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The extracellular matrix is a complex network of proteins and other molecules that are essential for the support, integrity, and structure of cells and tissues within the human body. The genes ZNF469 and PRDM5 each produce extracellular-matrix-related proteins that, when mutated, have been shown to result in the development of brittle cornea syndrome. This dysfunction results from aberrant protein function resulting in extracellular matrix disruption. Our group recently identified and published the first known associations between variants in these genes and aortic/arterial aneurysms and dissection diseases. This paper delineates the proposed effects of mutated ZNF469 and PRDM5 on various essential extracellular matrix components, including various collagens, TGF-B, clusterin, thrombospondin, and HAPLN-1, and reviews our recent reports associating single-nucleotide variants to these genes' development of aneurysmal and dissection diseases. [ABSTRACT FROM AUTHOR]

Background: We retrospectively evaluated early and intermediate outcomes of hybrid repair of complex thoracic aortic diseases involving an aberrant right subclavian artery. This paper aims to report features and available treatment options for this rare, hard-to-diagnose, and manage, aorta-related vascular condition. Methods: Between January 2012 and May 2019, 13 patients (mean age, 60.1 ± 9.3 years; nine men) underwent complex thoracic aorta repair surgery. Six patients had a thoracic aortic aneurysm, two had type A aortic dissection, and five had complicated type B aortic dissection. Hybrid repair strategies included de-branching in combination with single-stage aortic arch replacement with the frozen elephant trunk technique performed in four patients, thoracic endovascular aortic repair in six patients, and 2-stage hybrid repair consisting of a total arch replacement with a conventional/frozen elephant trunk (first stage) and subsequent endovascular repair (second stage) in three patients. Results: One early death occurred: a patient with acute type A aortic dissection, who underwent Bentall procedure and aortic arch replacement with the frozen elephant trunk technique, died in-hospital of multiorgan failure 41 days after the procedure. The remaining 12 patients were discharged in stable condition. The median follow-up duration was 36 months (2–71 months). Two late mortalities occurred: a patient with residual type A aortic dissection, who underwent arch replacement with the frozen elephant trunk technique, died of intracranial hemorrhage 3 months after the surgery. And 72 years old female patient died of acute exacerbation of chronic obstructive pulmonary disease 2 months after the surgery. Conclusion: Our study indicates that various hybrid strategies can be used to treat complex thoracic aortic diseases involving an aberrant right subclavian artery. The approach of choice depends on the features of disease pathology, the aortic segments involved, and the operating surgeon's experience. [ABSTRACT FROM AUTHOR]

Purpose: After endovascular aneurysm repair (EVAR), an increased [18F]FDG uptake may be observed at PET/CT, being common to both vascular graft/endograft infection (VGEI) and sterile post-surgical inflammation. Increased non-specific metabolic activity, due to foreign body reaction, can persist for several years after surgery, thus complicating the interpretation of PET/CT studies. In this paper, we aimed to assess [18F]FDG distribution at different time-points after the implant of abdominal Endurant® endografts in patients without suspicion of infection. Methods: We retrospectively evaluated [18F]FDG/CT in 16 oncological patients who underwent abdominal aortic aneurysm exclusion with Endurant® grafts. Patients had no clinical suspicion of infection and were followed up for at least 24 months after scan. [18F]FDG PET/CT scans were interpreted using both visual and semi-quantitative analyses. Results: The time between the EVAR procedure and [18F]FDG PET/CT ranged between 1 and 36 months. All grafts showed mild and diffuse [18F]FDG uptake without a focal pattern. Mean values of SUVmax were 2.63 ± 0.48 (95% CI 2.38–2.88); for SUVmean 1.90 ± 0.33 (95% CI 1.72–2.08); for T/B ratios 1.43 ± 0.41 (95% CI 1.21–1.65). SUVmax and SUVmean were not correlated to the time elapsed from the procedure, but we observed a declining trend in T/B ratio over time. Conclusions: Endovascular implant of Endurant® grafts does not cause a significant inflammatory reaction. The evidence of faint and diffuse [18F]FDG uptake along the graft can reliably exclude an infection, even in early post-procedural phases. Therefore, in patients with a low probability of VGEI, [18F]FDG PET/CT can also be performed immediately after EVAR. [ABSTRACT FROM AUTHOR]

