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Type B aortic dissection with high-risk features such as a large entry tear, false lumen, and circumferential dissection has a greater chance of progression and rupture without definitive surgical intervention. Traditional thoracic endovascular aortic repair of dissection with proximal zone 1 landing requires extensive aortic arch debranching to minimize the risk of ischemic stroke with endograft deployment. Recent developments in endovascular grafts for thoracic endovascular aortic repair have allowed for an optimized approach in challenging cases. We present the case of a 53-year-old male with circumferential type B aortic dissection and higher-risk features treated with a staged right-to-left carotid bypass and subsequent thoracic endovascular aortic repair with a GORE Ⓡ TAG Ⓡ Thoracic Branch Endoprosthesis for zone 1 proximal landing., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Background: Understanding the distinct anatomical differences between patients with type B aortic dissection (TBAD) and control patients (CPs) can enhance our knowledge of normal and pathological aortic dimensions. This study aimed to deepen our knowledge of these dimensions by measuring and comparing the anatomical indices of the aortic arch in male patients with TBAD and non-TBAD male patients., Methods: In this cross-sectional observational study, 62 TBAD patients (TBADPs) and 43 CPs were assessed. Using a fit centerline approach, we identified three pivotal anatomical landmarks: Point A, Point B, and Point C. These landmarks represented intersections of the aortic arch with the brachiocephalic trunk, left common carotid artery, and left subclavian artery, respectively. These points defined Zones 1, 2, and 3, which collectively span the entire proximal aorta from the proximal end of the aortic valve to Point C. Our analyses compared key anatomical indices such as diameter of the circumscribed circle (Dcirc), ellipticity, curvature, tortuosity between TBADP and CP at critical points and regions., Results: TBADPs showed a more circular cross-sectional shape at Points A, B and C, as indicated by reduced values of Dcirc_A (P = 0.031), ellipticity_A (P = 0.034) and ellipticity_B (P = 0.048), together with a significant decrease in Dcirc_C (P = 0.015) and ellipticity_C (P = 0.007). The aortic arch in TBADPs showed enhanced tortuosity in Zone 1 (p = 0.002) and extended elongation in Zone 3 (p = 0.001)., Conclusions: The study found that the aortic arch in male TBAD patients is more circular near its primary branches, has greater tortuosity in Zone 1, and is longer in Zone 3 compared to male control patients., (© 2024. The Author(s).)

Introduction: The treatment of acute type B aortic dissection (ATBAD) is currently a challenge for vascular surgeons, because of the early morbidity and mortality rates and the high risk of late aortic events up to 50% at 5 years. This study presents the initial outcomes of ATBAD treatment using optimal medical therapy alone or combined with proximal entry tear stent-graft coverage. Additionally, it provides an analysis of the evolution of the aortic diameter and its clinical consequences during the chronic phase in each group., Materials and Methods: Conducted as a retrospective, single-center study, we enrolled all consecutive ATBAD patients (n=130) treated between 2008 and 2020. The primary analysis studies the entire patient cohort based on their initial management approach, namely, medical treatment alone for uncomplicated ATBAD (n=67) or combined with stent-graft entry tear coverage (n=63). We also conducted a subgroup analysis to investigate factors associated with disease progression in the medical management group., Results: Median follow-up was 29.5 months. During this time aneurysmal evolution was observed in: 42.4% of cases in the medical group compared with 21.8% in the stent-graft group, primarily affecting the thoracic aorta. The stent-graft group exhibited significant aortic remodeling, with a decrease in false lumen (FL) and thoracic aortic diameters. Initial aortic diameter ≥40 mm and FL ≥22 mm were independent risk factors for aneurysmal degeneration. Five-year survival was consistent at 76.1% in both groups., Conclusion: This study confirms the safety and efficacy of stent-graft entry tear coverage for ATBAD. Initial thoracic endovascular aortic repair (TEVAR) appears to reduce late aortic events by promoting aortic remodeling. Considering TEVAR's safety and potential to prevent late aortic complications, it may be considered for uncomplicated ATBAD patients with an initial aortic diameter ≥40 mm or an FL ≥22 mm., Clinical Impact: This study validates the efficacy and safety of using endovascular stent grafts to seal the proximal entry tear in cases of acute type B aortic dissections, compared to optimal medical therapy. Aortic remodelling significantly benefits from endovascular stent graft coverage of the proximal entry tear. Given the heightened risk of late aortic events observed in the medical therapy cohort, there appears to be a necessity for including endovascular interventions in the management of uncomplicated acute type B aortic dissections, particularly when aortic diameter is ≥40 mm and false lumen diameter is ≥22 mm., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Acid Sphingomyelinase Deficiency (ASMD) is an ultra-rare autosomal recessive lysosomal storage disorder characterized by intracellular lipid accumulation resulting from reduced function of acid sphingomyelinase. Olipudase alfa, an enzyme replacement therapy, was recently approved in several countries for the treatment of the non-neurologic manifestations of ASMD. Studies demonstrate improvement in organomegaly, pulmonary function and lipid profiles with olipudase alfa, yet little is known about its impact on quality of life (QoL) for patients and caregivers. The purpose of this study is to better understand the real-life impact of ASMD on patients and caregivers and assess how olipudase alfa impacts QoL for pediatric patients and their caregivers., Methods: Caregivers of pediatric patients (≤ 18 years of age) with a confirmed diagnosis of ASMD that received olipudase alfa for at least 12 months were recruited in early 2022 through national patient organizations to participate in a global online questionnaire followed by semi-structured interviews. Ten caregivers of patients with ASMD who utilized olipudase alfa as an experimental therapy for pediatric patients participated in the study. Quantitative analysis of the results was undertaken, and qualitative data was analyzed using an inductive thematic approach., Results: Ten eligible participants completed questionnaires, and 8 of the 10 went on to participate in structured interviews. Symptom burden of ASMD and impact on symptomatology and quality of life after olipudase alfa use are reported here. Five themes emerged from analysis: (1) ASMD is a systemic disease with a wide array of manifestations that significantly impact QoL; (2) Olipudase alfa was associated with improvements in all non-neurologic manifestations of ASMD; (3) Participants perceived the risk associated with olipudase alfa to be low and the benefits to greatly outweigh any risk or burden; (4) Participants reported an unmet need to treat the neurologic manifestations of the disease despite the benefits of olipudase alfa in the management of non-neurological symptoms; (5) Participants felt all patients with ASMD need access to olipudase alfa based on the life-changing experience they perceived., Conclusions: These findings highlight the sustained positive impact olipudase alfa had in many domains that are deemed important to patients and families living with ASMD and outline the extensive unmet need for patients and families living with ASMD., (© 2024. The Author(s).)

