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(1) Background: The aortic arch (AA) branching model is challenging, considering the multiple anatomical variations documented in existing research. The bovine aortic arch (BAA) is the most prevalent anatomical variation among these. This variant of AA branching has long been considered a nonsymptomatic malformation, having been discovered incidentally during imaging investigations for other causes. However, more recent studies have demonstrated that BAA shows a frequent association with coarctation of the aorta (CoA), thoracic aortic disease (TAD), and stroke. At the same time, given the current context of increasing activity in the fields of interventional and surgical procedures in the aorta and its branches, it is very important to know the medical–surgical implications of this anatomical variant. (2) Methods: We conducted a comprehensive review using PubMed and Embase, focusing specifically on randomized trials and cohort analyses that examined the medical–surgical implications of BAA. We assessed information related to studied groups, medical procedures, and study outcomes. Initially, we identified 8454 studies, and after rigorous evaluation, we narrowed down our review to 25 articles. (3) Discussions: The intervention consisted of assessing the risks associated with BAA through different imaging investigation methods such as computer tomographic angiography (CTA), magnetic resonance imaging (MRI), or ultrasonography (US). The following results were evaluated: the prevalence of the BAA, the importance of imaging investigations in establishing the diagnosis and the therapeutic management and monitoring the evolution of patients with the BAA, the association of the BAA with CoA, TAD, and stroke, and the potential risks of interventional treatment in patients with the BAA. (4) Conclusions: The prevalence of the BAA differs both between different ethnic groups and between genders. Advanced imaging methods such as CTA and 4D flow MRI allow detailed descriptions of supra-aortic vascular anatomy and information about blood flow velocities, direction, and turbulence in the AA. US remains an easy and valuable imaging investigation, with the potential to detect and correctly diagnose the BAA and its hemodynamic implications. Anatomical variations in the AA are associated with increased rates of TAD, CoA, and stroke, necessitating early diagnosis and increased supervision of patients with such incidentally observed abnormalities. In addition, there is a need to further develop and refine the surgical techniques used and personalize them to the individual characteristics of patients with the BAA. [ABSTRACT FROM AUTHOR]

Background: There is a paucity of evidence on people with thoracic aortic aneurysm and dissection. We aimed to determine the prevalence of genetic variants and their associations with phenotypes. Methods: In this cross-sectional single-centre cohort study of consecutive patients who underwent endovascular or open-surgical repair of thoracic aortic aneurysm and dissection, genetic analysis was performed using four-stage Next Generation Sequencing, and findings were confirmed with Sanger sequencing. We collected personal and family history on comorbidities, clinical examination, anthropometrics, skeletal deformities, joint function, and ophthalmological measures. Cardiovascular risk and phenotype scores were calculated. Results: Ninety-five patients were eligible (mean age 54 ± 9 years, 70% males, 56% aortic dissection). One-fifth had a family history of aortic disease. Furthermore, 95% and 54% had a phenotype score of ≤5 and ≤2, respectively. There were no significant differences in the distribution of phenotype characteristics according to age, sex, aortic pathology, or performed invasive procedures. Genetic variants of uncertain significance were detected in 40% of patients, with classic mutations comprising 18% of all variants. We observed no significant association with cardiovascular and phenotype scores but with higher joint function scores (p = 0.015). Conclusion: Genetic variants are highly present in clinically relevant aortic pathologies. Variants appear to play a larger role than previously described. The different variants do not correlate with specific phenotypes, age, pathology, sex, or family history. [ABSTRACT FROM AUTHOR]

Advances in both imaging techniques and genetics have led to the recognition of a wide variety of aortic anomalies that can be grouped under the term 'hereditary thoracic aortic diseases'. The present review aims to summarize this very heterogeneous population's clinical, genetic, and imaging characteristics and to discuss the implications of the diagnosis for clinical counselling (on sports activity or pregnancy), medical therapies and surgical management. [ABSTRACT FROM AUTHOR]

Thoracic aortic disease (TAD) is often silent until a life-threatening complication occurs. However, genetic information can inform both identification and treatment at an early stage. Indeed, a diagnosis is important for personalised surveillance and intervention plans, as well as cascade screening of family members. Currently, only 20% of heritable TAD patients have a causative mutation identified and, consequently, further advances in genetic coverage are required to define the remaining molecular landscape. The rapid expansion of next generation sequencing technologies is providing a huge resource of genetic data, but a critical issue remains in functionally validating these findings. Induced pluripotent stem cells (iPSCs) are patient-derived, reprogrammed cell lines which allow mechanistic insights, complex modelling of genetic disease and a platform to study aortic genetic variants. This review will address the need for iPSCs as a frontline diagnostic tool to evaluate variants identified by genomic discovery studies and explore their evolving role in biological insight through to drug discovery.

An editorial is presented which expresses the views on the gender-related differences in cardiovascular care, emphasizing the continued prevalence of cardiovascular disease (CVD) as the leading cause of death in women. Topics include the heightened risks of CVD during pregnancy, challenges in managing cardiovascular issues in pregnant women, and the importance of multidisciplinary teams in tailoring care to address the unique needs of women with heart disease.

Abstract Background Both bicuspid aortic valve (BAV) and bovine aortic arch (BA) are considered as markers of thoracic aortic disease (TAD). But the association between them is not yet clear. This study aimed to explore the potential association of BAV and BA with TAD. Methods The study involved 449 participants who underwent their first aortic valve replacement in Fuwai Hospital from June 2017 to March 2018. All patients underwent multidetector computed tomography and echocardiography before surgery. The clinical characteristics were recorded to analyze the association between BAV, BA, and TAD. The univariate and multivariate logistic regression analyses were applied to identify the risk factors for TAD. Results BA accounted for 79.8% of the arch variants and was the most common aortic arch branching variant. BAV was present in 52.6% of the patients with BA and 38.1% of the patients with normal arch (NA). Among the 185 patients in the BAV subgroup, 50 had BA and 135 had NA. No significant differences were found in BAV anatomical phenotype, aortopathy phenotype, and valve function between BA and NA. The multivariate analysis showed that the presence of BAV and male sex were the risk predictors of TAD. BA was not a risk factor for TAD in either univariate or multivariate analysis. Conclusions The proportion of BAV in patients with BA was significantly higher than that of NA, but the BAV phenotype and aortopathy were not related to BA. BAV was a risk factor for TAD, whereas BA was not associated with TAD.

