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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 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.

Purpose Hybrid aortic arch repair (HAR) has been implemented for extended aortic arch and descending thoracic aortic disease since 2012 in our institution. This study aimed to estimate the early and mid-term efficacy and safety of HAR. Materials and Methods From 2007 to 2019, 56 patients underwent HAR for extended aortic arch disease, and 75 patients underwent total arch replacement (TAR) for arch-limited disease. HAR comprises 3 procedures: replacement of the aorta, reconstruction of all arch vessels, and thoracic endovascular aortic repair (TEVAR) from zone 0 to the descending aorta after cardiopulmonary bypass is off in 1 stage. The type II-1 HAR procedure, in which the ascending aorta and aortic arch distal to the brachiocephalic artery are replaced, was the most frequently selected procedure (40/56 patients). The outcomes of the type II-1 HAR procedure were compared with those of TAR using the Cox regression analysis. Results The median follow-up period was 36 months. In HAR, the operative mortality, in-hospital mortality, and postoperative permanent neurological deficits were not observed. The paraplegia rate was 1.8%. TEVAR-related complications occurred in 3 patients. Among the patients with non-ruptured atherosclerotic aortic arch aneurysm (31 type II-1 HAR patients and 36 TAR patients, the postoperative respiratory support time in those who underwent type II-1 HAR was quicker than in those who underwent TAR (pConclusions HAR, especially the type II-1 procedure, can treat extended aortic arch disease with acceptable survival outcomes. The development of TEVAR technology will further improve the outcomes of HAR in the future.

Although the frozen elephant trunk technique permits a complete single-stage treatment in patients with extended thoracic aortic disease, the problem of circulatory arrest time remains unsolved. We propose a simplified use of the Thoraflex Hybrid prosthesis to minimize the circulatory arrest time.

To investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease.Individuals with TBAD occurring at an age50 years enrolled in the National Registry of the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium were selected for analysis. Three cohorts were compared: Marfan syndrome (MFS TBAD), nonsyndromic familial TBAD (FTBAD), and sporadic TBAD. Demographics, comorbidities, aortic dissection details, and repair were compared.A total of 150 individuals met inclusion criteria (mean age at TBAD, 36.9 ± 9 years): 73 MFS TBAD, 42 FTBAD, and 35 sporadic TBAD. The cohort of sporadic TBAD had more male patients (71.4%) and fewer individuals of European descent (51.4%) compared with MFS TBAD (57.5% male, 84.9% European descent) and FTBAD (59.5% male, 90.5% European descent). There was a stepwise increase in hypertension prevalence across the cohorts (28.8% MFS, 59.5% FTBAD, 71.4% sporadic TBAD, P.001). Repair of the descending thoracic aorta was performed in 92 cases (67.1% in MFS, 61.9% in FTBAD, and 48.6% sporadic TBAD, P = .18) at a mean of 3.4 ± 5.4 years from TBAD. The repair extent varied. The largest extent of repair was in MFS TBAD, in which thoracoabdominal aortic aneurysm repair was performed in 56.2% compared with 35.7% FTBAD and 17.1% sporadic TBAD (P.001).Control of hypertension is an essential component of care to decrease the risk of TBAD. Over half of the young individuals with TBAD require aortic repair, and individuals with MFS undergo a larger anatomical extent of repair after TBAD.

To improve the knowledge about physical exercise in patients with Hereditable Thoracic Aortic Disease, insight to the patient perspectives is necessary. The aim of this study was to explore aspects related to physical exercise as highlighted by the patients themselves.Focus group interviews with 36 people with Marfan syndrome, Loeys-Dietz syndrome and vascular Ehlers Danlos syndrome were conducted. Inductive systematic condensation analysis was performed.Four themes related to physical exercise were elucidated by the participants: (1) Being diagnosed. (2) Considerations of physical exercise. (3) Body image and function. (4) Future perspectives. The four themes are mutually interrelated in terms of barriers, facilitators and strategies for dealing with physical exercise. Our findings indicate that the participants experience exercise as a consistent dilemma between what is healthy and what is risky. Inconsistent professional advice, non-engaging activities, unpredictable health conditions and a fear of exercising were factors that may contribute to inactivity and a sedentary lifestyle.The complexity and existential internal conflict related to physical exercise seemed to be a huge dilemma among persons with Hereditable Thoracic Aortic Disease. The balance between safe and healthy activities should be a research priority in these groups.Implications for rehabilitationPhysical activity and exercise pose a difficult dilemma for patients with Hereditable Thoracic Aortic Disease, in terms of what is healthy and what is dangerous.People with Hereditable Thoracic Aortic Disease need help to minimize concern, stress and anxiety associated with exercise.Individualized adapted programs including physical, psychological and social rehabilitation goals are most likely to be successful in encouraging exercise in these patient groups.

