Comparison of total percutaneous in situ microneedle puncture and chimney technique for left subclavian artery fenestration in thoracic endovascular aortic repair for type B aortic dissection.

OBJECTIVE: To compare the outcomes of totally percutaneous in situ microneedle puncture for left subclavian artery (LSA) fenestration (ISMF) and chimney technique in type B aortic dissection (TBAD) during thoracic endovascular aortic repair (TEVAR). MATERIALS AND METHODS: Data on patients who underwent either chimney-TEVAR (n = 89) or ISMF-TEVAR (n = 113) from October 2018 to April 2022 were analyzed retrospectively. The primary outcomes were mortality and major complications at 30 days and during follow-up. RESULTS: The technical success rate was 84.3% in the chimney group and 93.8% in the ISMF group (p = 0.027). The incidence of immediate endoleakage was significantly higher in the chimney than ISMF group (15.7% vs 6.2%, respectively; p = 0.027). The 1- and 3-year survival rates in the chimney and ISMF groups were 98.9% ± 1.1% vs 98.1% ± 0.9% and 86.5% ± 6.3% vs 92.6% ± 4.1%, respectively (log-rank p = 0.715). The 3-year rate of cumulative freedom from branch occlusion in the chimney and ISMF group was 95.4% ± 2.3% vs 100%, respectively (log-rank p = 0.023). CONCLUSION: Both ISMF-TEVAR and chimney-TEVAR achieved satisfactory short- and mid-term outcomes for the preservation of the LSA in patients with TBAD. ISMF-TEVAR appears to offer better clinical outcomes with higher patency and lower reintervention rates. However, ISMF-TEVAR had longer operation times with higher procedure expenses. CLINICAL RELEVANCE STATEMENT: When LSA revascularization is required during TEVAR, in situ, fenestration, and chimney techniques are all safe and effective methods; in situ, fenestration-TEVAR appears to offer better clinical outcomes, but takes longer and is more complicated. KEY POINTS: LSA revascularization during TEVAR reduces post-operative complication rates. Both in situ ISMF-TEVAR and chimney-TEVAR are safe and effective techniques for the preservation of the LSA during TEVAR. The chimney technique is associated with a higher incidence of endoleakage and branch occlusion, but ISMF-TEVAR is a more complicated and expensive technique.

Five Year Results of Endovascular versus Medical Therapy in Acute Type B Aortic Intramural Haematoma: Meta-Analysis of Reconstructed Time to Event Data.

OBJECTIVE: To evaluate outcomes in the follow up of thoracic endovascular aortic repair (TEVAR) vs. medical therapy in patients with acute type B aortic intramural haematoma (IMH). DATA SOURCES: The following sources were searched for articles meeting the inclusion criteria and published by July 2023: PubMed/MEDLINE, EMBASE, CENTRAL/CCTR (Cochrane Controlled Trials Register). REVIEW METHODS: This systematic review with pooled meta-analysis of time to event data followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines, and its protocol was registered on the public platform PROSPERO (CRD42023456222). The following were analysed: overall survival (all cause mortality), aortic related mortality, and restricted mean survival time. Certainty of evidence was evaluated through the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) tool. RESULTS: Eight studies met the eligibility criteria, including 1 015 patients (440 in the TEVAR group and 575 in the medical therapy group). All studies were observational, and the pooled cohort had a median follow up of 5.1 years. Compared with patients who received medical therapy alone, those who underwent TEVAR had a statistically significantly lower risk of all cause death (HR 0.44, 95% CI 0.30 - 0.65; p < .001; GRADE certainty: low), lower risk of aortic related death (HR 0.04, 95% CI 0.01 - 0.31; p = .002; GRADE certainty: low) and lifetime gain (restricted mean survival time was overall 201 days longer with TEVAR; p < .001). CONCLUSION: Thoracic endovascular aortic repair may be associated with lower risk of all cause and aortic related death compared with medical therapy in patients with acute type B IMH; however, the underlying data are not strong enough to draw robust clinical conclusions. Randomised controlled trials with large sample sizes and longer follow up are warranted to elucidate this question.

Midterm Outcomes of Endovascular vs. Medical Therapy for Uncomplicated Type B Aortic Dissection: Meta-Analysis of Reconstructed Time to Event Data.