Objective: To explore the risk factors for disease progression after initial treatment of type B thymomas using a predictive nomogram model., Methods: A single-center retrospective study of patients with type B thymoma was performed. The Cox proportional hazard model was used for univariate and multivariate analyses. Variables with statistical and clinical significance in the multivariate Cox regression were integrated into a nomogram to establish a predictive model for disease progression., Results: A total of 353 cases with type B thymoma were retrieved between January 2012 and December 2021. The median follow-up was 58 months (range: 1-128 months). The 10-year progression-free survival (PFS) was 91.8%. The final nomogram model included R0 resection status and Masaoka stage, with a concordance index of 0.880. Non-R0 resection and advanced Masaoka stage were negative prognostic factors for disease progression (p < 0.001). No benefits of postoperative radiotherapy (PORT) were observed in patients with advanced stage and non-R0 resection (p = 0.114 and 0.284, respectively)., Conclusion: The best treatment strategy for type B thymoma is the detection and achievement of R0 resection as early as possible. Long-term follow-up is necessary, especially for patients with advanced Masaoka stage and who have not achieved R0 resection. No prognostic benefits were observed for PORT., (© 2024. The Author(s).)

Introduction: In acute type B aortic dissection (TBAD) patients, thoracic endovascular aorta repair (TEVAR) and best medical treatment (BMT) have both been employed for the clinical management of this condition. The relative efficacy of TEVAR and BMT when used to manage cases of acute uncomplicated TBAD, however, remains to be clarified., Aim: To conduct a pooled meta-analysis comparing acute uncomplicated TBAD patient outcomes associated with primary TEVAR or BMT treatment., Material and Methods: Relevant articles published up to July 2023 were identified by searching the Web of Science, PubMed, and Wanfang databases. Pooled analyses of endpoints from these studies were then conducted., Results: Six relevant studies were included in this meta-analysis, involving 522 and 535 patients who underwent TEVAR and BMT treatment, respectively. No significant differences were observed between these two groups with respect to pooled hospitalization duration, re-intervention rates, early mortality, organ failure incidence, stroke incidence, or the incidence of retrograde type A dissection ( p = 0.89, 0.12, 0.09, 0.36, 0.09, and 0.95, respectively). TEVAR, however, was associated with significantly better pooled thrombosed/obliterated false lumen, late mortality, aorta-related mortality, and rupture rates relative to BMT ( p = 0.00001, 0.002, 0.0001, and 0.04, respectively). TEVAR was associated with a 7% pooled type I endoleak incidence rate. Endpoints exhibiting significant heterogeneity included hospitalization duration, thrombosed/obliterated false lumen rates, and rupture rates ( I 2 = 96%, 73%, and 61%, respectively)., Conclusions: While TEVAR and BMT yield similar short-term outcomes for acute uncomplicated TBAD patients, TEVAR may be associated with a better long-term patient prognosis., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2023 Termedia Sp. z o. o.)

Objectives: The objective of this study was to investigate the incidence, treatment and survival of Stanford type B aortic dissection (BTAD) during 20 years in the Finnish population., Methods: Data collection was made from the Nationwide Care Register for Health Care, Finnish National Institute for Health and Welfare. All patients over 15 years of age with BTAD from 2000 to 2019 were included in the study. A data search of the Registry of Death Cause (Statistic Finland) was carried out to identify the date and cause of death., Results: There were 1742 cases of BTAD during the study period. BTAD represented 45.6% of all aortic dissections leading to hospital admission. Incidence for BTAD was 1.62 per 100 000 inhabitants per year. The median survival was 12.7 years [95% confidence interval (CI) 9.63-14.7], 12.4 years (95% CI 10.5-14.4) and 8.6 years (95% CI 7.5-9.7) for patients treated with thoracic endovascular aortic repair (TEVAR), surgery and medical treatment (MT), respectively. Survival was significantly better after TEVAR and surgery, compared to MT only (P < 0.001). Age-adjusted survival was significantly better after TEVAR compared to patients treated with MT or surgery (hazard ratio 0.578, 95% CI 0.420-0.794, P < 0.001). Aortic-related death was the most common cause of death in all groups (41%)., Conclusions: The incidence of BTAD seems to be similar in the Finnish population compared to other populational studies. Patients treated with TEVAR had significantly better survival compared to other patients. A high risk for late aortic-related death should be recognized in patients with BTAD., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Objective: This study characterizes racial differences in presentation, as well as short- and long-term outcomes after endovascular treatment of thoracic aortic aneurysm (TAA) and type B aortic dissection (TBAD)., Methods: We queried the Gore Global Registry for Endovascular Aortic Treatment for thoracic endovascular aortic repairs (TEVARs) performed between 2010 and 2016 and followed through 2022. Pathologies represented were descending TAA, complicated TBAD, and uncomplicated TBAD. Using standard statistical tests, we compared overall and pathology-specific demographics, procedural factors, and outcomes among Black and White patients undergoing TEVAR., Results: We identified 438 TEVAR cases, including 236 descending TAA, 121 complicated TBAD, and 74 uncomplicated TBAD. Overall, Black patients were younger and had a higher incidence of renal insufficiency (P = .001), whereas White patients had more chronic obstructive pulmonary disease (P = .003) and cardiac arrhythmias (P = .037). In patients treated for descending TAA, Black patients had increased device- and procedure-related complications (34.3% vs 17.4%; P = .014), conversion to open repair (2.9% vs 0%; P = .011) and type II endoleak (5.7% vs 1.0%; P = .040), but no differences in mortality, length of hospital stay, or major adverse cardiovascular events. Whereas outcomes of TEVAR for uncomplicated TBAD were comparable, Black patients more frequently presented with complicated TBAD than White patients (Black, 40.5% vs White, 24.8%; P = .008) and had subsequently greater reintervention rates (28.1% vs 12.4%; P = .012), all-cause mortality (hazard ratio, 4.28; 95% confidence interval, 1.74-10.5; P = .002) and aortic-related mortality (hazard ratio, 16.7; 95% confidence interval, 1.49-186; P = .022)., Conclusions: Despite increased device- and procedure-related complications, similar short- and long-term outcomes are achieved in Black and White patients undergoing TEVAR for descending TAA and uncomplicated TBAD. However, Black patients are more likely to present with, require reintervention for, and suffer mortality from complicated TBAD., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Pheochromocytomas combined with aortic dissections are rare. Treatment of aortic dissection can be complicated by the presence of pheochromocytomas., Case Presentation: we present the case of a 48-year-old male who visited the hospital with chest and back pain for 13 h. Enhanced computed tomography (CT) revealed a type B aortic dissection combined with a left adrenal mass (72 mm). Elevated 24-h urinary vanillylmandelic acid levels can aid in the diagnosis of pheochromocytomas. Aortic dissection due to unstable hypertension secondary to pheochromocytoma is rare and complicates the procedure. Thoracic endovascular aortic repair was performed, and antihypertensive treatments were administered after surgery. After hypertension was addressed and the patient was stable, laparoscopic resection of the adrenal mass was performed., Conclusions: despite its rarity, it is important to consider pheochromocytoma as a differential factor for unstable hypertension when an aortic dissection is found., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Yi, Liu and Fan.)

