Search

Introduction: An aberrant right subclavian artery (ARSA) is an aortic anomaly that, in some cases, can be complicated with Kommerell's diverticulum (KD) at the origin of the ARSA. Progression and rupture of KD are associated with high mortality. Timely intervention is therefore required; however, there are no clinical guidelines for the most suitable intervention. Report: A 50 year old, previously healthy, male patient developed dysphagia. He was diagnosed with an aberrant right subclavian artery and KD. The KD increased in size from 4 – 7 cm within 2 months. He underwent single stage hybrid aortic repair involving an aortic valve replacement, total aortic arch debranchment, two thoracic endovascular aortic repair stents, and subclavian plugs. He developed a stroke during the post-operative period; however, all neurological symptoms had disappeared at 6 months and computed tomography showed no endoleaks and all supraortic vessels were open. Discussion: Literature on KD is limited; therefore, there is no consensus on KD treatment. Increasing awareness of rapidly developing KD will add to current knowledge of the disease. One stage cardiac and non-cardiac surgery was successfully performed with no long term complications.

Introduction: Symptomatic incomplete vascular rings resulting from aberrant right subclavian arteries (ARSA) are usually repaired with a subclavian to carotid transposition via a right thoracotomy or median sternotomy approach. We describe the successful use and long-term outcomes of a less invasive approach via a right collar incision for ARSA repair in a pediatric case series. Case presentations: Between January 2020 and December 2022, four pediatric patients with symptomatic ARSAs underwent operative repair using a right collar incision on the pediatric general and thoracic surgery service at a quaternary pediatric medical center. Demographic information, clinical characteristics and intra-operative complications were obtained from retrospective review of the medical record. Structured telephone interviews using a survey tool adapted from the Self Evaluation of Breathing Questionnaire and the PEDI-EAT 10 collected data on long-term symptomatic improvement.Two patients were male, and two were female. Age at time of operation ranged from 9 to 38 months. In all patients, the operative indication was dysphagia in the setting of an ARSA with no additional symptom etiology of symptoms identified. No patients required postoperative intensive care unit admission. Hospital length of stay was 2 days for all patients. There were no intra-operative complications. All patients experienced long-term symptomatic improvement following their operation with 100 % reporting symptomatic improvement and none reporting symptom recurrence. Conclusion: A right collar incision is a feasible and effective approach for subclavian to carotid transposition of ARSA in pediatric patients and should be considered for operative intervention of symptomatic aberrant right subclavian arteries.

An aberrant right subclavian artery (ARSA) is a rare variation of normal anatomy occurring in 0.5% to 1.8% of the population. No current guidelines are available regarding ARSA management, and surgical intervention should be evaluated carefully. Moreover, symptomatic patients with a dominant left arch and aberrant ARSA require a surgical approach from the right side of the chest for ligation and division of the aberrant artery at its origin on the aorta. The ARSA can then be reimplanted onto the right common carotid artery via a supraclavicular incision. The extensive mobilization in the chest allows for easy reimplantation in the supraclavicular region and eliminates reliance on the collateral circulation. Postoperative monitoring is reliable and easy with radial pulse examinations.

An aberrant right subclavian artery, the most common anatomic variant of the aortic arch, occurs in 0.5% of the population. Symptoms generally result from compression of the esophagus and/or trachea as the aberrant vessel passes posteriorly in the mediastinum. Treatment includes revascularization of the right subclavian artery from the right common carotid artery using a cervical approach combined with occlusion of the origin of the aberrant vessel from the thoracic aorta. We describe a hybrid treatment approach for a symptomatic aberrant right subclavian artery using cervical revascularization and branched thoracic stent graft coverage of the origin of the aberrant vessel.

