Single-Nucleus Transcriptomic Atlas of Human Pericoronary Epicardial Adipose Tissue in Normal and Pathological Conditions.

BACKGROUND: Pericoronary epicardial adipose tissue (EAT) is a unique visceral fat depot that surrounds the adventitia of the coronary arteries without any anatomic barrier. Clinical studies have demonstrated the association between EAT volume and increased risks for coronary artery disease (CAD). However, the cellular and molecular mechanisms underlying the association remain elusive. METHODS: We performed single-nucleus RNA sequencing on pericoronary EAT samples collected from 3 groups of subjects: patients undergoing coronary bypass surgery for severe CAD (n=8), patients with CAD with concomitant type 2 diabetes (n=8), and patients with valvular diseases but without concomitant CAD and type 2 diabetes as the control group (n=8). Comparative analyses were performed among groups, including cellular compositional analysis, cell type-resolved transcriptomic changes, gene coexpression network analysis, and intercellular communication analysis. Immunofluorescence staining was performed to confirm the presence of CAD-associated subclusters. RESULTS: Unsupervised clustering of 73 386 nuclei identified 15 clusters, encompassing all known cell types in the adipose tissue. Distinct subpopulations were identified within primary cell types, including adipocytes, adipose stem and progenitor cells, and macrophages. CD83high macrophages and FOSBhigh adipocytes were significantly expanded in CAD. In comparison to normal controls, both disease groups exhibited dysregulated pathways and altered secretome in the primary cell types. Nevertheless, minimal differences were noted between the disease groups in terms of cellular composition and transcriptome. In addition, our data highlight a potential interplay between dysregulated circadian clock and altered physiological functions in adipocytes of pericoronary EAT. ANXA1 (annexin A1) and SEMA3B (semaphorin 3B) were identified as important adipokines potentially involved in functional changes of pericoronary EAT and CAD pathogenesis. CONCLUSIONS: We built a complete single-nucleus transcriptomic atlas of human pericoronary EAT in normal and diseased conditions of CAD. Our study lays the foundation for developing novel therapeutic strategies for treating CAD by targeting and modifying pericoronary EAT functions.

Efficacy of Endovascular Repair Using Single Left Common Carotid Artery Stent Combined with Castor Single-Branched Stent-Graft in the Treatment of Regional Diseases of Zone 2 of the Aorta.

PURPOSE: To observe the short-term efficacy of thoracic endovascular aortic repair (TEVAR) using a single left common carotid artery chimney stent combined with a Castor single-branched stent-graft (SC-TEVAR) in the treatment of zone 2 (Z2) aortic diseases. MATERIALS AND METHODS: To conduct a retrospective analysis of 20 patients with Z2 aortic diseases who were treated in our department from June 2021 to April 2022. The lesions included true aortic degenerative aneurysms with diameter ≥5.0 cm and penetrating aortic ulcers with depth >1.0 cm or basal width >2.0 cm. All 20 patients accepted the SC-TEVAR treatment, which was a new hybrid method to assure the flow of the left common carotid artery (LCCA) and left subclavian artery (LSA). This method was defined as a concomitant chimney stent for LCCA and a Castor single-branched stent graft for the aorta and LSA. The baseline data and intraoperative data were collected to evaluate the safety and efficacy of this method. The patency of the target blood vessel and any associated complications were evaluated at 1 and 6 months postoperatively, to analyze the safety and efficacy of this new method. RESULTS: After discharge from the hospital, all patients were followed up by a specific follow-up team. At 6 monthly follow-up period, there were no cardiac events, stroke, hemiplegia, type I endoleak, type II endoleak, proximal stent graft-induced new entries, distal stent graft-induced new entries, wound infection, or bleeding. Only 1 patient developed an inguinal wound hematoma and got conservative treatment. Importantly, no patients developed stenosis or occlusion of the LCCA or LSA. The patency of branched arteries was 100%. The technical success rate was 90%. CONCLUSION: SC-TEVAR appears to be a new and relatively simple, safe, and effective treatment for Z2 aortic diseases. CLINICAL IMPACT: This was a single-center retrospective cohort study. A total of 20 patients with zone 2 aortic diseases accepted a new hybrid surgical method named SC-TEVAR. This method was not complicated and could be finished with only 3 peripheral artery exposure. The result showed no mortality, 100% patency of the branch artery, and 90% of technical success in 6 months of follow-up time. SC-TEVAR showed a satisfactory result in this retrospective study and could be promoted as an easy method to treat zone 2 aortic diseases.

