Search Results (269)

Open surgical repair continues to play an essential role in the contemporary management of thoracoabdominal aortic aneurysms, despite the dramatic advances and growing adoption of endovascular aortic techniques, which include the combination of thoracic endovascular aortic repair and complex endovascular aortic repair with fenestrated and branched endovascular repair. The relative advantages and limitations of open vs. endovascular approaches must be carefully balanced in each patient. However, open repair of thoracoabdominal aortic aneurysms continues to provide unique advantages in selected patients. This manuscript presents a narrative review of the current literature, addressing both open surgical repair and endovascular approaches in repairing thoracoabdominal aortic aneurysms. Full article

Heritable thoracic aortic diseases (HTAD) are inherited conditions that increase the risk of thoracic aortic aneurysms, dissections, and premature aortic rupture. Advances in human genetics and experimental models have transformed our understanding of these disorders from a phenotype-based classification system to a mechanism-based view involving extracellular matrix (ECM) architecture, transforming growth factor-β (TGFβ) signaling, and vascular smooth muscle cell contractility. Marfan syndrome, Loeys–Dietz syndrome, and nonsyndromic HTAD demonstrate how genetic mutations can disrupt the components that stabilize the aortic wall. These pathogenic mechanisms influence matrix organization, intracellular signaling, and the contractile machinery within the mechanically stressed proximal aorta. In this review, we summarize current mechanistic insights into the major forms of HTAD and discuss how new molecular and cellular concepts could influence surveillance, genetic counseling, and genotype-guided therapeutic strategies. Full article

The aorta is the largest vascular conduit in humans, comprising three layers and a multitude of varying cell types collectively maintaining homeostasis and normal aortic wall function. Amongst these layers, the tunica adventitia is the external-most layer, where microvessels, termed vasa vasorum, can be found. These comprise pericytes and endothelial cells (ECs) and provide nourishment to the tunica adventitia and the outer media layers in the thoracic aorta. Adjacent to these microvessels, stem/progenitor group populations can be found, together forming a perivascular niche. Eventually, however, many of these cells and components can become dysregulated and contribute to development of thoracic aortic aneurysm (TAA). The purpose of this narrative review is to evaluate the recent literature related to marker gene expression in tunica adventitia pericytes, as well as the contribution of these populations to the development of aneurysm in the thoracic aorta. Pericytes in TAA generally exhibit phenotypic changes, which could be driven, in part, by loss of fibroblast growth factor (FGF) signaling. These changes eventually lead to vasa vasorum remodeling in the thoracic aorta, in turn contributing to the development of TAA. Full article

Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening vascular disease lacking therapies that target underlying cell death pathways. Ferroptosis, an iron-dependent form of lipid peroxidation-driven cell death, has emerged as a key mechanism in vascular remodeling. We investigated whether exogenous ketosis induced by ketone ester (KE) supplementation can suppress ferroptosis and prevent TAAD. TAAD was induced in C57BL/6 mice using β-aminopropionitrile (BAPN). A subset of these mice received KE [(R)-3-hydroxybutyl (R)-3-hydroxybutyrate, 20 g/L] in their drinking water starting on day 15 of the BAPN treatment. Human aortic smooth muscle cells (HASMCs) were treated with the GPX4 inhibitor Ras-Selective Lethal 3 (RSL3) and β-hydroxybutyrate (β-OHB) to investigate ferroptotic markers, lipid peroxidation, and labile iron levels. KE supplementation significantly reduced TAAD incidence (69% → 43%) and improved survival rate (52% → 73%), while preserving aortic structure and reducing elastic fiber fragmentation. Transcriptomic analyses of human TAAD datasets (GSE153434 and GSE52093) and single-cell RNA sequencing data (GSE155468) revealed ferroptosis signatures characterized by decreased GPX4 and increased expression of iron metabolism genes. Mechanistically, KE suppressed BAPN-induced iron accumulation and lipid peroxidation in vivo. In HASMCs, β-OHB inhibited ferroptosis induced by GPX4 inhibition, decreasing lipid peroxidation and labile iron levels. KE restored GPX4 and SLC7A11 expression while suppressing HO-1 in vivo, with effects dependent on Nrf2 signaling in vitro. In summary, ketone ester supplementation protects against TAAD by inhibiting VSMC ferroptosis via GPX4 induction and HO-1 suppression, highlighting a potential therapeutic strategy for aortic disease. Full article