This case report describes a case of severe dysphagia lusoria secondary to an aberrant right subclavian artery causing compression of the esophagus. Our 62-year-old female patient presented with severe dysphagia and underwent right carotid–subclavian bypass with uncovered thoracic endovascular aortic repair and coil embolization of the aberrant right subclavian artery. This case is unique in that an uncovered dissection stent graft was used to avoid occluding the anatomic left subclavian artery and, therefore, avoid a left carotid–subclavian bypass. This case highlights a unique anatomic variant, its surgical repair, and the long-term improvement in the patient's quality of life.

A case of unilateral vascular hypoplasia is presented. A female patient was born with a complex aortic arch anatomy - a double aortic arch with an interrupted left arch. Surgical correction was performed at the age of 3 months. The patient was also noted to have had an ipsilateral large infantile haemangioma. These findings raised the suspicion of the diagnosis of PHACE syndrome. PHACE syndrome is an acronym for Posterior fossa abnormalities, Haemangioma, Arterial anomalies, Cardiac anomalies and Eye anomalies. Future research is needed to elucidate the underlying pathophysiology in PHACE syndrome.

A 30-year-old woman presented following a motor vehicle collision with a grade III blunt thoracic aortic injury and an aberrant right subclavian artery. Using intraoperative ultrasound and diagnostic subtraction angiography, we deployed an aortic endograft (cTAG; W.L. Gore & Associates), excluding the injury and aberrant right subclavian artery. The patient immediately lost arterial waveforms in her left arm, confirming incidental coverage of the left subclavian artery, likely due to the polytetrafluoroethylene sheath of the endograft. Her pulses returned after placement of a left subclavian chimney via retrograde brachial artery access.

Intramural Hematoma (IMH) forms part of the acute aortic syndrome, aortic dissection, and penetrating aortic ulcer. It is a life-threatening aortic disease that warrants prompt diagnosis and management. Like aortic dissections, it is classified using the Stanford classification system as type A (proximal to the origin of the left subclavian artery) and type B (distal to the origin of the left subclavian artery). Patients with type A IMH is generally managed surgically, and uncomplicated type B IMH is managed medically. The right subclavian artery arises typically from the brachiocephalic trunk. Aberrant right subclavian arteries (ARSA) are rare and derive directly from the aortic arch distal to the left subclavian artery. In this case report, a 73-year-old female presented with right-sided chest pain and shortness of breath. On examination, her heart rate was 100 bpm and blood Pressure was 185/85 and her ECG showed sinus rhythm. Following a CT scan, she was found to have a type B Aortic IMH with an ARSA. She was medically managed with vigorous blood pressure control. After a period of intravenous blood pressure treatment, she was treated with oral medication. Her subsequent CT scan showed that the hematoma was stable. She was followed up with MRI scanning 1 year later, which showed complete healing of the aorta with no changes in diameter. This case illustrates the importance of strict blood pressure management and follow-up imaging in patients presenting with type B IMH. It is important to monitor these patients regularly and where blood pressure control alone is not sufficient, further intervention may be required. Even though the complete resolution may be achieved as in this case, these patients will need to be kept under surveillance with repeated scans to monitor for any changes.

Horseshoe adrenal gland (HA) refers to congenital fusion of the adrenal glands across the midline - a rare anatomical variant often found in association with other congenital anomalies. Here we report 2 cases of HA associated with retro-aortic course of the right diaphragmatic crus, yet another rare anatomical variant, in a 61 your-old male and a 69-year-old female, who underwent CT examinations for unrelated reasons. Both patients also had additional vascular and vertebral anomalies. To the best of our knowledge, this is the first report to document association of 2 rare congenital anomalies: HA and retro-aortic right diaphragmatic crus.

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.

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.

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.)