Timing of Intervention and Long-Term Outcomes of Type B Aortic Intramural Hematoma with Intimal Disruption at Admission.

BACKGROUND: To compare the 30-day and long-term outcomes between patients with concomitant type B intramural hematoma and intimal disruption upon admission who underwent endovascular repair in the acute or subacute phases. METHODS: Data were extracted from January 1, 2010, to December 31, 2019. Logistic regression and Cox regression were performed to evaluate the impact of timing of intervention on 30-day and long-term outcomes, respectively. RESULTS: The study included 241 patients, among which 159 were in the acute group. No significant difference was observed in 30-day mortality (0.6% vs. 0%, P = 1), 30-day complication rate (2.5% vs. 1.2%, P = 0.664), long-term all-cause mortality (10.7% vs. 7.3%, P = 0.540), and aortic reintervention rate (2.5% vs. 2.4%, P = 1) between the acute and subacute group. In multivariable analysis, the timing of intervention was not associated with 30-day mortality (odds ratio (OR) = 0, 95% confidence interval CI: 0-Inf, P = 0.999), 30-day complication (OR = 0.30, 95% CI: 0.02-3.77, P = 0.348), long-term mortality (hazard ratio = 0.56, 95% CI: 0.20-1.61, P = 0.283), and aortic reintervention (OR = 0.97, 95% CI: 0.15-6.08, P = 0.970). CONCLUSIONS: For patients with concomitant type B intramural hematoma and intimal disruption upon admission, there is no significant difference in 30-day and long-term outcomes between those who undergo endovascular treatment in the acute or subacute phase.

A Multicenter Study of the Mid-term Outcomes of Patients with Uncomplicated Type B Aortic Dissection After Distal Porous Talos Stent-Graft Implantation.

BACKGROUND: The Talos stent-graft has extended length to improve aortic remodeling, and distal porous design to decrease the rate of spinal cord ischemia (SCI). This study retrospectively analyzed its mid-term outcomes for uncomplicated type B aortic dissection in a multicenter study. METHODS: The primary safety end point was 30-day major adverse events, including all-cause mortality, dissection-related mortality, conversion to open surgery, and device-related adverse events. The primary efficacy end point was treatment success at 12 months postoperation, defined as no technical failure or secondary dissection-related reintervention. The survival status of the patients was visualized using the Kaplan-Meier curve. Aortic growth was assessed at 4 levels, and SCI was evaluated at 12 months. RESULTS: 113 patients participated with a mean age of 54.4 (11.1) years and 71.7% (81/113) were male. The 30-day mortality was 0.9% (1/113), no conversions to open surgery or device-related adverse events were recorded. The 12-month treatment success rate was 99.1% (112/113), with no dissection-related reinterventions. There was no spinal cord or visceral ischemia at 12 months. At a median of 34 months follow-up, 9 further deaths were recorded and the 3-year survival rate was 91.7%. The percentage of aortic growth was 1.8% (2/111) at the tracheal bifurcation, 3.6% (4/111) below the left atrium, 6.0% (5/83) above the celiac artery, and 12.1% (9/74) below the lower renal artery. The total thrombosis rate of the false lumen at the stented segment was 80.5% (91/113). CONCLUSIONS: The results showed satisfactory results of Talos stent-graft in terms of safety and efficacy. More data are needed to confirm the long-term performance.

Surgical treatment patterns and clinical outcomes of type B aortic dissection involving the aortic arch.