OBJECTIVE: To evaluate outcomes of thoracic endovascular aortic repair (TEVAR) vs. medical therapy in uncomplicated type B aortic dissections (TBAD). DATA SOURCES: PubMed/MEDLINE, EMBASE, SciELO, LILACS, CENTRAL/CCTR, Google Scholar, and reference lists of relevant articles. REVIEW METHODS: This was a pooled meta-analysis of time to event data extracted from studies published by December 2022 for the following outcomes: all cause mortality, aortic related mortality, and late aortic interventions. Certainty of evidence was evaluated through the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) tool. RESULTS: Ten studies met the eligibility criteria (eight observational; two randomised trials), comprising 17 906 patients (2 332 patients in the TEVAR groups and 15 574 patients in the medical therapy group). Compared with patients who received medical therapy, patients who underwent TEVAR had a statistically significantly lower risk of all cause death (HR 0.79, 95% CI 0.72 - 0.87, p < .001; GRADE certainty: low) and lower risk of aortic related death (HR 0.43, 95% CI 0.30 - 0.62, p < .001; GRADE certainty: low) without statistically significant difference in the risk of late aortic interventions (HR 1.05, 95% CI 0.88 - 1.26, p = .56; GRADE certainty: low). In the subgroup analyses, TEVAR was associated with lower risk of all cause death when randomised controlled trials only were pooled (HR 0.44, 95% CI 0.23 - 0.83, p = .012; GRADE certainty: moderate), younger patients only (HR 0.56, 95% CI 0.47 - 0.67, p < .001; GRADE certainty: low), Western populations only (HR 0.85, 95% CI 0.77 - 0.93, p = .001; GRADE certainty: low) and non-Western populations only (HR 0.47, 95% CI 0.35 - 0.62, p < .001; GRADE certainty: low). For all cause mortality and aortic related mortality, restricted mean survival time was overall 396 days and 398 days longer with TEVAR (p < .001), respectively, which means that TEVAR was associated with lifetime gain. CONCLUSION: TEVAR may be associated with better midterm survival and lower risk of aortic related death in the follow up of patients treated for uncomplicated TBAD compared with medical therapy; however, randomised controlled trials with larger sample sizes and longer follow up are still warranted.

Analysis of Short-Term Clinical Outcomes and Influencing Factors in Patients with Acute Type B Aortic Intramural Hematoma Treated with Optimal Medical Therapy.

This study aimed to investigate the short-term predictors of aortic-related adverse events in patients with acute type B aortic intramural hematoma (IMH) initially treated with optimized medical therapy.A total of 157 patients with acute type B IMH were included in this study. These patients were divided into worsening group (n = 45) and stable group (n = 112) based on the incidence of aortic-related adverse events. The clinical data and imaging features of the two groups were compared. Multivariate logistic regression analysis of predictors of aortic-related adverse events in type B IMH was performed. Receiver operating characteristic (ROC) curve was applied to determine the optimal cutoff value for maximum descending aorta diameter (MDAD). Kaplan-Meier survival curve was used to analyze the incidence of aortic-related adverse events.Worsening and stable groups were statistically significant in diuretics, abnormal D-dimer level, observation endpoint systolic blood pressure (SBP), MDAD, aortic atherosclerosis, ulcer-like projection (ULP), and thickness of hematoma (P < 0.05). Multivariate logistic regression showed that abnormal D-dimer level (OR = 12.464, P = 0.025), MDAD (OR = 1.113, P = 0.030), and ULP (OR = 5.849, P = 0.022) were powerful independent risk factors for predicting aortic-related adverse events in type B IMH, and observation endpoint SBP within 100-120 mmHg (OR = 0.225, P = 0.014) was a protective factor for predicting aortic-related adverse events in type B IMH. The cutoff value of MDAD was 35.2 mm.Short-term imaging is recommended for type B IMH patients with abnormal D-dimer level, MDAD > 35.2 mm, and ULP. Blood pressure should also be strictly monitored and controlled during the acute phase of IMH.

Morphometric Predictors of Aortic Remodeling after Frozen Elephant Trunk Repair of Type A Dissection.

BACKGROUND: We analyzed the predictors of postoperative aortic remodeling after the frozen elephant trunk technique for type A dissection. METHODS: This retrospective study cohort comprised 20 patients who underwent the frozen elephant trunk technique for type A dissection. The incidence of aortic remodeling was evaluated at 4 levels: middle of the frozen elephant trunk, distal end of the frozen elephant trunk, 10th thoracic vertebra, and proximal to the celiac artery. Several parameters of the residual dissected aorta were analyzed at these 4 levels, and the predictors of aortic remodeling were investigated. RESULTS: The incidence of aortic remodeling was 85% at the middle of the frozen elephant trunk, 70% at the distal end of the frozen elephant trunk, 50% at the 10th thoracic vertebra, and 35% proximal to the celiac artery. In a total of 80 computed tomography images, there were significant differences between the aortic remodeling and nonremodeling groups in the true lumen diameter, false lumen diameter, true lumen/aortic diameter ratio, true lumen area, true lumen/aortic area ratio, and 2 parameters explaining the shape of the true lumen (created using elliptic Fourier analysis). Multivariate analysis revealed that the independent predictors of aortic remodeling were the true lumen/aortic area ratio and principal component 1. CONCLUSIONS: There were high incidences of aortic remodeling at the middle and distal end of the frozen elephant trunk. Multivariate analysis showed that the true lumen/aorta area ratio and the shape of the true lumen may be important predictors of aortic remodeling.