Abdominal aortic aneurysms (AAA) are life-threatening serious conditions that require effective and quick management. Although it is generally acknowledged that patients with AAA obtain the greatest benefit from endovascular repair (EVAR) compared to open surgical repair (OSR), there are few comparisons between the surgical approaches in Western versus Chinese patients. We aimed to perform a meta-analysis of studies in which EVAR was compared with OSR in the management of abdominal aortic aneurysms. We searched the Western literature through PubMed, OVID and Web of Science from 1991 until December 2018 and the Chinese-language literature from 1998 until December 2018. We pooled the results in January 2019 based on standardized inclusion and exclusion criteria and analyzed them using a conventional meta-analysis. Forty-five English papers with 31,074 AAA patients and twenty-one Chinese studies with 1,405 patients were included in this study. Chinese subjects were more likely to undergo endovascular repair than Western subjects (44.5% versus 41.5%, P = 0.012). The 30-day post-discharge mortality rate in Western studies was significantly lower for EVAR than for OSR (odds ratio (OR) = 0.481, P < 0.001). However, there was no significant reduction in the 30-day mortality rate following EVAR compared to OSR (OR = 0.733, P = 0.425) for Chinese patients. In Western patients, the postoperative complication rate of respiratory system and cardiac system was lower in the EVAR group than in the OSR group (OR = 0.270, P < 0.001 and OR = 0.411, P < 0.001, respectively), nevertheless, for Chinese patients, limb ischaemia was more common (OR = 1.539, P = 0.049) in the EVAR group. Whether in Western patients with an eight-year follow-up period or Chinese patients with a maximum four-year follow-up period, there was no significant difference between the EVAR and OSR groups in the all-cause death rate (hazard ratio (HR) = 1.026, P = 0.483 and HR = 1.173, P = 0.247, respectively). Chinese patients were more likely to receive EVAR than OSR and the 30-day mortality was significantly lower for EVAR than for OSR in Western patients but not in Chinese patients. Endovascular repair can be applied to Chinese patients with a reasonable safety margin. Further work is needed to explore the causes of these treatment differences. [ABSTRACT FROM AUTHOR]

The advent and refinement of complex endovascular techniques in the last two decades has revolutionized the field of vascular surgery. This has allowed an effective minimally invasive treatment of extensive disease involving the pararenal and the thoracoabdominal aorta. Fenestrated-branched EVAR (F/BEVAR) now represents a feasible technical solution to address these complex diseases, moving the proximal sealing zone above the renal-visceral vessels take-off and preserving their patency. The aim of this paper was to provide a narrative review on the peri-operative management of patients undergoing F/BEVAR procedures for juxtarenal abdominal aortic aneurysm (JAAA), pararenal abdominal aortic aneurysm (PRAA) or thoracoabdominal aortic aneurism (TAAA). It will focus on how to prevent, diagnose, and manage the complications ensuing from these complex interventions, in order to improve clinical outcomes. Indeed, F/BEVAR remains a technically, physiologically, and mentally demanding procedure. Intraoperative adverse events often require prolonged or additional procedures and complications may significantly impact a patient's quality of life, health status, and overall cost of care. The presence of standardized preoperative, perioperative, and postoperative pathways of care, together with surgeons and teams with significant experience in aortic surgery, should be considered as crucial points to improve clinical outcomes. Aggressive prevention, prompt diagnosis and timely rescue of any major adverse events following the procedure remain paramount clinical needs. [ABSTRACT FROM AUTHOR]