OBJECTIVE: In the present report, we have described the outcomes of endovascular repair, hybrid arch repair, and open surgical repair for type B dissection involving the aortic arch (B1-2, D). METHODS: Cases of endovascular repair, hybrid arch repair, and open surgical repair performed between January 2015 and December 2019 for aortic dissection designated as B1-2, D by the Society for Vascular Surgery/Society of Thoracic Surgeons classification were retrospectively analyzed. The primary end point was all-cause mortality at follow-up. The secondary end points included early mortality, early morbidities, and aortic-related late events. Kaplan-Meier curves were created to analyze survival from all-cause mortality and freedom from aortic-related late events in the endovascular, hybrid, and open groups. Propensity score matching and stratification (stratified by proximal dissection extension: B1, D and B2, D) were performed as sensitivity analyses to compare the outcomes among the three treatment patterns after controlling for major confounders. RESULTS: The present study included 151 patients (men, 79.5%; mean age, 47.3 ± 10.5 years), with 72 (47.7%) in the endovascular group, 46 (30.5%) in the hybrid group, and 33 (21.8%) in the open group. No significant difference was noted in early mortality between the endovascular, hybrid, and open groups (1.4% vs 2.2% vs 3.0%; P = .791). The incidence of early endoleak was significantly greater (33.3% vs 13.0% vs 6.1%; P = .002) and the incidence of renal function deterioration was less (4.2% vs 26.1% vs 24.2%; P = .001) after endovascular repair vs hybrid arch repair and open surgery. After a median follow-up of 40.0 months (range, 0-84.0 months), no significant differences were found in all-cause mortality (5.6% vs 4.3% vs 3.0%; P = 1.0), aortic-related late events (16.7% vs 15.2% vs 12.1%; P = .834), or late endoleak (9.7% vs 4.3% vs 6.1%; P = .630) after endovascular, hybrid, and open surgery. The propensity score matching and stratification analyses displayed consistent outcomes for early mortality, all-cause mortality, and aortic-related late events among the three groups. CONCLUSIONS: The mid- to long-term outcomes after endovascular repair, hybrid arch repair, and open surgical repair for type B dissection involving the aortic arch (B1-2, D) were favorable and comparable in selected patients. Extensive experience and multidisciplinary teamwork are prerequisites for individualized strategies for repair of B1-2, D.

Endovascular aortic arch repair with chimney technique for pseudoaneurysm.

BACKGROUND: Aortic pseudoaneurysm is a life-threatening clinical condition, and thoracic endovascular aortic repair (TEVAR) has been reported to have a relatively satisfactory effect in aortic pathologies. We summarized our single-centre experience using chimney TEVAR for aortic arch pseudoaneurysms with inadequate landing zones. METHODS: A retrospective study was conducted from October 2015 to August 2020, 32 patients with aortic arch pseudoaneurysms underwent chimney TEVAR to exclude an aortic lesion and reconstruct the supra-aortic branches, including 3 innominate artery, 12 left common carotid arteries and 29 left subclavian arteries. Follow-up computed tomography was suggested before discharge; at 3, 6, 12 months and yearly thereafter. RESULTS: The median age of 32 patients was 68.0 years (range, 28-81) with the mean max diameter of aneurysm of 47.9 ± 12.0 mm. Forty-four related supra-aortic branches were well preserved, and the technical success rate was 100%. The Type Ia endoleaks occurred in 3 (9%) patients. Two patients were lost to follow-up and 4 patients died during the follow-up period. The mean follow-up times was 46.5 ± 14.3 months. One patient died due to acute myocardial infarction just 10 days after chimney TEVAR and the other 3 patients passed away at 1.5 months, 20 months, and 31 months with non-aortic reasons. The 4.5-year survival estimate was 84.4%. The primary patency rate of the target supra-arch branch vessels was 97.7% (43/44), and no other aorta-related reinterventions and severe complications occurred. CONCLUSION: For aortic arch pseudoaneurysms with inadequate landing zones for TEVAR, the chimney technique seems to be feasible, with acceptable mid-term outcomes, and it could serve as an alternative minimally invasive approach to extend the landing zone. Slow flow type Ia endoleak could be treated conservatively after chimney TEVAR. Additional experience is needed, and the long-term durability of chimney TEVAR requires further follow-up.

EphrinB2 promotes the human aortic smooth muscle cell growth and migration via mediating F-actin remodeling.

