A 78-year-old man was diagnosed with a liver tumor on follow-up CT after thoracic aortic aneurysm surgery. Esophagogastroduodenoscopy revealed a type 2 tumor in the gastric antrum, a biopsy showed poorly differentiated adenocarcinoma, and CT revealed multiple liver metastases, resulting in a diagnosis of clinical Stage ⅣB(cT4aN0M1[HEP]). S-1/oxaliplatin (SOX)chemotherapy was started. However, after 9 courses of chemotherapy, the primary tumor continued to increase in size. Ramucirumab/paclitaxel(RAM/PTX)was started; after 3 courses, CT revealed shrinkage of the primary tumor and disappearance of multiple liver metastases. PET-CT showed no abnormal FDG accumulation in the stomach, surrounding lymph nodes, and liver. Therefore, the patient was considered to have a PR in efficacy, and a decision to perform conversion surgery was made based on the assumption that curative resection was possible. The patient underwent laparoscopic distal gastrectomy D2 lymph node dissection and Billroth Ⅰ reconstruction. The pathological result was M, Ant, type 2, por, ypT2N0M0, ypStage ⅠB, while the histological effect of the chemotherapy was Grade 0. The patient was treated with paclitaxel as adjuvant chemotherapy, which was discontinued 1 year after surgery owing to no recurrence. No recurrence has been noted during 2 years of follow-up.

A 69-year-old male was referred to our hospital due to extensive thoracic aortic aneurysm with right aortic arch and isolated left subclavian artery. We chose staged hybrid repair to avoid the risk of spinal cord injury and bilateral recurrent nerve paralysis. First, ascending aorta replacement with elephant trunk and reconstruction of the cervical branches were underwent. Second, we performed the thoracic endovascular aortic repair for aortic arch and descending aortic aneurysm. The postoperative course was satisfactory and, computed tomography (CT) showed successful aortic repair without any type of endoleak. He was discharged on the ninth day after the second operation. To our knowledge, this is the first report of surgical repair for an aortic aneurysm with right aortic arch and isolated subclavian artery.

A robust, accurate, and standardized approach to measurement of the aorta is critical to improve the predictive accuracy of these aortic measurements, and to investigate other aortic imaging biomarkers. Developing a comprehensive and generic schema for characterization of the aorta to enable investigators to standardize data that are collected across all aorta research. A systematic review of the literature was conducted to identify and assess schemata of aortic measurement and description. The schemata were reported and discussed to guide the synthesis of a comprehensive schema. We propose the International College of Angiology Aortic Research Schema as a comprehensive design that fills the gaps left behind by previously reported schemata. It is intended to be applicable for all clinically relevant purposes, including endograft development for aneurysm repair and for the accurate characterization of the aortic anatomy. This schema divides the aorta into 14 segments and 2 sections (thoracic and abdominal aortas). The segmentation proposed can be used in addition to specific measurements taken for any aneurysm including the neck, and maximal and minimal diameters of the aneurysm.

Williams-Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management more eventful. Association of infective endocarditis within the aortic arch and pseudoaneurysm formation is infrequent. We, hereby report a case of pseudoaneurysm formation and infective vegetation within the aortic arch in a patient with Williams syndrome and the role of transthoracic echocardiography in its perioperative management.

Blunt traumatic injuries to the brachiocephalic artery require surgical management. The operative technique used depends on the location of the injury, the patient's hemodynamic stability, and the surgeon's experience. Perfusion strategy can facilitate vascular control of the aortic arch and branch vessels. This report presents an urgent repair of a proximal posterior blunt traumatic brachiocephalic artery injury under circulatory arrest, with an excellent outcome.