Background: Contemporary management of uncomplicated type B aortic dissections (uTBAD) is based on the acuity and various morphological features. Medical therapy is mandatory, while the risks of early thoracic endovascular aortic repair (TEVAR) are balanced against the potential for rupture, complex surgery, and death. Improved aortic morphology following TEVAR is documented, but evidence for improved overall survival is lacking. The costs and impact on quality of life are also needed., Methods: The trial is a randomized, open-label, superiority clinical trial with parallel assignment of subjects at 23 clinical sites in Denmark, Norway, Sweden, Finland, and Iceland. Eligibility includes patients aged ≥ 18 with uTBAD of < 4 weeks duration. Recruited subjects will be randomized to either standard medical therapy (SMT) or SMT + TEVAR, where TEVAR must be performed between 2-12 weeks from the onset of symptoms., Discussion: This trial will evaluate the primary question of whether early TEVAR improves survival at 5 years among uTBAD patients. Moreover, the costs and the impact on quality of life should provide sorely needed data on other factors that play a role in treatment strategy decisions. The common Nordic healthcare model, with inclusion of all aortic centers, provides a favorable setting for carrying out this trial, while the robust healthcare registries ensure data validity., Trial Registration: ClinicalTrials.gov NCT05215587. Registered on January 31, 2022., (© 2023. The Author(s).)

Background: Extended downstream endovascular management has been applied in acute complicated type B aortic dissection (acTBAD), distally to standard thoracic endovascular aortic repair (TEVAR), using bare metal stents, with or without lamina disruption, using balloon inflation. The aim of this systematic review was to assess technical success, 30-day mortality, and mortality during follow-up in patients with acTBAD managed with the Provisional Extension To Induce Complete Attachment (PETTICOAT) or stent-assisted balloon-induced intimal disruption and relamination (STABILISE) technique., Methods: The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) 2020 statement was followed. A search of the English literature, via Ovid, using MEDLINE, EMBASE, and CENTRAL databases, until 30th August 2022, was executed. Randomized controlled trials and observational studies (published between 2000-2022), with ≥ 5 patients, reporting on technical success, 30-day mortality and mortality during the available follow-up among patients that underwent PETTICOAT or STABILISE technique for acTBAD were eligible. The Newcastle-Ottawa Scale was applied to assess the risk of bias. Primary outcomes were technical success and 30-day mortality, and secondary outcome was mortality during the available follow-up., Results: Thirteen studies were considered eligible, twelve in the quantitative analysis. In total, 418 patients with acTBAD managed with the PETTICOAT (83%) or STABILISE (17%) technique were included. Technical success ranged between 97-100%, 99% for the PETTICOAT and 100% for the STABILISE sub-cohort. Thirty-day mortality was estimated at 3.7% (12/321), 1.4% for the STABILISE and 4.4% for the PETTICOAT technique. All studies reported the mean available follow-up which was estimated at 20 months (range 3-168 months), 22 months (mean value) for the PETTICOAT and 17 months (mean value) for the STABILISE technique. Twenty-three patients died during follow-up, with an estimated mortality rate at 5.7% for the total cohort. The mortality during follow-up was 0% for the STABILISE and 7.0% for the PETTICOAT approach., Conclusions: Both, the PETTICOAT and STABILISE techniques presented less than 4% perioperative mortality in patients with acTBAD with high technical success rate. The mid-term mortality rate was at 6%. However, the heterogeneity in the available studies' highlights the need for further prospective studies, including larger volume and longer follow-up., Competing Interests: Tilo Kölbel has intellectual property with Cook Medical. All authors have completed the ICMJE uniform disclosure form and declare no support from any organization for the submitted work; no financial relationships with any organizations that might have an interest in the submitted work; no other relationships or activities that could appear to have influenced the submitted work., (Copyright: © 2023 The Author(s). Published by IMR Press.)