OBJECTIVES: To evaluate the potential effect of EphrinB2 in human thoracic aortic dissection (TAD) and to illustrate the mechanisms governing the role of EphrinB2 in the growth of human aortic smooth muscle cells (HASMC). METHODS: In the study, EphrinB2 expression was investigated by qRT-PCR and immunohistochemistry in 12 pairs of TAD and adjacent human tissues. HASMCs were used for in vitro experiments. Next, EphrinB2 overexpression and depletion in HASMCs were established by EphrinB2-overexpressing vectors and small interfering RNA, respectively. The transfection efficiency was evaluated by qRT-PCR and Western blot. The effects of overexpression and depletion of EphrinB2 on cell proliferation, migration, and invasion were tested in vitro. Cell Counting Kit-8, flow cytometry and transwell migration/invasion, and wound healing assay were used to explore the function of EphrinB2 on HASMC cell lines. The relationship between EphrinB2 and F-actin was assessed by Western blot, immunofluorescence, and Co-IP. RESULTS: We found that EphrinB2 was a prognostic biomarker of TAD patients. Moreover, EphrinB2 expression negatively correlated to aortic dissection tissues, and disease incidence of males, suggesting that EphrinB2 might act as a TAD suppressor by promoting proliferation or decreasing apoptosis in HASMC. Next, over-expression of EphrinB2 in HASMC lines drove cell proliferation, migration, and invasion, and inhibited apoptosis while knockdown EphrinB2 showed the opposite phenomenon, respectively. Furthermore, the level of F-actin in mRNA, protein, and distribution in HASMC cell lines highly matched with the expression of EphrinB2, which indicated that EphrinB2 could mediate the HASMC cytoskeleton via inducing F-actin. CONCLUSIONS: In conclusion, our results first provided the pivotal role of EphrinB2 in HASMC proliferation initiated by mediating F-actin and demonstrated a prognostic biomarker and the potential targets for therapy to prevent thoracic aortic dissection.

A systematic study of mosaicism in heritable thoracic aortic aneurysm and dissection.

Mosaicisms are often overlooked in routine molecular diagnosis. Although not common, they are of great significance for accurate diagnosis and genetic counseling. In this study, we systematically evaluated the frequency of mosaicisms in both asymptomatic parents and affected patients with thoracic aortic aneurysm and dissection (TAAD). Next-generation sequencing (NGS) data from 1085 patients was reanalyzed with a more lenient allele frequency to detect potential mosaic variants. In addition, parental mosaicisms were investigated in 80 TAAD families. Finally, a total of six mosaic variants were detected in our cohort. Three of them were identified in symptomatic patients and three were in asymptomatic parents. Notably, a low-level mosaic variant in TGFB2 was detected combined with a causative FBN1 variant in patient AD2001, which might partially explain the clinical heterogeneity in his family. Our study hinted that it is necessary and feasible to implement mosaicism analysis in routine molecular diagnosis.

HTAADVar: Aggregation and fully automated clinical interpretation of genetic variants in heritable thoracic aortic aneurysm and dissection.

PURPOSE: Early detection and pathogenicity interpretation of disease-associated variants are crucial but challenging in molecular diagnosis, especially for insidious and life-threatening diseases, such as heritable thoracic aortic aneurysm and dissection (HTAAD). In this study, we developed HTAADVar, an unbiased and fully automated system for the molecular diagnosis of HTAAD. METHODS: We developed HTAADVar (http://htaadvar.fwgenetics.org) under the American College of Medical Genetics and Genomics/Association for Molecular Pathology framework, with optimizations based on disease- and gene-specific knowledge, expert panel recommendations, and variant observations. HTAADVar provides variant interpretation with a self-built database through the web server and the stand-alone programs. RESULTS: We constructed an expert-reviewed database by integrating 4373 variants in HTAAD genes, with comprehensive metadata curated from 697 publications and an in-house study of 790 patients. We further developed an interpretation system to assess variants automatically. Notably, HTAADVar showed a multifold increase in performance compared with public tools, reaching a sensitivity of 92.64% and specificity of 70.83%. The molecular diagnostic yield of HTAADVar among 790 patients (42.03%) also matched the clinical data, independently demonstrating its good performance in clinical application. CONCLUSION: HTAADVar represents the first fully automated system for accurate variant interpretation for HTAAD. The framework of HTAADVar could also be generalized for the molecular diagnosis of other genetic diseases.

Endovascular repair of acute zone 0 intramural hematoma with most proximal tear or ulcer-like projection in the descending aorta.