Background: In 2020, the first pre-assembled bioprosthetic aortic valved conduit (AVC) was approved in the United States. This study compares its anatomic and functional outcomes to traditional hand-sewn composite conduits in patients undergoing aortic root replacement. Methods: This retrospective study compared 118 patients receiving the pre-assembled AVC (2021–2023) versus 66 patients with hand-sewn conduits (2012–2020) after elective bio-Bentall procedures. Primary outcomes were post-operative mortality and complication rates. Secondary outcomes included anatomic and hemodynamic changes. Graft dimensions were obtained from post-operative computed tomography (CT). Echocardiographic parameters were collected at early and late follow-up. Between-group differences and longitudinal changes were assessed using linear mixed-effects models. Results: Groups were comparable in age (pre-assembled 63 ± 11 vs. hand-sewn 64 ± 11 years) and predominantly male. Despite significantly higher concomitant hemiarch rates in pre-assembled conduits (91.5% vs. 28.8%, p < 0.001), 30-day mortality, stroke, and reoperation for bleeding were comparable between groups. Pre-assembled conduits demonstrated superior hemodynamics with lower baseline peak gradients (Δ 9.1 mmHg, p < 0.001), lower mean gradients (Δ 5.3 mmHg, p < 0.001), and larger indexed effective orifice area (Δ 0.27 cm²/m², p = 0.018). Annual rates of hemodynamic and dimensional change were minimal and comparable between groups. Kaplan–Meier analysis showed no survival difference at 3 years. Conclusions: The pre-assembled AVC demonstrates equivalent safety and superior early hemodynamic performance compared to hand-sewn conduits, with stable mid-term anatomic and functional outcomes. Full article

Objectives: Several measurement techniques have been proposed to address the non-circular geometry of the aortic root. The Laplace diameter metric incorporates the cloverleaf anatomy of the aortic root and is derived via measurement of sinus-to-commissure lengths with subsequent doubling of the largest radius from the center. This study compares the conventional sinus-to-sinus with the novel Laplace method for sizing the aortic root and quantifying its implication on surgical decision-making. Methods: Patients undergoing surveillance at a high-volume aortic center were categorized by aortic root morphology as nondilated, non-syndromic dilated, bicuspid aortic valve and Marfan syndrome. Aortic root diameters by sinus-to-sinus and Laplace diameter methods were measured on computed tomography, compared using paired t-tests, and correlated using Spearman rank coefficients. Results: Of the 1297 patients assessed, 530 were included in the final analysis (nondilated n = 113, non-syndromic dilated n = 347, bicuspid aortic valve n = 50, Marfan syndrome n = 17). Aortic root diameters were significantly larger by Laplace than sinus-to-sinus diameter across all groups (sinus-to-sinus: 1.9 ± 5.5 mm; Laplace: 44.9 ± 7.0 mm; 95% confidence interval 2.72–3.34; p < 0.0001). Although Laplace and sinus-to-sinus diameter were correlated (Spearman r = 0.6789, 95% CI 0.6–0.7; p < 0.0001), the relationship was non-linear (R² = 0.492). Laplace diameter increased the proportion of patients meeting surgical thresholds (2022 AHA/ACC guidelines) versus sinus-to-sinus: nondilated 0% vs. 1.77%, non-syndromic dilated 4.9% vs. 25.1%, bicuspid aortic valve 10.0% vs. 26.0%, and Marfan syndrome 23.5% vs. 52.9%. Conclusions: On average, Laplace diameter exceeded sinus-to-sinus diameter by 3 mm and would extend surgical eligibility to an additional 21% of patients under current guidelines. Full article