Background: Soft markers are nonspecific findings detected by ultrasonography during the second trimester that are often transient and nonpathologic but may imply an increased risk of underlying fetal aneuploidy. However, large-scale prospectively stratified studies focusing on the prevalence of chromosomal aberrations, including copy number variants, in fetuses with different types of isolated soft markers have rarely been published in the literature., Objective: This study aimed to investigate clinical outcomes in fetuses with isolated soft markers by single nucleotide polymorphism array with long-term follow-up and to propose a diagnostic algorithm based on specific types of soft markers., Study Design: The prevalence of fetal isolated soft markers was 13.2% (7869 of 59,503). A total of 2466 fetuses with ultrasonographic soft markers during the second trimester, which were subjected to single nucleotide polymorphism array with long-term follow-up, were selected in this prospective study over a 5-year period. Soft markers were categorized into 12 groups. The demographic profile and chromosomal microarray analysis detection results were analyzed and compared among different groups., Results: The overall prevalence of chromosomal aberrations in fetuses with soft markers was 4.3% (107 of 2466), which comprised 40.2% with numeric chromosomal abnormalities, 48.6% with pathogenic copy number variants, and 11.2% with likely pathogenic copy number variants. The incidence of numeric chromosomal abnormalities was significantly higher in multiple soft markers (5.5% vs 1.5%; P=.001) and the thickened nuchal fold group (8.3% vs 1.7%; P=.024). Meanwhile, the incidence of pathogenic copy number variants was significantly higher in multiple soft markers (5.5% vs 2.4%; P=.046) and the short femur length group (6.6% vs 2.2%; P<.0001). The incidences of pathogenic copy number variants in fetuses with isolated echogenic intracardiac focus, enlarged cisterna magna, choroid plexus cysts, echogenic bowel, or single umbilical artery were lower than 1.5%. The normal infant rate in fetuses without chromosomal aberrations was 91.7%; however, it was significantly lower in the mild ventriculomegaly (86.2% vs 93.0%; P<.0001) and short femur length groups (71.4% vs 93.6%; P<.0001)., Conclusion: The potential chromosomal aberrations and clinical prognoses varied widely among different types of isolated soft markers. Pathogenic copy number variants are more often present in specific soft markers, especially when multiple soft markers are found. Thus, a specific soft marker type-based prenatal genetic testing algorithm was proposed., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Transradial is becoming the access of choice for coronary angiography (CAG). Arteria lusoria (AL) poses a challenge for right transradial access because it can cause difficulty in accessing the ascending aorta. Of 18,686 patients who underwent CAG in Geisinger Medical Center from 2012 to 2018, 6 had a diagnosis of AL. Four underwent attempted right radial access, in 3 cases before AL was identified. All were successful, and one patient had successful right transradial percutaneous coronary intervention. CAG and PCI can be successfully performed using right radial access in patients with AL., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: There is scarce information about the prevalence of anomalies and anatomical variations of the main great thoracic vessels in dogs, particularly in dogs without congenital heart disease., Animals: The study included 878 privately owned dogs., Material and Methods: Computerized tomography (CT) thoracic studies carried out between 2011 and 2014 for a variety of reasons were reviewed. The prevalence of anomalies and anatomical variations of the aorta and vena cava, the arterial branches of the aortic arch and the main branches of the intrathoracic veins in dogs with no evidence of congenital heart disease was evaluated. Poor-quality CTs, CTs with thoracic pathology that impaired visualization or those of young dogs with clinical evidence or suspicion of congenital cardiac disease were excluded., Results: Eight hundred two CT studies were analysed. Eight dogs (1%) showed an anatomic anomaly. The most common anomaly was an aberrant retroesophageal right subclavian artery (n = 7, 0.8%). One dog showed a dilated azygos vein secondary to an interrupted vena cava. Three types of branching of the common carotid arteries were observed: both arteries arising at the same point (type I: n = 506/742; 68.2%), separated (type II: n = 212/742; 28.6%) or from a common trunk (type III: n = 24/742; 3.2%)., Conclusions: Major anatomical variations or anomalies of the main great thoracic vessels in dogs without congenital cardiac disease were rare. An aberrant retroesophageal right subclavian artery was the most common anomaly found. Three slight variations of