OBJECTIVE: In the present study, we have reported the midterm results of endovascular repair of acute zone 0 intramural hematomas (IMHs) with the most proximal tear or ulcer-like projection (ULP) in the descending aorta. METHODS: Data from patients with acute zone 0 IMH with the most proximal tear or ULP in the descending aorta from January 1, 2010, to December 31, 2019, were retrospectively reviewed. We performed Kaplan-Meier curves to calculate the intervention-free survival and survival after endovascular or open surgical repair. We used propensity score matching to compare the outcomes of endovascular and open surgical repair. RESULTS: The present study included 99 patients. Of the 99 patients, 34 had initially received medical treatment. The 0.5-, 1-, and 3-year intervention-free survival rates for the 34 patients were 23.5%, 17.6%, and 14.7%, respectively. Of the 99 patients, 51 had undergone endovascular therapy, 27 after initial medical treatment. Most of these 51 patients had had a maximal diameter of the ascending aorta of <50 mm and a maximal diameter of IMH in the ascending aorta of <10 mm. The 1-, 3-, and 5-year survival rate for the endovascular group was 98.0%. Finally, 42 patients had undergone open surgery (3 after medical treatment), and the 1-, 3-, 5-year survival rates were all 92.9%. After propensity score matching, no statistically significant difference was found in the 30-day and follow-up mortality. However, endovascular repair was associated with a shorter operation time (69 vs 314 minutes; P < .001), shorter length of intensive care unit stay (24 vs 70 hours; P = .001), and shorter length of hospital stay (7 vs 12 days; P = .011). CONCLUSIONS: For patients with acute zone 0 IMH and the most proximal tear or ULP in the descending aorta, in addition to open surgery, endovascular repair is an option if the maximal diameter of the ascending aorta is <50 mm and the maximal diameter of the IMH in the ascending aorta is <10 mm.

Survival and aortic remodeling outcomes in patients with type B aortic intramural hematoma in the endovascular era: An observational cohort study.

BACKGROUND: The late outcomes of patients with type B aortic intramural hematoma (IMH) receiving medical treatment (MT) have varied greatly. Whether endovascular stent graft placement in the descending aorta will provide beneficial effects for patients with type B IMH has remained uncertain. We have presented the survival and aortic remodeling outcomes for patients with type B IMH stratified by the treatment received (MT vs endovascular treatment [ET]). METHODS: The participants were consecutively recruited from September 2010 to August 2017 from an institutional registry for type B IMH at Fuwai Hospital. The cohort was divided into two subgroups according to their treatment (MT vs ET). Kaplan-Meier estimations and propensity score-matched analysis were used to evaluate the outcomes after MT and ET. RESULTS: The cohort included 347 patients with type B IMH (189 in the MT subgroup and 158 in the ET subgroup). During hospitalization, two patients (1.1%) in the MT subgroup and one patient (0.6%) in the ET subgroup had died. During follow-up (median, 3.4 years; interquartile range, 2.3-4.5 years; total patient-years, 1191.1), 36 patients had died of all causes. The cumulative probability of death was 0.03 per patient-year. The Kaplan-Meier estimated survival rates at 5 years were higher for the ET subgroup (94.9%) than for the MT subgroup (84.2%; P = .001). Cox regression analysis showed that ET was associated with a lower risk of death (hazard ratio, 0.32; 95% confidence interval, 0.15-0.69; P = .004). Follow-up computed tomography scans were completed for 244 patients. The incidence of IMH resolution in the ET subgroup (53.5%) was higher than that in the MT subgroup (33.3%; P = .003). CONCLUSIONS: The present findings revealed different survival and aortic remodeling outcomes according to MT vs ET for consecutive patients with type B IMH. The survival rate was improved for the patients in the ET subgroup compared with that for the MT subgroup. Also, late progression to aortic dissection was less frequent in the ET subgroup.

Early Results of a Novel Gutter-Free Chimney Stent-Graft System to Treat Aortic Arch Dissection: Single-Center Data from a Prospective Clinical Trial.

AIMS: Discuss the clinical value, technique characteristics, and early follow-up results of a newly designed gutter-free chimney stent-graft system for aortic arch pathology. METHODS AND RESULTS: About 13 patients with aortic arch dissection were enrolled in a clinical trial testing a novel gutter-free stent-graft between February 2019 and December 2020. All 13 patients were male, age 52.6±10.4 years. The implantation time was 14.0±6.9 minutes; total procedure time was 89.5±19.8 minutes. The volume of contrast was 79.6±7.2 ml. And 15 aortic stent-grafts were implanted, and all 13 patients had chimney branch stent-grafts implanted into the left subclavian artery (LSA). There were 3 (23.1%) cases of immediate type Ιa endoleak after thoracic endovascular aortic repair (TEVAR), and 7.7% type Ιa endoleaks occurred in delayed fashion. Survival at 2 years was 100%, and the 2-year patency of chimney stent-grafts was 100%. CONCLUSIONS: This study reports early success with good freedom from endoleak using a novel stent-graft designed for chimney TEVAR to treat aortic arch dissection. Postoperative survival and patency of the branch stent-grafts were excellent. Additional data from this multicenter clinical trial will be forthcoming.

