Case Of Thoracic Aortic Aneurysm Research Articles

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

BackgroundTatton-Brown-Rahman syndrome (TBRS) is a rare disorder, caused by DNMT3A heterozygous pathogenic variants, and first described in 2014. TBRS is characterised by overgrowth, intellectual disability, facial dysmorphism, hypotonia and musculoskeletal...

Long-term results of etiology-based thoracic endovascular aortic repair: a single-center experience

The use of thoracic endovascular aortic repair (TEVAR) for thoracic aortic aneurysm (TAA) and Stanford type B aortic dissection (TBAD) has been increasing; however, in terms of etiology, the differences of long term after TEVAR outcomes remain unexplored. Thus, we investigated etiology-specific long-term results of TEVAR for TAA and TBAD. A total of 421 TEVAR procedures were performed at our institution from July 2007 to December 2021; 249 TAA cases and 172 TBAD cases were included. Traumatic aortic dissection and aortic injury cases were excluded. The mean observation duration was 5.7 years. The overall 30-day mortality rate was 1.4% (n = 6), with 1.2% (n = 3) in the TAA group and 1.7% (n = 3) in the TBAD group. The overall incidence of postoperative stroke was 0.9% (n = 4), with 1.2% (n = 3) and 0.6% (n = 1) in the TAA and TBAD groups, respectively (p = 0.90). Paraplegia developed in 1.7% (n = 7) of patients, with 2.4% (n = 6) in the TAA group and 0.6% (n = 1) in the TBAD group. Freedom from aortic-related death was not significantly different between the two etiologies; however, thoracic reintervention was more common in the TBAD group (p = 0.003), with endoleak being the most common indication for reintervention. Additionally, retrograde type A aortic dissection occurred in four TBAD cases, while migration occurred in three TAA cases. The perioperative results of TEVAR for TAA and TBAD were satisfactory. The long-term results were unfavorable owing to the occurrence of etiology-specific and common complications. In terms of the high frequency of reintervention, the long-term complications associated with TEVAR are etiology specific.

Genetic liability to elevated circulating IP-10, IFNγ and SCGFβ levels in relation to thoracic aortic aneurysm: A mendelian randomization study

Inflammation is associated with thoracic aortic aneurysm (TAA) but the effects of each circulating inflammatory factor on TAA remain unclear. In this study, we explored the relationship between circulating inflammatory factors and TAA risk using Mendelian randomization (MR) approach based on summary statistics from the latest genome-wide association study (GWAS) of 41 circulating inflammatory factors in 8293 Finns and a GWAS involving 1351 TAA cases and 18,295 controls of European ancestry. In univariable MR, higher interferon gamma-induced protein 10 (IP-10) levels, higher interferon gamma (IFNγ) levels and higher stem cell growth factor beta (SCGFβ) levels were associated with an increased risk of TAA (OR = 1.37, 95 % CI = 1.17–1.59, p = 7.42 × 10−5; OR = 1.43, 95 % CI = 1.19–1.74, p = 2.04 × 10−4; OR = 1.27, 95 % CI = 1.09–1.48, p = 2.40 × 10−3, respectively). In multivariable MR, the patterns of associations for the three cytokines remained adjusting for each other or smoking, but were attenuated differently with adjustment for other cardiovascular risk factors, especially for lipids and body mass index. Bidirectional MR approach did not identify any significant associations between cytokines and risk factors. Our results indicated that circulating cytokines may play mediation roles in the pathogenesis of TAA. Further studies are needed to determine whether these biomarkers can be used to prevent and treat TAA.

Outcomes of open repair for descending thoracic and thoracoabdominal aortic aneurysm in recent 10 years: experience of a high-volume centre in Korea.