Paraplegia After Open Surgical Repair Versus Thoracic Endovascular Aortic Repair for Thoracic Aortic Disease: A Retrospective Analysis of Japanese Administrative Data.

OBJECTIVES: To comparatively examine the risk of postoperative paraplegia between open surgical descending aortic repair and thoracic endovascular aortic repair (TEVAR) among patients with thoracic aortic disease. DESIGN: Retrospective cohort study. SETTING: Acute-care hospitals in Japan. PARTICIPANTS: A total of 6,202 patients diagnosed with thoracic aortic disease. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The main outcome of this study was the incidence of postoperative paraplegia. Multiple logistic regression models, using inverse probability of treatment weighting and an instrumental variable (ratio of TEVAR use to open surgical repair and TEVAR uses), showed that the odds ratios of paraplegia for TEVAR (relative to open surgical descending aortic repair) were 0.81 (95% confidence interval: 0.42-1.59; p = 0.55) in the inverse probability of treatment-weighted model and 0.88 (0.42-1.86; p = 0.75) in the instrumental-variable model. CONCLUSIONS: There were no statistical differences in the risk of paraplegia between open surgical repair and TEVAR in patients with thoracic aortic disease. Improved perioperative management for open surgical repair may have contributed to the similarly low incidence of paraplegia in these two surgery types.

Long-term outcomes of total arch replacement versus proximal aortic replacement in acute type A aortic dissection: Meta-analysis of Kaplan-Meier-derived individual patient data.

OBJECTIVES: To evaluate the long-term outcomes of a conservative approach (with proximal aortic replacement with or without hemiarch replacement) versus an aggressive approach (with total aortic arch replacement) in the treatment of acute type A aortic dissection (ATAAD). METHODS: We performed a pooled analysis of Kaplan-Meier-derived individual patient data from studies with follow-up comparing the aforementioned approaches to treat patients with ATAAD. RESULTS: Eighteen studies met our eligibility criteria, comprising 5243 patients with follow-up (Conservative: 3676 patients; Aggressive: 1567 patients). We observed a statistically significant difference in overall survival favoring the aggressive approach (hazard ratios [HR] 0.86, 95% confidence interval [CI] 0.76-0.98, p = .022), but no statistically significant difference in the risk of reoperation (HR 0.89, 95% CI 0.66-1.2, p = .439) in the overall follow-up. Landmark analyses revealed that, in the first 3 months after the procedure, mortality rates were comparable between conservative and aggressive approaches (HR 1.04, 95% CI 0.88-1.24, p = .627), but the results beyond 3 months showed improved survival in patients undergoing the aggressive surgical procedure (HR 0.71, 95% CI 0.59-0.85, p < .001). The landmark analyses also revealed that, in the first 7 years after the procedure, reoperation rates were comparable between the approaches (HR 1.03, 95% CI 0.76-1.40, p = .848), but the results beyond 7 years showed a lower risk of reoperation in patients undergoing the aggressive surgical procedure (HR 0.10, 95% CI 0.01-0.75, p = .025). CONCLUSION: The aggressive approach seems to confer better long-term survival and lower risk of the need for reoperation in the follow-up of patients treated for ATAAD.

Genetic Architecture of Abdominal Aortic Aneurysm in the Million Veteran Program.

BACKGROUND: Abdominal aortic aneurysm (AAA) is an important cause of cardiovascular mortality; however, its genetic determinants remain incompletely defined. In total, 10 previously identified risk loci explain a small fraction of AAA heritability. METHODS: We performed a genome-wide association study in the Million Veteran Program testing ≈18 million DNA sequence variants with AAA (7642 cases and 172 172 controls) in veterans of European ancestry with independent replication in up to 4972 cases and 99 858 controls. We then used mendelian randomization to examine the causal effects of blood pressure on AAA. We examined the association of AAA risk variants with aneurysms in the lower extremity, cerebral, and iliac arterial beds, and derived a genome-wide polygenic risk score (PRS) to identify a subset of the population at greater risk for disease. RESULTS: Through a genome-wide association study, we identified 14 novel loci, bringing the total number of known significant AAA loci to 24. In our mendelian randomization analysis, we demonstrate that a genetic increase of 10 mm Hg in diastolic blood pressure (odds ratio, 1.43 [95% CI, 1.24-1.66]; P=1.6×10-6), as opposed to systolic blood pressure (odds ratio, 1.06 [95% CI, 0.97-1.15]; P=0.2), likely has a causal relationship with AAA development. We observed that 19 of 24 AAA risk variants associate with aneurysms in at least 1 other vascular territory. A 29-variant PRS was strongly associated with AAA (odds ratioPRS, 1.26 [95% CI, 1.18-1.36]; PPRS=2.7×10-11 per SD increase in PRS), independent of family history and smoking risk factors (odds ratioPRS+family history+smoking, 1.24 [95% CI, 1.14-1.35]; PPRS=1.27×10-6). Using this PRS, we identified a subset of the population with AAA prevalence greater than that observed in screening trials informing current guidelines. CONCLUSIONS: We identify novel AAA genetic associations with therapeutic implications and identify a subset of the population at significantly increased genetic risk of AAA independent of family history. Our data suggest that extending current screening guidelines to include testing to identify those with high polygenic AAA risk, once the cost of genotyping becomes comparable with that of screening ultrasound, would significantly increase the yield of current screening at reasonable cost.