ObjectiveThe main purpose of this study was to evaluate the safety and efficacy of Castor single-branched stent-graft combined with fenestrated technique in treatment of thoracic aortic disease (TAD) with unfavorable proximal landing area (PLZ) and isolated left vertebral artery (ILVA).MethodsFrom January 2018 to March 2022, 8 patients with TAD (6 patients with type B aortic dissections, 1 patient with type B intramural hematomas, and 1 patient with thoracic aortic aneurysm) underwent thoracic endovascular aortic repair with fenestrated Castor stent-graft due to the existence of ILVA and unfavorable PLZ. Demographic characteristics, surgical details, postoperative complications, follow-up and postoperative CTA imaging results were collected and analyzed.ResultsThe primary technical success rate was 100%. The mean operation time was 115 min (range, 70–180 min). All the left subclavian arteries (LSAs) and ILVAs of the eight patients were revascularized by fenestrated Castor stent-grafts. During the follow-up period, no deaths and complications were observed. No internal leakage, aortic rupture, retrograde type A dissection were found on computed tomography angiography. All of the LSAs and ILVAs maintained patency without stenosis.ConclusionCastor single-branched stent-graft implantation combined with fenestration technique may be safe and feasible for TAD patients with ILVA and unfavorable PLZ.

BackgroundWe studied acute lung injury (ALI) in thoracic aortic disease (TAD) patients and investigated the predictive effect of interleukin-6 (IL-6) in acute lung injury after thoracic aortic disease.MethodsData on 188 TAD patients, who underwent surgery between January 2016 to December 2021 at our hospital, were enrolled in. We analyzed acute lung injury using two patient groups. Patients with No-ALI were 65 and those with ALI were 123. Univariate logistic, LASSO binary logistic regression model and multivariable logistic regression analysis were performed for acute lung injury.ResultsPreoperative IL-6 level was lower (15.80[3.10,43.30] vs. 47.70[21.40,91.60] pg/ml, p 18 pg/ml (area under the curve: AUC = 0.727). Univariate logistic regression analysis showed 19 features for TAD appeared to be early postoperative risk factors of acute lung injury. Using LASSO binary logistic regression, 19 features were reduced to 9 potential predictors (i.e., Scrpost + PLTpost + CPB > 182 min + D-dimerpost + D-dimerpre + Hypertension + Age > 58 years + IL6 > 18 pg/ml + IL6). Multivariable logistic regression analysis showed that Postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml were early postoperative risk factors for ALI after TAD, and the odds ratios (ORs) of postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml were 1.006 (1.002–1.01), 4.717 (1.306–19.294) and 2.96 (1.184–7.497), respectively. When postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml (AUC = 0.819), the 95% confidence interval [CI] was 0.741 to 0.898. Correction curves were nearly diagonal, suggesting that the nomogram fit well. The DCA curve was then drawn to demonstrate clinical applicability. The DCA curve showed that the threshold probability of a patient is in the range of 30% to 90%.ConclusionsThe inclusion of interleukin-6 demonstrated good performance in predicting ALI after TAD surgery.

Background: Both bicuspid aortic valve (BAV) and bovine aortic arch (BA) are considered as markers of thoracic aortic disease (TAD). But the association between them is not yet clear. This study aimed to explore the potential association of BAV and BA with TAD. Methods: The study involved 449 participants who underwent their first aortic valve replacement in Fuwai Hospital from June 2017 to March 2018. All patients underwent multidetector computed tomography and echocardiography before surgery. The clinical characteristics were recorded to analyze the association between BAV, BA, and TAD. The univariate and multivariate logistic regression analyses were applied to identify the risk factors for TAD. Results: BA accounted for 79.8% of the arch variants and was the most common aortic arch branching variant. BAV was present in 52.6% of the patients with BA and 38.1% of the patients with normal arch (NA). Among the 185 patients in the BAV subgroup, 50 had BA and 135 had NA. No significant differences were found in BAV anatomical phenotype, aortopathy phenotype, and valve function between BA and NA. The multivariate analysis showed that the presence of BAV and male sex were the risk predictors of TAD. BA was not a risk factor for TAD in either univariate or multivariate analysis. Conclusions: The proportion of BAV in patients with BA was significantly higher than that of NA, but the BAV phenotype and aortopathy were not related to BA. BAV was a risk factor for TAD, whereas BA was not associated with TAD. [ABSTRACT FROM AUTHOR]

Advances in both imaging techniques and genetics have led to the recognition of a wide variety of aortic anomalies that can be grouped under the term ‘hereditary thoracic aortic diseases’. The present review aims to summarize this very heterogeneous population’s clinical, genetic, and imaging characteristics and to discuss the implications of the diagnosis for clinical counselling (on sports activity or pregnancy), medical therapies and surgical management.

ObjectiveTo present our experience of preserving the isolated left vertebral artery (ILVA) with physician-modified fenestration (PM-F) or in situ fenestration (ISF) during thoracic endovascular aortic repair (TEVAR) for aortic pathologies involving aortic arch.MethodsThis is a single-center, retrospective, observational cohort study. Between June 2016 and December 2021, 9 patients (8 men; median age 60.0 years old) underwent TEVAR with ILVA reconstruction (PM-F, n = 6; ISF, n = 3) were identified and analyzed.ResultsThe technical success rate was 100%. No early (

Background: Open surgical repair (OSR) for TAAs is associated with a high perioperative morbidity and mortality, depending on the extent of repair. Also, because of the high mortality rates associated with OSR; stable patients with uncomplicated type B AD frequently obtain medical treatment. The aim of this work is evaluation the efficacy and predictors of outcomes of early thoracic endovascular aortic repair in patients with uncomplicated type B aortic dissection and patients with thoracic aortic aneurysms. Methods: A total of 25 patients with uncomplicated type B AD and descending thoracic aortic aneurysm who underwent endovascular aortic repair in National Heart Institute in the period between 2015 and 2017 were included in the study. Results: Of the 25 patients, 5 patients had a descending thoracic aortic aneurysm, and 20 patients had uncomplicated type B AD, the total mortality rate was 8 % throughout the follow-up period. Procedure related complications were observed in 8 % of the study population, where 2 patients developed type I endoleak, which resolved spontaneously during the follow up period. Both death and endoleak occurred in subacute and chronic cases, while using TEVAR in acute AD and aortic aneurysms showed no complications. Conclusion: Early thoracic endovascular aortic repair, along with optimal medical therapy, in uncomplicated type B aortic dissections and thoracic aortic aneurysms is associated with better outcome. [ABSTRACT FROM AUTHOR]