Contemporary guidelines on thoracic aortic repair provide inconsistent reporting standards for the definition of aortic arch classification in Types I, II and III. The different reported criteria cannot be used interchangeably, due to a very low level of concordance, and this finding has relevant implications for the comparisons between studies using different classifications, and between different datasets of multicentre trials, which are not consistently analyzed with the same criteria. Also, the reported definitions, which were originally proposed for predicting difficult carotid stenting and therefore were conceived for healthy aortic arches, can be influenced by the pathological derangements of the aortic wall, including aneurysms and dissections. In this respect, the Madhwal’s classification, which is based on the diameter of the left common carotid artery, appears to be the more suitable one for aortic arch classification in patients with thoracic aortic disease because it provides relevant clinical information along with an adequate reproducibility.

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.

OBJECTIVES: In Brazil, descending thoracic aorta disease, including aneurysms and dissections, is managed preferentially by endovascular treatment, owing to its feasibility and good results. In this study, we analyzed endovascular treatment of isolated descending thoracic aortic disease cases in the Brazilian public health system over a 12-year period. METHODS: Public data from procedures performed from 2008 to 2019 were extracted using web scraping techniques to assess procedure type frequency (elective or urgency), mortality, and governmental costs. RESULTS: A total of 5,595 procedures were analyzed, the vast majority of which were urgent procedures (61.82% vs. 38.18%). In-hospital mortality was lower for elective than for urgent surgeries (4.96 vs.10.32% p=0.008). An average of R$16,845.86 and R$20,012.04 was paid per elective and emergency procedure, respectively, with no statistical difference (p=0.095). CONCLUSION: Elective procedures were associated with lower mortality than urgent procedures. There was no statistically significant difference between elective and urgent procedures regarding costs.

Background Zone 2 thoracic endovascular aortic repair (TEVAR) is performed for the treatment of various thoracic aortic diseases involving the left subclavian artery. This study aimed to analyze the late clinical outcomes of zone 2 hybrid TEVAR according to the various indications. Methods A total of 48 patients who underwent zone 2 TEVAR at our institution between December, 2010 and July, 2020 were enrolled. The indications were aortic aneurysm (AA, n = 15), acute type B aortic dissection (AD, n = 14), penetrating aortic ulcer (PAU, n = 8), traumatic aortic injury (TAI, n = 8), and others (n = 3). The clinical outcomes including early complications and mid-term aortic measurements were retrospectively reviewed. Results The technical success rate was 100% and in-hospital mortality occurred in one patient. The early postoperative complications included stroke (n = 1), transient spinal cord ischemia (n = 1), neck wound hematoma (n = 1), and left phrenic or vagus nerve injury (n = 9). In patients with AD, positive remodeling was observed in ten patients (76.9%) (false lumen regression in the entire or thoracic aorta [n = 9], false lumen thrombosis in the thoracic aorta [n = 1]). However, in patients with AA, increased aneurysm was found in six patients (40%). Persistent aneurysmal growth was found in patients with a maximal aortic diameter of > 60 mm on initial imaging (4/6, 50%). No aortic expansion was observed in those with TAI or PAU. Endoleak was noted in five patients (10.4%), and among them, aortic reintervention was required only in patients with large AAs. Conclusions Zone 2 hybrid TEVAR was associated with an acceptable early complication rate and provided acceptable mid-term aortic results for patients with AD, PAU, and TAI. However, patients with large AAs were at increased risk of aortic reintervention. In cases of large AA, clinicians should carefully consider whether zone 2 hybrid TEVAR or open surgical repair will be more effective for the patient.

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

INTRODUCTION: Arterial tortuosity has emerged as a predictor of adverse outcomes in congenital aortopathies using 3D reconstructed images. We validated a new method to estimate aortic arch tortuosity on 2D CT. We hypothesize that arch tortuosity may identify bicuspid aortic valve (BAV) patients at high risk to develop thoracic aortic aneurysms or aortic dissections (TAD). METHODS: BAV subjects with chest CT scans were retrospectively identified in our clinical records and matched to tricuspid aortic valve (TAV) controls by age, gender, and presentation with TAD. Subjects with prior ascending aortic intervention were excluded. Measurements included aortic arch tortuosity, length, angle, width and height. Total aortic tortuosity was estimated in subjects with available abdominal images. RESULTS: 120 BAV and 234 TAV subjects were included. Our 2D measurements were highly correlated with 3D midline arch measurements and had high inter- and intra-observer reliability. Compared to TAV, BAV subjects had increased arch tortuosity (median 1.76 [Q1-Q3: 1.62-1.95] vs. 1.63 [1.53-1.78], P < 0.01), length (149 [136-160] vs. 135 [122-152] mm, P < 0.01),height (46 [41-53]vs. 39[34-7]mm, P < 0.01),and vertex acuity (70[61-77]vs.75 [68-81] degree, P < 0.01). In a multivariable analysis, arch tortuosity remained independently associated with BAV after adjusting for aortic diameter and other clinical characteristics. CONCLUSIONS: We found that aortic arch tortuosity is significantly increased in BAV and may identify BAV patients who are at increased risk for TAD. Further studies to evaluate the association between tortuosity and clinical outcomes are in progress.

