Human STAT1 gain of function with chronic mucocutaneous candidiasis: A comprehensive review for strengthening the connection between bedside observations and laboratory research.

Germline human heterozygous STAT1 gain-of-function (GOF) variants were first discovered a common cause of chronic mucocutaneous candidiasis (CMC) in 2011. Since then, numerous STAT1 GOF variants have been identified. A variety of clinical phenotypes, including fungal, viral, and bacterial infections, endocrine disorders, autoimmunity, malignancy, and aneurysms, have recently been revealed for STAT1 GOF variants, which has led to the expansion of the clinical spectrum associated with STAT1 GOF. Among this broad range of complications, it has been determined that invasive infections, aneurysms, and malignancies are poor prognostic factors for STAT1 GOF. The effectiveness of JAK inhibitors as a therapeutic option has been established, although further investigation of their long-term utility and side effects is needed. In contrast to the advancements in treatment options, the precise molecular mechanism underlying STAT1 GOF remains undetermined. Two primary hypotheses for this mechanism involve impaired STAT1 dephosphorylation and increased STAT1 protein levels, both of which are still controversial. A precise understanding of the molecular mechanism is essential for not only advancing diagnostics but also developing therapeutic interventions. Here, we provide a comprehensive review of STAT1 GOF with the aim of establishing a stronger connection between bedside observations and laboratory research.

Arterial aneurysm and dissection: toward the evolving phenotype of Tatton-Brown-Rahman syndrome.

BACKGROUND: Tatton-Brown-Rahman syndrome (TBRS) is a rare disorder, caused by DNMT3A heterozygous pathogenic variants, and first described in 2014. TBRS is characterised by overgrowth, intellectual disability, facial dysmorphism, hypotonia and musculoskeletal features, as well as neurological and psychiatric features. Cardiac manifestations have also been reported, mainly congenital malformations such as atrial septal defect, ventricular septal defect and cardiac valvular disease. Aortic dilatation has rarely been described. METHODS: Here we have undertaken a detailed clinical and molecular description of eight previously unreported individuals, who had TBRS and arterial dilatation and/or dissection, mainly thoracic aortic aneurysm (TAA). We have also reviewed the seven previously published cases of TAA in individuals with TBRS to try to better delineate the vascular phenotype and to determine specific follow-up for this condition. RESULTS: We include eight new patients with TBRS who presented with arterial aneurysms mainly involving aorta. Three of these patients presented with dissection that required critical surgery. CONCLUSIONS: Arterial aneurysms and dissections are a potentially lethal, age-dependent manifestation. The prevalence of aortic disease in individuals with TBRS is far in excess of that expected in the general population. This cohort, together with individuals previously published, illustrates the importance to consider dilatation/dissection, mainly in aorta but also in other arteries. Arterial vascular weakness may therefore also be a cardinal feature of TBRS and vascular surveillance is recommended.

Midterm outcomes of arteriovenous fistula aneurysm repair with simultaneous inflow revision or outflow tract revascularization.

OBJECTIVE: The aim of this study was to investigate the 2-year outcomes of extensive revisions aimed at simultaneously addressing arteriovenous fistula (AVF) aneurysms, outflow stenosis, and/or high flow volumes. METHODS: This was a retrospective cohort study in a tertiary referral center. The study population comprised hemodialysis patients with aneurysmal AVFs requiring revision. Subjects were eligible if they were ≥18 years of age and had aneurysm repair associated with inflow revision or treatment of outflow lesions. Patients were followed-up for a minimum of 2 years, and the primary outcome measure was cumulative patency. RESULTS: Our cohort comprised 93 patients with a mean age of 56 ± 12 years. The most frequent access type was brachiocephalic fistula (61%), and the median access age at the time of revision was 66 months (interquartile range, 46-93 months). The median access flow volume was 2300 ml/min (interquartile range, 1281-2900), and 31 subjects (33%) presented aneurysms associated with both high flow and venous obstruction. Aneurysm repair consisted of aneurysmorraphy in the overwhelming majority (95%). Inflow revision was performed in a total of 49 cases (53%), and outflow tract obstruction was treated in 71 patients (72%), with 27 patients (29%) receiving a simultaneous 3-component revision. At 2 years, the primary, primary assisted, and cumulative patencies were 60% ± 1%, 91% ± 3%, and 94% ± 3%, respectively. The most frequent indication for first-time reinterventions was outflow tract stenosis (24%). Aneurysm recurrence was observed in 13 subjects (14%) during the entire follow-up and was responsible for reinterventions in seven of them (8%), with a 2-year freedom from aneurysm-related reintervention rate of 95% ± 3%. CONCLUSIONS: Extensive AVF revision with simultaneous aneurysm repair, inflow revision, and outflow tract revascularization is feasible, safe, and associated with favorable 2-year patency rates.