As it remains unclear whether there are sex-based differences in clinical outcomes after thoracic endovascular aortic repair (TEVAR), this meta-analysis aimed to evaluate differences in early outcomes and overall survival between female and male patients who underwent TEVAR. The PubMed, Embase, Web of Science, and Cochrane Central databases were searched for eligible studies published through June 10, 2023, that reported sex-based differences in clinical outcomes after TEVAR. The primary outcome was operative mortality; second outcomes included stroke, spinal cord ischemia, acute kidney injury, hospital length of stay, and overall survival. Patient characteristics, operative data, and early outcomes were aggregated using the random-effects model, presenting pooled risk ratio (RR) or standardized mean difference along with their corresponding 95% confidence intervals (CIs). Overall survival was assessed by reconstructing individual patient data to generate sex-specific pooled Kaplan-Meier curves. This study was registered in PROSPERO (CRD42023426069). Of the 1785 studies retrieved, 14 studies met all eligibility criteria, encompassing a total of 17,374 patients, comprising 5026 female and 12,348 male patients. Female patients were older, had a smaller maximum aortic diameter, had lower rates of smoking and coronary artery disease, and had higher rates of anemia. Intraoperatively, female patients were more likely to use iliac conduits and require blood transfusions. There were no sex-based differences in operative mortality (RR: 1.12, 95% CI: 0.90-1.40; P =.309), stroke (RR: 1.14, 95% CI: 0.95-1.38; P =.165), spinal cord ischemia (RR: 1.33, 95% CI: 0.83-2.14; P =.234), acute kidney injury (RR: 0.78, 95% CI: 0.52-1.17; P =.228), and hospital length of stay (standardized mean difference: 0.09, 95% CI: −0.03 to 0.20; P =.141). Pooled Kaplan-Meier estimates showed a worse overall survival in female patients compared with male patients (87.2% vs 89.8% at 2 years, log-rank P =.001). Among patients treated by TEVAR, female sex was not associated with increased risk of operative mortality or major morbidity. However, female patients exhibited a lower overall survival after TEVAR compared with male patients. [ABSTRACT FROM AUTHOR]

Abstract Background The application of cardiovascular magnetic resonance angiography (CMRA) for the assessment of thoracic aortic disease is often associated with prolonged and unpredictable acquisition times and residual motion artefacts. To overcome these limitations, we have integrated undersampled acquisition with image-based navigators and inline non-rigid motion correction to enable a free-breathing, contrast-free Cartesian CMRA framework for the visualization of the thoracic aorta in a short and predictable scan of 3 min. Methods 35 patients with thoracic aortic disease (36 ± 13y, 14 female) were prospectively enrolled in this single-center study. The proposed 3D T2-prepared balanced steady state free precession (bSSFP) sequence with image-based navigator (iNAV) was compared to the clinical 3D T2-prepared bSSFP with diaphragmatic-navigator gating (dNAV), in terms of image acquisition time. Three cardiologists blinded to iNAV vs. dNAV acquisition, recorded image quality scores across four aortic segments and their overall diagnostic confidence. Contrast ratio (CR) and relative standard deviation (RSD) of signal intensity (SI) in the corresponding segments were estimated. Co-axial aortic dimensions in six landmarks were measured by two readers to evaluate the agreement between the two methods, along with inter-observer and intra-observer agreement. Kolmogorov–Smirnov test, Mann–Whitney U (MWU), Bland–Altman analysis (BAA), intraclass correlation coefficient (ICC) were used for statistical analysis. Results The scan time for the iNAV-based approach was significantly shorter (3.1 ± 0.5 min vs. 12.0 ± 3.0 min for dNAV, P = 0.005). Reconstruction was performed inline in 3.0 ± 0.3 min. Diagnostic confidence was similar for the proposed iNAV versus dNAV for all three reviewers (Reviewer 1: 3.9 ± 0.3 vs. 3.8 ± 0.4, P = 0.7; Reviewer 2: 4.0 ± 0.2 vs. 3.9 ± 0.3, P = 0.4; Reviewer 3: 3.8 ± 0.4 vs. 3.7 ± 0.6, P = 0.3). The proposed method yielded higher image quality scores in terms of artefacts from respiratory motion, and non-diagnostic images due to signal inhomogeneity were observed less frequently. While the dNAV approach outperformed the iNAV method in the CR assessment, the iNAV sequence showed improved signal homogeneity along the entire thoracic aorta [RSD SI 5.1 (4.4, 6.5) vs. 6.5 (4.6, 8.6), P = 0.002]. BAA showed a mean difference of

Pathogenic variants in ACTA2, encoding smooth muscle α‐actin, predispose to thoracic aortic aneurysms and dissections. ACTA2 variants altering arginine 179 predispose to a more severe, multisystemic disease termed smooth muscle dysfunction syndrome (SMDS; OMIM 613834). Vascular complications of SMDS include patent ductus arteriosus (PDA) or aortopulmonary window, early‐onset thoracic aortic disease (TAD), moyamoya‐like cerebrovascular disease, and primary pulmonary hypertension. Patients also have dysfunction of other smooth muscle‐dependent systems, including congenital mydriasis, hypotonic bladder, and gut hypoperistalsis. Here, we describe five patients with novel heterozygous ACTA2 missense variants, p.Arg179Gly, p.Met46Arg, p.Thr204Ile, p.Arg39Cys, and p.Ile66Asn, who have clinical complications that align or overlap with SMDS. Patients with the ACTA2 p.Arg179Gly and p.Thr204Ile variants display classic features of SMDS. The patient with the ACTA2 p.Met46Arg variant exhibits exclusively vascular complications of SMDS, including early‐onset TAD, PDA, and moyamoya‐like cerebrovascular disease. The patient with the ACTA2 p.Ile66Asn variant has an unusual vascular complication, a large fusiform internal carotid artery aneurysm. The patient with the ACTA2 p.Arg39Cys variant has pulmonary, gastrointestinal, and genitourinary complications of SMDS but no vascular manifestations. Identifying pathogenic ACTA2 variants associated with features of SMDS is critical for aggressive surveillance and management of vascular and nonvascular complications and delineating the molecular pathogenesis of SMDS. [ABSTRACT FROM AUTHOR]