The human aorta is a high-risk area for vascular diseases, which are commonly restored by thoracic endovascular aortic repair. In this paper, we report a promising shear-activated targeted nanoparticle drug delivery strategy to assist in the treatment of coarctation of the aorta and aortic aneurysm. Idealized three-dimensional geometric models of coarctation of the aorta and aortic aneurysm are designed, respectively. The unique hemodynamic environment of the diseased aorta is used to improve nanoparticle drug delivery. Micro-carriers with nanoparticle drugs would be targeting activated to release nanoparticle drugs by local abnormal shear stress rate (SSR). Coarctation of the aorta provides a high SSR hemodynamic environment, while the aortic aneurysm is exposed to low SSR. We propose a method to calculate the SSR thresholds for the diseased aorta. Results show that the upstream near-wall area of the diseased location is an ideal injection location for the micro-carriers, which could be activated by the abnormal SSR. Released nanoparticle drugs would be successfully targeted delivered to the aortic diseased wall. Besides, the high diffusivity of the micro-carriers and nanoparticle drugs has a significant impact on the surface drug concentrations of the diseased aortic walls, especially for aortic aneurysms. This study preliminary demonstrates the feasibility of shear-activated targeted nanoparticle drug delivery in the treatment of aortic diseases and provides a theoretical basis for developing the drug delivery system and novel therapy. [ABSTRACT FROM AUTHOR]

As the global population ages, the issue of abdominal aortic aneurysm continues to grow. With the evolution of new devices and refined operative technique, aneurysm treatment via endovascular aortic repair is becoming increasingly favourable. This, however, is not without drawbacks, where regular surveillance is paramount to long‐term success and detection of post‐procedure complications. Of these complications, endoleak is the most notable and poses the greatest risk of potential future aortic rupture. The purpose of this review paper is to discuss the armada of imaging modalities used in the detection and evaluation of endoleak and their varying usefulness. Plain abdominal X‐ray is a cost‐effective tool in detecting gross graft abnormalities such as stent migration or deformity (kinking or fracture). Though it may raise suspicion for endoleak, X‐ray does not allow accurate classification of endoleak type when used alone. Duplex ultrasonography quantifies both aortic anatomy and real time flow dynamics. Most screening programmes are conducted using two‐dimensional ultrasound. Unfortunately, observer and equipment variability may lead to surveillance discrepancies—but reduced when utilising a dedicated vascular sonography laboratory. Contrast enhanced ultrasonography is a promising alternative to computed tomography, though still is emerging. Computed tomography angiography certainly has disadvantages (ionising radiation, contrast‐nephropathy, limited differentiation of endoleak type)—however, it provides near‐real surgical dimensions and highlights graft complications and concomitant disease (such as neighbouring infection). With widespread availability and short scan time, it certainly remains valuable in surveillance. Magnetic resonance angiography has a similar sensitivity to computed tomography (minus the radiation), however is plagued by movement and metal artefact. Other novel modalities in endoleak surveillance include four‐dimensional ultrasound, multiplanar intra‐operative probes, nuclear medicine and wall stress analysis. [ABSTRACT FROM AUTHOR]

Acute type A aortic dissection is a surgical emergency and management of such pathology can be complex with poor outcomes when there is organ malperfusion. Carotid artery involvement is present in 30% of patients diagnosed with acute type A aortic dissection, and given its emergency and complex nature, there is much controversy regarding the approach, extent of treatment, and timing of the intervention. It is clear that the occurrence of cerebral malperfusion adds an extra layer of complexity to the decision-making framework for treatment. Standardization and validation of the optimal management approach is required, and this should ideally be addressed with large-scale studies. Nonetheless, current literature supports the need for rapid recognition and diagnosis of acute type A aortic dissection with cerebral malperfusion, immediate and extensive surgical repair, and the appropriate use of cerebral perfusion techniques. This paper aims to discuss the current evidence regarding the impact of carotid artery involvement in both the management and outcomes of acute type A aortic dissection. [ABSTRACT FROM AUTHOR]