Treatment strategy for splenic artery aneurysms and novel classification based on imaging.

OBJECTIVE: The incidence of splenic artery aneurysms (SAAs) has increased with advances in imaging techniques, necessitating a comprehensive classification to guide treatment strategies. This study aims to propose a novel classification system for SAAs based on aneurysm characteristics and to review treatment outcomes at our center. METHODS: This retrospective study included 113 patients with SAAs admitted to Peking Union Medical College Hospital from January 2019 to December 2023, assessed using computed tomography angiography or digital subtraction angiography. A new classification system was devised based on the aneurysm location, morphology, integrity, and parent artery anatomy. Treatment strategies were determined based on these characteristics, with interventions ranging from endovascular therapy to laparoscopic and open surgery. Patients were followed up after the intervention to assess mortality, complications, reinterventions, and aneurysm-related outcomes. RESULTS: The study cohort of 113 patients with 127 SAAs had a predominance of female patients (63.7%) and a mean age of 52.7 years. The SAAs were classified into five types, with type I being the most common. The intervention techniques varied across types, with sac embolization, covered stent implantation, and artery embolization being the most frequently used. The overall technical success rate was 94.7%, with perioperative complication and reintervention rates of 25.0% and 0.9%, respectively, and no deaths within 30 days after the intervention. The median follow-up duration was 21 months, with overall complications rate of 3.5% and no aneurysm-related complications or deaths. CONCLUSIONS: The proposed classification system effectively guides the selection of treatment strategies for SAAs, incorporating key anatomical and morphological features. This system facilitated high technical success and low complication rates, underscoring the importance of tailored techniques in managing SAAs.

Outcomes of one-stage and two-stage aneurysm repair in arteriovenous fistulae.

OBJECTIVE: Maintenance of long-term arteriovenous access is important in long-term care for patients with end-stage renal disease. Arteriovenous access is associated in the long term with the development of fistula aneurysms (FAs). This study aims to evaluate the outcomes of staged FA treatment in dialysis access arteriovenous fistulae (AVF). METHODS: A retrospective review of all patients over a 12-year period with primary autogenous AVF was undertaken at a single center. Patients undergoing elective open aneurysm repair were identified and were categorized into three groups: single FA repair (single, control group) and staged and unstaged repair of two FAs (staged and unstaged). A staged repair was a procedure in which the initial intent was to treat both aneurysms in the AVF and in which the most symptomatic aneurysm was treated first. When the incision from the first surgery had healed, the second symptomatic aneurysm in the AVF was treated. An unstaged repair was a procedure in which the initial intent was to repair both symptomatic aneurysms simultaneously. All patients had a fistulogram before the FA repair. Thirty-day outcomes, cannulation failure, line placement, reintervention, and functional dialysis (continuous hemodialysis for 3 consecutive months) were examined. RESULTS: Five hundred twenty-seven patients presented with FA that met requirements for open intervention; 44% underwent single FA repair, whereas the remaining 34% and 22% underwent staged and unstaged repair of two FAs, respectively. The majority of patients were diabetic and Hispanic. Ninety-one percent of the patients required percutaneous interventions of the outflow tract (37%) and the central veins (54%). Thirty-day major adverse cardiovascular events were equivalent across all modalities. Thirty-day morbidity and early thrombosis (<18 days) were significantly higher in the unstaged group (4.3%) compared with the two other groups (1.3% and 2.1%, single and staged, respectively), which led to an increased need for a short-term tunneled catheter (8.9%) compared with the two other groups (3.4% and 4.4%, single and staged, respectively), Unstaged repair resulted in an increased incidence of secondary procedures (5.0%) compared with the two other groups (2.6% and 3.1%, single and staged, respectively). Functional dialysis at 5 years was equivalent in the single and staged groups but was significantly decreased in the unstaged group. CONCLUSIONS: Open interventions are successful therapeutic modalities for FAs, but unstaged rather than staged repair of two concurrent FAs results in a higher early thrombosis, an increased secondary intervention rate, and a need for a short-term tunneled central line. Staged and single FA repairs have equivalent results. In the setting of two symptomatic FAs, staged repair is recommended.

Aneurysmal Disease in Patients With Takayasu Arteritis.