Familial Clustering of Aortic Size, Aneurysms, and Dissections in the Community.

BACKGROUND: Ruptured aortic aneurysm and aortic dissections are potentially preventable disorders associated with high mortality. Screening of individuals at risk may translate into elective surgical interventions and lowered mortality. It is uncertain if the risk of aortic dilation of varying degrees aggregates within families. METHODS: We investigated the risk of having thoracic and abdominal aortic sizes in the highest quartile (measured by computed tomography scans and indexed for body size) if at least 1 parent did so in the Framingham Heart Study cohorts, and estimated the incidence rates and hazard ratios of developing aortic aneurysm or dissection among first-degree relatives of those with aortic aneurysm or dissection, in comparison with age- and sex-matched controls (1:10 for aortic aneurysm and 1:100 for aortic dissection) using the Danish nationwide administrative registries. RESULTS: In the Framingham Heart Study, offspring (n=235) whose parent(s) had a sex- and age-standardized aortic size in the upper quartile had a multivariable-adjusted ≈3-fold increased odds ratio of belonging to the upper quartile themselves. In Denmark, a total of 68 939 individuals (mean age, 42 years) had a first-degree relative with aortic aneurysm and 7209 persons (mean age, 39 years) had a first-degree relative with aortic dissection. During an average follow-up of 7 years, first-degree relatives of patients with aortic aneurysm and dissection had a hazard ratio of 6.70 (95% CI, 5.96-7.52) for developing aortic aneurysm and a hazard ratio of 9.24 (95% CI, 5.53-15.44) for dissection in comparison with matched controls. These estimates remained unchanged on adjusting for several comorbidities, including prevalent hypertension, bicuspid aortic valve, and the Marfan syndrome. For both aortic aneurysm and dissections, the absolute event rates approached 1 per 1000 person-years for first-degree relatives versus 11 to 13 (aortic aneurysm) and 2 to 3 (aortic dissections) per 100 000 person-years among controls. CONCLUSIONS: Increased aortic size, a precursor of aortic aneurysm and a risk factor for dissection, clusters in families. The incidence rates of aortic aneurysm and dissections approach the incidence rates of other common cardiovascular conditions in first-degree relatives, supporting the use of systematic screening for these conditions.

Intracranial Aneurysms Are Associated With Marfan Syndrome: Single Cohort Retrospective Study in 118 Patients Using Brain Imaging.

BACKGROUND AND PURPOSE: The association between intracranial aneurysms (IAs) and Marfan syndrome (MFS) is controversial. We aimed to evaluate the prevalence and characteristics of IAs in patients with MFS using brain imaging and compare it with the general population. METHODS: Between 2007 and 2020, 118 patients with confirmed MFS who underwent brain imaging were enrolled and classified into 2 groups; IA group versus non-IA. Demographic data were acquired from their medical records, including age, sex, comorbidities, and aortic diseases. Two readers reviewed all brain images independently regarding the presence, morphology, size, and location of IAs. All data were compared between both groups, and IA characteristics in MFS were analyzed using a database of controls with IAs. RESULTS: The prevalence of IAs was 11.9% in patients with MFS. IA group were significantly older in age (44.6±12.1 years in IA versus 36.8±14.0 years in non-IA, P=0.039) and had female predominance (71.4% in IA versus 43.3% in non-IA, P=0.047). All IAs were unruptured, and there was no subarachnoid hemorrhage during the follow-up period (mean; 53.5±43.3 months). The mean diameter of IAs was significantly larger (4.23±1.80 mm in MFS versus 3.04±1.57 mm in control, P=0.004). IAs with MFS were frequently located in the vertebrobasilar artery (33.3% in MFS versus 2.1% in control, P=0.002) and more common in fusiform morphology (13.3% in MFS versus 1.1% in control, P=0.048). CONCLUSIONS: This large-cohort study demonstrated a high prevalence and differential features of IAs in MFS, which may support the association between IAs and MFS.