Introduction: Current guidelines recommend patients with thoracic aortic disease (TAD) including inherited aortopathies to avoid heavy exercise. However, evidence supporting the negative advice on exercise is scarce. We aimed to provide an up-to-date systematic review of the available evidence on risks and benefits of exercise and sports participation in TAD patients. Areas covered: A systematic search was performed in Medline, Embase and Web of Science: thoracic aortic aneurysm or thoracic aortic dissection or inheritable aortopathies including Marfan Syndrome (MFS), Loeys-Dietz syndrome, Turner Syndrome, Ehlers-Danlos syndrome, bicuspid aortic valve (BAV) and sports, exercise or athletes. The resulting 1,652 manuscripts were reviewed by two independent observers. Eventually, 26 studies and 12 case-reports were included, reporting on thoracic aortic dimensions in athletes, exercise related acute aortic dissections, and exercise in BAV and MFS patients. Expert opinion: Blood pressure elevation during exercise may be associated with an increased risk of acute aortic dissection; however, no controlled trials have longitudinally evaluated the effect of exercise on survival or the risk of aortic dissection in TAD patients. Mouse-model studies suggest beneficial effects of exercise in the setting of a dilated aorta in MFS. There is a clear need for prospective research in this field.

Congenital abnormalities of the thoracic aorta encompass a variety of disorders with variable clinical manifestations ranging from asymptomatic to life threatening. A variety of imaging modalities are available for the evaluation of these anomalies with computed tomography (CT) commonly preferred due to its excellent spatial resolution and rapid acquisitions, avoiding the need of general anesthesia or even sedation. We review the embryology, imaging findings, and associations of multiple congenital thoracic aorta malformations with emphasis in the role of CT angiography in the evaluation of these pathologies.

Background. In families with an inherited form of non-syndromic thoracic aortic disease (TAAD), aortic diameter alone is not a reliable marker for disease occurrence or progression. To identify other parameters of aortic function, we studied aortic stiffness in families with TAAD. We also compared diameter measurements obtained by transthoracic echocardiography (TTE) and magnetic resonance imaging (MRI). Methods. Seven families, including 116 individuals, with non-syndromic TAAD, were studied. The aortic diameter was measured by TTE and MRI. Aortic stiffness was assessed as local distensibility in the ascending aorta and as regional and global pulse wave velocity (PWV). Individuals with a dilated thoracic aorta (n = 21) were compared with those without aortic dilatation (n = 95). Results. Ascending aortic diameter measured by TTE strongly correlated with the diameter measured by MRI (r2 = 0.93). The individuals with dilated aortas were older than those without dilatation (49 vs 37 years old). Ascending aortic diameter increased and distensibility decreased with increasing age; while, PWV increased with age and diameter. Some young subjects without aortic dilatation showed increased aortic stiffness. Individuals with a dilated thoracic aorta had significantly higher PWV and lower distensibility, measured by MRI than individuals without dilatation. Conclusions. Diameters measured with TTE agree with those measured by MRI. Aortic stiffness might be a complementary marker for aortic disease and progression when used with aortic diameter, especially in young individuals. Originally included in thesis in manuscript form.

Introduction Thoracic endovascular aortic repair (TEVAR) has become an accepted treatment for thoracic aortic disease. However, the principal complications relate to coverage of the thoracic aortic wall and deliberate occlusion of aortic branches over a potentially long segment. Complications include risk of stroke, spinal cord ischaemia (SCI) and arterial insufficiency to the left arm (left arm ischaemia (LAI)). This study specifically scrutinised the development of SCI and LAI after TEVAR for interventions for thoracic aortic disease from 1999 to 2020. In particular, those who underwent extra-anatomical bypass (both immediate and late) were compared to the length of thoracic aortic coverage by the stent graft. Materials and methods Ninety-eight patients underwent TEVAR. The presenting symptoms, pathology, procedural and follow-up data were collected prospectively with particular evidence of stroke, SCI and LAI both immediate onset and after 48 h of graft placement. Results Fifty underwent TEVAR for an aneurysm (thoracoabdominal aortic aneurysm), 22 for dissection, 19 for acute transection and 7 for intramural haematoma/pseudoaneurysm of the thoracic aorta. Twenty-nine (30%) required a debranching procedure to increase the proximal landing zone (1 aorto-carotid subclavian bypass, 10 carotid/carotid subclavian bypass and 18 carotid/subclavian bypass). Ten patients (10%) died within 30 days of TEVAR. Twenty-four grafts covered the left subclavian artery origin without a carotid/subclavian bypass. Five required a delayed carotid/subclavian bypass for LAI (4) and SCI (1). Six developed immediate signs of SCI after TEVAR and these 11 (group i) had a mean (SD) length of coverage of the thoracic aorta of 30.2 (10.6) cm compared to 21.5 (11.2) cm (group g) in those who had no LAI or SCI post TEVAR, p Conclusions In this series, delayed carotid/subclavian bypass may be required for chronic arm ischaemia and less so for SCI. The length of coverage of thoracic aorta during TEVAR is a factor in the development of delayed SCI and LAI occurrence. Carotid subclavian bypass is required for certain patients undergoing TEVAR (particularly if greater than 20 cm of thoracic aorta is covered).