Although recent studies and guidelines suggest the preferred outcomes after surgical repair of thoracic aortic aneurysm (TAA) and thoracoabdominal aortic aneurysm (TAAA), published results are limited to those from high-volume hospitals and based on old data gathered before optimal management was established. Here, we analysed our outcomes over the previous 10 years from cases of open TAA and TAAA repair to offer updated and real-world results of those complex procedures performed in a high-volume centre. From November 2013 to April 2022, 212 consecutive adult patients who underwent open TAA and TAAA repair were enrolled. We analysed early and late outcomes after surgery, including postoperative complications and mortality. There were 154 (73%) men, and the median age at surgery was 61 years. Intraoperative death occurred in 1 patient due to uncontrolled bleeding. Nine patients (4%) died during follow-up, and the survival estimates at 5 years were 94 ± 3% and 95 ± 3% after descending TAA and TAAA repair, respectively. Ten patients (4%) suffered from spinal cord ischaemic injury (9 with paraplegia and 1 with paresthesia), but permanent paraplegia persisted in only 1 case. We report very low postoperative complication rates and excellent early and late survival rates after open TAA and TAAA repair from our recent 10-year data analysis. These findings may assist when choosing treatment options for these complicated diseases.

Early and midterm outcomes of in situ fenestration via adjustable puncture needle for Ankura aortic stent graft: A single-center experience.

An analysis was conducted to demonstrate early and midterm outcomes of using adjustable puncture needle-based in situ fenestration (ISF) technique for Ankura aortic stent graft to reconstruct the supra-arch branch in thoracic endovascular aortic repair (TEVAR). A retrospective analysis of 68 cases of aortic lesions with insufficient proximal anchoring area admitted to our department from March 2017 to December 2021 was performed. In situ fenestration thoracic endovascular repair (TEVAR) was performed at the same time during the operation. Among them, there were fifty-eight cases of thoracic aortic dissection, seven cases of thoracic aortic aneurysm, and three cases of thoracic aortic ulcer. Intraoperative adjustable puncture with needle was used for the Ankura aorta stent graft to perform ISF and reconstruction of the supra-arch branch. The success rate of ISF was 94.1% (64/68). Only in four cases, attempts were made to create ISF in the left subclavian artery (LSA), and all the four were unsuccessful because of sharp and tortuosity angle. Among them, forty-four cases were only LSA fenestration, and one case was left common carotid artery (LCCA) fenestration + LSA embolism. LSA + left vertebral artery (LVA) fenestration was performed in two cases, LSA fenestration +LCCA chimney was performed in fourteen cases, LCCA + LSA fenestration + innominate artery (INA) chimney was performed in one case, and LCCA fenestration + INA chimney + LSA embolism was performed in two cases. The overall neurologic event rate was 3.1% (spinal cord ischemia 0, with stroke observed in two cases). Postoperatively, one patient (1.6%) died on the third day after TEVAR due to the retrograde dissections. There were four cases of endoleak (6.3%; three type I and one type II). The average follow-up was 29.2 ± 14.4months, and no patient died during the follow-up period. Three cases of endoleak disappeared, and one case did not increase further. In addition, other two cases of retrograde dissections were observed at 3 and 5months after TEVAR. Fortunately, they were scheduled for emergency surgery with ascending aorta replacement and improved well. There was only one case of LSA occlusion after ISF which was reconstructed by endovascular treatment. Two patients were found with distal stent graft-induced new entries (dSINE), and TEVAR was performed again during the follow-up period. Early and midterm outcomes demonstrated that ISF via the adjustable puncture needle device for the Ankura aorta stent graft is a feasible and effective treatment method, which can achieve high technical success and satisfactory short-term results.

Retrograde type A aortic dissection during or after thoracic endovascular aortic repair: a single center 16-year experience.