Variants of Unknown Significance in Genes Associated with Heritable Thoracic Aortic Disease Can Be Low Penetrant "Risk Variants".

Thoracic aortic aneurysms leading to acute aortic dissections are a preventable cause of premature deaths if individuals at risk can be identified. Individuals with early-onset aortic dissections without a family history or syndromic features have an increased burden of rare genetic variants of unknown significance (VUSs) in genes with pathogenic variants for heritable thoracic aortic disease (HTAD). We assessed the role of VUSs in the development of disease using both in vitro enzymatic assays and mouse models. VUSs in LOX and MYLK identified in individuals with acute aortic dissections were assayed to determine whether they disrupted enzymatic activity. A subset of VUSs reduced enzymatic activity compared to the wild-type proteins but less than pathogenic variants. Additionally, a Myh11 variant, p.Arg247Cys, which does not cause aortic disease in either humans or mice, was crossed with the Acta2-/- mouse, which has aortic enlargement with age while Acta2+/- mice do not. Acta2+/-Myh11R247C/R247C mice have aortic dilation by 3 months of age without medial degeneration, indicating that two variants not known to cause disease do lead to aortic enlargement in combination. Furthermore, the addition of Myh11R247C/R247C to the Acta2-/- mouse model accelerates aortic enlargement and increases medial degeneration. Therefore, our results emphasize the need for a classification system for variants in Mendelian genes that goes beyond the 5-tier system of pathogenic, likely pathogenic, VUS, likely benign, and benign, and includes a designation for low-penetrant "risk variants" that trigger disease either in combination with other risk factors or in a stochastic manner.

LTBP3 Pathogenic Variants Predispose Individuals to Thoracic Aortic Aneurysms and Dissections.

The major diseases affecting the thoracic aorta are aneurysms and acute dissections, and pathogenic variants in 11 genes are confirmed to lead to heritable thoracic aortic disease. However, many families in which multiple members have thoracic aortic disease do not have alterations in the known aortopathy genes. Genes highly expressed in the aorta were assessed for rare variants in exome sequencing data from such families, and compound rare heterozygous variants (p.Pro45Argfs∗25 and p.Glu750∗) in LTBP3 were identified in affected members of one family. A homozygous variant (p.Asn678_Gly681delinsThrCys) that introduces an additional cysteine into an epidermal growth factor (EGF)-like domain in the corresponding protein, latent TGF-ß binding protein (LTBP-3), was identified in a second family. Individuals with compound heterozygous or homozygous variants in these families have aneurysms and dissections of the thoracic aorta, as well as aneurysms of the abdominal aorta and other arteries, along with dental abnormalities and short stature. Heterozygous carriers of the p.Asn678_Gly681delinsThrCys variant have later onset of thoracic aortic disease, as well as dental abnormalities. In these families, LTBP3 variants segregated with thoracic aortic disease with a combined LOD score of 3.9. Additionally, heterozygous rare LTBP3 variants were found in individuals with early onset of acute aortic dissections, and some of these variants disrupted LTBP-3 levels or EGF-like domains. When compared to wild-type mice, Ltbp3-/- mice have enlarged aortic roots and ascending aortas. In summary, homozygous LTBP3 pathogenic variants predispose individuals to thoracic aortic aneurysms and dissections, along with the previously described skeletal and dental abnormalities.

Management of Descending Thoracic Aortic Diseases: Similarities and Differences Among Cardiovascular Guidelines.

Cardiovascular societies have developed recommendations regarding the management of thoracic aortic diseases. While improvements in treatment have been observed during the past decade in regard to patient selection, thoracic endovascular aortic repair (TEVAR) and associated techniques, and high-volume centralization, the broad expansion of TEVAR has raised considerations about its indications, appropriateness, limitations, and application. The aim of this systematic review was to assess the similarities and differences among current cardiovascular societies' guidelines for the management of thoracic aortic diseases. The MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials were searched from January 2009 to May 2020. The initial search identified 990 articles. After exclusion of duplicate or inappropriate articles, the final analysis included 5 articles from cardiovascular societies published between 2010 and 2020. Selected controversial topics were analyzed, including diagnosis, imaging, spinal cord ischemia prevention, and management of the most important thoracic aortic pathologies. The analysis included data concerning the therapeutic approach in acute and chronic type B aortic dissection, penetrating aortic ulcer, intramural hematoma, thoracic aortic aneurysm, and traumatic aortic injury, as well a discussion of inflammatory aneurysms, aortitis, and genetic syndromes. The review presents consistent and controversial recommendations, as well as "gray zone" issues that need further investigation. There was significant overlap and agreement among the 5 societies regarding the management of thoracic aortic diseases. Especially in dissection and aneurysm management, TEVAR has established its role as the treatment of choice. However, robust evidence is still needed in many aspects of the management of thoracic aortic pathologies.