Introduction: Aortic aneurysm is a life-threatening disease resulted from progressive dilatation of the aorta, which can be subdivided into thoracic and abdominal aortic aneurysms. Sustained subcutaneous angiotensin II infusion can induce aortic aneurysms in mice. However, the relevance of using angiotensin II induction model to study aneurysm disease and the degree of commonality between species remain elusive. Methods: We utilized scRNA-seq to infer aortic cell sub-structures and transcriptional profiles in clinical patient TAAs and AAAs, as well as mouse models of corresponding diseases (Ang II induction) and in healthy mouse aorta. Unbiased comparison between mice and humans explored the possible reasonability and utility of mouse Ang II-induced aortic aneurysm as a model for human aortic aneurysm diseases. Meanwhile, we performed comparative analysis of aortic aneurysms between TAA and AAA in both organisms. Results and Discussion: We demonstrated similarities and differences of changes in the components of human and mouse cell types, and our unbiased comparison between mouse and human identified well conserved subpopulations of SMCs and macrophages. Furthermore, the results of our comparative analyses suggested different biological functions and distinct potential pathogenic genes for thoracic and abdominal aortic aneurysms. MIF and SPP1 signaling networks participated in aortic aneurysm in both organisms. This study maps aortic aneurysm and offers opportunities for future researches to investigate the potential of subpopulations or marker genes as therapy targets.

Type A aortic dissections (TAAD) are devastating aortic complications. Patients with Marfan syndrome, a bicuspid aortic valve or a thoracic aortic aneurysm have an increased risk to develop a TAAD. These predisposing conditions are characterized by a histologically thin intimal layer and hardly any atherosclerosis. Little is known about the susceptibility for atherosclerosis in patients with a type A aortic dissection.

Paraplegia remains the most devastating complication following thoracoabdominal aortic surgery. Motor-evoked potential (MEP) monitoring has been widely used to assess intraoperative motor function. MEP amplitude is affected by various factors, including anesthetic agents and measurement time; however, there are no reports regarding MEP monitoring using remimazolam in thoracoabdominal aortic surgery. A 57-year-old woman underwent open repair of a thoracic descending aorta for a chronic dissecting aortic aneurysm under remimazolam and remifentanil anesthesia. The administration rate of remimazolam was adjusted using spectral edge frequency of SedLine®, which ranged from 0.2 to 1.0 mg/kg/h after anesthetic induction with 12 mg/kg/h. Muscle MEPs were obtained using subdermal needle electrodes at the abductor pollicis brevis muscle and abductor hallucis. There were no significant changes, which were defined as a 50% reduction of MEP amplitude from each baseline value, including during split circulation. On postoperative day one, she had no motor deficits nor signs of intraoperative awareness. Remimazolam might be well tolerated for MEP monitoring in patients undergoing thoracic descending aortic aneurysm surgery.

The objective was to investigate the prevalence of abdominal aortic aneurysms (AAAs) and the diameters of the aorta and common iliac arteries (CIAs) in a Korean cohort and secondly to analyze the differences in aortic diameter by comparison with a European cohort. The Korean cohort included participants ≥ 50 years who consented to AAA screening and data were analysed retrospectively. Aortic and common iliac diameters were measured using the outer-to-outer diameter method and prevalence rates were calculated. Common risk factors such as smoking, body mass index, pulmonary disease, hypertension, diabetes, hyperlipidaemia, ischaemic heart disease, and cerebrovascular disease were reported in association with AAA occurrence and AAA development. The aortic diameters were then compared with those in a Belgian cohort of 2487 participants identified in the Liège AAA Screening Program. An aortic size index (ASI) was also calculated to account for the potential size differences in the Belgian and Korean populations. A total of 3124 Korean participants were examined using ultrasound. The prevalence of AAAs in this cohort was 0.7%. The combined prevalence of subaneurysmal dilatation and AAA was 1.5%. The prevalence in male smokers older than 65 years was 2.7% (19/715). The mean infrarenal aortic diameter was 17.3 ± 3.1 mm in men and 15.7 ± 2.7 mm in women; the corresponding values in Belgian participants were 19.4 ± 3.0 mm in men and 17.9 ± 2.4 mm in women. The median aortic size index was 0.99 (interquartile range 0.88-1.12). The mean infrarenal aortic diameter was significantly smaller in the Korean cohort than in the Belgian cohort. Considering the observed prevalence of AAAs in different age groups, the age groups which would contribute to most cases was male persons above 66 years in both cohorts.