This article aims to investigate the incidence rate of retrograde type A aortic dissection (RTAD) and the risk factors of RTAD in relation to thoracic endovascular aortic repair (TEVAR). Patients with thoracic aortic disease who underwent TEVAR at Henan Provincial People's Hospital from January 2004 to December 2019 were enrolled in the present research. The risk factors associated with RTAD following TEVAR using univariate and multiple logistic regression analyses. During the study period, A total of 1,688 TEVAR patients were included in this study, and of these, 1,592 cases were included in the type B aortic dissection (TBAD) group, and 96 cases were included in the non-TBAD group. There were 1,230 cases of aortic dissection and 362 cases of aortic intramural hematoma and/or penetrating ulcer in the TBAD group. The non-TBAD group included 68 cases of thoracic aortic aneurysm, 21 cases of thoracic aortic pseudoaneurysm, and seven cases of congenital aortic coarctation. The overall incidence rate of RTAD was 1.1% (18/1,688) in patients, all of which occurred in the TBAD group. The cohort comprised 18 RTAD patients with an average age of 56.78, consisting of 13 males and 5 females. Among them, 13 individuals exhibited hypertension. Ten instances happened within the TEVAR perioperative period, including two cases during the surgery, six cases occurred within three months, two cases occurred after one year, and the longest interval was 72 months following TEVAR. TEVAR was successfully implemented in 17 patients, while the operation technique was temporarily altered in one case. The new entry position for RTAD was identified as the proximal region of the stent graft (SG) in 13 patients, while in five cases, the entry site was more than 2 cm away from the proximal region of the SG. 17 cases were at the greater curvature of the aorta, and one case was at the lesser curvature. Multivariate logistic regression analysis revealed that the SG oversizing ratio is a relevant risk factor for RTAD. However, ascending aortic diameter, aortic arch type, SG type, and anchored region were not directly related to the occurrence of RTAD. RTAD is a rare yet catastrophic complication. It could occur both during the procedure, early and late postoperative periods. Maintaining an appropriate SG oversizing ratio is crucial to minimize the risk of RTAD.

Causal associations between CD40/CD40L and aortic diseases: A mendelian randomization study.

Background: CD40 and CD40L have been reported as associated with aortic dissection (AD) and aortic aneurysm (AA), but the causality of the associations has not been established yet. Methods: We conducted a two-sample Mendelian randomization (MR) study to assess the causal inference between CD40/CD40L and aortic diseases including AD and AA. The instrumental variables (IVs) for CD40 and CD40L were selected from a high-quality protein quantitative trait loci dataset released by a genomic study involving 30,931 individuals of European ancestry. The genome-wide association studies summary statistics for AD and AA were from the FinnGen Release 7, with 288638 controls for all outcomes of interests, 680 cases for AD and 6,092 cases for AA, also from European ancestry. For AA subtypes, there were 5,881 cases of thoracic AA (TAA) and 2,434 cases of abdominal AA (AAA) respectively. Inverse-variance weighted and Wald ratio were applied for calculating causal estimates. Horizontal pleiotropy and heterogeneity were assessed using MR-Egger regression analysis and Cochran Q test, respectively. Leave-one-out analyses were further performed. Results: Three single-nucleotide polymorphisms (SNPs) for CD40 and one SNP for CD40L were selected as IVs. We found genetic proxied CD40 levels inversely associated with the risk of AD (odds ratio [OR]: 0.777, 95% confidence interval [CI]: 0.618-0.978, p = 0.031) and AA (OR: 0.905, 95% CI: 0.837-0.978, p = 0.012), consistent across TAA (both p < 0.050). There were trends of increased risks of AD and AA in the presence of CD40L while not reaching statistical significance. No significant horizontal pleiotropy or heterogeneity was observed. Conclusion: Our MR study provides evidence supporting the causal association between CD40 and the reduced risks of both AD and AA.

Deep Learning-based Automated Aortic Area and Distensibility Assessment: the Multi-Ethnic Study of Atherosclerosis (MESA).

This study details application of deep learning for automatic segmentation of the ascending and descending aorta from 2D phase-contrast cine magnetic resonance imaging for automatic aortic analysis on the large MESA cohort with assessment on an external cohort of thoracic aortic aneurysm (TAA) patients. This study includes images and corresponding analysis of the ascending and descending aorta at the pulmonary artery bifurcation from the MESA study. Train, validation, and internal test sets consisted of 1123 studies (24,282 images), 374 studies (8067 images), and 375 studies (8069 images), respectively. The external test set of TAAs consisted of 37 studies (3224 images). CNN performance was evaluated utilizing a dice coefficient and concordance correlation coefficients (CCC) of geometric parameters. Dice coefficients were as high as 97.55% (CI: 97.47-97.62%) and 93.56% (CI: 84.63-96.68%) on the internal and external test of TAAs, respectively. CCC for maximum and minimum and ascending aortic area were 0.969 and 0.950, respectively, on the internal test set and 0.997 and 0.995, respectively, for the external test. The absolute differences between manual and deep learning segmentations for ascending and descending aortic distensibility were 0.0194 × 10-4 ± 9.67 × 10-4 and 0.002 ± 0.001mmHg-1, respectively, on the internal test set and 0.44 × 10-4 ± 20.4 × 10-4 and 0.002 ± 0.001mmHg-1, respectively, on the external test set. We successfully developed a U-Net-based aortic segmentation and analysis algorithm in both MESA and in external cases of TAA.