Objective: Aortic remodeling of the thoracic aorta has been studied in patients treated with medical or endovascular therapy for the treatment of acute aortic dissections; however, particular attention has not yet focused on identifying specific growth patterns and rates across all aortic zones. Additionally, previous studies have not delineated between dissections with and without visceral aortic involvement, and we hypothesize that these two cohorts may exhibit distinct differences. The aim of this study is to investigate aortic behavior over time in medically managed acute Society for Vascular Surgery/Society of Thoracic Surgeons (SVS/STS) type B dissections with visceral aortic involvement and identify potential associations of subsequent aortic behavior with clinical outcomes., Methods: A single-center retrospective review was performed of all patients between 2010 and 2020 with acute SVS/STS type B aortic dissections with visceral aortic involvement that were not surgically managed. Short-axis centerline measurements of the true/false lumen and total aortic diameter (TAD) were taken at standardized locations relative to aortic anatomy within each aortic zone, including nondissected zones. Measurements were taken at the time of diagnosis and at six subsequent yearly intervals. Diameter changes over time were evaluated using repeated measures mixed models linear growth analysis. Aortic enlargement was classified by growth in TAD ≥5 mm in either the thoracic (thoracic segment enlargement [TSE], zone 0-4) or visceral segments (visceral segment enlargement [VSE], zone 5-9)., Results: A total of 78 patients were identified with a median length of follow-up of 3.3 years (interquartile range [IQR], 1.3-6.6 years). Follow-up past 5 years was seen in 31% of the cohort. For the entire cohort, mean thoracic growth in TAD was 2.0 ± 2.0 mm/year, and visceral growth in TAD was 2.5 ± 2.4 mm/year. TSE was observed in 65% of patients, with a median time until onset of 0.8 years (IQR, 0.4-2.3 years). VSE was observed in 57% of the cohort, with a median time until onset of 1.6 years (IQR, 0.9-3.3 years). Repeat measures mixed models linear growth analysis identified significant predictable linear growth in all aortic zones except for the nondissected zones 0-2. Odds for TSE are significantly increased in patients with known genetically triggered aortic conditions (odds ratio [OR], 2; 95% confidence interval [CI], 1.8-4.5; P = .044) and in cases where the dissection entry tear was in either zone 1 or 2 (OR, 4.8; 95% CI, 1.2-8.4; P = .044). In adjusted regression analysis, odds for intervention in the thoracic aorta were significantly increased in patients with rapid TSE in zone 3 (OR, 3.6; 95% CI, 1.1-8.4; P = .045). Similarly, odds for intervention targeting the visceral aortic segment were significantly increased in patients with zone 9 VSE (OR, 9.3; 95% CI, 1.1-13.3; P = .014). Odds for 5-year all-cause mortality were significantly increased in cases with large thoracic aneurysms (OR, 6.1; 95% CI, 1.1-14.9; P = .042)., Conclusions: Aortic enlargement was present in the majority of patients with medically managed acute SVS/STS Type B aortic dissections with visceral aortic involvement, with analysis demonstrating predictable linear growth in all dissected zones. Patients with aortic enlargement demonstrated higher gross changes in diameter in addition to higher yearly rates of change compared with all comers. Odds for enlargement were impacted by both patient demographic and anatomic dissection characteristics. Growth in zone 3 and zone 9 significantly increased odds for aortic intervention. Odds for 5-year mortality were significantly increased in the presence of large thoracic aneurysms. Results highlight risk of progressive degeneration beyond acute phase in SVS/STS Type B aortic dissections with visceral aortic involvement, with life-long surveillance remaining crucial in management of dissections., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To provide the 5-year outcomes of the use of a composite device (proximal covered stent graft + distal bare stent) for endovascular repair of patients with acute, type B aortic dissection complicated by aortic rupture and/or malperfusion., Methods: Study of Thoracic Aortic Type B Dissection Using Endoluminal Repair (STABLE) II was a prospective, multicenter study of the Zenith Dissection Endovascular System (William Cook Europe). Patients were enrolled between August 2012 and January 2015 at sites in the United States and Japan. Five-year follow-up was completed by January 2020., Results: In total, 73 patients (mean age: 60.7 ± 10.9 years; 65.8% male) with acute type B dissection complicated by malperfusion (72.6%), rupture (21.9%), or both (5.5%) were enrolled. Patients were treated with either a composite device (79.5%) or the proximal stent graft alone (no distal bare stent, 20.5%). Dissections were more extensive in patients who received the composite device (408.9 ± 121.3 mm) than in patients who did not receive a bare stent (315.9 ± 100.1 mm). The mean follow-up was 1209.4 ± 754.6 days. Freedom from all-cause mortality was 80.3% ± 4.7% at 1 year and 68.9% ± 7.3% at 5 years. Freedom from dissection-related mortality remained at 97.1% ± 2.1% from 1-year through 5-year follow-up. Within the stent-graft region, the rate of either complete thrombosis or elimination of the false lumen increased over time (82.1% of all patients at 5 years vs 55.7% at first postprocedure computed tomography), with a higher rate at 5 years in patients who received the composite device (90.5%) compared with patients without the bare stent (57.1%). Throughout the follow-up, overall true lumen diameter increased within the stent-graft region, and overall false lumen diameter decreased. At 5 years, 20.7% of patients experienced a decrease in maximum transaortic diameter within the stent-graft region, 17.2% experienced an increase, and 62.1% experienced no change. Distal to the treated segment (but within the dissected aorta), 23.1% of patients experience no change in transaortic diameter at 5 years; a bare stent was deployed in all these patients at the procedure. Five-year freedom from all secondary intervention was 70.7% ± 7.2%., Conclusions: These 5-year outcomes indicate a low rate of dissection-related mortality for the Zenith Dissection Endovascular System in the treatment of patients with acute, complicated type B aortic dissection. Further, these data suggest a positive influence of composite device use on false lumen thrombosis. Continuous monitoring for distal aortic growth is necessary in all patients., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

A 63-year-old man had presented for emergency endovascular treatment of acute type B aortic dissection complicated by acute occlusion of the true lumen of the infrarenal aorta and a Crawford type III thoracoabdominal aortic aneurysm. These findings precluded the use of a conventional endovascular approach. A novel technique was developed with the insertion of guidewires through the left femoral and subclavian arteries to deliver stent grafts to cover the dissection entry tear and exclude the thoracoabdominal aortic aneurysm. A femorofemoral bypass was performed to preserve the circulation. The procedure and follow-up course were uneventful. This technique appears to be a promising tool for thoracic endovascular aortic repair in emergency setting. More experience with the method is warranted., (© 2022 The Author(s).)

