advancements to perform fetal CMR. In total, 20 publications on cardiac gating techniques in the human fetus were analyzed., Results: Fetal MRI is a safe imaging method with no developmental impairments found to be associated with in utero exposure to MRI. Fetal CMR is challenging due to general drawbacks (e. g., fetal motion) and specific limitations such as the difficulty to generate a cardiac gating signal to achieve high spatiotemporal resolution. Promising technical advancements include new methods for fetal cardiac gating, based on novel post-processing approaches and an external hardware device, as well as motion compensation and acceleration techniques., Conclusion: Newly developed direct and indirect gating approaches were successfully applied to achieve high-quality morphologic and functional imaging as well as quantitative assessment of fetal hemodynamics in research settings. In cases when prenatal echocardiography is limited, e. g., by an unfavorable fetal position in utero, or when its results are inconclusive, fetal CMR could potentially serve as a valuable adjunct in the prenatal assessment of congenital cardiovascular malformations. However, sufficient data on the diagnostic performance and clinical benefit of new fetal CMR techniques is still lacking., Key Points: · New fetal cardiac gating methods allow high-quality fetal CMR.. · Motion compensation and acceleration techniques allow for improvement of image quality.. · Fetal CMR could potentially serve as an adjunct to fetal echocardiography in the future.., Citation Format: · Knapp J, Tavares de Sousa M, Schönnagel BP. Fetal Cardiovascular MRI - A Systemic Review of the Literature: Challenges, New Technical Developments, and Perspectives. Fortschr Röntgenstr 2022; 194: 841 - 851., Competing Interests: PD Dr. Schönnagel and PD Dr. Tavares de Sousa are co-founders and stake-holders of northh medical GmbH, the developing company of the DUS device used for fetal cardic gating mentioned in this review., (Thieme. All rights reserved.)

The best treatment for a right-sided aortic arch (RAA) and Kommerell diverticulum (KD) has not been determined due to the rarity of these conditions. The current trend in the treatment of this disease is to increase the endovascular approach without a sternotomy. We describe a rare condition with an association of an RAA with a KD of an aberrant left subclavian artery and an anomalous right vertebral artery originating from the aortic arch (AVA). The left vertebral artery was missing. Also, there was an incomplete circle of Willis due to the absence of the left and right posterior communication arteries. Therefore, the AVA was the only artery to supply the vertebral-basilar system. In our case, a simple thoracic endovascular aortic repair was not suitable because of the sharply curved arch and short landing zone. Also, a debranching thoracic endovascular aortic repair was not appropriate because that approach would not permit reconstruction of the AVA. The patient successfully underwent a total arch replacement with the frozen elephant trunk technique. This procedure could be an effective option for patients with RAAs with KDs associated with another arch vessel anomaly., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Kommerell diverticulum (KD) is a congenital vascular disease associated with dilatation at an aberrant subclavian artery's origin. The surgical repair should be considered for the symptomatic patients due to the adjacent organ's compression by the aneurysmal change of KD. An appropriate approach should be selected for the open repair to suit the anatomical type of disease. We reported a 50-year-old female diagnosed with KD undergoing the single-stage open repair through the right thoracotomy with the straight skin incision with rib cross., (© 2022. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

Purpose: We present a case of an aberrant right subclavian artery (ARSA) with extremely rare vascular anomalies., Case Report: A 69-year-old woman was suspected to have right internal carotid artery (ICA) stenosis. Computed tomography angiography demonstrated an ARSA and hypoplasia of the right ICA. The proximal segment of the right vertebral artery (VA) was aplasia, and a right type 1 proatlantal artery (PA) arose from the right common carotid artery. Cerebral angiography demonstrated segmental dysplasia of the right ICA. The ascending intrapetrous segment and the ascending foramen lacerum-horizontal intracavernous segment of the right ICA demonstrated hypoplasia. The collateral pathways promoted reconstitution of each of the distal segments. Left internal carotid angiography demonstrated anterior communicating artery aneurysm and sufficient cross flow to the contralateral middle cerebral artery via the AcomA., Discussion: A type 1 PA with an ARSA may result in the regression of the right dorsal aorta with persistence of the first cervical intersegmental artery. Although there are few findings of a relationship between an ARSA and intracranial artery anomalies, a developmental error of the right dorsal aorta may cause such complex vascular anomalies., Conclusion: Knowledge of anatomical variations in patients with ARSA is useful when performing angiography or endovascular therapy, as well as during clinical follow-up., (© 2022. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

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., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)