A Deep Learning-Based and Fully Automated Pipeline for Thoracic Aorta Geometric Analysis and Planning for Endovascular Repair from Computed Tomography.

Feasibility assessment and planning of thoracic endovascular aortic repair (TEVAR) require computed tomography (CT)-based analysis of geometric aortic features to identify adequate landing zones (LZs) for endograft deployment. However, no consensus exists on how to take the necessary measurements from CT image data. We trained and applied a fully automated pipeline embedding a convolutional neural network (CNN), which feeds on 3D CT images to automatically segment the thoracic aorta, detects proximal landing zones (PLZs), and quantifies geometric features that are relevant for TEVAR planning. For 465 CT scans, the thoracic aorta and pulmonary arteries were manually segmented; 395 randomly selected scans with the corresponding ground truth segmentations were used to train a CNN with a 3D U-Net architecture. The remaining 70 scans were used for testing. The trained CNN was embedded within computational geometry processing pipeline which provides aortic metrics of interest for TEVAR planning. The resulting metrics included aortic arch centerline radius of curvature, proximal landing zones (PLZs) maximum diameters, angulation, and tortuosity. These parameters were statistically analyzed to compare standard arches vs. arches with a common origin of the innominate and left carotid artery (CILCA). The trained CNN yielded a mean Dice score of 0.95 and was able to generalize to 9 pathological cases of thoracic aortic aneurysm, providing accurate segmentations. CILCA arches were characterized by significantly greater angulation (p = 0.015) and tortuosity (p = 0.048) in PLZ 3 vs. standard arches. For both arch configurations, comparisons among PLZs revealed statistically significant differences in maximum zone diameters (p < 0.0001), angulation (p < 0.0001), and tortuosity (p < 0.0001). Our tool allows clinicians to obtain objective and repeatable PLZs mapping, and a range of automatically derived complex aortic metrics.

Three Cases of Thoracic Aortic Aneurysm with Aberrant Right Subclavian Artery

Three Cases of Thoracic Aortic Aneurysm with Aberrant Right Subclavian Artery

A RARE PRESENTATION OF THORACIC AORTIC ANEURYSM RUPTURE

TOPIC: Signs and Symptoms of Chest Diseases TYPE: Medical Student/Resident Case Reports INTRODUCTION: Thoracic aortic aneurysm (TAA) is an uncommon clinical condition, commonly associated with atherosclerosis. It is mostly asymptomatic but aneurysm exceeding 6 cm are at risk of yearly rate of rupture or dissection of at least 6.9% and a death rate of 11.8%. We present to you a case of TAA with rupture presenting with cough, hemoptysis and pneumonitis. CASE PRESENTATION: Patient is a 68 years male who presented to the ED with cough, hemoptysis, chest discomfort for 2-3 days. He had intermittent episodes of fever. COVID test was negative 3 days ago. Past medical history was significant for HTN, Asthma, COPD, Diabetes mellitus, tobacco use disorder. Patient was afebrile with BP of 137/73, HR 72, RR 16, O2 saturation of 89% in room air. Physical examination including chest was unremarkable. Initial workup including CBC was remarkable for WBC of 12500, Hgb of 10.5, INR 0.8, PTT 24, Troponin 5 cm, smokers, COPD, advanced age, hypertension. Thoracic endovascular stent graft surgery, under epidural anesthesia, is the preferred surgical treatment especially in old TAA patients as in our case because of low morbidity, mortality and hospital stay. CONCLUSIONS: Rupture of TAAs is still very rare, but should remain on the differential for a patient with cough, hemoptysis with features of pneumonitis as it is associated with very high morbidity and mortality rates. REFERENCE #1: CL F, Esk, MK ari. Thoracic endovascular aneurysm Repair (TEVAR) for Ruptured THORACIC aortic aneurysms. Published May 2, 2017 REFERENCE #2: Inam, H., Zahid, I., Khan, S.D. et al. Hemoptysis secondary to rupture of infected aortic aneurysm– a case report. J Cardiothorac Surg 14, 144 (2019). https://doi.org/10.1186/s13019-019-0959-y REFERENCE #3: Sun D, Mehta S. Hemoptysis caused by erosion of thoracic aortic aneurysm. CMAJ. 2010;182(4):E186. doi:10.1503/cmaj.090447 DISCLOSURES: No relevant relationships by Luna Khanal, source=Web Response No relevant relationships by Adarsha Ojha, source=Web Response