Ascending aortic aneurysm is a pathology that is important to be supervised and treated. During the years the aorta dilates, it becomes stiff, and its elastic properties decrease. In some cases, the aortic wall can rupture leading to aortic dissection with a high mortality rate. The main reference standard to measure when the patient needs to undertake surgery is the aortic diameter. However, the aortic diameter was shown not to be sufficient to predict aortic dissection, implying other characteristics should be considered. Therefore, the main objective of this work is to assess in-vivo the elastic properties of four different quadrants of the ascending aorta and compare the results with equivalent properties obtained ex-vivo. The database consists of 73 cine-MRI sequences of thoracic aorta acquired in axial orientation at the level of the pulmonary trunk. All the patients have dilated aorta and surgery is required. The exams were acquired just prior to surgery, each consisting of 30 slices on average across the cardiac cycle. Multiple deep learning architectures have been explored with different hyperparameters and settings to automatically segment the contour of the aorta on each image and then automatically calculate the aortic compliance. A semantic segmentation U-Net network outperforms the rest explored networks with a Dice score of 98.09% (±0.96%) and a Hausdorff distance of 4.88 mm (±1.70 mm). Local aortic compliance and local aortic wall strain were calculated from the segmented surfaces for each quadrant and then compared with elastic properties obtained ex-vivo. Good agreement was observed between Young's modulus and in-vivo strain. Our results suggest that the lateral and posterior quadrants are the stiffest. In contrast, the medial and anterior quadrants have the lowest aortic stiffness. The in-vivo stiffness tendency agrees with the values obtained ex-vivo. We can conclude that our automatic segmentation method is robust and compatible with clinical practice (thanks to a graphical user interface), while the in-vivo elastic properties are reliable and compatible with the ex-vivo ones.

Thoracic aortic disease affects people of all ages and the majority of those aged <60 years have an underlying genetic cause. There is presently no effective medical therapy for thoracic aneurysm and surgery remains the principal intervention. Unlike abdominal aortic aneurysm, for which the inflammatory/atherosclerotic pathogenesis is well established, the mechanism of thoracic aneurysm is less understood. This paper examines the key cell signaling systems responsible for the growth and development of the aorta, homeostasis of endothelial and vascular smooth muscle cells and interactions between pathways. The evidence supporting a role for individual signaling pathways in pathogenesis of thoracic aortic aneurysm is examined and potential novel therapeutic approaches are reviewed. Several key signaling pathways, notably TGF-β, WNT, NOTCH, PI3K/AKT and ANGII contribute to growth, proliferation, cell phenotype and survival for both vascular smooth muscle and endothelial cells. There is crosstalk between pathways, and between vascular smooth muscle and endothelial cells, with both synergistic and antagonistic interactions. A common feature of the activation of each is response to injury or abnormal cell stress. Considerable experimental evidence supports a contribution of each of these pathways to aneurysm formation. Although human information is less, there is sufficient data to implicate each pathway in the pathogenesis of human thoracic aneurysm. As some pathways i.e., WNT and NOTCH, play key roles in tissue growth and organogenesis in early life, it is possible that dysregulation of these pathways results in an abnormal aortic architecture even in infancy, thereby setting the stage for aneurysm development in later life. Given the fine tuning of these signaling systems, functional polymorphisms in key signaling elements may set up a future risk of thoracic aneurysm. Multiple novel therapeutic agents have been developed, targeting cell signaling pathways, predominantly in cancer medicine. Future investigations addressing cell specific targeting, reduced toxicity and also less intense treatment effects may hold promise for effective new medical treatments of thoracic aortic aneurysm.