OBJECTIVE: Takayasu arteritis (TA) leads to stenotic disease. Aneurysmal lesions are rarer. This study assessed the main characteristics of aneurysmal disease in a Canadian cohort of patients with TA. METHODS: This monocentric retrospective study included patients with TA followed at the Mount Sinai Hospital Vasculitis Clinic in Toronto. Diagnosis of TA was based on clinical findings and/or satisfied the 1990 American College of Rheumatology classification criteria. RESULTS: Seventy-four patients were included. At any time, aneurysmal disease was found in 23 (31%) patients. Median disease duration was 9.0 (IQR 7.0-19.0) years. Prior hypertension (P = 0.02), fever (P = 0.04), and seizure disorders (P = 0.03) were more common. Limb claudication was less frequent (P = 0.01). Persistent and/or new aneurysms were demonstrated in 22/23 patients at follow-up. Thoracic aorta aneurysm (13/22) was most common, followed by abdominal aorta (8/22), subclavian (7/22), and carotid (6/22) artery disease. Aortic valve regurgitation was more frequent (9/23 vs 3/48; P = 0.001). Twenty-one patients had been treated with glucocorticoids (median 6.1 years [IQR 3.7-8.1]). Methotrexate, azathioprine, and leflunomide were repeatedly used. Infliximab (7/23) was used more often (P = 0.04), whereas tocilizumab was received by only 4 patients with aneurysmal disease (P = 0.01). Patients with aneurysms suffered more frequent relapses (2.0 [IQR 0.0-4.0] vs 1.0 [IQR 0.0-2.0], P = 0.04). CONCLUSION: Aneurysmal disease was found in a significant proportion of patients with TA. Given that aneurysms may carry a risk of rupture, and are associated with a higher rate of relapse, this finding should be reported systematically in TA studies.

Drug Delivery Angle for Various Atherosclerosis and Aneurysm Percentages of the Carotid Artery.

Stroke is the second cause of mortality among adult males and the first cause of death in adult females all around the world. It is also recognized as one of the most important causes of morbidity and dementia in adults. Stenosis or rupture of the only channels of the blood supply from the heart to the brain (carotid arteries) is among the main causes of stroke. In this regard, treatment of the lesions of carotid arteries, including atherosclerosis and aneurysm, could be a huge step in preventing stroke and improving brain performance. Targeted drug delivery by drug-carrying nanoparticles is the latest method for optimal delivery of drug to the damaged parts of the artery. In this study, a wide range of carotid artery lesions, including different percentages of atherosclerosis and aneurysm, were considered. After analyzing the dynamics of the fluid flow in different damaged regions and selecting the magnetic framework with proper ligand (Fe3O4@MOF) as the drug carrier, the size of the particles and their number per cycle were analyzed. Based on the results, the particle size of 100 nm and the use of 300 particles per injection at each cardiac cycle can result in maximum drug delivery to the target site. Then, the effect of the hospital bed angle on drug delivery was investigated. The results showed a unique optimal drug delivery angle for each extent of atherosclerosis or aneurysm. For example, in a 50% aneurysm, drug delivery at an angle of 30° is about 387% higher than that at an angle of 15°. Finally, simulation of real geometry indicated the effectiveness of simple geometry instead of real geometry for the simulation of carotid arteries, which can remarkably decrease the computational time and costs.

A Qualitative Systematic Review of Endovascular Management of Renal Artery Aneurysms.

PURPOSE: To perform a qualitative systematic review of endovascular management of renal artery aneurysms (RAAs). MATERIALS AND METHODS: A comprehensive electronic search of PubMed, MEDLINE, Embase, Google Scholar, and Cochrane databases from 2000 to 2022 was performed using the search terms "renal artery," "aneurysm," and "endovascular." Means of outcome measures were calculated with a primary end point focused on RAA-related mortality and rupture. Secondary end points included reintervention rate and renal infarction. RESULTS: Twenty-six, single-center, retrospective, observational studies were included. There were 454 RAAs treated in 427 patients using endovascular techniques. Mean age was 53.8 years, with a female predominance (62%). A variety of endovascular treatments of RAA were used with excellent technical success (96%), renal parenchymal preservation, and a low rate of moderate/severe adverse events (AEs). Primary coil embolization was the most commonly used technique (44.7%). There was an overall AE rate of 22.9%, of which 6.7% were moderate/severe and there was 0% periprocedural mortality. The most common AE was renal infarction (49 patients, 11.5%); however, renal function was preserved in 84% of patients. Nephrectomy rate was 0.4%. Computed tomography (CT) angiography was the most common imaging follow-up modality used in 72% of studies. Only 9 studies (34%) reported anticoagulant use. Although the risk of delayed aneurysm reperfusion warrants clinical and imaging surveillance, relatively few patients (3%) required reintervention in this cohort. CONCLUSIONS: Endovascular management of RAA is a technically feasible treatment option with low rates of AEs and reintervention. The present study highlights the techniques available for interventional radiologists, a need for standardization of AE reporting, anticoagulation therapy, and follow-up imaging.