Similar Midterm Clinical Outcomes and Aortic Remodeling in Bifurcated and Straight Aortic Stent Grafts Used to Treat Isolated Abdominal Aortic Dissection.

PURPOSE: The optimal treatment for isolated abdominal aortic dissection (IAAD) is currently unknown. We compared the effects of straight and bifurcated aortic stent grafts on postoperative aortic remodeling in patients with IAAD. MATERIALS AND METHODS: From February 2012 to December 2019, 57 patients with IAAD were treated using endovascular methods, including either a bifurcated or a straight aortic stent graft. The clinical features, risk factors, computed tomography angiograms, midterm follow-up results, and aortic remodeling of these patients were reviewed and analyzed. RESULTS: In total, 44 (77%) patients were treated with a bifurcated graft and 13 (23%) patients were treated with a straight graft. Patients treated with straight grafts had fewer common iliac arteries involved (38% vs 73%, p=0.023), the dissection length was shorter (76.3 ± 40.0 vs 116.2 ± 56.7 mm, p=0.011), and the preoperative aortic diameter (26.0 ± 5.6 vs 35.2 ± 12.1 mm) and the false lumen diameter (13.1 ± 5.2 vs 21.2 ± 11.3 mm) were smaller. During the procedure, there were 3 (5.3%) type I endoleaks, 1 (1.8%) surgical conversion and 1 (1.8%) partial renal artery coverage without perioperative mortality. Patients with straight grafts had shorter operative time (96.5 ± 24.4 vs 144.2 ± 49.0 minutes, p<0.0001). The median follow-up duration was 37.6 ± 21.0 (range = 3-89) months with 1 (1.8%) aortic-related death. Type A aortic dissection occurred in 1 (1.8%) patient. New descending aortic dissection occurred in 3 (5.3%) patients, and 1 patient advanced to type A aortic dissection 3 months later. Two (3.5%) patients had limb occlusion. There was no significant difference in aortic remodeling, survival, and freedom from all adverse events between the 2 treatment strategies. CONCLUSIONS: Endovascular treatment provides a safe, minimally invasive treatment for IAAD in midterm follow-up. Compression of the true lumen at the aortic bifurcation is the main concern after treatment with a bifurcated graft. Straight grafts are an excellent alternative for some patients, with the benefit of reduced procedural time, effective aortic remodeling, and excellent clinical prognosis. More experience is needed to offer clear recommendations for making treatment decisions as well as determine long-term effectiveness and durability.

Midterm Results of Retrograde In Situ Needle Fenestration During Thoracic Endovascular Aortic Repair of Aortic Arch Pathologies.

PURPOSE: To evaluate the safety and feasibility of the in situ needle fenestration (ISNF) technique for reconstruction of the left subclavian artery (LSA) during thoracic endovascular aortic repair (TEVAR) of complicated aortic arch pathologies. MATERIALS AND METHODS: A retrospective review was conducted from January 2014 to December 2019 of 50 patients (mean age 60.2±11.1; 45 men) who underwent ISNF to revascularize the LSA during TEVAR. Twenty-one of the patients also required revascularization of the left common carotid artery (LCCA; n=19) and innominate artery (IA; n=2) using physician-modified in vitro fenestration. Overall, 73 supra-aortic branches were targeted for revascularization. RESULTS: ISNF was successful in 48 patients (96%); one LSA could not be stented and a tortuous LSA prevented the needle from fenestrating the graft. No perioperative major adverse event occurred. There were no type I and 4 type III endoleaks (8%), 3 of which occurred among the first 20 cases. Types II and IV endoleaks were found in 3 (6%) and 6 (12%) cases, respectively; all disappeared during a median follow-up of 15 months (range 3-66). One death (2%) occurred within 12 months due to cerebral hemorrhage. Two patients (4%) required open reinterventions at 6 and 62 months. CONCLUSION: ISNF for revascularization of the LSA during TEVAR seems to be feasible with acceptable midterm outcomes. The learning curve and evolving patient selection criteria affected technical success, complications, and the need for reinterventions. Long-term durability requires further evaluation.