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The study of Arterial blood flow is a fascinating topic as arterial disease is responsible of death in many nations. This paper investigated the conduct of blood flow across a tapered artery with stenosis. This study has been simply explained by the use of a mathematical model that is appropriate. The graphical representations were created to support the results of the study. For various values of tapering angle, it is discovered that wall shear stress enhance when a peak is reached, then decreases. We also found that the velocity of the blood flow reduces with radius and also for distinct values of tapering angle. It is also evidenced that the blood flow rate reduces as the radius and tapering angle increase. [ABSTRACT FROM AUTHOR]

Acute aortic syndrome is a group of interlinked conditions with common presenting symptoms, including aortic dissection, penetrating atherosclerotic ulcer, and intramural hematoma. Pharmacological management of acute aortic syndrome is a growing area, with key themes to address the underlying inflammatory pathways believed to be the cause. Research into interleukins, matrix metalloproteinases, and granulocyte macrophage colony-stimulating factor are just some of the many immunological properties being investigated and translated into medical therapies. Stem cell experiments may indicate further advances in the pathologies of acute aortic syndrome. The study of pharmacogenomics to improve treatment across different genomes is also a novel area outlined in this paper. [ABSTRACT FROM AUTHOR]

Objective: To systematically review the rate of morbidity and mortality associated with the use of E-vita hybrid stent graft and ThoraflexTM in patients undergoing complex aortic surgery. Methods: A comprehensive search was undertaken among the four major databases to identify published data about E-vita or ThoraflexTM in patients undergoing repair of thoracic aortic aneurysms. Results: In total, 28 papers were included in the study, encompassing a total of 2,161 patients (1,919 E-vita and 242 ThoraflexTM). Patients undergoing surgery with E-vita or ThoraflexTM were of similar age and sex. The number of patients undergoing non-elective repair with ThoraflexTM was higher than with E-vita (35.2% vs. 28.7%, respectively). Cardiopulmonary bypass time was associated with increasing mortality in E-vita patients, however a meta-analysis of proportions showed higher 30-day mortality, permanent neurological deficit, and one-year mortality for ThoraflexTM patients. Direct statistical comparisons between E-vita and ThoraflexTM was not possible due to heterogeneity of studies. Conclusion: Although there are limited studies available, the available data suggests that mortality and morbidity are lower for the E-vita device in thoracic aortic aneurysm surgery than for ThoraflexTM. Long-term data of comparative studies do not yet exist to assess viability of these procedures. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to examine contemporary results of the frozen elephant trunk (FET) procedure in an all-comers patient cohort. Methods: Between January 2017 and May 2024, a total of 132 consecutive patients with either aortic aneurysm (n = 32), acute aortic dissection (n = 32), or chronic aortic dissection (n = 68) underwent total aortic arch replacement employing the FET technique. In-hospital data were collected prospectively and included preoperative characteristics, intraoperative data, and follow-up results. Results: The median cardiopulmonary bypass time, cardiac ischemia time, and selective antegrade cerebral perfusion time were 180 (161–205), 89 (70–113), and 45 (38–54) min, respectively. Total 30-day mortality rate was 7.6% (n = 10). The rate of major postoperative neurological complications was 6.8% (n = 9) for perioperative stroke and 2.3% (n = 3) for permanent spinal cord injury. Five patients (3.8%) required hemofiltration at the time of discharge due to postoperative kidney injury. Rates of subsequent endovascular and open aortic repair following primary FET were 40.9% (n = 54) and 3.8% (n = 5), respectively. The median time to reintervention was 86 (30–439) days. The median follow-up time was 25 (8–52) months, and overall survival rates at 1, 2, and 3 years were 89%, 89%, and 87%, respectively. Conclusions: Our data are consistent with current reports, indicating that the FET technique is a valuable adjunct in treating extensive aortic arch pathologies. The procedure provides an increasingly safe and effective option for complete aortic arch replacement, even in patients requiring a redo procedure. [ABSTRACT FROM AUTHOR]