The Natural History of Splenic Artery Aneurysms: Factors That Predict Aneurysm Growth.

PURPOSE: To examine the natural history of splenic artery aneurysms (SAAs) at a single institution and assess the effect of patient factors and aneurysm characteristics on aneurysm growth. MATERIALS AND METHODS: This single-center retrospective study included patients with SAAs who underwent serial imaging over 30 years (1990-2020). Data regarding patient demographics and aneurysm characteristics were collected. The variables contributing to aneurysm growth were assessed using nonparametric tests for continuous variables and chi-square test for categorical variables. Multivariable linear regression was performed using aneurysm growth rate as a continuous dependent variable. RESULTS: A total of 132 patients were included in this study. The median maximum diameter of the SAAs was 15.8 mm (range, 4.0-50.0 mm). Growth over time was observed in 39% of the aneurysms, whereas the remaining 61% were stable in size. Of aneurysms that increased in size, the median aneurysm growth rate was 0.60 mm/y (range, 0.03-5.00 mm/y). Maximum aneurysm diameter of >2 cm and the presence of >50% mural thrombus were significant positive predictors for aneurysm growth (P = .020 and P = .022, respectively). Greater than 50% rim calcification was a significant negative predictor for aneurysm growth (P = .009) in multivariate analysis. CONCLUSIONS: A larger baseline SAA size, presence of mural thrombus, and lack of rim calcification are associated with increased aneurysm growth rate.

Smooth Muscle Cell Klf4 Expression Is Not Required for Phenotype Modulation or Aneurysm Formation in Marfan Syndrome Mice-Brief Report.

BACKGROUND: Smooth muscle cell (SMC) phenotypic reprogramming toward a mixed synthetic-proteolytic state is a central feature of aortic root aneurysm in Marfan syndrome (MFS). Previous work identified Klf4 as a potential mediator of SMC plasticity in MFS. METHODS: MFS (Fbn1C1041G/+) mouse strains with an inducible vascular SMC fluorescent reporter (MFSSMC) with or without SMC-specific deletion of Klf4 exons 2 to 3 (MFSSMC-Klf4Δ) were generated. Simultaneous SMC tracing and Klf4 loss-of-function (Klf4Δ mice) was induced at 6 weeks of age. Aneurysm growth was assessed via serial echocardiography (4-24 weeks). Twenty-four-week-old mice were assessed via histology, RNA in situ hybridization, and aortic single-cell RNA sequencing. RESULTS: MFS mice demonstrated progressive aortic root dilatation compared with control (WTSMC) mice regardless of Klf4 genotype (P<0.001), but there was no difference in aneurysm growth in MFSSMC-Klf4Δ versus MFSSMC (P=0.884). Efficient SMC Klf4 deletion was confirmed via lineage-stratified genotyping, RNA in situ hybridization, and immunohistochemistry. Single-cell RNA sequencing of traced SMCs revealed a highly similar pattern of phenotype modulation marked by loss of contractile markers (eg, Myh11, Cnn1) and heightened expression of matrix genes (eg, Col1a1, Fn1) between Klf4 genotypes. Pseudotemporal quantitation of SMC dedifferentiation confirmed that Klf4 deletion did not alter the global extent of phenotype modulation, but reduced expression of 23 genes during this phenotype transition in MFSSMC-Klf4Δmice, including multiple chondrogenic genes expressed by only the most severely dedifferentiated SMCs (eg, Cytl1, Tnfrsf11b). CONCLUSIONS: Klf4 is not required to initiate SMC phenotype modulation in MFS aneurysm but may exert regulatory control over chondrogenic genes expressed in highly dedifferentiated SMCs.

Aortic Stress Activates an Adaptive Program in Thoracic Aortic Smooth Muscle Cells That Maintains Aortic Strength and Protects Against Aneurysm and Dissection in Mice.