Background: The incidence and prognosis of aortoesophageal fistula (AEF) has not been clarified. The clinical characteristics and surgical outcomes of AEF were investigated., Methods: The clinical data of patients who underwent surgical treatment for AEF from January 2020 to December 2021 that were registered in the Japan Cardiovascular Surgery Database (JCVSD) were analyzed., Results: During the period, 123 patients (71.0 [IQR: 61.0-78.0] years old; 76.4% men) underwent surgical treatment for AEF. The prevalence of secondary AEF was 61%. Secondary AEF after aortic grafting was the most frequent (n = 40; 32.5%), followed by AEF after thoracic endovascular aortic repair (TEVAR) (n = 30; 24.4%). Operative mortality was observed in 23 patients (18.7%). TEVAR for AEF (p = 0.019), postoperative bleeding (p = 0.047), stroke (p = 0.004), renal failure (p < 0.001), newly required hemodialysis (p = 0.023), pneumonia (p = 0.003), multisystem failure (p < 0.001), and dyslipidemia (p = 0.02) were associated with risk factors of operative mortality after surgical treatment of AEF on univariable logistic regression analyses., Conclusions: This first nationwide study on the surgical treatment for AEF demonstrated a higher incidence of secondary AEF than primary AEF. Both open surgical repair and TEVAR for AEF were associated with high operative mortality. TEVAR and dyslipidemia were risk factors for operative mortality. Precautions and further improved treatment strategies for AEF are still required., (Copyright © 2024. Published by Elsevier Inc.)

To provide an adequate proximal landing zone, left subclavian artery (LSA) reconstruction has become an important part of thoracic endovascular aortic repair (TEVAR). This study evaluates the short and medium term efficacy of a novel unibody single-branched stent graft for zone 2 TEVAR. Fifty-two patients (mean age, 56 ± 10.9 years; 42 men) with distal aortic arch lesions requiring LSA reconstruction received unibody single-branched stents from September 2019 to March 2021. Computed tomography angiography was performed 6, 12, and 24 months after surgery to observe stent morphology, branch patency, endoleaks, stent-related adverse events, and changes in the diameter of true and false lumens. All stents were deployed adequately, and the technical success rate was 100%. The mean operation time was 121.8 ± 47.0 min. The mean postoperative hospital stay was 6.2 ± 3.7 days, and the mean follow-up was 16.8 ± 5.2 months (range, 12–24 months). During follow-up, there were no deaths and complications such as stent displacement or fracture, stenosis, fracture, occlusion, and type Ia endoleaks. The patency rate of the branched segment was 100%. In 42 patients with aortic dissection (AD), the true lumen diameter of the aortic isthmus was 29.4 ± 2.9 mm after surgery, significantly larger than before surgery (20.6 ± 5.4 mm, P < 0.05). Postoperative aortic isthmus false lumen diameter was significantly smaller than that before operation (6.1 ± 5.2 mm vs. 16.0 ± 7.6 mm, P < 0.05). The new unibody single-branched stent for zone 2 TEVAR is safe and accurate, and its efficacy is good in the short and medium term.

Abstract Background Left subclavian artery revascularization (LSA) is frequently performed in the setting of thoracic endovascular repair (TEVAR). The purpose of this study was to compare different techniques for LSA revascularization during TEVAR. Methods We performed a single center’s retrospective cohort study from 2016 to 2019. Patients were categorized by LSA revascularization methods, including direct coverage without revascularization (Unrevascularized), carotid-subclavian bypass (CSB), fenestrated TEVAR (F-TEVAR). Indications, demographics, operation details, and outcomes were analyzed using standard statistical analysis. Results 171 patients underwent TEVAR with LSA coverage, 16.4% (n = 28) were unrevascularized and the remaining patients underwent CSB (n = 100 [58.5%]) or F-TEVAR (n = 43 [25.1%]). Demographics were similar between the unrevascularized and revascularized groups, except for procedure urgent status (p = 0.005). The incidence of postoperative spinal cord ischemia was significantly higher between unrevascularized and revascularized group (10.7% vs. 1.4%; p = 0.032). There was no difference in 30-day and mid-term rates of mortality, stroke, and left upper extremity ischemia. CSB was more likely time-consuming than F-TEVAR [3.25 (2.83–4) vs. 2 (1.67–2.67) hours, p = 0], but there were no statistically significant differences in 30-day or midterm outcomes for CSB versus F-TEVAR. During a mean follow-up time of 24.8 months, estimates survival rates had no difference. Conclusions LSA revascularization in zone 2 TEVAR is necessary which is associated with a low 30-day rate of spinal cord ischemia. When LSA revascularization is required during TEVAR, CSB and F-TEVAR are all safe and effective methods, and F-TEVAR appears to offer equivalent clinical outcomes as a less time-consuming and minimally invasive alternative.

Thoracic aortic aneurysms are silent yet deadly clinical entities which may elude detection until an acutely life-threatening aortic dissection or rupture occurs. Approximately 20% of patients with thoracic aortic aneurysms or dissection have a positive family history, indicating a strong genetic component to the aetiology. Genetic screening in such hereditary thoracic aortic disease (HTAD) may thus be beneficial in detecting causative genetic mutations in affected patients, identifying asymptomatic family members who may be at risk, and in guiding the optimal timing of preventative surgery in those with confirmed genetic aortopathy. Genetic screening can facilitate personalised aortic care tailored to an individual's specific genetic abnormality, with the aim of mitigating the significant morbidity burden and premature mortality associated with HTAD. This review examines the rationale for genetic screening in HTAD, its potential applications, current limitations and potential future directions. [ABSTRACT FROM AUTHOR]

Despite the recent technical developments, surgery on the thoracic aorta remains challenging and is associated with significant mortality and morbidity. Decisions about when and if to operate are based on a balance between surgical risk and the hazard of aortic rupture. These decisions are sometimes difficult in elective cases of thoracic aortic diseases, including aneurysms and dissections. Abnormal wall stress derived from flow alterations influences disease progression. Therefore, a better understanding of the complex hemodynamic environment inside the aortic lumen will facilitate patient-specific risk assessments of complications, which enable clinicians to provide timely prophylactic interventions. Time-resolved 3D phase-contrast (4D flow) MRI has many advantages for the in vivo assessment of flow dynamics. Recent developments in 4D flow imaging techniques has led to significant advances in our understanding of physiological flow dynamics in healthy subjects and patients with thoracic aortic diseases. In this clinically focused review of thoracic aortic diseases, we demonstrate the clinical advances acquired with 4D flow MRI from published studies. We provide a systematic overview of key evidences and considerations regarding normal thoracic aortas, thoracic aortic aneurysms, aortic dissections, and thoracic aortas with prosthetic graft replacement. [ABSTRACT FROM AUTHOR]