Regulatory variants in TCF7L2 are associated with thoracic aortic aneurysm

Thoracic aortic aneurysm (TAA) is characterized by dilation of the aortic root or ascending/descending aorta. TAA is a heritable disease that can be potentially life threatening. While 10%-20% of TAA cases are caused by rare, pathogenic variants in single genes, the origin of the majority of TAA cases remains unknown. A previous study implicated common variants in FBN1 with TAA disease risk. Here, we report a genome-wide scan of 1,351 TAA-affected individuals and 18,295 control individuals from the Cardiovascular Health Improvement Project and Michigan Genomics Initiative at the University of Michigan. We identified a genome-wide significant association with TAA for variants within the third intron of TCF7L2 following replication with meta-analysis of four additional independent cohorts. Common variants in this locus are the strongest known genetic risk factor for type 2 diabetes. Although evidence indicates the presence of different causal variants for TAA and type 2 diabetes at this locus, we observed an opposite direction of effect. The genetic association for TAA colocalizes with an aortic eQTL of TCF7L2, suggesting a functional relationship. These analyses predict an association of higher expression of TCF7L2 with TAA disease risk. Invitro, we show that upregulation of TCF7L2 is associated with BCL2 repression promoting vascular smooth muscle cell apoptosis, a key driver of TAA disease.

Transapical thoracic endovascular aortic repair with a frozen elephant trunk for thoracic aortic aneurysm with shaggy aorta.

Surgical repair for thoracic aortic aneurysm (TAA) with shaggy aorta remains challenging due to the risk of cerebral infarction or spinal cord ischemia. Here we report a case of TAA with shaggy aorta successfully treated with transapical endovascular repair under extracorporeal membrane oxygenation (ECMO) support.

Numerical investigation of patient-specific thoracic aortic aneurysms and comparison with normal subject via computational fluid dynamics (CFD).

Vascular hemodynamics play an important role in cardiovascular diseases. This work aimed to investigate the effects of an increase in ascending aortic diameter (AAD) on hemodynamics throughout a cardiac cycle for real patients. In this study, two scans of thoracic aortic aneurysm (TAA) subject with different AADs (42.94mm and 48.01mm) and a scan of a normal subject (19.81mm) were analyzed to assess the effects of hemodynamics on the progression of TAA with the same flow rate. Real-patient aortic geometries were scanned by computed tomography angiography (CTA), and steady and pulsatile flow conditions were used to simulate real patient aortic geometries. Aortic arches were obtained from routine clinical scans. Computational fluid dynamics (CFD) simulations were performed with in vivo boundary conditions, and 3D Navier-Stokes equations were solved by a UDF (user-defined function) code defining a real cardiac cycle of one patient using Fourier series (FS). Wall shear stress (WSS) and pressure distributions were presented from normal subject to TAA cases. The results show that during the peak systolic phase pressure load increased by 18.56% from normal subject to TAA case 1 and by 23.8% from normal subject to TAA case 2 in the aneurysm region. It is concluded that although overall WSS increased in aneurysm cases but was low in dilatation areas. As a result, abnormal changes in WSS and higher pressure load may lead to rupture and risk of further dilatation. CFD simulations were highly effective to guide clinical predictions and assess the progress of aneurysm regions in case of early surgical intervention. Graphical abstract.

A Case of Thoracic Aortic Aneurysm with Hemoptysis as the First Symptom

This article reports a case of thoracic aortic aneurysm with hemoptysis as the first clinical manifestation, hemoptysis as the first clinical manifestation is very uncommon and to the best of our knowledge, only twenty of such cases have been previously reported. The case report presented here constitutes the longest course of the disease reported in medical literature, and good recovery after thoracic endovascular aortic repair, may help us to increase the understanding and diagnosis of the disease.