BACKGROUND: When aortic cells are under stress, such as increased hemodynamic pressure, they adapt to the environment by modifying their functions, allowing the aorta to maintain its strength. To understand the regulation of this adaptive response, we examined transcriptomic and epigenomic programs in aortic smooth muscle cells (SMCs) during the adaptive response to AngII (angiotensin II) infusion and determined its importance in protecting against aortic aneurysm and dissection (AAD). METHODS: We performed single-cell RNA sequencing and single-cell sequencing assay for transposase-accessible chromatin (scATAC-seq) analyses in a mouse model of sporadic AAD induced by AngII infusion. We also examined the direct effects of YAP (yes-associated protein) on the SMC adaptive response in vitro. The role of YAP in AAD development was further evaluated in AngII-infused mice with SMC-specific Yap deletion. RESULTS: In wild-type mice, AngII infusion increased medial thickness in the thoracic aorta. Single-cell RNA sequencing analysis revealed an adaptive response in thoracic SMCs characterized by upregulated genes with roles in wound healing, elastin and collagen production, proliferation, migration, cytoskeleton organization, cell-matrix focal adhesion, and PI3K-PKB/Akt (phosphoinositide-3-kinase-protein kinase B/Akt) and TGF-ß (transforming growth factor beta) signaling. ScATAC-seq analysis showed increased chromatin accessibility at regulatory regions of adaptive genes and revealed the mechanical sensor YAP/transcriptional enhanced associate domains as a top candidate transcription complex driving the expression of these genes (eg, Lox, Col5a2, Tgfb2). In cultured human aortic SMCs, cyclic stretch activated YAP, which directly bound to adaptive gene regulatory regions (eg, Lox) and increased their transcript abundance. SMC-specific Yap deletion in mice compromised this adaptive response in SMCs, leading to an increased AAD incidence. CONCLUSIONS: Aortic stress triggers the systemic epigenetic induction of an adaptive response (eg, wound healing, proliferation, matrix organization) in thoracic aortic SMCs that depends on functional biomechanical signal transduction (eg, YAP signaling). Our study highlights the importance of the adaptive response in maintaining aortic homeostasis and preventing AAD in mice.

The Role of Long Noncoding RNAs in Vascular Smooth Muscle Cell Phenotype and the Pathogenesis of Cardiovascular and Cerebrovascular Aneurysms.

ABSTRACT: Aneurysms are localized dilations of blood vessels, which can expand to 50% of the original diameter. They are more common in cardiovascular and cerebrovascular vessels. Rupture is one of the most dangerous complications. The pathophysiology of aneurysms is complex and diverse, often associated with progressive vessel wall dysfunction resulting from vascular smooth muscle cell death and abnormal extracellular matrix synthesis and degradation. Multiple studies have shown that long noncoding RNAs (lncRNAs) play a significant role in the progression of cardiovascular and cerebrovascular diseases. Therefore, it is necessary to find and summarize them. LncRNAs control gene expression and disease progression by regulating target mRNA or miRNA and are biomarkers for the diagnosis and prognosis of aneurysmal cardiovascular and cerebrovascular diseases. This review explores the role, mechanism, and clinical value of lncRNAs in aneurysms, providing new insights for a deeper understanding of the pathogenesis of cardiovascular and cerebrovascular aneurysms.

Popliteal artery aneurysm ultrasound criteria for reporting characteristics.

INTRODUCTION: Duplex ultrasound (DUS) is the modality of choice for surveillance of popliteal artery aneurysms (PAAs). However, noninvasive vascular laboratories have no standard guidelines for reporting results. This study assessed reports of PAA DUS for inclusion of information pertinent to operative decision-making and timing of surveillance. METHODS: This study was a retrospective review of a multi-institutional repository that was queried for all patients with a PAA from 2008 to 2022 and confirmed via manual chart review. DUS reports were abstracted and images were individually annotated for features of interest including dimensions, flow abnormalities, and percent thrombus burden. RESULTS: A total of 166 PAAs in 130 patients had at least one DUS available for viewing. Postoperative surveillance of PAAs was performed at several intervals: the first at 30 months (IQR 3.7-113, n = 44), the second at 64 months (IQR 20-172, n = 31), and the third at 152 months (IQR 46-217, n = 16) after the operation. The largest diameter of operative PAAs (median 27.5 mm, IQR 21.8-38.0) was significantly greater than nonoperative PAAs (median 20.9 mm, IQR 16.7-27.3); p < 0.01. Fewer than 33 (21%) reports commented on patency of distal runoff. We calculated an average percent thrombus of 60% (IQR 19-81) in nonoperative PAAs, which is significantly smaller than 75% (IQR 58-89) in operative PAAs; p < 0.01. CONCLUSION: In this multi-institutional retrospective study, PAAs are often not followed at intervals recommended by the Society for Vascular Surgery guidelines and do not include all measurements necessary for clinical decision-making in the multi-institutional repository studied. There should be standardization of PAA DUS protocols performed by all noninvasive vascular laboratories to ensure completeness of PAA DUS images and inclusion of characteristics pertinent to clinical decision-making in radiology reports.

Targeting myeloperoxidase to stabilize unruptured aneurysm: an imaging-guided approach.