Ehlers-Danlos is a connective tissue disorder characterized by hypermobile joints, hyperextensible skin, and increased bleeding tendency. We report a case involving endovascular repair of an intussusception of aortic dissection that occurred in an Ehlers-Danlos patient following surgical repair of an intercostal artery bleed. Given the rarity of Ehlers-Danlos, continued reporting of treatment outcomes is important for refining complication management in this population. Our experience lends support for endovascular rescue of failed aortic repair that may arise in Ehlers-Danlos patients and contributes to the otherwise small body of literature concerning endovascular intervention in this population overall.

That children present with hepatic encephalopathy (HE) in the setting of acute liver failure (ALF) is accepted and a recognized prognostic factor for survival [1,2]. What is less understood is the impact of chronic liver disease (CLD) on the neuro-cognitive and -psychiatric development and outcomes of children with chronic liver disease early in life. Much is extrapolated from the adult literature or from work in experimental models. But what distinguishes children is that central nervous system development, characterized by massive brain growth, is ongoing at the time of liver disease, arguably exposing them to unique risks, something which cannot be extrapolated from adults. The purpose of this brief review is to summarize what is distinctive about the neurocognition of children with CLD or having presented CLD or portosystemic bypass in childhood., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Open wounds of the eye are the most common ocular injuries in children in Tajikistan. Assessment of visual functions in the outcome of such wounds is the most important task for developing a rehabilitation plan and predicting the prospects for the recovery of vision, especially in young patients., Objective: To assess visual acuity (VA) in the outcome of a penetrating wound of the eyeball in children., Material and Methods: The study retrospectively analyzed VA in the outcome of type B open trauma of scleral (12.1%), corneal (59.1%), and scleral-corneal (28.8%) localization in 132 children (132 eyes) older than 4 years (mean age 8.3 years), who were admitted to the hospital on the first day after injury. The exclusion criteria from the main selection were: the presence of an intraocular foreign body, the age of patient higher than 4 years old, and the time of admission more than 24 hours post the moment of injury., Results: Visual acuity was checked at the time of discharge from the hospital (average stay was 19.02 days) and amounted to 0-light projection in 50 (37.9%) children; 0.01-0.1 in 43 (32.6%); 0.2-0.3 in 29 (22.0%); above 0.4 in 10 (7.6%). High rate of vascular reactions to trauma was noted on admission on the first day - intraocular hemorrhage (28.8%), hyphema (10.6%), endophthalmitis (5.3%) as a result of a penetrating injury to the eyeball - as well as their negative effect on vision at discharge., Conclusion: Upon discharge from the hospital, in 92.4% of children the visual acuity in the outcome was 0.3 and below. Adverse visual outcomes significantly correlated with the depth of the injury (correlation coefficient 0.5931) and vascular reactions to trauma (correlation coefficient 0.503333).

Background: Four-dimensional flow magnetic resonance imaging (4D flow MRI) can accurately visualize and quantify flow and provide hemodynamic information such as wall shear stress (WSS). This imaging technique can be used to obtain more insight in the hemodynamic changes during cardiac cycle in the true and false lumen of uncomplicated acute Type B Aortic Dissection (TBAD). Gaining more insight of these forces in the false lumen in uncomplicated TBAD during optimal medical treatment, might result in prediction of adverse outcomes., Methods: A porcine aorta dissection model with an artificial dissection was positioned in a validated ex-vivo circulatory system with physiological pulsatile flow. 4D flow MR images with 3 set heartrates (HR; 60 bpm, 80 bpm and 100 bpm) were acquired. False lumen volume per cycle (FLV), mean and peak systolic WSS were determined from 4D flow MRI data. For validation, the experiment was repeated with a second porcine aorta dissection model., Results: During both experiments an increase in FLV (initial experiment: ΔFLV = 2.05 ml, p < 0.001 , repeated experiment: ΔFLV = 1.08 ml, p = 0.005 ) and peak WSS (initial experiment: ΔWSS = 1.2 Pa, p = 0.004 , repeated experiment: ΔWSS = 1.79 Pa, p = 0.016 ) was observed when HR increased from 60 to 80 bpm. Raising the HR from 80 to 100 bpm, no significant increase in FLV ( p = 0.073, p = 0.139 ) was seen during both experiments. The false lumen mean WSS increased significant during initial (2.71 to 3.85 Pa; p = 0.013 ) and non-significant during repeated experiment (3.22 to 4.00 Pa; p = 0.320 )., Conclusion: 4D flow MRI provides insight into hemodynamic dimensions including WSS. Our ex-vivo experiments showed that an increase in HR from 60 to 80 bpm resulted in a significant increase of FLV and WSS of the false lumen. We suggest that strict heart rate control is of major importance to reduce the mean and peak WSS in uncomplicated acute TBAD. Because of the limitations of an ex-vivo study, 4D flow MRI will have to be performed in clinical setting to determine whether this imaging model would be of value to predict the course of uncomplicated TBAD.

