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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.

Endovascular therapies have had a considerable impact on contemporary management of thoracic aortic disease. Still, with the anatomic challenges of the aortic arch, endovascular experience with devices that traverse the arch and deploy in the Zone 0 position remains limited. We report the first Canadian experience with the RelayBranch Thoracic Stent Graft (Terumo Aortic, Sunrise, FL) with Zone 0 deployment for total endovascular aortic arch repair in a patient at very high risk for redo open surgery. We demonstrate safe deployment of the device and successful treatment of a type 1A endoleak. Features of the RelayBranch design that mitigate challenges of arch deployment are also discussed. Résumé: Les traitements endovasculaires ont eu un impact considérable sur la gestion contemporaine des pathologies de l'aorte thoracique. Pourtant, en raison des contraintes anatomiques de la crosse aortique, l'expérience endovasculaire avec des dispositifs qui traversent la crosse et se déploient dans la zone 0 reste limitée. Nous rapportons la première expérience canadienne de l'endoprothèse thoracique RelayBranch avec déploiement (Terumo Aortic, Sunrise, FL) en zone 0 pour une réparation endovasculaire totale de la crosse aortique chez un patient présentant un risque très élevé de reprise de chirurgie ouverte. Nous décrivons le déploiement en toute sécurité du dispositif et le traitement réussi d'une endofuite de type 1A. Enfin, nous examinons les caractéristiques du système RelayBranch qui limitent les difficultés liées au déploiement du dispositif dans la crosse aortique.

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 Non-syndromic heritable thoracic aortic disease (nsHTAD) is an autosomal dominant disorder with high mortality rate if undetected. Familial evaluation could be useful to identify high-risk patients early. Purpose To assess the yield of clinical and genetic screening in a cohort of patients with suspected nsHTAD. Methods We collected clinical and genetic data about patients with suspected nsHTAD treated in a specialized clinic. Bicuspid aortic valve cases were excluded. Genetic study was performed with next-generation-sequencing, including at least 30 related genes. All first degree relatives were offered evaluation according to current guidelines. Results Twenty-five index cases were analysed (mean age: 48.3 years, male: 64%). Sixteen patients (64%) presented with acute aortic dissection (postmortem diagnosis was performed in 6 cases with sudden cardiac death). Hypertension was reported in 13 cases (52%) and 8 patients (32%) had smoking history. Family history of aortic aneurysm or dissection was identified in 13 cases (52%). Eighty-three first-degree relatives were evaluated. Clinically affected family members were detected in 10 families (40%). Genetic cause of the disease was identified in 6 families (24%). Table 1 describes main characteristics of index cases with pathogenic variants. Combined clinical and genetic screening was positive in 12 families (48%) and identified 24 relatives (29%) with aortic dilatation or carrier status for the disease. Conclusions The combination of clinical and genetic screening in suspected nsHTAD is a useful tool for early detection of the disease in family members at risk and for the prevention of future complications. Funding Acknowledgement Type of funding sources: None. Table 1