Inflammation plays a key role in pathogenesis and rupture of aneurysms. Non-invasively and dynamically monitoring aneurysm inflammation is critical. This study evaluated myeloperoxidase (MPO) as an imaging biomarker and therapeutic target for aneurysm inflammation using an elastase-induced rabbit model treated with or without 4-aminobenzoic acid hydrazide (ABAH), an irreversible inhibitor of MPO. Myeloperoxidase-sensitive magnetic resonance imaging (MRI) using Mn-TyrEDTA, a peroxidase activity-dependent contrast agent, revealed weak contrast enhancement in contralateral arteries and decreased contrast enhancement in aneurysm walls with ABAH treatment, indicating MPO activity decreased and inflammation mitigated. This was supported by reduced immune cell infiltration, matrix metalloproteinases (MMP-2 and - 9) activity, ROS production and arterial wall destruction on histology. Finally, the aneurysm expansion rate remained < 50% throughout the study in the ABAH(+) group, but increased gradually in the ABAH(-) group. Our results suggest that inhibition of MPO attenuated inflammation and expansion of experimental aneurysm and MPO-sensitive MRI showed promise as a noninvasive tool for monitoring aneurysm inflammation.

Pilot Study on Feasibility and Outcome of a Nerve-Preserving Aortoiliac Exposure Technique.

BACKGROUND: Dysfunctional ejaculation is a common complication following open aortoiliac aneurysm surgery. It may occur in 49-63% of patients and is caused by iatrogenic damage to the sympathetic lumbar splanchnic nerves and superior hypogastric plexus. A nerve-preserving operative technique based on a unilateral right-sided approach to the abdominal aorta, was implemented in clinical practice. The aim of this pilot study was to establish the safety and feasibility of the technique, and whether a sympathetic pathway and ejaculatory function was preserved. METHODS: Patients were asked to fill out questionnaires preoperatively, and 6 weeks, 6 months, and 9 months postoperatively. The International Index of Erectile Function, Cleveland Clinic Incontinence Score (CCIS), Patient assessment of constipation symptoms (Pac-Sym), and International Consultation on Incontinence Questionnaire on male lower urinary tract symptoms were used. Surgeons were asked to complete a technical feasibility questionnaire. RESULTS: Twenty-four patients undergoing aortoiliac aneurysm surgery were included. The nerve-sparing phase of the procedure added an average of 5-10 min of operating time and was technically feasible in twenty-two patients. No major complications occurred during nerve-sparing exposure. Fifteen of twenty-four patients were sexually active at some point throughout the study. No postoperative loss of ejaculation was seen in sexually active patients. CCIS, Pac-sym, International Index of Erectile Function, and Incontinence Questionnaire on male lower urinary tract symptoms scores remained similar throughout the study. CONCLUSIONS: Nerve-preserving aortoiliac reconstruction surgery is safe and feasible. Ejaculatory function is preserved. Given the low number of patients in the study, further research is needed to provide robust data.

The Management of Aneurysmal Degeneration of Lower Extremity Cryopreserved Saphenous Vein Bypass Grafts.

BACKGROUND: The use of cryopreserved saphenous veins (CSVs) for the treatment of lower extremity peripheral arterial disease is an attractive option when there is no available autogenous vein. Prior studies found CSVs are at risk for aneurysmal degeneration requiring reoperation. As the management of these complications and patient outcomes is not well described, the objective of this case series is to describe the open and endovascular management of degenerative CSVs at a tertiary community center. METHODS: All CSVs implanted for lower extremity bypass at our institution between 2001 and 2021 were retrospectively reviewed. All CSVs with evidence of aneurysmal change were included in this study. CSVs with evidence of active infection were excluded. The decision to intervene was left to the discretion of the operating surgeon. Demographic data, indications for the index operation, and details about subsequent interventions for degenerative CSVs were recorded. Study end points included limb salvage and continued patency. Demographic data, indications for the index operation, and details about subsequent interventions for degenerative CSVs were recorded. RESULTS: Seventeen bypasses were identified to have aneurysmal degeneration in 13 patients in the absence of infection between 2001 and 2021. Nine of the 13 patients were male, and the average age and body mass index during the index procedure were 72 and 28, respectively. Indications for the index bypass included acute limb ischemia (9), popliteal aneurysm (2), and chronic limb threatening ischemia with Rutherford's class IV (5) and V (1). The mean time between the index procedure and first graft revision due to aneurysmal changes was 4 years. Most of the aneurysms did not occur at the site of anastomosis with 13 occurring in the body of the graft. Thirteen grafts were managed with open surgery and 3 were managed with endovascular techniques. All endovascular repairs were managed via covered stenting. Patients were followed for an average duration of 7 years from the initial bypass and 2 years from their last aneurysmal repair. Limb salvage in this cohort was 87% with 2 limbs requiring amputation, all of whom underwent open reconstruction. The mortality rate in this series was 54% and no patients died due to complications from their graft. Continued patency on Kaplan Meier survival curve analysis was 79% at 6 months, 65% at 1 year, 54% at 3 years, and 27% at 5 years. CONCLUSIONS: In our experience, aneurysmal degeneration of CSV grafts was mostly managed with standard open surgical techniques, although endovascular therapy also proved acceptable. Limb salvage rates and continued patency of repair at 1 year in this cohort were acceptable. This case series highlights the importance of diligent surveillance for patients with CSVs.