Background: The application of cardiovascular magnetic resonance angiography (CMRA) for the assessment of thoracic aortic disease is often associated with prolonged and unpredictable acquisition times and residual motion artefacts. To overcome these limitations, we have integrated undersampled acquisition with image-based navigators and inline non-rigid motion correction to enable a free-breathing, contrast-free Cartesian CMRA framework for the visualization of the thoracic aorta in a short and predictable scan of 3 min. Methods: 35 patients with thoracic aortic disease (36 ± 13y, 14 female) were prospectively enrolled in this single-center study. The proposed 3D T2-prepared balanced steady state free precession (bSSFP) sequence with image-based navigator (iNAV) was compared to the clinical 3D T2-prepared bSSFP with diaphragmatic-navigator gating (dNAV), in terms of image acquisition time. Three cardiologists blinded to iNAV vs. dNAV acquisition, recorded image quality scores across four aortic segments and their overall diagnostic confidence. Contrast ratio (CR) and relative standard deviation (RSD) of signal intensity (SI) in the corresponding segments were estimated. Co-axial aortic dimensions in six landmarks were measured by two readers to evaluate the agreement between the two methods, along with inter-observer and intra-observer agreement. Kolmogorov–Smirnov test, Mann–Whitney U (MWU), Bland–Altman analysis (BAA), intraclass correlation coefficient (ICC) were used for statistical analysis. Results: The scan time for the iNAV-based approach was significantly shorter (3.1 ± 0.5 min vs. 12.0 ± 3.0 min for dNAV, P = 0.005). Reconstruction was performed inline in 3.0 ± 0.3 min. Diagnostic confidence was similar for the proposed iNAV versus dNAV for all three reviewers (Reviewer 1: 3.9 ± 0.3 vs. 3.8 ± 0.4, P = 0.7; Reviewer 2: 4.0 ± 0.2 vs. 3.9 ± 0.3, P = 0.4; Reviewer 3: 3.8 ± 0.4 vs. 3.7 ± 0.6, P = 0.3). The proposed method yielded higher image quality scores in terms of artefacts from respiratory motion, and non-diagnostic images due to signal inhomogeneity were observed less frequently. While the dNAV approach outperformed the iNAV method in the CR assessment, the iNAV sequence showed improved signal homogeneity along the entire thoracic aorta [RSD SI 5.1 (4.4, 6.5) vs. 6.5 (4.6, 8.6), P = 0.002]. BAA showed a mean difference of < 0.05 cm across the 6 landmarks between the two datasets. ICC showed excellent inter- and intra-observer reproducibility. Conclusions: Thoracic aortic iNAV-based CMRA with fast acquisition (~ 3 min) and inline reconstruction (3 min) is proposed, resulting in high diagnostic confidence and reproducible aortic measurements. [ABSTRACT FROM AUTHOR]

Chronic cough has a broad differential, and thoracic aortic aneurysm (TAA) is a rare but potentially life-threatening etiology. We present a giant arch TAA in a non-dyspneic, Pacific Islander man with significant tobacco-use history who presented with chronic cough with no acute pulmonary process noted on imaging. Given the high mortality rates associated with thoracic aortic aneurysms, the purpose of this report is to highlight the importance of keeping TAA as a rare differential for chronic cough, particularly when caring for patients with elevated risk. Recognition of patients with thoracic aortic disease who have a class I indication for surgical intervention (meaning there is evidence or general agreement that surgery will be beneficial, useful, and effective) as well as prompt evaluation of their anatomical landmarks in the perioperative period is critical. Imaging and, in particular, computed tomography remain the optimal modalities to screen for thoracic aortic disease. [ABSTRACT FROM AUTHOR]

OBJECTIVE: We aim to present a novel surgical technique of Frozen Elephant Trunk (FET) to treat complex thoracic aortic diseases in one stage and report its short-term outcomes. PATIENTS AND METHODS: Between December 2019 and 30 April 2021, twenty-five patients underwent FET operation at Viet Duc University Hospital. The mean age of the patients was 55.9 (±9.9, range 33-72) years. Eighteen (72%) of the patients were men. Thoracic aortic aneurysm was presented in three (12%) patients. Among seventeen (68%) of the patients undergoing the aortic dissection, eleven (44%) were treated acute type A aortic dissection. Type A intramural hematoma was presented in three (12%) patients. Four (16%) of the patients had undergone previous aortic operations, four (16%) of them had Marphan syndrome and two (11.1%) of them had stage 3 chronic kidney disease. All patients underwent FET procedure by unique protocol. Brain protection was achieved by antegrade bilateral selective cerebral perfusion and moderate hypothermia (28°C) in all cases; besides cerebral tissue oximetry monitoring was used to control brain oxygenation. RESULTS: There were no perioperative deaths, and all patients are still alive during mild-term follow-up period. Sixteen (88.9%) patients received isolated FET, while a Bentall procedure during FET was performed in two (8%) patients and right coronary artery bypass was in one (4%) case. The duration of cardiopulmonary bypass, cross-clamping, circulatory arrest, and total operation were 176.7 (±48.1, range 102-330), 106 (±39.8, range 63-205), 32.7 (±9.6, range 20-58), and 365.6 (±53.6, range 270-480) min, respectively. There was no bleeding following surgery. Prolonged ventilation required tracheotomy was documented in two (8%) patients, hemodialysis caused acute renal failure was in five (20%) patients, cerebral shock was in one (4%) patient, and type 1A endoleak in 2 (8%) patients. CONCLUSIONS: Our modification of FET technique was feasible, effective, and safe, with good postoperative outcomes. [ABSTRACT FROM AUTHOR]