Background Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS) and related heritable thoracic aortic diseases (HTAD) are well-known for their aortic complications. Myocardial dysfunction and arrhythmia are less known in this setting but have been increasingly reported as additional causes of morbidity and mortality. Related to the rarity of the disorders, data on the prevalence of these features and clinical characteristics of the patients are difficult to obtain, calling for a multicentre initiative. Purpose To study the prevalence of myocardial dysfunction and arrhythmia in patients with HTAD and describe their clinical and genetic profile. Methods Nine centres from seven countries participated in this multicentre retrospective study. Medical records of patients 12 years or older carrying a (likely) pathogenic variant in the FBN1 gene, LDS genes (TGFBR1, TGFBR2, TGFB2, TGFB3 and SMAD3) or ACTA2 gene were screened. Patients presenting myocardial dysfunction and/or arrhythmia were identified, and clinical and genetic data were collected. Myocardial dysfunction included (a)symptomatic reduced ejection fraction (EF Results In total, 3219 patients with HTAD were screened: 2761 with a variant in FBN1, 385 with a variant in one of the LDS genes (TGFBR1, TGFBR2, TGFB2, TGFB3 and SMAD3) and 73 carrying a variant in ACTA2. Myocardial dysfunction and arrhythmia were not reported in patients carrying an ACTA2 variant. Myocardial dysfunction was observed in patients with a variant in FBN1 and the LDS genes, without significant differences in prevalence (2.3% vs. 1.8%, p=0.563). Patients with a variant in the LDS genes presenting myocardial dysfunction were younger than patients carrying a variant in FBN1 (25±11 years vs. 39±17 years, p=0.034). The prevalence of VT/VF/SCD was similar in patients with a variant in one of the LDS genes compared to those with a variant in FBN1 (1.6% vs. 0.8%, p=0.132) and there was no difference in age at time of event (26±13 years vs. 33±14 years, p=0.289). Among patients with a variant in the LDS genes, the prevalence of VT/VF/SCD was highest in patients carrying a variant in the TGFBR2 gene and was significantly higher compared to patients with a variant in FBN1 (3.4% vs. 0.8%, p=0.017). In contrast, AF/AFL was significantly more often reported in patients with a variant in FBN1 compared to those with a variant in one of the LDS genes (1.7% vs. 0.3%, p=0.033). Conclusions Myocardial dysfunction and arrhythmia are rare features in patients with HTAD. They occur predominantly in patients with a variant in FBN1 and LDS genes, but were not reported in patients carrying a variant in the ACTA2 gene. Further analysis to identify other contributing factors is necessary. Funding Acknowledgement Type of funding sources: None. Figure 1

In this monthly feature, Sarah Jane Palmer delves into topical news, the latest research and what the experts are saying on subjects related to cardiology and cardiac nursing practice

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.

Idiopathic necrotizing aortitis is characterized by lymphoplasmacytic or giant cell-associated inflammation of the aorta, with no specific identifiable cause. We present the case of a 79-year-old man who sought medical attention from his primary care physician because of worsening shortness of breath. The patient underwent an elective ascending aorta, hemiarch, and aortic valve replacement. Histological examination of the aortic specimen demonstrated an unusually thin aorta with features consistent with necrotizing aortitis with giant cell infiltration.

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.

Background: Contemporary outcomes after surgical management of thoracic aortic disease have improved; however, the impact of sex-related differences is poorly understood. Methods: A total of 1653 patients (498 [30.1%] female) underwent thoracic aortic surgery with hypothermic circulatory arrest between 2002 and 2017 in 10 institutions of the Canadian Thoracic Aortic Collaborative. Outcomes of interest were in-hospital death, stroke, and a modified Society of Thoracic Surgeons–defined composite for mortality or major morbidity (stroke, renal failure, deep sternal wound infection, reoperation, prolonged ventilation). Multivariable logistic regression was used to determine independent predictors of these outcomes. Results: Women were older (mean±SD, 66±13 years versus 61±13 years; P P P =0.02), stroke (8.8% versus 5.5%; P =0.01), and Society of Thoracic Surgeons–defined composite end point for mortality or major morbidity (31% versus 27%; P =0.04). On multivariable analyses, female sex was an independent predictor of mortality (odds ratio, 1.81; P P P Conclusions: Women experience worse outcomes after thoracic aortic surgery with hypothermic circulatory arrest. Further investigation is required to better delineate which measures may reduce sex-related outcome differences after complex aortic surgery.

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.

Objective: To explore the effect of aortic stent implantation on patients in the treatment of thoracic aortic diseases. Methods: Selected patients from Yunnan Fuwai Cardiovascular (YFC) Hospital as a sample group to carry out the study, the main participants were thoracic aortic aneurysm patients admitted from June 2020 to June 2021. The number of patients involved were 80. The patients are divided into two groups and different treatment methods were adopted. A comparative analysis of the effects of aortic stent placement on patients was conducted. Results: Before treatment, there was no significant difference in the levels of various inflammatory factors between the two groups of patients, P>0.05. After treatment, the data indicators of the two groups were significantly different, as there were significant differences in the surgical indicators among the two groups of patients, P

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 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).

The ability to manage acute aortic syndromes or catastrophes begins with a detailed understanding of the anatomy and pathophysiology of the thoracic aorta. This chapter reviews the embryology, anatomy, histology and pathophysiology of the normal and diseased thoracic aorta. We begin with explanations of the embryologic origin of the aorta and its thoracic branches. The histology of the aortic wall is then detailed. Next, the various forms of thoracic aortic disease are outlined with an emphasis on their histology and pathophysiology, and finally, several important inherited forms of thoracic aortic disease are outlined.