Background Decision-making regarding the operability of thoracic aortic disease in nonagenarian patients remains controversial because outcomes of open surgical repair of the thoracic aorta are unclear. We investigated the surgical and nonsurgical outcomes of acute thoracic aortic syndrome treatment in nonagenarians. Methods After evaluating data in our institute from April 2016 to March 2020, we included 10 nonagenarians who needed surgical intervention on the thoracic aorta via a median sternotomy for acute thoracic aortic syndrome. The mean age of the cohort was 91.9 ± 2.1 years. Five patients underwent open surgical repair of the thoracic aorta (surgical group), and 5 refused surgery (nonsurgical group). All patients in the surgical group performed activities of daily living independently, with a mean clinical frailty scale of 3.2 ± 0.4. The surgical group included 4 patients with type A aortic dissection and one with a ruptured thoracic aortic aneurysm. Hemiarch replacement was performed in 3 patients and total arch replacement in 2. The mean follow-up period was 17.8 ± 5.1 months. Results Hospital mortality rates were 0% in the surgical and 80% in the nonsurgical group. The mean length of hospitalization was 28.4 ± 6.7 days in the surgical group. The 1-year survival rates were 100% in the surgical group and 20% in the nonsurgical group. Conclusion Open surgical repair for acute thoracic aortic syndrome via median sternotomy is a reasonable treatment option even in nonagenarians. Involvement of family members is important for decision-making to devise the optimal treatment strategy (surgical vs. medical).

Objectives To inform the design of a clinical trial of a targeted screening programme for relatives of individuals affected by thoracic aortic disease, we performed a consensus exercise as to the acceptability of screening, the optimal sequence and choice of tests, long-term patient management, and choice of trial design. Methods Working with the Aortic Dissection Awareness UK & Ireland patient association, we performed a Delphi exercise with clinical experts, patients, and carers, consisting of three rounds of consultation followed by a final multi-stakeholder face-to-face workshop. Results Thirty-five experts and 84 members of the public took part in the surveys, with 164 patients and clinicians attending the final workshop. There was substantial agreement on the need for a targeted screening pathway that would employ a combined approach (imaging + genetic testing). The target population would include the first- and second-degree adult (> 15 years) relatives, with no upper age limit of affected patients. Disagreement persisted about the screening process, sequence, personnel, the imaging method to adopt, computed tomography (CT) scan vs magnetic resonance imaging (MRI), and the specifics of a potential trial, including willingness to undergo randomisation, and measures of effectiveness and acceptability. Conclusion A Delphi process, initiated by patients, identified areas of uncertainty with respect to behaviour, process, and the design of a targeted screening programme for thoracic aortic disease that requires further research prior to any future trial.

Thoracic aortic disease has usually been treated with surgery. Thoracic endovascular aortic repair is challenging if supra-aortic vessels are involved. In situ fenestration of the main graft from a retrograde approach while using bare-metal stents as bridge stents appears to be a safe and practical technique.

A number of congenital defects and acquired disease processes affect the thoracic aorta, and traditionally, computed tomography (CT) has been the mainstay of imaging, especially in evaluation of the acute aorta. However, recent advances in magnetic resonance (MR) imaging such as electrocardiographically (ECG) triggered breath-hold sequences and ultrafast 3-dimensional MR angiography (MRA) are bringing MR imaging to the forefront of imaging of the thoracic aorta. By providing high-resolution morphological imaging and sophisticated vascular flow analysis for functional data, this modality can provide a comprehensive, reproducible evaluation of the thoracic aorta. In this review, we discuss the role of MR imaging in the evaluation of thoracic aorta pathology along with pertinent examples of aortic abnormalities.