Electrocardiogram (ECG)-gated contrast-enhanced computed tomography angiography (CTA) is the reference method for follow-up of ascending aortic aneurysms but delivers substantially higher radiation doses than ECG-gated non-contrast CT (NCCT). NCCT can be acquired at a lower dose while enabling measurements of the aortic outer diameter. This study aimed to quantify the radiation dose of both techniques and determine whether a significant difference exists in ascending thoracic aorta diameter measurements between NCCT and CTA. Eighty patients who underwent ECG-gated cardiac CT for suspected coronary artery disease were retrospectively analyzed. Three observers measured the ascending aortic diameter at the level of the pulmonary artery in a plane perpendicular to the aorta on both NCCT and CTA images. Inter-rater reliability was assessed using intraclass correlation coefficients, and paired samples t-tests were used to evaluate measurement differences. Dose-length products (DLP) were collected. Median DLP values were 16.1 mGy·cm (interquartile range 11.8–25.1) for NCCT and 190.3 mGy·cm (interquartile range 120.5–298.9) for CTA. NCCT measurements were consistently larger than CTA measurements, with mean differences of 2.1 ± 0.8 mm, 2.6 ± 0.96 mm, and 2.9 ± 1.09 mm for the senior radiologist, junior radiologist, and resident, respectively (all p < 0.001). Inter-observer agreement was excellent (ICC = 0.99, p < 0.001). NCCT delivered an 11.8-fold lower radiation dose than CTA. NCCT may replace CTA for ascending aortic diameter follow-up if measurements are adjusted by approximately 2–3 mm relative to CTA-derived inner-diameter thresholds. Full article

Introduction: Tuberculous aneurysm of the thoracic aorta (TBAA) is an extremely rare but potentially fatal manifestation of tuberculosis (TB). Clinical presentation may include hemoptysis in the absence of parenchymal lung abnormalities. Case report: We presented a 62-year-old male with cough, chest pain, and minimal hemoptysis. Diagnostic evaluation confirmed an aneurysm of the descending thoracic aorta at a site previously treated with endovascular repair, with no imaging findings suggestive of pulmonary TB. Bronchoscopy revealed blood in the main bronchi without an identifiable endobronchial source. The diagnosis of TB was established by polymerase chain reaction (PCR) testing of bronchial aspirate obtained during bronchoscopy. Emergency surgical intervention was recommended because of an impending aortic rupture, but the patient declined surgery. Standard antituberculous therapy was initiated, and the patient subsequently developed drug-induced liver injury, prompting temporary cessation of treatment. The clinical course was later complicated by the development of an aortoesophageal fistula (AEF), with significant implications for prognosis. Conclusions: Early recognition of TBAA, along with a multidisciplinary approach that integrates advanced diagnostic modalities, timely vascular intervention, and carefully managed antituberculous therapy, is essential to reduce mortality and optimize treatment outcomes. Full article

Objective: To evaluate early and mid-term outcomes of thoracic endovascular aortic repair (TEVAR) using the Castor single-branched aortic stent graft in a real-world multicenter Italian experience. Methods: This retrospective, nonrandomized, multicenter study included all consecutive patients treated with the Castor stent graft between January 2019 and April 2025 in eight Italian centers. The device was used in patients with thoracic aortic pathologies requiring TEVAR in proximal landing zones 1 or 2. Primary endpoints included technical success, intraoperative major adverse events (MAEs), and deployment accuracy. Secondary endpoints were aortic-related mortality, neurological complications, reinterventions, and endoleaks. Results: Fifty-one patients (mean age 68.8 ± 8 years, 75.5% male) were treated, primarily for type B aortic dissection (45.1%) and thoracic aortic aneurysm (27.5%). Proximal landing was in zone 2 in 92.1% and zone 1 in 7.8% of cases. The technical success rate was 94.1%, with three cases (5.8%) of intraoperative type Ia endoleak. No intraoperative deaths or major adverse events occurred. Two cases of minor embolic stroke (3.9%) were observed, both in zone 1 procedures involving left common carotid artery revascularization. At a median follow-up of 22.3 months (range 2–58), no additional endoleaks or neurological events were reported, with 100% branch patency rate. Conclusions: The Castor single-branched stent graft is a feasible option for TEVAR in zone 2, with high technical success and low rates of neurological complications. Accurate case selection and procedural planning are essential. Full article