Background: Stanford type-B aortic dissection (TBAD) is commonly treated by thoracic endovascular aortic repair (TEVAR). Usually, the implanted stent-grafts will not cover the entire dissection-affected region for those patients with dissection extending beyond the thoracic aorta, thus the fate of the uncovered aortic segment is uncertain. This study used 3-dimensional measurement of aortic morphological changes to classify the different remodeling effects of TBAD patients after TEVAR, and hypothesized that not only initial morphological features, but also their change over time at follow-up are associated with the remodeling., Methods: Forty-one TBAD patients underwent TEVAR and CT-angiography before and after the intervention (twice or more follow-ups) were included in this study. According to the false-lumen volume variations post-TEVAR, patients who had abdominal aortic expansion at the second follow-up were classified into the Enlarged (n =12, 29%) and remaining into the Stable group (n = 29, 71%). 3D morphological parameters were extracted on precise reconstruction of imaging datasets. Statistical differences in 3D morphological parameters over time between the 2 groups and the relationship among these parameters were analyzed., Results: In the Enlarged group, the number of all tears before TEVAR was significantly higher (P = 0.022), and the size of all tears at the first and second follow-up post-TEVAR were significantly higher than that in the Stable group (P = 0.008 and P = 0.007). The location of the primary tear was significantly higher (P = 0.031) in the Stable group. The cross-sectional analysis of several slices below the primary tear before TEVAR shows different shape features of the false lumen in the Stable (cone-like) and Enlarged (hourglass-like) groups. The number of tears before TEVAR has a positive correlation with the post-TEVAR development of dissection (r = 0.683, P = 0.00)., Conclusion: The results in this study indicated that the TBAD patients with larger tear areas, more re-entry tears and with the primary tear proximal to the arch would face a higher risk of negative remodeling after TEVAR., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: Several factors determining differences between types A and B aortic dissection (AD) have been reported; however, little data exist examining their differences in left ventricular hypertrophy (LVH). We compared the prevalence of LVH in patients with types A and B AD., Methods: We retrospectively analyzed 334 patients with acute AD (227 type A; 107 type B). Concentric hypertrophy (CH; increased left ventricular mass index [LVMI] and relative wall thickness [RWT]) is one of four types of left ventricular (LV) geometry thought to be most associated with hypertension. We compared LVMI and the prevalence of CH in patients with types A or B AD. Multivariate logistic regression analyses of variables associated with type B AD were performed., Results: Comparing type A and B AD, LVMI (95 ± 26 vs.107 ± 28, p <0.001) and prevalence of CH (26% vs. 44%, p = 0.001) were higher in type B AD. In multivariate analysis, CH was an independent factor associated with type B AD (odds ratio: 2.62, confidence interval: 1.54-4.47, p <0.001)., Conclusions: Our data suggested LVH was more prevalent in type B than in type A AD. Considering LVH usually results from hypertension, patients with type B AD may be more affected by hypertension than those with type A.

Objective: Previous publications have clearly established a correlation between timing of thoracic endovascular aortic repair (TEVAR) and complications after treatment of complicated acute type B aortic dissection (ATBAD). However, the temporal association of TEVAR with morbidity after uncomplicated presentations is poorly understood and has not previously been examined using real-world national data. Therefore, the objective of this analysis was to determine whether TEVAR timing of uncomplicated ATBAD (UATBAD) is associated with postoperative complications., Methods: The Vascular Quality Initiative TEVAR and complex endovascular aneurysm repair registry was analyzed from 2010 to 2019. Procedures performed for non-dissection-related disease as well as for ATBAD with malperfusion or rupture were excluded. Because of inherent differences between timing cohorts, propensity score matching was performed to ensure like comparisons. Univariate and multivariable analysis after matching was used to determine differences between timing groups (symptom onset to TEVAR: acute, 1-14 days; subacute, 15-90 days) for postoperative mortality, in-hospital complications, and reintervention., Results: A total of 688 cases meeting inclusion criteria were identified. After matching 187 patients in each of the 1- to 14-day and 15- to 90-day treatment groups, there were no statistically significant differences between groups. On univariate analysis, the 1- to 14-day treatment group had a higher proportion of cases requiring reintervention within 30 days (15.3%) compared with UATBAD patients undergoing TEVAR within 15 to 90 days (5.2%; P = .02). There was also a difference (P = .007) at 1 year, with 33.8% of the 1- to 14-day UATBAD patients undergoing reintervention compared with 14.5% for the 15- to 90-day group. There were no statistically significant differences on multivariable analysis for long-term survival, complications, or long-term reintervention. There was a trend toward significance (P = .08) with the 1- to 14-day group having 2.3 times the odds of requiring an in-hospital reintervention compared with the 15- to 90-day group., Conclusions: Timing of TEVAR for UATBAD does not appear to predict mortality or postoperative complications. However, there is a strong association between repair within 1 to 14 days and higher risk of reintervention. This may in part be related to the 1- to 14-day group's representing an inherently higher anatomic or physiologic risk population that cannot be entirely accounted for with propensity analysis. The role of optimal timing to intervention should be incorporated into future study design of TEVAR trials for UATBAD., (Copyright © 2020 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Pasteurella multocida is the etiological agent responsible for several diseases in a wide range of hosts around the world and thus, causes serious economic losses. Acute septicemia associated with capsular type B P. multocida has recently emerged in Europe and continuous outbreaks of these acute processes have been described in Spain since they were first detected in pigs in 2009 and cattle in 2015. The scarcity of studies on the antimicrobial susceptibility of this capsular type of P. multocida and growing concern about the general increase of antimicrobial resistance mean that studies related to the performance of type B P. multocida against antibiotics are necessary to establish accurate treatments and to monitor antimicrobial resistances., Results: Seventy-six isolates of P. multocida type B from pigs and cattle with acute septicemia were tested for susceptibility to 10 different antimicrobials. Bovine isolates were susceptible to all the antibiotics we tested except for lincomycin (94.4% of isolates were resistant). However, the antimicrobials we tested were less effective against swine isolates, of which none were susceptible to lincomycin. Furthermore, 29.3% swine isolates were resistant to tetracycline, 27.6% to penicillin, 20.7% to oxytetracycline, 17.3% to chloramphenicol, 15.5% to gentamicin, and 3.4% to enrofloxacin; no resistance to ceftiofur was detected. No multidrug resistant isolates were detected from cattle, while 25.86% of swine isolates were resistant to three or more antibiotic classes., Conclusions: In this study, the lower resistance rates and multidrug resistant isolates reported for P. multocida type B derived from cattle compared to those isolated from pigs may be related to the increased use of antibiotics in the porcine industry in Spain. Lincomycin is not recommended for the treatment of acute septicemia in pigs or cattle, rather, the use of ceftiofur, enrofloxacin, or gentamicin is indicated as an emergency treatment in the early stages of disease; once the susceptibility results are known, the use of tetracyclines, penicillin, or chloramphenicol should be prioritized. The increase in multidrug resistant isolates and antimicrobial resistance rates indicates that more attention should be paid to prevention as well as the responsible use of antibiotics.