The elephant trunks, either conventional or frozen represent the major technical improvements in the treatment of complex thoracic aortic disease. In the last decades, these useful techniques progressively evolved along with the introduction of new devices to facilitate the procedure and ameliorate post-operative results. The latest multi-branched hybrid FET prostheses give us the opportunity to greatly facilitate graft implantation and reduce operative times. The following review will provide an overview of the FET technique throughout the current available devices, possible surgical indications and principal surgical steps. © 2018 The Japanese Association for Thoracic Surgery

Background Large gutters after chimney procedures are one of the main causes of type I endoleak (EL-I). This study aims to evaluate a new tailored planning named “Over-SIRIX,” based on Osirix Imaging Software, to choose the correct main graft oversizing in order to minimize EL-I incidence. Methods From 2008 to 2015, 34 patients were treated with parallel grafts for aortic arch diseases at our institution. The study included 22 patients with single stent and antegrade flow configuration; they were divided into 2 groups (PRE- and POST-“Over-SIRIX”). “Over-SIRIX” was carried out in the retrospective group (PRE-“Over-SIRIX”), and it was used to plan the endovascular procedure in the prospective group (POST-“Over-SIRIX”). Through the multiplanar reconstruction (MPR) of the preoperative computed tomography angiography (CTA), the proximal neck of the chimney grafts was studied. Stent and endograft configurations were drawn in order to minimize the “gutters.” To obtain the ideal main graft sizing (I-Size), a formula was used by adding the custom sizing (C-Size) to the disease oversizing (D-Over). The same MPR imaging was evaluated on postoperative CTA to study gutters area and presence of EL-I. Results The mean I-Size was 41.67 mm that was equivalent to an ideal oversizing of 19.3% (range 10–28%). The gutters area decreased from 7.3 to 1.7 mm 2 (PRE/POST) and EL-I rate from 28.5% to 0% (PRE/POST). Gutters area bigger than 7.5 mm 2 and planning made without “Over-SIRIX” were significantly associated ( P Conclusions “Over-SIRIX” appears to be a feasible method to customize planning during chimney technique, reducing the risk of EL-I which is significantly related to the presence and size of the gutters.

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

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.

Background The contemporary incidence and prevalence of thoracic aortic aneurysm and dissection (TAAD) remains understudied. Epidemiological research on TAAD has been limited by incomplete case identification within administrative data sources. The objectives of this study were to develop a case identification method and determine the incidence and prevalence of TAAD, based on data from Manitoba, Canada. Methods Hospital records and medical claims housed at the Manitoba Centre for Health Policy, and data from the Manitoba Thoracic Aortic Diseases Clinic were used to develop a case definition and identify patients with TAAD in Manitoba, Canada. The age-standardized incidence and prevalence of thoracic aortic disease was determined and stratified by sex from 1998 to 2016. Results We identified 4264 patients with TAAD, of whom 63% were male. The age standardized incidence was 2 times higher in men, peaking at 42 cases per 100,000 in 2013. Although the overall incidence was lower in female patients, the number of incident cases increased 68% compared with 25% in male patients. In hospitalized patients, the incidence and prevalence of aneurysms increased over time, whereas the incidence of dissection and rupture has remained stable. Conclusions The use of a novel case identification method provided a more complete description of the epidemiology of TAAD. The incidence and prevalence of TAAD is rising, more so in female patients over time. These data support increased resource allocation to thoracic aortic diseases clinics to ensure appropriate monitoring, treatment, and follow-up for the growing number patients with thoracic aortic disease.

Background: To evaluate the early safety and clinical performance of the new low-profile RelayPro Thoracic Stent-Graft System in patients with thoracic aortic disease. Methods: This was an international, prospective, single-arm study in patients diagnosed with thoracic aorta disease (aneurysm, pseudoaneurysm, dissection, penetrating atherosclerotic ulcer, or intramural hematoma) and treated with a RelayPro stent-graft (in bare stent and/or nonbare stent configurations). The primary endpoints were freedom from aneurysm or dissectionrelated mortality and stent-graft performance. Results: A total of 31 patients were treated with the RelayPro thoracic stent-graft between 2014 and 2015 at 8 sites in Italy and Spain. Mean age was 72.1 (+/- 10.2) years and 77% were male, 74% with hypertension, and 42% with a history of smoking. Twenty-four (77%) had aneurysms (fusiform in 46%, saccular in 42%, pseudoaneurysm in 12%); 5 (16%) had penetrating atherosclerotic ulcer; and 2 (6%) had chronic Type B dissection. Mean vascular access diameter was 9.1 mm (6-13 mm); 7 patients (23%) had vascular access of 7 mm or less. Technical success was 100% (primary, 90%; assisted primary, 10%). Freedom from aneurysm/dissection-related mortality through 30 days was 100%. Freedom from device-related major adverse events through 30 days was 94%. At 1 year, there was 1 (3%) type Ib and 1 (3%) type II endoleak, 1 (3%) nonaneurysm-related late death, and 1 (3%) secondary intervention (to correct type Ib endoleak). Conclusions: The RelayPro has a 3-4 French profile reduction to allow endovascular repair of thoracic aortic disease in patients with smaller anatomies. This study shows good initial stent-graft performance and a favorable early safety profile.