Splenic Artery Infectious Aneurysms in Infective Endocarditis - An Observational Study and Comprehensive Literature Review.

BACKGROUND: To determine the prevalence, the clinical and radiological features, associated factors, treatment, and outcome of splenic artery aneurysms (SAAs) in infective endocarditis (IE). METHODS: We retrospectively reviewed 474 consecutive patients admitted to our institution with definite IE (2005-2020). RESULTS: Six patients had SAAs (1.3%; 3 women; mean age: 50 years). In all cases, the diagnosis was obtained by abdominal computed tomography angiography (CTA). SAAs-IE were solitary and saccular with a mean diameter of 30 mm (range: 10-90 mm). SAAs-IE were intrasplenic (n = 4) or hilar (n = 2). Streptococcus spp. were the predominant organisms (n = 4). In all cases, a left-sided native valve was involved (aortic, n = 3; mitral, n = 2; mitral-aortic, n = 1). SAAs were silent in half patients and were revealed by abdominal pain (n = 2) and by the resurgence of fever after cardiac surgery (n = 1). All patients underwent emergent valve replacement. One patient died within 24 hr from multiorgan failure. For the others, uneventful coil embolization was performed in 4 patients after valve replacement (3 diagnosed early and 1 at 8 weeks). In the remaining patient, SAA-IE diagnosed at abdominal CTA at day 16, with complete resolution under appropriate antibiotherapy alone. CONCLUSIONS: SAAs-IE are a rare occurrence that may be clinically silent. SAAs-IE can be intrasplenic or hilar in location. Endovascular treatment in this context was safe. According to current guidelines, radiologic screening by abdominal CTA allowed the detection of silent SAAs which could be managed by endovascular treatment to prevent rupture. The delayed formation of these SAAs could justify a CTA control at the end of antibiotherapy.

Prosthetic Conduit Use Does Not Adversely Impact Outcomes after Open Repair of Popliteal Artery Aneurysms.

BACKGROUND: Single segment, greater saphenous vein (GSV) conduit is considered the optimal bypass conduit among patients undergoing bypass surgery for peripheral artery disease (PAD). While this data has been extrapolated to patients undergoing bypass for popliteal artery aneurysms (PAAs), the pathophysiology of PAA is inherently different when compared to PAD, and the impact of conduit type on long-term outcomes after open repair of PAA remains unclear. METHODS: A multicenter database of five regional hospitals was retrospectively reviewed for all patients with PAA undergoing open surgical repair. Data were collected on demographic information, operative details, medications, and postoperative outcomes. Kaplan-Meier curves were used to compare freedom from major adverse limb events (MALE) following GSV versus prosthetic bypass. Cox proportional hazards model was used to identify patient-level characteristics associated with MALE, which was defined as major ipsilateral limb amputation or reintervention for graft patency. RESULTS: From 1999 to 2020, a total of 101 patients with PAA underwent open exclusion and bypass surgery. Median follow-up period was 4.2 years (interquartile range, 1.3-7.4 years), and complete data were available for 99 (98.0%) patients. The majority of patients were male (99.0%) and Caucasian (93.9%). Only 11.1% of procedures were emergent, with the remainder (88.9%) being elective. All patients underwent medial exposure with a below-knee popliteal bypass target (100%). Bypass conduits included GSV (69.7%), prosthetic conduit (28.3%), and 2 (2.0%) alternative conduits (one spliced arm vein, one cryopreserved vein). Patients undergoing prosthetic bypass were older (72 vs. 66 years, P = 0.001) and had similar rates of medical comorbidities. Compared with the GSV group, patients with prosthetic conduits were more frequently placed on postoperative anticoagulation (60.7% vs. 23.2%, P < 0.001). Conduit type did not impact postoperative complication rates (P = NS each). MALE rates were low overall (19.2% at 2 years), and similar when stratified by conduit type (log rank P = 0.47). On multivariable analysis, emergent bypass was associated with MALE (hazard ratio [HR] 5.73, 95% confidence interval [CI] 2.07-15.85, P < 0.001). Prosthetic conduit usage (HR 1.00, 95% CI, 0.40-2.51, P = 0.99) and postoperative anticoagulation (HR 1.02, 95% CI 0.42-2.50, P = 0.97) were not associated with MALE. CONCLUSIONS: Open repair of PAA is associated with excellent long-term outcomes. Prosthetic bypass is a comparable alternative to autogenous conduit for below-knee popliteal bypass targets, and lack of suitable GSV should not prohibit open surgical repair when indicated.