Radial self-navigated native magnetic resonance angiography in comparison to navigator-gated contrast-enhanced MRA of the entire thoracic aorta in an aortic patient collective.

BACKGROUND: The native balanced steady state with free precession (bSSFP) magnetic resonance angiography (MRA) technique has been shown to provide high diagnostic image quality for thoracic aortic disease. This study compares a 3D radial respiratory self-navigated native MRA (native-SN-MRA) based on a bSSFP sequence with conventional Cartesian, 3D, contrast-enhanced MRA (CE-MRA) with navigator-gated respiration control for image quality of the entire thoracic aorta. METHODS: Thirty-one aortic native-SN-MRA were compared retrospectively (63.9 ± 10.3 years) to 61 CE-MRA (63.1 ± 11.7 years) serving as a reference standard. Image quality was evaluated at the aortic root/ascending aorta, aortic arch and descending aorta. Scan time was recorded. In 10 patients with both MRA sequences, aortic pathologies were evaluated and normal and pathologic aortic diameters were measured. The influence of artifacts on image quality was analyzed. RESULTS: Compared to the overall image quality of CE-MRA, the overall image quality of native-SN-MRA was superior for all segments analyzed (aortic root/ascending, p < 0.001; arch, p < 0.001, and descending, p = 0.005). Regarding artifacts, the image quality of native-SN-MRA remained superior at the aortic root/ascending aorta and aortic arch before and after correction for confounders of surgical material (i.e., susceptibility-related artifacts) (p = 0.008 both) suggesting a benefit in terms of motion artifacts. Native-SN-MRA showed a trend towards superior intraindividual image quality, but without statistical significance. Intraindividually, the sensitivity and specificity for the detection of aortic disease were 100% for native-SN-MRA. Aortic diameters did not show a significant difference (p = 0.899). The scan time of the native-SN-MRA was significantly reduced, with a mean of 05:56 ± 01:32 min vs. 08:51 ± 02:57 min in the CE-MRA (p < 0.001). CONCLUSIONS: Superior image quality of the entire thoracic aorta, also regarding artifacts, can be achieved with native-SN-MRA, especially in motion prone segments, in addition to a shorter acquisition time.

Mid-Aortic Syndrome in Williams-Beuren Syndrome with an Atypical Small-Sized Deletion of Chromosome 7q11.23 Misdiagnosed as Takayasu Arteritis.

Mid-aortic syndrome (MAS) is a rare condition characterized by stenosis of the distal thoracic and/or abdominal aorta. Williams-Beuren syndrome (WBS) is a relatively rare cause of MAS. We report a case of incidentally diagnosed MAS caused by WBS without typical manifestations caused by an atypical small-sized deletion in chromosome 7q11.23, which was initially misdiagnosed as Takayasu arteritis.

Changes in Health Perception among Patients with Aortic Diseases in a Severe COVID-19 Area in the West of Germany: A Longitudinal Study between the First and Second Wave of the COVID-19 Pandemic.

Background and Objectives: The rapid spread of the novel coronavirus disease (COVID-19) has become the most challenging global health pandemic since the 1918 flu. In Germany, more than 3.4 million cases are confirmed so far, including 83,000 deaths. Increased fatality rates among patients with pre-existing cardiovascular diseases (CVD) represent this group at particular risk. The aim of this study was to evaluate changes in health perception among patients with aortic diseases during the first (w1) and second wave (w2) of the COVID-19 pandemic in Germany. Material and Methods: Patients (n = 262) diagnosed with aortic disease participated in telephone interviews during w1 and w2. The perception of COVID-19 as a threat was examined using relevant items of the Brief Illness Perception (BIP) questionnaire. Results: The BIP score increased from 9.18 (SD = 7.132) to 14.58 (SD = 6.956) between w1 and w2 (p < 0.001). Although this population is at high risk their overall perception of COVID-19 as a threat was low in the beginning, but surged during w2. Main reasons were increased effects on personal life and elevated concerns about the pandemic, but did not include the educational aspect of COVID-19. Conclusions: Tailored risk communication strengthens the mental health of people in a public health crisis and ensures the success of governmental guidelines.

Unibody design for aortic disease with a narrow aortic bifurcation: tips and tricks for success.