WITHDRAWN: Endovascular Repair with Stent-Graft of Symptomatic Tuberculous Aortic Pseudoaneurysm

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Identification of gross deletions in FBN1 gene by MLPA.

BACKGROUND: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by mutations in the FBN1 gene. Approximately 90% of classic MFS patients have a FBN1 mutation that can be identified by single-gene sequencing or gene-panel sequencing targeting FBN1. However, a small proportion of MFS patients carry a large genomic deletion in FBN1, which cannot be detected by routine sequencing. Here, we performed an MLPA (multiplex ligation-dependent probe amplification) test to detect large deletions and/or duplications in FBN1 and TGFBR2 in 115 unrelated Chinese patients with suspected MFS or early-onset aneurysm/dissection. RESULTS: Five novel large deletions encompassing a single exon or multiple exons in the FBN1 gene were characterized in five unrelated patients, of which four were proven by Sanger sequencing, and the breakpoints were identified. Three of them met the revised Ghent criteria when genetic results were not available, and the other two patients were highly suspected and diagnosed with MFS until the FBN1 deletions were identified. CONCLUSIONS: Our finding expands the mutation spectrum of large FBN1 deletions and emphasizes the importance of screening for large FBN1 deletions in clinical genetic testing, especially for those with classic Marfan phenotype.

Evaluation of associations between outflow morphology and rupture risk of abdominal aortic aneurysms.

PURPOSE: This study aimed to evaluate the association between the outflow morphology and abdominal aortic aneurysm (AAA) rupture risk, to find risk factors for future prediction models. MATERIALS AND METHODS: We retrospectively analyzed 46 patients with ruptured AAAs and 46 patients with stable AAAs using a 1:1 match for sex, age, and maximum aneurysm diameter. The chi-square test, paired t-test, and Wilcoxon signed-rank test were used to compare variables. Logistic regression was performed to evaluate variables potentially associated with AAA rupture. Receiver operating characteristic curve analysis and the area under the curve (AUC) were used to assess the regression models. RESULTS: Ruptured AAAs had a shorter proximal aortic neck (median (interquartile range, IQR): 24.0 (9.4-34.2) mm vs. 33.3 (20.0-52.8) mm, p = 0.004), higher tortuosity (median(IQR): 1.35 (1.23-1.49) vs. 1.29 (1.23-1.39), p = 0.036), and smaller minimum luminal area of the right common iliac artery (CIA) (median (IQR): 86.7 (69.9-126.4) mm2 vs. 118.9 (86.3-164.1)mm2, p = 0.001) and left CIA (median(IQR): 92.2 (67.3,125.1) mm2 vs. 110.7 (80.12, 161.1) mm2, p = 0.010) than stable AAA did. Multiple regression analysis demonstrated significant associations of the minimum luminal area of the bilateral CIAs (odds ratio [OR] = 0.996, 95 % confidence interval [CI] 0.991-0.999, p = 0.037), neck length (OR = 0.969, 95 % CI 0.941-0.993, p = 0.017), and aneurysm tortuosity (OR = 1.031, 95 % CI 1.003-1.063, p = 0.038) with ruptured AAAs. The AUC of this regression model was 0.762 (95 % CI 0.664-0.860, p < 0.001). CONCLUSIONS: The smaller minimum luminal area of the CIA is associated with an increased risk of rupture. This study highlights the potential of utilizing outflow parameters as novel and additional tools in risk assessment. It also provides a compelling rationale to further intensify research in this area.

Endovascular repair for thoracic aortic pseudoaneurysm induced by pedicle screw implantation: a case report with 8 years follow-up.

BACKGROUND: Pedicle screw instrument surgeries can result in the development of aortic pseudoaneurysm, which is a rare yet potentially severe complication; therefore, the purpose of this work is to describe the case of pseudoaneurysm of the thoracic aorta caused by the severe migration of a pedicle screw after surgery. CASE PRESENTATION: We herein report a patient who underwent endovascular repair for the pseudoaneurysm of the descending thoracic aorta following thoracic vertebral fixation surgery. A 28-80 mm covered stent was initially inserted through the right femoral artery, and intraoperative aortography revealed a minor extravasation of contrast material. Subsequently, an additional 28-140 mm covered stent was implanted. The patient recovered well during the 8-year follow-up period. CONCLUSIONS: Vascular complications resulting from spinal surgery are severe and rare, necessitating early diagnosis and intervention.