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.

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.

Current guidelines on the management of thoracic aortic disease recommend that the ascending aorta be replaced when it reaches the size of 5.5 cm. Recently emerging data suggest that this criterion may need to be shifted to the left, signifying a recommendation to operate on patients with smaller aortic sizes. The data that support the need for a leftward shift in the guidelines include (1) novel and more granular data on the natural history of ascending aortic aneurysm between 5 and 6 cm showing that 2 hinge risk points exist—one at 5.25 cm, and the other at 5.75 cm; (2) aortic diameter before the moment of aortic dissection is at least 7 mm smaller than postdissection aortic size; (3) the advent of a semiautomated centerline method of imaging assessment seems to underestimate true ascending aortic size; (4) aortic surgery in the present era is very safe and its benefits outweigh the associated risks; (5) genetic testing via high-throughput next-generation sequencing identifies genetic defects responsible for aortic catastrophes at smaller aortic sizes; and (6) familial aortic dissection occurrence suggests that family members of an aortic dissection victim who harbor a sizable aneurysm should be operated on regardless of aortic size.

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.

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.

Although not commonly encountered in daily clinical practice, with the exception of atheromatous disease in the older population, disorders of the thoracic aorta often present dramatically and require urgent evaluation. Echocardiography is ideally suited for this purpose, particularly in the critical care setting where both transthoracic echocardiogram (TTE) and transoesophageal echocardiography (TOE) can play important roles in the bedside diagnosis and management of afflicted patients. An understanding of the anatomical relationships of the aorta within the thoracic cavity is important, in addition to specific pertinent findings within each of the different pathologies. The physician should be on the alert for complications following acute myocardial infarction and echocardiography is central to the majority of diagnoses. A baseline echo for all critically ill patients who have suffered an infarction is recommended. Constrictive pericarditis, when encountered is usually unexpected, and can be life-threatening if not diagnosed quickly in the critical care setting. As such, the physician needs to know certain echocardiographic features that assist in the diagnosis.

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.

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.

Introduction/Objective Sex disparity is reported across all forms of cardiovascular diseases. Only few studies have focused on sex differences in thoracic aortic disease pathology. We aim to identify and understand sex differences in this patient group to bridge the knowledge gap and improve clinicopathologic outcomes. Methods/Case Report This is a retrospective analysis of 83 proximal thoracic aortic aneurysm and dissection (TAAD) cases treated at a single quaternary care center in 2019. Chart review was done for demographics. Consensus criteria (Stone JR et al. Cardiovasc Pathol 2015; 24:267-78; Halushka MK et al. Cardiovasc Pathol 2016; 25:247-57) and a scoring system (Waters KM et al. Cardiovasc Pathol 2017; 30:6-11) were used for pathology reporting. Clinical correlation was also made. Pearson’s chi-square test was used for statistical analysis. Results (if a Case Study enter NA) 83 patients (61 male and 22 female) were retrieved. Overall thoracic aortopathy was higher among males, accounting for 73.4% of individuals with TAAD. In a subgroup analysis, there was no sex difference in dissection, aortic root involvement, and bicuspid aortic valve (p>0.05). Genetic aortopathy was more prevalent in females than males (27.2% vs 9.8%, p=0.04) alongside early age at first aortic event (median age: 31y vs 52y). Histopathologically, females had frequent translamellar mucoid extracellular matrix accumulation (45.4% vs 22.9%, p=0.04), extensive (54.5% vs 27.8%, p=0.02) and severe (59% vs 34.4%, p=0.04) elastic fiber fragmentation, higher band like (9% vs 6.5%, p>0.05) plus extensive (13.6% vs 4.9%, p>0.05) smooth muscle nuclei loss, and extensive (13.6% vs 1.6%, p=0.01) plus dense (4.5% vs 1.6%, p>0.05) laminar medial collapse than males. Conclusion In our patient population, females have a lower prevalence of thoracic aortic disease treated with open repair. However, those who develop TAAD harbor a greater burden of wall pathology and probable worse outcomes. We recommend sex-based analysis of all research on thoracic aortic diseases.