This Society for Vascular Surgery/Society of Thoracic Surgeons (SVS/STS) document illustrates and defines the overall nomenclature associated with type B aortic dissection. The contents describe a new classification system for practical use and reporting that includes the aortic arch. Chronicity of aortic dissection is also defined along with nomenclature in patients with prior aortic repair and other aortic pathologic processes, such as intramural hematoma and penetrating atherosclerotic ulcer. Complicated vs uncomplicated dissections are clearly defined with a new high-risk grouping that will undoubtedly grow in reporting and controversy. Follow-up criteria are also discussed with nomenclature for false lumen status in addition to measurement criteria and definitions of aortic remodeling. Overall, the document provides a facile framework of language that will allow more granular discussions and reporting of aortic dissection in the future., (Copyright © 2020 Society for Vascular Surgery and The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Effectiveness and optimal timing of endovascular treatment for type B aortic dissection (AD) remain controversial., Method: An extensive search of literature (January 1999-December 2017) was conducted using PubMed, Cochrane Library and Science-Direct databases for studies on endovascular repair for acute/chronic type B AD; ≥10 patients; not reviews; and reporting predefined baseline data and ≥50% of predefined study outcomes, which were extracted and analysed according to Preferred Reporting Items for Systematic reviews and Meta-Analyses. Primary outcomes were in-hospital all-cause mortality and aorta-related mortality., Result: Based on 92 publications (5956 patients), pooled estimate for overall in-hospital mortality was 7.0% [95% CI, 6.2%-7.8%]. Major perioperative complications included stroke (4.2% [3.6%-4.9%]), spinal cord ischemia (3.3% [2.8%-3.9%]), retrograde type A AD (3.2% [2.7%-3.9%]), type I endoleak (4.9% [3.8%-6.2%]), visceral ischemia (3.1% [2.5%-3.8%]) and acute renal failure requiring haemodialysis (5.1% [4.3%-5.9%]). Mid-term mortality incidence was 8.9% [7.2%-10.9%], and secondary intervention rate was 12.5% [10.5%-15.0%] with 6.1% [5.3%-7.2%] conversion to open surgery. Institutions with ≥40 endovascular treatment caseload had significantly lower rates of in-hospital and aorta-related mortality, stroke, type I endoleak, renal failure and retrograde type A AD. Patients treated in the acute phase had significantly higher incidence of in-hospital or aorta-related mortality and renal failure. Patients with chronic dissection required significantly more often secondary intervention during follow-up., Conclusion: Endovascular stent-graft for type B AD therefore appeared feasible and safe with a low incidence of mortality and perioperative complications, particularly for delayed intervention and centres with ≥40 caseload. Standardized and long-term follow-up data are warranted., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: The objective of this study was to compare short-term outcomes in patients who underwent thoracic endovascular aortic repair (TEVAR) with stent grafts alone or with a composite device design (stent graft plus bare-metal aortic stent) for acute type B aortic dissection in the setting of malperfusion., Methods: This retrospective analysis included patients with acute (≤14 days of symptom onset) complicated type B dissection in the setting of malperfusion who were treated with stent grafts alone (TEVAR cohort) at two European institutions vs those who underwent TEVAR with a composite device design (Cook Medical, Bloomington, Ind) in the investigational STABLE I feasibility study and STABLE II pivotal study (STABLE cohort). Preoperative characteristics and 30-day outcomes (including mortality, malperfusion-related mortality, morbidity, and secondary interventions) were compared between the two groups., Results: The TEVAR cohort (41 patients; mean age, 58.8 ± 12.7 years; 78.0% male) and the STABLE cohort (84 patients; mean age, 57.8 ± 11.7 years; 71.4% male) were largely similar in preoperative medical characteristics, with more STABLE patients presenting with a history of hypertension (79.8% vs 58.5%; P = .018). The TEVAR and STABLE groups had similar lengths of dissection (451.8 ± 112.7 mm vs 411.8 ± 116.4 mm; P = .10) and similar proximal and distal extent of dissection. At presentation, the two groups exhibited comparable organ system involvement in malperfusion: renal (53.7% TEVAR, 57.1% STABLE), gastrointestinal (41.5% TEVAR, 44.0% STABLE), lower extremities (34.1% TEVAR, 52.4% STABLE), and spinal cord (9.8% TEVAR, 2.4% STABLE). The 30-day rate of all-cause mortality was 17.1% (7/41) in the TEVAR group and 8.3% (7/84) in the STABLE group (P = .22). The 30-day rate of malperfusion-related mortality (deaths from bowel/mesenteric ischemia or multiple organ failure) was 12% (5/41) in the TEVAR group and 2.4% (2/84) in the STABLE group (P = .038). The 30-day morbidity, for the TEVAR and STABLE groups, respectively, included bowel ischemia (9.8% [4/41] vs 2.4% [2/84]; P = .09), renal failure requiring dialysis (7.3% [3/41] vs 9.5% [8/84]; P > .99), paraplegia or paraparesis (4.9% [2/41] vs 3.6% [3/84]; P = .66), and stroke (2.4% [1/41] vs 10.7% [9/84]; P = .16). The occurrence of 30-day secondary intervention was similar in the TEVAR and STABLE groups (7.3% [3/41] vs 7.1% [6/84]; P > .99). True lumen expansion in the abdominal aorta was significantly greater in the STABLE group., Conclusions: In patients with acute type B aortic dissection in the setting of branch vessel malperfusion, the use of a composite device with proximal stent grafts and distal bare aortic stent appeared to result in lower malperfusion-related mortality than the use of stent grafts alone. The 30-day rates of morbidity and secondary interventions were similar between the groups., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