BACKGROUND: Surgical management of patients with abdominal aortic diseases associated with distal narrowing is a challenging situation. OBJECTIVES: To evaluate outcomes of unibody bifurcated endovascular stent graft repair. METHODS: This is a retrospective, observational, multi-institutional database study of a cohort of consecutive cases, approved by the local Ethics Committee. Records were reviewed of patients diagnosed from 2010 to 2020 with "shaggy" aorta, saccular aneurysm, penetrating aortic ulcer, and isolated aortic dissection located in the infrarenal abdominal aorta. All patients were treated with a unibody bifurcated stent graft. Main outcomes were technical success, procedure complications, long-term patency, and mortality in the follow-up period up to 5 years. Data on demographics, comorbidities, surgical management, and outcomes were analyzed. RESULTS: Twenty-three patients were treated with unibody bifurcated stent graft repair, including 7 cases of "shaggy" aorta, 3 isolated dissections of the abdominal aorta, 4 penetrating aortic ulcers, and 9 saccular aneurysms. Immediate technical success was achieved in 100% of cases. At follow-up, all stent grafts remained patent and there were no limb occlusions. The patients were symptom-free and reported no complications related to the procedure. There were 5 deaths during the follow-up period (median= 4 years), but none were related to the procedure and there were no aorta-related deaths. CONCLUSIONS: The present study shows that unibody bifurcated stent grafting is safe and effective in this group of patients with narrow distal abdominal aorta and complex aortic pathology. The results were similar for both infrarenal aortic aneurysms and aorto-iliac atherosclerotic disease.

CONTEXTO: O manejo cirúrgico de pacientes com doenças da aorta abdominal associadas ao estreitamento distal é desafiador. OBJETIVOS: Avaliar os desfechos do reparo endovascular com endoprótese bifurcada do tipo monobloco. MÉTODOS: Foi realizado um estudo retrospectivo, de coorte observacional, de banco de dados multi-institucional, com casos consecutivos, após aprovação pelo Comitê de Ética local. Foram analisados prontuários de pacientes com diagnóstico de shaggy aorta, aneurisma sacular, úlcera penetrante da aorta e dissecção isolada da aorta localizados na aorta abdominal infrarrenal entre 2010 e 2020; todos os pacientes foram tratados com endoprótese bifurcada do tipo monobloco. Os principais desfechos foram sucesso técnico, complicações relacionadas ao procedimento, perviedade de longo prazo e mortalidade no seguimento de até 5 anos. Foram incluídos dados demográficos, comorbidades, manejo cirúrgico e desfechos. RESULTADOS: Vinte e três pacientes foram tratados com endoprótese bifurcada do tipo monobloco, incluindo 7 casos de shaggy aorta, 3 dissecções isoladas da aorta abdominal, 4 úlceras penetrantes da aorta e 9 aneurismas saculares. Sucesso técnico imediato foi obtido em 100% dos casos. No seguimento, todas as endopróteses permaneceram pérvias e não houve oclusões de membros. Os pacientes estavam sem sintomas e não relataram complicações relacionadas ao procedimento. Ocorreram 5 óbitos durante o seguimento (mediana = 4 anos), mas nenhum relacionado ao procedimento ou à aorta. CONCLUSÕES: O presente estudo mostra que a endoprótese bifurcada do tipo monobloco é segura e eficaz neste grupo de pacientes com estreitamento distal da aorta abdominal e patologia aórtica complexa. Os resultados foram semelhantes para aneurismas da aorta infrarrenal e doença aterosclerótica aortoilíaca.

Sex-Related Differences in Patients Undergoing Thoracic Aortic Surgery.

BACKGROUND: Contemporary outcomes after surgical management of thoracic aortic disease have improved; however, the impact of sex-related differences is poorly understood. METHODS: A total of 1653 patients (498 [30.1%] female) underwent thoracic aortic surgery with hypothermic circulatory arrest between 2002 and 2017 in 10 institutions of the Canadian Thoracic Aortic Collaborative. Outcomes of interest were in-hospital death, stroke, and a modified Society of Thoracic Surgeons-defined composite for mortality or major morbidity (stroke, renal failure, deep sternal wound infection, reoperation, prolonged ventilation). Multivariable logistic regression was used to determine independent predictors of these outcomes. RESULTS: Women were older (mean±SD, 66±13 years versus 61±13 years; P<0.001), with more hypertension and renal failure, but had less coronary disease, less previous cardiac surgery, and higher ejection fraction than men. Rates of aortic dissection were similar between women and men. Rates of hemiarch, and total arch repair were similar between the sexes; however, women underwent less aortic root reconstruction including aortic root replacement, Ross, or valve-sparing root operations (29% versus 45%; P<0.001). Men experienced longer cross-clamp and cardiopulmonary bypass times, but similar durations of circulatory arrest, methods of cerebral perfusion, and nadir temperatures. Women experienced a higher rate of mortality (11% versus 7.4%; P=0.02), stroke (8.8% versus 5.5%; P=0.01), and Society of Thoracic Surgeons-defined composite end point for mortality or major morbidity (31% versus 27%; P=0.04). On multivariable analyses, female sex was an independent predictor of mortality (odds ratio, 1.81; P<0.001), stroke (odds ratio, 1.90; P<0.001), and Society of Thoracic Surgeons-defined composite end point for mortality or major morbidity (odds ratio, 1.40; P<0.001). CONCLUSIONS: Women experience worse outcomes after thoracic aortic surgery with hypothermic circulatory arrest. Further investigation is required to better delineate which measures may reduce sex-related outcome differences after complex aortic surgery.