Background: The best approach for aortic root disease remains controversial. Composite valve-graft conduit (CVG) replacement offers good results at short-term and long-term follow-up; on the other hand, valve-sparing aortic root replacement (VSARR) has proven to be an excellent treatment alternative. This study aimed to analyse the outcomes after VSARR and compare whether preoperative moderate or severe aortic regurgitation (AR) and or the need for aortic valve repair (AVR) during this procedure influenced survival and freedom from reoperation rates.Methods: From September 2005 to June 2018, 104 patients underwent VSARR using the reimplantation technique: 64% presented with preoperative moderate or severe AR, concomitant AVR was performed in 43.3%, Marfan syndrome was present in 16.3%, and 12.5% had a bicuspid aortic valve. Complete follow-up was obtained in 91% of the sample, echocardiographic results were available for 86% and the mean follow-up time was 1,893 days.Results: In-hospital mortality was 2.9% and one death occurred 42 days after hospital discharge. In the latest echocardiographic assessment, 88.3% presented with mild AR or better. Freedom from reoperation at 8 years was 95.4%. There was no case of endocarditis and one patient had a stroke 2 years after the operation. There were no between-group differences in morbidity, mortality and complications during the follow-up.Conclusion: VSARR can be performed with low mortality rates and reasonable durability of the aortic valve. Neither moderate or severe AR nor the need for aortic valve repair during the procedure altered survival and freedom from reoperation. [ABSTRACT FROM AUTHOR]

Objective: With an aging population and advancements in imaging, recurrence of thoracic aortic dissection is becoming more common., Methods: All patients enrolled in the International Registry of Aortic Dissection from 1996 to 2023 with type A and type B acute aortic dissection were identified. Among them, initial dissection and recurrent dissection were discerned. The study period was categorized into 3 eras: historic era, 1996 to 2005; middle era, 2006 to 2015; most recent era, 2016 to 2023. Propensity score matching was applied between initial dissection and recurrent dissection. Outcome of interests included long-term survival and cumulative incidence of major aortic events defined by the composite of reintervention, aortic rupture, and new dissection., Results: The proportion of recurrent dissection increased from 5.9% in the historic era to 8.0% in the most recent era in the entire dissection cohort. In patients with type A dissection, propensity score matching between initial dissection and recurrent dissection yielded 326 matched pairs. Kaplan-Meier curves showed similar long-term survival between the 2 groups. However, the cumulative incidence of major aortic events was significantly higher in the recurrent dissection group (40.3% ± 6.2% vs 17.8% ± 5.1% at 4 years in the initial dissection group, P = .02). For type B dissection, 316 matched pairs were observed after propensity score matching. Long-term survival and the incidence of major aortic events were equivalent between the 2 groups., Conclusions: The case volume of recurrent dissection or the ability to detect recurrent dissection has increased over time. Acute type A recurrent dissection was associated with a higher risk of major aortic events than initial dissection. Further judicious follow-up may be crucial after type A recurrent dissection., Competing Interests: Conflict of Interest Statement G.J.A. receives consulting fees from Terumo Aortic. I.S. receives institutional research support from Abbott, AtriCure, Artivion, Boston Scientific, Edwards, Medtronic, and Terumo Aortic. None were related to this manuscript. All other authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Thoracic aortic disease (TAD) is often silent until a life-threatening complication occurs. However, genetic information can inform both identification and treatment at an early stage. Indeed, a diagnosis is important for personalised surveillance and intervention plans, as well as cascade screening of family members. Currently, only 20% of heritable TAD patients have a causative mutation identified and, consequently, further advances in genetic coverage are required to define the remaining molecular landscape. The rapid expansion of next generation sequencing technologies is providing a huge resource of genetic data, but a critical issue remains in functionally validating these findings. Induced pluripotent stem cells (iPSCs) are patient-derived, reprogrammed cell lines which allow mechanistic insights, complex modelling of genetic disease and a platform to study aortic genetic variants. This review will address the need for iPSCs as a frontline diagnostic tool to evaluate variants identified by genomic discovery studies and explore their evolving role in biological insight through to drug discovery., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Singh, Shetty, Jacob, Bayraktar and Sinha.)

Open in new tab Download slide Open in new tab Download slide The aim of this study was to investigate whether the 'bovine' arch [i.e. arch variant with a common origin of the innominate and left carotid artery (CILCA)] is associated with an increased risk of thoracic aortic disease (TAD). The study was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The PubMed, EMBASE and Cochrane databases were searched to identify all case series reporting about CILCA arch and TAD between January 2008 and December 2018. A total of 485 studies were screened. The prevalence of CILCA arch was assessed, and data analysis was performed considering the difference in the risk of TAD for presence versus absence of CILCA arch. Eight studies enrolling 11 381 subjects were retrieved for quantitative analysis. The proportion of TAD among CILCA arch patients was higher [41.5% (28.1–56.4)] than the proportion among patients with standard arch configuration 34.0% (20.1–51.4). The odds ratio of developing TAD was 1.4 times higher in subjects with CILCA arch (95% confidence interval 1.068–1.839). The test for an overall effect indicated a significant association between CILCA arch and TAD (P  < 0.015). The I 2 was 78.1% with a value of P < 0.001 for heterogeneity. The Egger test did not show evidence of publication bias (P  = 0.317). In conclusion, our meta-analysis supports the hypothesis of a correlation between the presence of CILCA arch and the onset of TAD. Our results warrant a specific and long-term surveillance for patients with this anatomical variant, and a thorough awareness of its potential clinical implications during image interpretation. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES Our goal was to identify anatomical and physiological factors that could predict the amount of cerebrospinal fluid (CSF) drainage in patients undergoing elective endovascular repair of descending thoracic and thoracoabdominal aortic disease. METHODS All consecutive elective endovascular procedures performed for descending thoracic or thoracoabdominal aortic disease between January 2015 and December 2017 were included in the study. Routine use of CSF drainage was established in all patients. The goal of drainage was to reach a spinal fluid pressure of 10–12 mmHg by draining in 5–15-ml aliquots. The number of visible intercostal and lumbar segmental arteries (SAs) was evaluated before and after endovascular repair. The covering ratio of SAs was calculated as covered preoperative SAs/total preoperative SAs. RESULTS Twenty-four consecutive patients were included in the final analysis. The indication for the intervention was a descending thoracic aneurysm in 13 cases, a thoracoabdominal aneurysm in 4 cases and a chronic type B dissection in 7 cases. The procedure performed was thoracic endovascular aortic repair in 20 cases and fenestrated endovascular aneurysm repair in 4 cases. None of the patients developed spinal cord ischaemia. The mean volume of CSF drained was 46 ml. The mean length of aortic coverage was 231 mm. The mean number of total preoperative SAs and of covered preoperative SAs was, respectively, 22 and 9. The volume of CSF drained was significantly correlated with all these variables (length of aortic coverage, total visible SAs and covered SAs), but the most powerful correlation was individuated with the covering ratio of SAs. CONCLUSIONS Our findings suggest that the percentage of intercostal and lumbar SAs covered by placement of a stent graft can predict the volume of CSF drained in patients undergoing elective endovascular repair of descending thoracic and thoracoabdominal aortic disease. [ABSTRACT FROM AUTHOR]