common carotid artery branching were identified. These findings might be of relevance for surgical or catheterization procedures., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Congenital aortic arch variations are more common in patients with thoracic aortic disease for reasons unknown. Additionally, little is understood about their relation to type B aortic dissections (TBAD) specifically. We investigated the prevalence of variant aortic arch anatomy in patients with TBAD compared with controls. To understand the implications of how variant aortic arch anatomy may contribute to degenerative aortic disease, we compared flow hemodynamics of three variations of aortic arches using four-dimensional flow magnetic resonance imaging (4D flow MRI)., Methods: Arch anatomy on computed tomography imaging was reviewed and compared between patients with TBAD and age/sex-matched controls free of aortic pathology. Arch variants were defined as follows: common origin of innominate and left common carotid artery (bovine arch), aberrant right subclavian artery, and right-sided aortic arch. Demographics, TBAD characteristics, and follow-up data were abstracted. Patients with TBAD with variant and conventional aortic arches were compared. Additionally, three matched healthy controls with conventional, bovine, and aberrant right subclavian artery arches underwent 4D flow MRI evaluation to assess if there were differences in flow patterns by arch type. Indices of regional hemodynamic wall sheer stress were compared., Results: Computed tomography scans of 185 patients with TBAD (mean age, 58.1 ± 12.4 years; 72.4% males; 71.4% Caucasian) and 367 controls (mean age, 62.5 ± 13.4 years; 67% males; 77.9% Caucasian) were reviewed. Variant arch anatomy was more prevalent in patients with TBAD (40.5% vs 24.5%; P < .001). In patients with TBAD, there were no differences in the mean age of presentation and descending thoracic aorta diameter among those with variant or conventional arch anatomy. Patients with TBAD with variant arch anatomy had a higher percentage of dissection related thoracic aortic repairs (54.7% vs 33.6%; P = .004) with repairs occurring predominantly in the acute phase. 4D flow MRI demonstrated a higher systolic wall shear stress along the inner curve of the bovine arch compared with the conventional aberrant right subclavian artery arches., Conclusions: Variant aortic arch anatomy is significantly more prevalent in patients with TBAD. patients with TBAD with variant arch anatomy had a higher percentage of dissection-related aortic repair. Preliminary 4D flow MRI data show differences in hemodynamic flow patterns between variant and conventional arches. Studies of long-term outcomes based on arch anatomy may offer additional insight to TBAD genesis and possibly influence management decisions., (Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To characterize the morphology of type B aortic dissection with aberrant right subclavian artery (ARSA) and present early and midterm outcomes of total endovascular treatment for affected patients., Methods: From January 2010 to December 2015, patients with ARSA and type B aortic dissection treated with total endovascular techniques were enrolled. The angle of the aortic arch was measured on pre-operative CTA. Sixty age and gender matched normal aortic arch patients with type B aortic dissection served as controls. Primary outcomes were technical success, 30 day mortality, and late survival. Secondary outcomes included in hospital morbidity, re-intervention rate, and patency of the subclavian artery., Results: A total of 13 patients (8 men, 5 women; mean age 58 years) were included. The mean angle of the aortic arch in patients with ARSA was significantly smaller than in normal aortic arch patients (117.2° ± 10.8° vs. 124.2° ± 9.4°, respectively; p = .024). Simple thoracic endovascular aortic repair (TEVAR) and TEVAR plus a parallel graft technique were performed in six and seven patients, respectively. Primary technique success was achieved in 11 of the 13 (84.6%) patients. A bird beak configuration occurred significantly more frequently in patients with ARSA than in normal aortic arch patients (91.7% vs. 48.3%, respectively; p = .035). The median follow-up time was 36 months. One patient received a secondary procedure because of a new onset entry tear at the distal end of the stent graft. No posterior circulation stroke, permanent spinal cord ischaemia, or ischaemia of the upper arm was observed., Conclusions: Type B aortic dissection with ARSA was associated with a steep aortic arch. Total endovascular treatment for these patients was feasible and safe. Stent grafts with better flexibility and appropriate extension of the proximal landing zone with a parallel graft technique are suggested based on the observed outcomes., (Copyright © 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.)