Familial thoracic aortic aneurysm and dissection (FTAAD), caused by the pathogenic Myh11 K1256del variant, is characterized by impaired aortic contractility; however, how reduced contractility predisposes the aorta to dissection remains incompletely understood. In this study, we performed a data-driven trans-omic upstream analysis using Genome Enhancer to identify key regulatory mechanisms in aortas from Myh11 K1256del mice under baseline conditions, without exposure to exogenous pathological stimuli. Transcriptome analysis revealed enrichment of genes related to smooth muscle contraction and regulation of myosin light chain phosphatase activity. Upstream computational analysis of regulatory regions identified nuclear factor of activated T cells 1 and lymphoid enhancer-binding factor 1 as major transcription factors, and further highlighted Rho-associated, coiled-coil-containing protein kinase 1 (ROCK1) as a predicted central regulator of the dysregulated transcriptional network. Druggability analysis suggested ROCK1 and the JunB proto-oncogene AP-1 transcription factor subunit as potential therapeutic targets. Furthermore, it predicted 51 candidate therapeutants, including atorvastatin, GSK-269962A, and atovaquone. These findings indicate that even in the absence of overt pathological stimulation, aortic tissue carrying the Myh11 K1256del variant exhibits a transcriptional program centered on ROCK signaling, which may prime the aorta for maladaptive responses to additional stress and may enhance susceptibility to dissection. This computational analysis requires experimental validation, but may provide a hypothesis-generating framework for development of preventive pharmacological interventions against FTAAD. Full article

Zone 2 thoracic endovascular aortic repair (TEVAR) frequently requires left subclavian artery (LSA) preservation to maintain vertebrobasilar and upper-extremity perfusion while obtaining a durable proximal seal. Dedicated single-branch endografts were developed to standardize this step and to facilitate a reproducible fully endovascular strategy. Two main device concepts currently shape this field: integrated unibody branch platforms, represented by Castor and the second-generation Cratos, and modular retrograde-branch systems, represented by the Gore TAG Thoracic Branch Endoprosthesis (TBE). The Castor/Cratos evidence base is broader and older, and is mainly centered on type B aortic dissection, with prospective multicenter and real-world data showing favorable branch patency and aortic remodeling. By contrast, TBE evidence is expanding rapidly and is supported by prospective midterm data in arch aneurysms as well as by increasingly large post-commercial series and comparative analyses across zones 0–2. Beyond outcomes, the two platforms differ substantially in branch directionality, potential contribution to proximal fixation, modularity, branch diameter range, proximal landing requirements, access profile, and regulatory/off-the-shelf availability, all of which have direct consequences for anatomical suitability in dissection, aneurysm disease, and trauma. This narrative review synthesizes current evidence and proposes an anatomy-first, pathology-aware framework for selecting between Castor/Cratos and TBE in totally endovascular zone 2 TEVAR with LSA revascularization. Full article

Thoracic aortopathies, including aneurysm and dissection, are complex vascular disorders characterized by structural alterations of the aortic wall that disrupt normal haemodynamics. Altered shear stress, turbulent flow, and endothelial dysfunction promote thrombus formation and modulate systemic hemostasis via platelet activation and the von Willebrand factor–ADAMTS13 axis. The Total Thrombus-Formation Analysis System (T-TAS) is a microfluidic, flow-dependent assay that quantitatively evaluates thrombus formation under physiological shear conditions. Although studied in various cardiovascular contexts, its application in aortopathies remains largely unexplored, and no prospective studies have validated its clinical utility. Integrating T-TAS with computational haemodynamic approaches, such as two-way fluid–structure interaction simulations, enables assessment of the interplay between blood flow, vessel wall mechanics, pulse wave propagation, and local shear patterns. Patient-specific modelling, including individualized flow profiles, pressure distributions, and wall properties, may enhance mechanistic insights. Genetic variants in Fibrillin-1 gene (FBN1), Transforming Growth Factor Beta Receptor 1/2 (TGFBR1/2), Actin Alpha 2 (ACTA 2), and Myosin Heavy Chain 11 (MYH11) further contribute to structural vascular heterogeneity and diverse systemic haemostatic phenotypes, highlighting the need for personalized assessment. T-TAS should currently be considered an exploratory research tool rather than a validated diagnostic or prognostic method. This narrative review proposes a hypothesis-generating framework integrating structural, haemodynamic, molecular, and functional perspectives. Combining flow-based thrombosis assays with advanced modelling may inform future translational studies, improve mechanistic understanding of thrombus formation, and support personalized risk stratification and management in patients with thoracic aortopathies. Full article