This review provides a general overview of the consensus statement from the 2010 more recent updates AHA/ACC Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease, and highlights current practice patterns. The suggestions herein are intended to facilitate clinical decision making in the management of thoracic aortic disease. The main intent of this report is to highlight screening, surveillance, initial and definitive management of thoracic aortic disease, and special populations that should be considered. [ABSTRACT FROM AUTHOR]

Surgery on the arch or descending aorta is associated with significant risk of neurological complications. As a consequence of intubation and sedation, early neurologic injury may remain unnoticed. Biomarkers to aid in the initial diagnostics could prove of great value as immediate intervention is critical. Twenty-three patients operated in the thoracic aorta with significant risk of perioperative neurological injury were included. Cerebrospinal fluid (CSF) and serum were obtained preoperatively and in the first and second postoperative days and assessed with a panel of 92 neurological-related proteins. Three patients suffered spinal cord injury (SCI), eight delirium, and nine hallucinations. There were markers in both serum and CSF that differed between the affected and non-affected patients (SCI; IL6, GFAP, CSPG4, delirium; TR4, EZH2, hallucinations; NF1). The study identifies markers in serum and CSF that reflect the occurrence of neurologic insults following aortic surgery, which may aid in the care of these patients. [ABSTRACT FROM AUTHOR]

Purpose: To analyze the results of isolated left subclavian artery (LSA) revascularization during thoracic endovascular aortic repair (TEVAR) using carotid-subclavian bypass (CSbp) or chimney grafts (CGs).Methods: A retrospective multicenter, observational study identified 73 patients (mean age 68±13 years, range 22-87; 56 men) with acute or chronic thoracic aortic lesions who underwent TEVAR with isolated LSA revascularization using either CSbp (n=42) or CGs (n=31) from January 2010 and February 2017. Primary endpoints were TEVAR-related mortality, postoperative stroke, freedom from type Ia endoleak, and LSA patency.Results: Primary technical success was achieved in all cases. Early TEVAR-related mortality was 4.2% (CSbp 2% vs CG 6%, p=0.571). Two (3%) patients had major ischemic strokes (one in each group). Mean follow-up was 24±21 months (range 1-72; median 15). Estimated freedom from TEVAR-related mortality was 93%±3% (95% CI 84.3% to 97.0%) at 12 and 36 months, with no significant difference between CSbp and CG (p=0.258). Aortic reintervention did not differ between the groups (CSbp 5% vs CG 6%, p=0.356); nor did freedom from type Ia endoleak (CSbp 98% vs CG 87%, p=0.134). Gutter-related endoleaks occurred in 4 (13%) CG patients, but none of the patients experienced sac enlargement or the need for reintervention and none died. Primary patency of the LSA was 100% for the entire group during the observation period.Conclusion: In our experience, LSA revascularization proved most satisfactory and equally effective with both the CSbp and CG techniques, without discernible differences at midterm follow-up. [ABSTRACT FROM AUTHOR]

The impact of sex on outcomes of thoracic endovascular aortic repair (TEVAR) represents an area of increased interest over the last decade, and long-term data are lacking. The aim of the present study was to investigate sex-related differences in long-term outcomes after TEVAR using real-world data from the Global Registry for Endovascular Aortic Treatment. Data were obtained retrospectively after querying the multicenter, sponsored Global Registry for Endovascular Aortic Treatment. Patients treated with TEVAR between December 2010 and January 2021 were selected regardless of the type of thoracic aortic disease. The primary outcome was sex-specific all-cause mortality at 5 years and maximum follow-up. Secondary outcomes were sex-specific all-cause mortality at 30 days and 1 year, and aorta-related mortality, major adverse cardiac events, neurological complications, and device-related complications or reinterventions at 30 days, 1 year, 5 years, and maximum follow-up. A total of 805 patients were analyzed; 535 (66.5%) were males. Females were older (median, 66 years [interquartile range (IQR), 57-75 years] vs 69 years [IQR, 59-78 years], P <.001). Males had more frequently a history of coronary artery bypass grafting and renal insufficiency (8.7% vs 3.7% [ P =.010] and 22.4% vs 11.6% [ P <.001]). The median follow-up was 3.46 years (IQR, 1.49-4.99 years) for males and 3.18 years (IQR, 1.29-4.86 years) for females. Indications for TEVAR were mostly descending thoracic aortic aneurysms (n = 307 [38.1%]) type B aortic dissections (n = 250 [31.1%]) or others (n = 248 [30.8%]). Freedom from 5-year all-cause mortality was similar for males and females (67% [95% CI, 62.1-72.2] vs 65.9% [95% CI, 58.5-74.2]; P =.847), and there were no differences in secondary outcomes. Multivariable Cox regression showed females to have lower all-cause mortality rates; however, this difference did not reach statistical significance (hazard ratio, 0.97; 95% CI, 0.72-1.30; P =.834). Additional subgroup analyses based on the indication for TEVAR did not identify differences between both sexes for the primary and secondary outcomes except more endoleak type II in females with complicated type B aortic dissection (1.8% vs 12.1%; P =.023). The present analysis suggests that long-term outcomes of TEVAR performed irrespective of the type of aortic disease are similar for males and females. Further studies are needed to clarify existing controversies regarding the impact of sex on outcomes of TEVAR. [ABSTRACT FROM AUTHOR]

Currently, total arch replacement (TAR) using frozen elephant trunk (FET) technique is gaining popularity for various thoracic aortic pathologies. FROZENIX is the first commercialized open stent-graft (OSG) in Japan, and it is a useful alternative to previously self-made OSG. FROZENIX is easy to deploy compared with formerly launched OSGs. However, some challenges have been identified with FROZENIX. Herein, we present the cases of two patients in whom we encountered serious unexpected kinking of FROZENIX during TAR performed using FET, which was related to its structural property. The clinical course and the bailout are described. [ABSTRACT FROM AUTHOR]