Multimodal Study of the Superior Mesenteric Artery Wall.

BACKGROUND: To quantitatively analyze histological and fiber structure of the superior mesenteric artery (SMA) wall and to further explore the possible relationship between the architecture and histology changes of vessel wall and the occurrence of related diseases. METHODS: Histological and fiber structure analysis were performed on SMA specimens obtained from 22 cadavers. The SMA specimens were divided into initial, curved, and distal segments, and each segment was separated into the anterior and posterior walls. RESULTS: From the initial to the curved to the distal segment, the ratio of elastin decreased (31.4% ± 6.0%, 21.1% ± 5.8%, 18.6% ± 4.7%, respectively; P < 0.001), whereas the ratio of smooth muscle actin (24.5% ± 8.7%, 30.5% ± 6.8%, 36.1% ± 7.3%, respectively; P < 0.001) increased. Elastic fiber longitudinal amplitude of angular undulation was highest in the initial segment [7° (3.25°, 15°)] and lowest in the curved segment [2° (1°, 5°)]. In SMA curved segment, the anterior wall, when compared with the posterior wall, demonstrated a lower ratio of elastin (19.0% ± 5.8% vs. 23.3% ± 5.0%; P = 0.010) and collagen (41.4% ± 12.3% vs. 49.0% ± 10.2%; P = 0.032), a lower elastic fiber longitudinal amplitude of angular undulation [1° (1°, 5°) vs. 3° (2°, 5.25°); P = 0.013], a lower average fiber diameter (8.06 ± 0.36 pixels vs. 8.45 ± 0.50 pixels; P = 0.005), and a lower average segment length (17.96 ± 1.59 pixels vs. 20.05 ± 2.33 pixels; P = 0.001). CONCLUSIONS: SMA wall structure varies along the circumferential and axial directions, the presence of dense undulated elastic fiber protects the SMA initial segment of from dissection and aneurysm, but highly cross-linked collagen fiber here increases the likelihood of plaque formation. In the anterior wall of the curved segment, lower elastin and collagen content, lower elastic fiber undulation, and higher degree of collagen fiber cross-linking leads to the occurrence of SMA dissection and aneurysm. In the distal segment, high levels of vascular smooth muscle cells and bundles of long collagen fiber offer protection against the development of SMA-related diseases.

Impact of arteriovenous fistula aneurysms on a UK dialysis populations' perception of vascular access.

BACKGROUND: Clinically-oriented outcome measures are increasingly being recognized as lacking in consideration of factors important to patients. There is an emerging move of guideline bodies advocating a more patient-centred approach. Aneurysms in autogenous arteriovenous fistula (AVF) can be considered unsightly and a constant reminder for patients of their dependence on dialysis. However, their impact on patient's perception has not previously been reported. METHODS: Between April 2017-18, the Vascular Access Questionnaire (VAQ) was administered to prevalent haemodialysis patients across ten dialysis units via structured interviews, as part of a quality improvement project. Data for the subgroup of patients with aneurysmal AVF (categorised as per classification by Valenti et al.), were retrospectively evaluated and compared to the wider cohort. RESULTS: Data were collected for 539 patients (median age: 66 years; 59% male), of whom 195 (36%) had aneurysmal AVF, with Type 2 morphology (cannulation site) being the most common (75%). Duration of AVF was found to be significantly associated with aneurysmal development, with estimated likelihoods of 11%, 43% and 61% after one, five and ten years, respectively. Interestingly, patients with diabetes had a significantly lower prevalence of aneurysmal development than those that were non-diabetic (25% vs. 43%, p < 0.001). Overall VAQ scores were not found to differ significantly by aneurysm status (p = 0.816) or across morphology types (p = 0.277). However, patients with aneurysmal AVF were significantly more concerned with the appearance of their AVF (p < 0.001) than the wider cohort. Despite this, patients with aneurysmal AVF gave significantly higher scores for satisfaction and ease of use and lower scores for bruising and clotting (p < 0.05). CONCLUSIONS: Aneurysmal AVF are often cited as an important factor by patients for not proceeding with fistula formation. In this evaluation of patient reported experiences, those with aneurysmal AVF reported high satisfaction levels. This may help clinicians highlight positive patient reported outcomes of aneurysmal AVF during preprocedural consent processes.