This paper presents a case study of a patient that underwent surgery for a ruptured abdominal aneurysm. The postoperative course was complicated by resistant hypertension and tachycardia. A suprarenal mass was detected in the computed tomography scan with radiological suspicion of pheochromocytoma. Few cases of pheochromocytoma coexisting with aneurysms have been reported. Management of cardiovascular stability is crucial in such cases. Despite the lack of evidence, pheochromocytomas might have a role in the etiology of aortic aneurysms. [ABSTRACT FROM AUTHOR]

Inflammatory aortic diseases are broadly classified into three categories according to the degree of inflammation: atherosclerosis, atherosclerosis with excessive inflammation, and aortitis/periaortitis. This paper presents a case of a 39-year old man with aneurysmal dilatation of thoracic aorta and aortic valve insufficiency. The aortic wall showed thickening and wrinkled "tree bark" appearance as well as apparent scarring of the intima. Histological examination revealed intimal hyperplasia, a granulomatous/giant cell pattern in the inner tunica media, a few epithelioid macrophages, abundant chronic lymphoplasmacytic and histiocytic inflammation and discrete fibrinoid necrosis. The histological findings were indicative of Horton's disease, but no typical clinical features were present. The case illustrates the difficulties involved in diagnosing inflammatory aortic diseases where it may be challenging to arrive at a specific diagnosis despite the knowledge of medical history, and available macroscopic and histological findings. [ABSTRACT FROM AUTHOR]

Background/Objectives: Pulmonary diseases are common in patients with thoracic aortic aneurysm (TAA) and abdominal aortic aneurysm (AAA). Although high prevalences of chronic obstructive pulmonary disease and lung cancer (LC) are known, the prevalence of these and other pulmonary diseases regarding their relation to the outcome of TAA and/or AAA are not determined. Methods: Pulmonary diseases present at aortic aneurysm (AA) diagnosis and follow-up periods and cause of death of 952 patients with TAA, AAA, or TAA + AAA (including thoracoabdominal AA) treated at our institution in Japan were retrospectively analyzed. Cox regression analysis was used to investigate potential risk factors of mortality. Results: The mean patient age was 72.4 years, and the median follow-up was 4.92 years. At diagnosis, 528 (55.5%) patients had pulmonary diseases, including emphysema without interstitial lung disease (ILD) or LC, LC, idiopathic pulmonary fibrosis (IPF) without LC, non-IPF ILD without LC, and interstitial lung abnormalities (ILAs) without LC in 250, 85, 65, 15, and 58 patients, respectively. During follow-up, LC and acute exacerbation (AE) of IPF developed in 50 and 12 patients, respectively. In 213 patients who died, there were 45 (21.1%) aortic disease-related deaths. Other causes of death included LC (27.7%), cardiovascular events (9.4%), pneumonia (5.6%), and interstitial lung disease (4.7%). In a multivariate Cox regression hazard model, age; larger maximum aneurysm diameter; and coexisting LC, IPF, or concomitant cancer were associated with poor prognosis. Conclusions: In patients with AA, not only age and aneurysm diameter but also coexisting LC and IPF were prognostic factors for mortality. [ABSTRACT FROM AUTHOR]

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder of platelet function. The frequent occurrence of alloimmunization due to repeated platelet transfusions is the major complication of the disease. Achieving hemostasis in these patients with anti-GPIIb-IIIa antibodies during surgical procedures is a significant challenge due to the high risk of bleeding. Recombinant activated factor VII (rFVIIa) is an effective agent for achieving hemostasis in alloimmunized Glanzmann's thrombasthenia patients. The key clinical question was to determine whether abdominal aortic aneurysm surgery can be safely performed with rFVIIa in Glanzmann's thrombasthenia patients with anti-GPIIb/IIIa antibodies and whether long-term antiplatelet therapy is suitable for these patients. The patient underwent endovascular aneurysm repair with intensive rFVIIa administration, experiencing neither bleeding nor thrombosis. Data regarding the surgical management of Glanzmann's thrombasthenia patients with anti-GPIIb-IIIa antibodies and the use of antithrombotics in this high-risk population are still very limited. Sharing clinical experience can be valuable for hematologists managing similar cases. [ABSTRACT FROM AUTHOR]