Aortic aneurysms represent a very common pathology that can affect any segment of the aorta. These types of aneurysms can be localized on the thoracic segment or on the abdominal portion, with the latter being more frequent. Though there are similarities between thoracic and abdominal aortic aneurysms, these pathologies are distinct entities. In this article, we undertook a review regarding the different mechanisms that can lead to the development of aortic aneurysm, and we tried to identify the different manners of treatment. For a long time, aortic wall aneurysms may evolve in an asymptomatic manner, but this progressive dilatation of the aneurysm can lead to a potentially fatal complication consisting in aortic rupture. Because there are limited therapies that may delay or prevent the development of acute aortic syndromes, surgical management remains the most common manner of treatment. Even though, surgical management has improved much in the last years, thus becoming less invasive and sophisticated, the morbi-mortality linked to these therapies remains increased. The identification of the cellular and molecular networks triggering the formation of aneurysm would permit the discovery of modern therapeutic targets. Molecular and cellular mechanisms are gaining a bigger importance in the complex pathogenesis of aortic aneurysms. Future studies must be developed to compare the findings seen in human tissue and animal models of aortic aneurysm, so that clinically relevant conclusions about the aortic aneurysm formation and the pharmacological possibility of pathogenic pathways blockage can be drawn.

Hemodynamics in aortic dissection (AD) is closely associated with the risk of aortic aneurysm, rupture, and malperfusion. Altered blood flow in patients with AD can lead to severe complications such as visceral malperfusion. In this study, we aimed to investigate the effect of cannulation flow on hemodynamics in AD using a fluid-structure interaction simulation. We developed a specific-idealized AD model that included an intimal tear in the descending thoracic aorta, a re-entry tear in the left iliac artery, and nine branches. Two different cannulation methods were tested: (1) axillary cannulation (AC) only through the brachiocephalic trunk and (2) combined axillary and femoral cannulation (AFC) through the brachiocephalic trunk and the right common iliac artery. AC was found to result in the development of a pressure difference between the true lumen and false lumen, owing to the difference in the flow rate through each lumen. This pressure difference collapsed the true lumen, disturbing blood flow to the celiac and superior mesenteric arteries. However, in AFC, the pressure levels between the two lumens were similar, and no collapse occurred. Moreover, the visceral flow was higher than that in AC. Lastly, the stiffness of the intimal flap affected the true lumen's collapse.

Marfan syndrome (MFS) is a rare connective tissue disorder caused by mutations in FBN1. Patients with MFS notably suffer from aortic aneurysm and dissection. Despite considerable effort, animal models have proven to be poorly predictive for therapeutic intervention in human aortic disease. Patient-derived induced pluripotent stem cells can be differentiated into vascular smooth muscle cells (VSMCs) and recapitulate major features of MFS. We have screened 1,022 small molecules in our in vitro model, exploiting the highly proteolytic nature of MFS VSMCs, and identified 36 effective compounds. Further analysis identified GSK3β as a recurring target in the compound screen. GSK3β inhibition/knockdown did not ameliorate the proliferation defect in MFS-VSMCs but improved MFS-VSMC proteolysis and apoptosis and partially rescued fibrillin-1 deposition. To conclude, we have identified GSK3β as a novel target for MFS, forming the foundation for future work in MFS and other aortic diseases.

Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening cardiovascular disease with severe extracellular matrix (ECM) remodeling that lacks efficient early stage diagnosis and nonsurgical therapy. A disintegrin and metalloproteinase with thrombospondin motif 7 (ADAMTS-7) is recognized as a novel locus for human coronary artery atherosclerosis. Previous work by us and others showed that ADAMTS-7 promoted atherosclerosis, postinjury neointima formation, and vascular calcification. However, whether ADAMTS-7 is involved in TAAD pathogenesis is unknown. We aimed to explore the alterations in ADAMTS-7 expression in human and mouse TAAD, and investigate the role of ADAMTS-7 in TAAD formation. A case-control study of TAAD patients (N = 86) and healthy participants (N = 88) was performed. The plasma ADAMTS-7 levels were markedly increased in TAAD patients within 24 h and peaked in 7 days. A TAAD mouse model was induced with 0.5% β-aminopropionitrile (BAPN) in drinking water. ELISA analysis of mouse plasma, Western blotting, and immunohistochemical staining of aorta showed an increase in ADAMTS-7 in the early stage of TAAD. Moreover, ADAMTS-7-deficient mice exhibited significantly attenuated TAAD formation and TAAD rupture-related mortality in both male and female mice, which was accompanied by reduced artery dilation and inhibited elastin degradation. ADAMTS-7 deficiency caused repressed inflammatory response and complement system activation during TAAD formation. An increase in plasma ADAMTS-7 is a novel biomarker for human TAAD. ADAMTS-7 deficiency attenuates BAPN-induced murine TAAD. ADAMTS-7 is a potential novel target for TAAD diagnosis and therapy. KEY MESSAGES: A case-control study revealed increased plasma ADAMTS-7 is a risk factor for TAAD. ADAMTS-7 was elevated in plasma and aorta at early stage of mouse TAAD. ADAMTS-7 knockout attenuated mouse TAAD formation and mortality in both sexes.

Thoracic aortic aneurysms (TAA) may be associated with complications such as rupture and dissection, which can lead to a fatal outcome. Increased central arterial stiffness has been proposed to be present in patients with TAA compared to unmatched controls. We aimed to assess whether wall properties in patients with TAA are also altered when compared to a matched control group. Applanation tonometry was performed in 74 adults with TAA and 74 sex, age, weight, height, and left ventricular ejection fraction matched controls. Subsequently analysis of the pulse wave was done using the SphygmoCor System. For comparing the two groups, AIx was adjusted to a heart rate of 75/min (AIx@75). 148 1-to-1 matched participants were included in the final model. There was no significant difference in the Alx@75 between the TAA group and the matched control group [mean (SD) of 24.7 (11.2) % and 22.8 (11.2) %, p = 0.240]. Adjusted for known cardiovascular risk factors, there was no association between TAA and AIx@75. Patients with TAA showed comparable arterial wall properties to cardiovascular risk factor matched controls. Since higher arterial stiffness is associated with TAA progression, it remains to be investigated if increased central arterial stiffness is a relevant factor of TAA emergence.

Objectives. Paraplegia is devastating complication associated with thoracic and thoracoabdominal aortic aneurysm repair. Vast evidence has been gathered on pre-, peri- and postoperative protective adjuncts aiming to minimize spinal cord ischemia. This review focuses on the pretreatment phase of open surgical or endovascular aortic procedures and gathers the experimental data on the interventional preconditioning and priming methods that increase the spinal cord ischemic tolerance. Design. By the start of March 2021, a systematic review was performed in PubMed, Scopus and Web of Science core collection to identify the articles that reported (i) either an ischemic preconditioning, remote ischemic preconditioning or priming method prior to (ii) experimental spinal cord ischemia performed in endovascular or open surgical fashion mimicking either thoracic, abdominal or thoracoabdominal aortic aneurysm procedures. (iii) The outcomes were reported via neurological, motor-evoked potential, somatosensory-evoked potential, histopathological, immunohistochemical, physiological analysis, or in different combinations of these measurements. Results. The search yielded 7802 articles, and 57 articles were included in the systematic review. The articles were assessed by the evaluated species, the utilized pretreatment, the measured protective effects, and the suggested underlying mechanisms. Conclusions. The reviewed articles showed several possible mechanisms in ischemic and remote ischemic preconditioning for prevention of spinal cord ischemia. The main suggested method for priming was arteriogenetic stimulus. Future studies should confirm these hints of arteriogenetic stimulus with more precise quantification of the protective recruitment process.