Objective: The main purpose of this study was to evaluate the safety and efficacy of Castor single-branched stent-graft combined with fenestrated technique in treatment of thoracic aortic disease (TAD) with unfavorable proximal landing area (PLZ) and isolated left vertebral artery (ILVA)., Methods: From January 2018 to March 2022, 8 patients with TAD (6 patients with type B aortic dissections, 1 patient with type B intramural hematomas, and 1 patient with thoracic aortic aneurysm) underwent thoracic endovascular aortic repair with fenestrated Castor stent-graft due to the existence of ILVA and unfavorable PLZ. Demographic characteristics, surgical details, postoperative complications, follow-up and postoperative CTA imaging results were collected and analyzed., Results: The primary technical success rate was 100%. The mean operation time was 115 min (range, 70-180 min). All the left subclavian arteries (LSAs) and ILVAs of the eight patients were revascularized by fenestrated Castor stent-grafts. During the follow-up period, no deaths and complications were observed. No internal leakage, aortic rupture, retrograde type A dissection were found on computed tomography angiography. All of the LSAs and ILVAs maintained patency without stenosis., Conclusion: Castor single-branched stent-graft implantation combined with fenestration technique may be safe and feasible for TAD patients with ILVA and unfavorable PLZ., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Wang, Fang, Song, Wei, Meng, Bai, Liu and Zhao.)

Background: We studied acute lung injury (ALI) in thoracic aortic disease (TAD) patients and investigated the predictive effect of interleukin-6 (IL-6) in acute lung injury after thoracic aortic disease., Methods: Data on 188 TAD patients, who underwent surgery between January 2016 to December 2021 at our hospital, were enrolled in. We analyzed acute lung injury using two patient groups. Patients with No-ALI were 65 and those with ALI were 123. Univariate logistic, LASSO binary logistic regression model and multivariable logistic regression analysis were performed for acute lung injury., Results: Preoperative IL-6 level was lower (15.80[3.10,43.30] vs. 47.70[21.40,91.60] pg/ml, p  < 0.001) in No-ALI group than in ALI group. The cut-off points, determined by the ROC curve, were preoperative IL-6 > 18 pg/ml (area under the curve: AUC = 0.727). Univariate logistic regression analysis showed 19 features for TAD appeared to be early postoperative risk factors of acute lung injury. Using LASSO binary logistic regression, 19 features were reduced to 9 potential predictors (i.e., Scrpost + PLTpost + CPB > 182 min + D-dimerpost + D-dimerpre + Hypertension + Age > 58 years + IL6 > 18 pg/ml + IL6). Multivariable logistic regression analysis showed that Postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml were early postoperative risk factors for ALI after TAD, and the odds ratios (ORs) of postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml were 1.006 (1.002-1.01), 4.717 (1.306-19.294) and 2.96 (1.184-7.497), respectively. When postoperative creatinine, CPB > 182 min and IL-6 > 18 pg/ml (AUC = 0.819), the 95% confidence interval [CI] was 0.741 to 0.898. Correction curves were nearly diagonal, suggesting that the nomogram fit well. The DCA curve was then drawn to demonstrate clinical applicability. The DCA curve showed that the threshold probability of a patient is in the range of 30% to 90%., Conclusions: The inclusion of interleukin-6 demonstrated good performance in predicting ALI after TAD surgery., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Li, Feng, Wang, Li, Zhu, Sun and Wu.)

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OBJECTIVES: Cerebral malperfusion for patients with acute type A aortic dissection (AAAD) remains an unsolved problem. The present study aimed to evaluate our management of cerebral perfusion and identify predictors of perioperative cerebral malperfusion in patients undergoing surgical repair of AAAD. METHODS: Between January 2004 and December 2015, 137 consecutive patients with AAAD underwent aortic replacement at Tsuchiya General Hospital. The status of the dissected supra-aortic branch vessels (SABVs) was classified as patent or thrombosis by preoperative computed tomographic angiography. Intraoperative cerebral perfusion was monitored by transcutaneous carotid echo and regional oxygen saturation. In cases with neurological symptoms or cerebral malperfusion, quick cerebral perfusion was immediately started using a quick cutdown technique. We assessed clinical outcomes, including mortality and complications, and analysed predictors of early mortality and cerebral malperfusion. RESULTS: The early mortality rate was 8.0%. Postoperative cerebral injury was observed in 4 patients (2.9%). Nineteen patients had perioperative cerebral malperfusion. There were no postoperative cerebral injuries in the patients in whom intraoperative cerebral malperfusion was corrected. Multivariable analysis revealed that preoperative shock (odds ratio [OR] 22.60, P < 0.0001) and extension of dissection to the abdominal aorta (OR 9.31, P = 0.0064) were significant risk factors for early mortality. Preoperative neurological symptoms (OR 12.40, P = 0.0006) and partial or complete thrombosis of the SABV (OR 64.10, P < 0.0001) were identified as independent predictors of perioperative cerebral malperfusion. CONCLUSIONS: Perioperative cerebral perfusion should be carefully managed, especially in the patients with preoperative neurological symptoms or partial or complete thrombosis of the SABV. [ABSTRACT FROM AUTHOR]

Copyright of Gefaesschirurgie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Thoracic aortic diseases, including aneurysms and dissections of the thoracic aorta, are a major cause of morbidity and mortality. Risk factors for thoracic aortic disease include increased hemodynamic forces on the ascending aorta, typically due to poorly controlled hypertension, and heritable genetic variants. The altered genes predisposing to thoracic aortic disease either disrupt smooth muscle cell (SMC) contraction or adherence to an impaired extracellular matrix, or decrease canonical transforming growth factor beta (TGF-β) signaling. Paradoxically, TGF-β hyperactivity has been postulated to be the primary driver for the disease. More recently, it has been proposed that the response of aortic SMCs to the hemodynamic load on a structurally defective aorta is the primary driver of thoracic aortic disease, and that TGF-β overactivity in diseased aortas is a secondary, unproductive response to restore tissue function. The engineering of mouse models of inherited aortopathies has identified potential therapeutic agents to prevent thoracic aortic disease. [ABSTRACT FROM AUTHOR]