Single-Cell Analysis of the Normal Mouse Aorta Reveals Functionally Distinct Endothelial Cell Populations.

BACKGROUND: The cells that form the arterial wall contribute to multiple vascular diseases. The extent of cellular heterogeneity within these populations has not been fully characterized. Recent advances in single-cell RNA-sequencing make it possible to identify and characterize cellular subpopulations. METHODS: We validate a method for generating a droplet-based single-cell atlas of gene expression in a normal blood vessel. Enzymatic dissociation of 4 whole mouse aortas was followed by single-cell sequencing of >10 000 cells. RESULTS: Clustering analysis of gene expression from aortic cells identified 10 populations of cells representing each of the main arterial cell types: fibroblasts, vascular smooth muscle cells, endothelial cells (ECs), and immune cells, including monocytes, macrophages, and lymphocytes. The most significant cellular heterogeneity was seen in the 3 distinct EC populations. Gene set enrichment analysis of these EC subpopulations identified a lymphatic EC cluster and 2 other populations more specialized in lipoprotein handling, angiogenesis, and extracellular matrix production. These subpopulations persist and exhibit similar changes in gene expression in response to a Western diet. Immunofluorescence for Vcam1 and Cd36 demonstrates regional heterogeneity in EC populations throughout the aorta. CONCLUSIONS: We present a comprehensive single-cell atlas of all cells in the aorta. By integrating expression from >1900 genes per cell, we are better able to characterize cellular heterogeneity compared with conventional approaches. Gene expression signatures identify cell subpopulations with vascular disease-relevant functions.

Factors associated with in-hospital complications and long-term implications of these complications in elderly patients undergoing endovascular aneurysm repair.

OBJECTIVE: Perioperative complications in elderly patients undergoing endovascular aneurysm repair (EVAR) occur frequently. Although perioperative mortality has been well-described in the elderly patient population, factors associated with in-hospital complications and their impact on long-term survival remain poorly characterized. METHODS: We identified all patients undergoing elective EVAR for infrarenal AAA within the Vascular Quality Initiative registry (2003-2018) and compared in-hospital complication rates between elderly (age ≥75) and nonelderly patients (<75). We used logistic regression to identify independent factors associated with in-hospital complications, whereas Kaplan-Meier analysis and Cox proportional hazards models were used to determine associations between complications and long-term survival. To assess the effect of complications on early and late survival, we stratified survival periods into the first 30 days after discharge, and between 1 and 6 months, 7 and 12 months, and 1 and 8 years after the index procedure. To investigate the implications of in-hospital morbidity on long-term outcomes, we estimated the adjusted population-attributable fractions of individual complications on both perioperative and long-term survival. RESULTS: We identified 17,156 elderly patients and 19,922 nonelderly patients. Elderly patients experienced higher complication rates compared with nonelderly patients (17% vs 10%; P < .001). The factors with the strongest associations with morbidity in elderly patients were anemia (odds ratio [OR], 2.4; 95% confidence interval [CI], 2.2-2.6), female gender (OR, 1.9; 95% CI, 1.7-2.1), and large AAA diameter (OR, 1.7; 95% CI, 1.6-1.9). Patients with any in-hospital complication had lower unadjusted survival estimates than patients without complications at 1 year (83% vs 95%; P < .001), 5 years (66% vs 80%; P < .001), and 8 years (60% vs 72%; P < .001). After risk adjustment, in-hospital complications were independently associated with higher mortality, although the association attenuated over time (first month after discharge: hazard ratio [HR], 5.9; 95% CI, 3.9-9.1; 1-6 months after the procedure: HR, 2.1; 95% CI, 1.7-2.7; P < .001; 7-12 months after the procedure: HR, 1.5; 95% CI, 1.1-1.9; 1-8 years after the procedure: HR, 1.2; 95% CI, 1.01-1.3). Of all deaths occurring within 8 years after procedure, 9.5% were independently associated with in-hospital complications. Complications with the greatest impact on long-term mortality were renal dysfunction (2.4%), blood transfusion (3.4%), and reintubations (2.4%). CONCLUSIONS: Elderly patients are at higher risk for in-hospital complications after EVAR. These in-hospital complications have a significant impact on both short- and long-term survival. To further improve the delivery of EVAR care nationally, quality improvement efforts should be focused on preventing postoperative morbidity in elderly patients, as well as refining out of hospital surveillance strategies for subjects who experience in-hospital complications to improve overall survival.