Background: The widespread use of fluoroquinolones has been associated with the formation, dissection, and rupture of aortic aneurysms. Arterial biomarkers are established predictors of cardiovascular events. The present study was designed to investigate the effect of quinolones on arterial stiffness and aortic size for the first time. Methods: We studied 28 subjects receiving short-term (<15 days) antibiotic therapy involving quinolones and 27 age- and sex-matched subjects receiving an alternative to quinolone antibiotics. The follow-up period was approximately 2 months. The study's primary endpoint was the carotid–femoral pulse wave velocity (cfPWV) difference between the two groups 2 months after therapy initiation. Secondary endpoints were the augmentation index corrected for heart rate (AIx@75) and sonographically assessed aortic diameters 2 months after the initial treatment. Results: Subjects had similar values of arterial biomarkers, blood pressure measurements, and aortic diameters at baseline. At follow-up, no significant change was observed between the two groups regarding the hemodynamic parameters and arterial biomarkers (p > 0.05 for all), i.e., cfPWV (7.9 ± 2.6 m/s for the control group vs. 8.1 ± 2.4 m/s for the fluoroquinolones group; p = 0.79), AIx@75 (22.6 ± 9.0% for the control group vs. 26.6 ± 8.1% for the fluoroquinolones group; p = 0.09), and aortic diameters. Conclusions: To our knowledge, FRAGILES is the first study to provide insights into the possible effects of fluoroquinolones on arterial biomarkers, showing that, at least in the short term, treatment with fluoroquinolones does not affect aortic function and diameter. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Upper gastrointestinal bleeding due to primary aortoesophageal fistula is a rare clinical condition burdened with high mortality rate. However, the outcomes are closely related to the level of clinical awareness, the complementary and multidisciplinary approach during the diagnostic workup, and the selected treatment option. We present an atypical case of an aneurysm of the thoracoabdominal aorta complicated with primary aortoesophageal fistula (AEF). A 55‐year‐old male with no previous diseases, presented with prolonged and intense back pain and upper gastrointestinal bleeding. The gastroscopy detected an unusual culprit lesion in the distal esophagus resembling an esophageal wall defect, and the computed tomography revealed an aneurysm of the thoracoabdominal aorta, remarkable surrounding hematoma, and active contrast extravasation. Despite the urgent surgical repair, a lethal outcome occurred. AEF patients require high clinical awareness and complementary multidisciplinary approach in order to provide a rapid diagnosis and optimal treatment. [ABSTRACT FROM AUTHOR]

This paper explores information sharing in multidisciplinary clinical collaboration between three hospitals. Our study draws on qualitative interviews with surgeons and radiologists in two county hospitals and one university hospital. The analysis shows that the actors shared a restricted amount of information about the patients they have in common and that different actors used the shared information in different ways. However, much communication was still needed to clarify and negotiate the meaning of shared data and its implications for collaborative care. To conclude, while the arguments for a shared information space may appear convincing, the communication practice observed should illustrate that IS also needs to support the communicative process in clinical collaborative work. [ABSTRACT FROM AUTHOR]

Infected or mycotic aortic aneurysms (MAAs) are a rare type of aneurysms. Due to the high risk of rupture, MAAs are life-threatening conditions. Early diagnosis and treatment are necessary, yet MAAs are usually found coincidentally. We report 10 patients with MAAs in whom macroscopically, similar coined-sized lesions of the inner aortic wall were seen in all cases. When a coin-sized lesion in the inner aortic wall is seen during open surgical repair of an aortic aneurysm, the surgeon should consider an infectious cause. Microbiological tissue samples should be collected, and additional targeted antibiotic therapy should be started. [ABSTRACT FROM AUTHOR]