Thoracic Endovascular Aortic Repair (TEVAR): A Case Report

Thoracic aortic aneurysm (TAA) is generally a disease of the elderly which remains mostly asymptomatic. It is often detected incidentally with imaging studies of the chest done for other reasons. We present a 55year-old smoker, normotensive and non-diabetic male patient who was diagnosed as a case of TAA and treated by endovascular means with thoracic endovascular aortic repair (TEVAR) technique. Due to small caliber femoral artery, thoracic endograft was deployed through a Dacron graft conduit of 10 mm diameter which was anastomosed to the common iliac artery. It was a hybrid procedure done in cardiac catheterization laboratory under general anesthesia. Completion angiogram revealed good technical success with no endoleak or neurological deficit. Patient improved symptomatically after TEVAR.&#x0D; Bangladesh Heart Journal 2019; 34(2) : 146-150

A Case of Type I Debranching Complicated by Anastomotic Pseudoaneurysm: Do Not Ask Too Much of the Ascending Aorta.

Treatment of aortic arch aneurysm with standard open surgery is technically demanding, and associated morbidity and mortality are not insignificant. In high-risk patients, hybrid procedures with debranching and reimplantation or bypass of the aortic arch vessel followed by thoracic endovascular aortic repair (TEVAR) in the aortic arch represent a valid alternative to open surgery. However, when the ascending aorta is mildly dilated, the risk of retrograde dissection increases sharply. Here, we report a case of thoracic aortic aneurysm, with normal ascending aorta diameter, treated with Type I debranching and anterograde TEVAR complicated by anastomotic pseudoaneurysm and acute endocarditis, treated ultimately with ascending aortic repair and aortic valve replacement.

Is there a prospect for hybrid aortic arch surgery?

The surge of endovascular repair of aortic aneurysm in current modern aortic surgery practice has been the key for surgical management of elective cases of thoracic aortic aneurysms. This has paved way for the combined hybrid approach to be amongst the armamentarium for the management of aortic arch disease. The pivotal understanding of the aortic arch natural history coupled with device technology advancement allowed surgeons insight into delivery of hybrid surgery with acceptable morbidity and mortality results. This review article provides current insights into hybrid technique of aortic arch aneurysm repair and the evidences behind its applicability to arch surgery. It is aimed to highlight the challenges encountered for this innovative approach and correlate its challenges to those that are met by the conventional open aortic arch repair.

Association of Hemodynamic Behavior in the Thoracic Aortic Aneurysm to the Intraluminal Thrombus Prediction: A Two-Way Fluid Structure Coupling Investigation

Clotting of blood elements or intraluminal thrombus (ILT) is known to develop within aortic aneurysm sacs, and is clinically associated with the dilation and rupturing of aneurysms. However, the underlying factors that generate ILT are still unclear. We hypothesize that ILT can form under the influence of unfavorable hemodynamic patterns. This paper presents evidence for one such type of flow dynamics that could give rise to ILT within the aneurysm sac. Image-based patient-specific fluid–structure-interaction modeling of three cases of thoracic aortic aneurysms was performed, using retrospective CT images to investigate the formation of ILT as a result of local hemodynamic of aneurysm. This study showed that the formation of the ILT was associated with a vortex observed near the aortic narrowing, upstream of the aneurysm sac. This vortex could subject the blood elements to elevated stresses before directing them into the sac. The recirculation flow within the aneurysm sac may trap these activated blood elements, thus, leading to the formation of ILT during early diastole. One primary cause for ILT, as indicated in this study, could be attributed to the sharp curvature at the aortic narrowing (or isthmus) that gives rise to the vortex. Our study also showed that the size and location of the aneurysm have a direct impact on the duration and location of the vortex, which could influence the formation of the ILT.

ステントグラフト内挿術により嚥下障害・嗄声・喘息様症状が改善した胸部大動脈瘤の1例

ステントグラフト内挿術により嚥下障害・嗄声・喘息様症状が改善した胸部大動脈瘤の1例