Exercise recommendations for those who have Marfan syndrome or other genetic predisposition for thoracic aortic disease remain controversial and at times ambiguous. There are no outcomes studies to help guide recommendations. In this review, we examine the guidelines regarding exercise and inherited aortic conditions, the theoretical reasoning and circumstantial evidence that support the guidelines, as well as the knowledge gaps that continue to exist.

The patient with thoracic aortic disease can present for open or endovascular repair. Thoracic endovascular aortic repair (TEVAR) has emerged as a minimally invasive option for a multitude of aortic pathology, including dissections, aneurysms, traumatic injuries, and ulcers. Postoperative management of these patients depends on the extent of procedure, whether it was open or endovascular, and, finally, on the preoperative comorbidities present. While procedural success has catapulted TEVAR to popularity, midterm results have been mixed. Additionally, periprocedural complications such as paraplegia and renal failure remain a significant morbidity in these patients.

Arterial tortuosity is emerging as a common feature in genetically mediated thoracic aortic disease that may be prognostic. This review will summarize recent literature on arterial tortuosity in the setting of genetic arteriopathies.Although arterial tortuosity has been primarily described in Loeys-Dietz syndrome due to TGFBR1 and TGFBR2 mutations and in arterial tortuosity syndrome due to SLC210A mutations, recent studies that use quantitative measures of tortuosity suggest that tortuosity is present in many other genetic conditions associated with aortic dilation and dissection. The mechanisms of the development of tortuosity in these disorders are not fully understood, but are founded in the concept that there is abnormal, pathologic arterial lengthening in a fixed space, resulting in more tortuous vessels. Further studies suggest that patients with increased arterial tortuosity are at increased risk of adverse cardiovascular events, including aortic surgery, aortic dissection, and death.Arterial tortuosity is commonly present in genetically mediated aortic disease. Given the suboptimal performance of aortic dimension alone in predicting aortic dissection, quantification of tortuosity may augment the current algorithms for determining risk in patients with aortic disease.

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. Electronic supplementary material The online version of this article (10.1007/s12265-018-9835-8) contains supplementary material, which is available to authorized users.

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.

BACKGROUND Endovascular treatment of thoracic aortic disease currently allows to treat high-risk patients with better results than open repair. It represents the first option for treatment according to the most recent guidelines. The aim of the study is to evaluate the early results of the low-profile Zenith Alpha Thoracic Endoprosthesis (ZATE). METHODS Between October 2012 and July 2015, 14 asymptomatic patients were treated with ZATE. 10 patients were male, mean age was 71, 7 years (range 58-85 years). 8 patients presented with atherosclerotic aortic aneurysm (7 thoracic aneurysm and 1 type I thoracoabdominal aneurysm), 2 chronic type B aortic dissection, 1 type IV endoleak 5 years after TEVAR, 3 aortic arch penetrating ulcers complicated by pseudoaneurysm. Hybrid staged procedures in 11 patients included 7 total aortic arch debranching (1 single [innominate] chimney stent-graft + carotid-carotid-subclavian), 2 carotid-carotid-subclavian artery bypass, 2 carotid-subclavian artery bypass. RESULTS No 30-day mortality or major complications were observed. The mean length of stay was 7.3 days (range 4-14 days). Mean procedure time, X ray time and Contrast load were 115 minutes (range 90-150 minutes), 20 minutes (range 10-30) and 79 mL (range 40-120 mL) respectively. 25 stent-grafts were implanted. The mean follow-up was 21 months (range 14-32 months). No mortality and no major complications were observed during the follow-up. In case of arch debranching with landing 0 and 1 zone the mean distance between the beginning of the endoprosthesis and the debranching inflow vessel was 5.5±2.4 mm. CONCLUSIONS The use of ZATE could be a viable alternative for treating patients with aortic arch proximal landing zone to facilitate the precise deployment. Larger case studies and longer follow-up are needed.