In Situ Fenestration and Carotid-Subclavian Bypass for Left Subclavian Artery Revascularization During Thoracic Endovascular Aortic Repair.

PURPOSE: To evaluate the safety and feasibility of left subclavian artery (LSA) revascularization techniques during thoracic endovascular aortic repair (TEVAR)-the in situ needle fenestration (ISNF) technique and the carotid-subclavian bypass (CS-Bp)-for complicated aortic pathologies. METHODS: A retrospective single-center observational study was conducted to identify all patients with thoracic aortic pathologies who underwent TEVAR with LSA revascularization using either CS-Bp or ISNFs from January 2014 to December 2020. RESULTS: One hundred and twelve consecutive patients who received TEVAR with LSA revascularization were included. Among them, 69 received CS-Bp and 43 received ISNF (29 using the Futhrough adjustable puncture needles, 14 using the binding stent-graft puncture systems). Technical success, defined as achieving aortic arch pathology exclusion and LSA preservation, was attained in 99.1% patients. Early mortality was 0.9%. Major adverse events within 30 days, including one cerebral hemorrhage, one cervical incision hemorrhage, one stroke and two paraplegia, were exclusively observed in the CS-Bp group. Immediate type I, II and III endoleaks occurred in 0%, 4.7% and 2.3% in the ISNF group, respectively, compared to 0%, 2.9% and 0% in the CS-Bp group.One hundred and eight (97.2%) patients were available for follow-up at a median 50 (maiximum of 103) months, revealing a LSA patency rates of 99.1%. Six patients died during follow-ups-five in the CS-Bp group and one in the ISNF group. Cause of death include one aortic-related stent-graft infection, three non-related and two with unknow causes. The survival exhibited no significantly different between the ISNF (97.7%) and CS-Bp (89.9%) groups (p = 0.22). CONCLUSIONS: Both CS-Bp and ISNF are feasible techniques for LSA reconstruction in TEVAR. ISNF, whether using Futhrough or BPS, seems to be competitive with CS-Bp.

Elongation of the proximal descending thoracic aorta and associated hemodynamics increase the risk of acute type B aortic dissection.

BACKGROUND: Acute type B aortic dissection (ATBAD) is a life-threatening aortic disease. However, little information is available on predicting and understanding of ATBAD. OBJECTIVE: The study sought to explore the underlying mechanism of ATBAD by analyzing the morphological and hemodynamic characteristics related to aortic length. METHODS: The length and tortuosity of the segment and the whole aorta in the ATBAD group (n= 163) and control group (n= 120) were measured. A fixed anatomic landmark from the distal of left subclavian artery (LSA) to the superior border of sixth thoracic vertebra was proposed as the proximal descending thoracic aorta (PDTA), and the dimensionless parameter, length ratio, was introduced to eliminate the individual differences. The significant morphological parameters were filtrated and the associations between parameters were investigated using statistical approaches. Furthermore, how aortic morphology influenced ATBAD was explored based on idealized aortic models and hemodynamic-related metrics. RESULTS: The PDTA length was significantly increased in the ATBAD group compared with the control group and had a strong positive correlation with the whole aortic length (r= 0.89). The length ratio (LR2) and tortuosity (T2) of PDTA in the ATBAD group were significantly increased (0.15 ± 0.02 vs 0.12 ± 0.02 and 1.73 ± 0.48 vs 1.50 ± 0.36; P< 0.001), and LR2 was positive correlation with T2 (r= 0.73). In receiver-operating curve analysis, the area under the curve was 0.835 for LR2 and 0.641 for T2. Low and oscillatory shear (LOS) was positive correlation with LR2, and the elevated LOS occurred in the distal of LSA. CONCLUSION: Elongation of PDTA is associated with ATBAD, and the length ratio is a novel predictor. Elongated PDTA induced more aggressive hemodynamic forces, and high LOS regions may correspond to the entry tear location. The synergy of the morphological variation and aggressive hemodynamics creates contributory conditions for ATBAD.