Thoracoabdominal aortic aneurysms (TAAAs) are uncommon and usually silent until rupture, causing a substantial burden to the health care system. Aneurysm growth and rupture prediction is mainly based on aneurysm diameter measurement by imaging modalities, meaning that the biology of aneurysm growth is not part of a potentially more adequate surveillance of aortic aneurysm patients. Alternatives or complementary options for aortic aneurysm surveillance are an ongoing, non-addressed open issue of vascular medicine. The application of different biomarkers has been discussed, yet so far, an adequate candidate for aortic aneurysm surveillance, if it comes to the thoracic or thoracoabdominal aorta, preferably without radiation exposure, has not been named. Elastin breakdown, as a component of aortic wall degeneration primarily driven by matrix metalloproteinases (MMPs), is a core element of aneurysm development. Desmosine is an elastin-specific cross-link increasingly studied as a circulating or urinary biomarker of compromised aortic wall integrity and disease activity. Accordingly, this review investigated whether plasma desmosine (pDES), a highly specific marker of elastin degradation, could be used as a non-invasive biomarker for detecting aortic aneurysms and assessing their risk profile. The existing literature of desmosine in fields of aortic pathologies in the acute and chronic setting will be assessed based on the current literature; furthermore, future perspectives of desmosine as a biomarker of aortic pathologies, such as aortic aneurysm dynamics, will be discussed. Full article

Background/Objectives: Thoracic aortic aneurysms have long been associated with germline mutations such as FBN1, TGFBR2, and COL3A1, which predispose to Marfan, Loeys–Dietz, and Ehlers–Danlos syndromes, respectively. However, recent research has identified a correlation between the JAK2 V617F somatic mutation and thoracic aortic aneurysm formation. This review aims to synthesize the current evidence on the relationship between JAK2 V617F and TAA development. Methods: A literature review was conducted using PubMed reviewed articles up to June 2025. Search terms included “thoracic aortic aneurysm”, “somatic mutations” and “JAK2 V617F”. Relevant clinical datasets and population-based cohort studies were identified and evaluated. Results: The available studies demonstrated a consistent association between JAK2 V617F and thoracic aortic aneurysm formation, with JAK2 V617F variant allele frequency (VAF) being a valuable biomarker of aneurysm risk. The mutation is accompanied by the onset of increased cytokine production, pro-inflammatory leukocytes, and elevated expression levels of MMPs—all of which drive elastin degradation and are classically associated with thoracic aortic aneurysm development. Conclusions: Compelling emerging evidence supports an association between the JAK2 V617F somatic mutation and the formation of thoracic aortic aneurysms, with VAF acting as a valuable biomarker for aneurysm risk. However, no studies have evaluated whether increasing VAF influences aneurysm growth rate, highlighting the need for future clinical research. Full article

Background/Objectives: Ascending thoracic aortic aneurysms (aTAAs) pose a high risk of dissection and rupture. Though more prevalent in males, females may experience worse outcomes. Growth rate is considered a part of risk assessment, yet data in non-syndromic females without valve abnormalities remain limited. This study aims to assess whether aTAA growth differs between non-syndromic females and males with normal aortic valve morphology. Methods: The systematic review followed the PRISMA 2020 guideline. The final search was completed in April 2025, with guidance from a certified librarian. Included studies were RCTs or observational studies of non-syndromic adults with aTAA reporting sex-specific data and included ≥10 females. Prior dissection, valve replacement, or surgery were excluded. In addition to the original search, 11 articles were identified as likely to contain sex-specific data, and the corresponding authors were contacted. The protocol is registered in PROSPERO (CRD420251025890). Meta-analysis was not performed due to high heterogeneity and limited study numbers. Results: Of 2629 identified studies, 73 studies were screened in full-text, and only three met the inclusion criteria. The most common exclusion reason was lack of appropriately sex-stratified data. Two authors out of the 11 contacted replied with additional datasets, resulting in a total of five studies being included. Of the five included studies, three found faster growth rates in females. Reported growth rates in females varied notably, ranging from −0.7–1.74 mm/year. Conclusions: Evidence on sex differences in aTAA growth among non-syndromic patients with normal aortic valves remains inconclusive. Three of the five studies reported faster growth in females. Standardization in future research is needed. Full article