OBJECTIVE: This study investigated the growth and behavior of the ascending aorta in patients with descending thoracic aortic disease. METHODS: We examined 200 patients with descending thoracic aortic disease including acute type B dissection (n = 95), chronic type B dissection (n = 38), intramural hematoma (n = 23), and thoracoabdominal aortic aneurysms (n = 44). Images from computed tomography and magnetic resonance imaging were evaluated after three-dimensional reconstruction to examine the growth rate in those with >1 year of imaging follow-up (n = 108). Survival data were derived from all 200 patients in this study. RESULTS: Average proximal aortic dimensions at the index image were relatively small, measuring 3.65 ± 0.51 cm in the root, 3.67 ± 0.48 cm in the ascending aorta, and 3.50 ± 0.44 cm in the proximal arch. Average growth rate was low for the aortic root, ascending aorta, and proximal arch at 0.36 ± 0.64 mm/y, 0.26 ± 0.44 mm/y, and 0.25 ± 0.44 mm/y, respectively. There was no difference in baseline proximal aortic dimensions and growth rate between the four subgroups. An index aortic diameter ≥4.1 cm grew faster than those

An acute aortic dissection is a life-threatening cardiovascular condition that is preventable if individuals at risk are identified. Pathogenic variants in 11 genes confer a highly penetrant, dominantly inherited risk for aortic aneurysms and dissections with or without syndromic features (e.g.

To examine the potential of aortic arch variants, specifically bovine aortic arch, isolated left vertebral artery, and aberrant right subclavian artery, as markers for thoracic aortic disease (TAD).We screened imaging data of 556 patients undergoing surgery due to TAD for presence of aortic arch variations. Demographic data were collected during chart review and compared with a historical control group of 4617 patients.Out of 556 patients with TAD, 33.5% (186 patients) demonstrated anomalies of the aortic arch, compared with 18.2% in the control group (P.001). Three hundred seventy (66.5%) had no anomaly of the aortic arch. Bovine aortic arch emerged as the most common anomalous branch pattern with a prevalence of 24.6% (n = 137). Thirty-five patients (6.3%) had an isolated left vertebral artery, and 10 patients (1.8%) had an aberrant right subclavian artery. When compared with the control group, all 3 arch variations showed significant higher prevalence in patients with TAD (P.001). Patients with aortic aneurysms and anomalous branch patterns had hypertension less frequently (73.5% vs 81.8%; P = .048), but had a higher rate of bicuspid aortic valve (40.8% vs 30.6%; P = .042) when compared with patients with aneurysms but normal aortic arch anatomy. Patients with aortic branch variations were significantly younger (58.6 ± 13.7 years vs 62.4 ± 12.9 years; P = .002) and needed intervention for the aortic arch more frequently than patients with normal arch anatomy (46% vs 34.6%; P = .023).Aortic arch variations are significantly more common in patients with TAD than in the general population. Atypical branching variants may warrant consideration as potential anatomic markers for future development of TAD.

Ancient historical texts describe the presence of aortic pathology conditions, although the surgical treatment of thoracic aortic disease remained insurmountable until the 19th century. Surgical treatment of thoracic aortic disease then progressed along with advances in surgical technique, conduit production, cardiopulmonary bypass, and endovascular technology. Despite radical advances in aortic surgery, principles established by surgical pioneers of the 19th century hold firm to this day.

Endovascular approaches to the aortic arch are challenged by unique anatomy and physiology of this area. Simple application of conventional endovascular technology and technique for abdominal or descending thoracic aortic disease to the aortic arch is insufficient to achieve effective and durable repairs. Appreciation of these challenges has led to developments in endovascular technology as well as complex strategies to deal with individual patient anatomy that hold the potential for continued improved outcomes in both the short and the long term.

Thoracic endovascular aortic repair (TEVAR) has been proven to be the optimal therapy for patients with a ruptured descending thoracic aortic pathology. In these emergent settings, TEVAR provides a rapid delivery of treatment to an unstable patient with a lethal disease. Typically, the greatest challenge is maintaining hemodynamic stability until the time of graft deployment. In this report, we describe our technique of performing awake TEVAR for ruptured descending thoracic aortic disease.

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.

Thoracic endovascular aortic repair (TEVAR) has become an alternative treatment option for acute thoracic aortic disease. This review focuses on current endovascular treatment of acute thoracic aortic disease and future directions of TEVAR. TEVAR is a promising alternative approach to open surgery, with lower early mortality and morbidity rates, especially in high-risk cohorts. Furthermore, with accumulating experience and improving device technology and imaging modalities, TEVAR has become safer and has potential to expand treatment options to include ascending and arch pathologies.

To examine the risk of thoracic aortic disease (TAD) when one or more first-degree relatives are affected, and to relate the risk of family history to the risk of other cardiopulmonary comorbidity.Population-based, matched, case-control study.Registry-based investigation.All cases, nationwide, of TAD diagnosed 2001-2005 in individuals born 1932 or later (n=2436) were identified, and a random control-group (n=12 152) matched for age, sex and geography was generated. First-degree relatives were identified in the Multigeneration Registry. Family history of TAD was assessed by cross-linking nationwide health registries.None.Family history was present in 108 cases (4.4%), compared with 93 (0.77%) controls (p0.0001). The risk of TAD increased with number of affected relatives: OR 5.8 (95% CI 4.3 to 7.7) vs OR 20 (2.2 to 179) with one versus two or more affected relatives. The relative risk of TAD was highest in the youngest (≤49 years) age group and slightly more pronounced in women than in men (OR 7.2 (4.2 to 12) vs OR 5.5 (3.9 to 7.7)). Among cardiopulmonary comorbidities, heart failure conferred the highest relative risk, OR 6.3 (4.1 to 9.8).Family history confers a significantly increased (sixfold to 20-fold) relative risk of TAD. The effect is more pronounced in women and in younger subjects, and is not conveyed by cardiopulmonary comorbidity. Knowledge of family history is important to counselling, treatment indications, surveillance and screening protocols.

FUNDAMENTO: Ainda nao foram analisadas as caracteristicas epidemiologicas das doencas da aorta toracica (DAT) no estado de Sao Paulo e no Brasil, assim como o seu impacto na sobrevida desses pacientes. OBJETIVOS: Avaliar o impacto da mortalidade das DAT e caracteriza-la epidemiologicamente. METODOS: Analise retrospectiva dos dados do Sistema Unico de Saude para os codigos de DAT do registro de internacoes, de procedimentos e dos obitos, a partir do Codigo Internacional de Doencas (CID-10), registrados na Secretaria de Saude do Estado de Sao Paulo durante o periodo de janeiro de 1998 a dezembro de 2007. RESULTADOS: Foram 9.465 obitos por DAT, 5.500 homens (58,1%) e 3.965 mulheres (41,9%); 6.721 disseccoes (71%) e 2.744 aneurismas, 86,3% diagnosticados no IML. Foram 6.109 internacoes, 67,9% do sexo masculino, sendo que 21,2% evoluiram a obito (69% homens), com proporcoes semelhantes de disseccao e aneurisma entre os sexos, respectivamente 54% e 46%, porem com mortalidade distinta. Os homens com DAT morrem mais que as mulheres (OR = 1,5). A distribuicao etaria para obitos e internacoes foi semelhante, com predominio na sexta decada. Foram 3.572 operacoes (58% das internacoes) com mortalidade de 20,3% (os pacientes mantidos em tratamento medicamentoso apresentaram mortalidade de 22,6%; p = 0,047). O numero de internacoes, de cirurgias, de obitos dos pacientes internados e geral de obitos por DAT foi progressivamente superior ao aumento populacional no decorrer do tempo. CONCLUSOES: Atuacoes especificas na identificacao precoce desses pacientes, assim como a viabilizacao do seu atendimento, devem ser implementadas para reduzir a aparente progressiva mortalidade por DAT imposta a nossa populacao.

Purpose To investigate the midterm outcomes of thoracic endovascular aneurysm repair (TEVAR) with the use of the Seal stent graft for four categories of thoracic aortic disease. Materials and Methods This retrospective multicenter study evaluated the records of 216 Korean patients who underwent TEVAR with the Seal stent graft during 2007–2010. The study outcomes were ( i ) perioperative death, ( ii ) endoleak, ( iii ) repeat intervention, ( iv ) aortic-related death, and ( v ) all sudden unexplained late deaths. Results The overall technical success rate was 94% (203 cases), and the disease-specific rates were 97% (88 cases) for aneurysms, 96% (71 cases) for dissections, 82% (32 cases) for traumatic aortic disease, and 100% (12 cases) for intramural hematoma and/or penetrating aortic ulcer. There were 6 acute surgical conversions (2 for aneurysms and 4 for dissections). There were 18 endoleaks, 4 retrograde ascending aortic dissections, and 6 stent graft–induced new entries. The 1-, 3-, and 5-year overall survival rates were 93% ± 3, 90% ± 4, and 90% ± 4, respectively. Conclusions TEVAR with the Seal thoracic stent graft provided a high technical success rate and low mortality and complication rates during midterm follow-up. However, additional long-term studies are needed to evaluate the durability and late complications associated with this device.

In thoracic stentgrafting, a short proximal landing zone may necessitate intentional occlusion of the left subclavian artery, which may lead to brain, spinal cord, and left arm malperfusion. A scalloped stentgraft is designed for adequate proximal sealing and preserving the left subclavian artery flow. We performed a systematic review, describe the required pre-operative planning and details of the perioperative technique used and report our own experience. In literature, only 29 patients were reported so far using a proximal scalloped stentgraft. Results show a high technical success rate and low complication rate. These results are comparable to the results from our own institution. From these first small series the scalloped thoracic endovascular aortic repair appears to be a safe and relatively simple solution for thoracic aortic disease involving the left subclavian artery.