The timing and choice of surgical method for type B aortic dissection, is still a topic of much debate. We performed total arch replacement using frozen elephant trunk (TAR-FET) as a means of preventing distant aortic events, such as retrograde type A aortic dissection (RTAD). We conducted analysis of 142 patients with acute type B dissection who were admitted between January of 2010 and July of 2017. Fifty-five cases required surgical intervention to treat enlargement of the false lumen diameter and ULP formation 2 weeks after the onset of symptoms. 17 TAR-FET were performed with a mean of 42±26 days period from onset to surgery. There were no complications of RTAD or paraplegic, and 90% of patient demonstrated aortic event free survival (5 years) and false lumen reduction ratio of 35%. Based on our analysis, using TAR-FET properly avoids serious complications like RTAD, and is a viable treatment option for type B dissection. (This is a translation of J Jpn Coll Angiol 2018; 58: 151-157.)., Competing Interests: Disclosure StatementNone of the authors have conflicts of interest to report., (Copyright © 2019 Annals of Vascular Diseases.)

Background: Niemann-Pick diseases are rare inherited lipid storage disorders caused by mutations in the SMPD1, NPC1 , and NPC2 genes. The aim of this study was to assess the mutation spectrum of a cohort of Iranian Niemann-Pick patients. Methods: A consanguineous couple with a child suspected of having Niemann-Pick disease type A (died at age 2) was screened for gene mutations in the SMPD1 gene. Sanger sequencing was performed for all exons and exon-intron boundary regions. A literature review on SMPD1, NPC1 , and NPC2 genes mutations in Iran was conducted using published original papers on this subject. Results: A novel frameshift c.762delG (p.Leu256fs*) at a heterozygous state was identified in the parents. According to the review study, identified mutations in 39 Iranian patients were concentrated in exon 2 of the SMPD1 gene and exons 8 and 9 of the NPC1 gene. Conclusion: Niemann-Pick diseases genes mutation analysis ( SMPD1, NPC1 , and NPC2 ) in Iran shows the genetic heterogeneity of these diseases in this country. More studies with larger sample sizes should be conducted to further examine genetic changes associated with Niemann-Pick diseases in Iran., (© 2019 Iran University of Medical Sciences.)

Acute aortic dissection (AAD) cases are thought to have high blood pressure (BP) on admission; however, little data are available on BP prior to admission. The purpose of this study was to investigate systolic blood pressure (SBP) very early after symptom onset and before hospital transfer in patients with AAD to determine whether SBPs were high, and also whether SBPs were higher or lower compared with SBPs at hospital admission. We obtained results using three-year data derived from the Tokyo Acute Aortic Super Network Database. First, we selected 830 patients with AAD for which the "duration from symptom onset to first medical contact by ambulance crews" (SO-FMC) was within 60 min. We examined the SBPs of such patients. Next, we selected 222 patients with AAD whose SBPs were measured both at FMC, within 15 min after symptom onset, and at hospital admission, and compared SBPs at FMC with those at hospital admission. Among types A (n = 190) and B (n = 117), in patients with an SO-FMC ≤ 15 min, the median SBP was 100 mmHg and 178 mmHg (p < 0.001), respectively; 9% and 50% (p < 0.001) of such patients, respectively, exhibited an SBP ≥ 180 mmHg; and 43% and 10% (p < 0.001) of such patients, respectively, had an SBP < 90 mmHg. Of patients with types A (n = 124) and B (n = 98) AAD whose SBPs were measured both at FMC, within 15 min after symptom onset, and at hospital admission, SBPs at FMC were higher than those at hospital admission for the SBP ≥ 180 mmHg subgroups of both type A (194 mmHg vs. 159 mmHg, p < 0.001) and type B (199 mmHg vs. 186 mmHg, p < 0.001). Approximately 10 min after symptom onset and before hospital transfer, the measured SBPs of many patients with type A AAD were not necessarily high. However, the SBPs of cases with type B AAD were high as previously reported for SBP on admission. In addition, for the subgroup of SBP ≥ 180 mmHg at FMC within 15 min after symptom onset, SBPs at FMC were significantly higher than those at hospital admission for both types A and B; the higher SBP at symptom onset may have been partially associated with being a trigger of AD.

Objective: We report the final 5-year results from the Study of Thoracic Aortic Type B Dissection Using Endoluminal Repair (STABLE I) study, a prospective, single-arm, multicenter study of the Zenith Dissection Endovascular System (William Cook Europe, Aps, Bjaeverskov, Denmark), a pathology-specific device comprising a proximal stent graft with barbs and a distal bare stainless steel stent for the treatment of patients with complicated type B aortic dissection., Methods: The study prospectively enrolled 86 patients (mean age, 59 years; 73% male) at sites in the United States, Europe, and Australia from 2007 to 2012. Treatment occurred during the acute phase (≤14 days after symptom onset) in 55 patients and during the nonacute phase (>14 days; all treated ≤90 days) in 31 patients. Five-year clinical and imaging follow-up was available for 88.5% of eligible patients., Results: The 30-day all-cause mortality rate was 5.5% (3 of 55) for acute and 3.2% (1 of 31) for nonacute patients (P > .99). The 5-year freedom from all-cause mortality was 79.9% ± 6.2% for acute and 70.1% ± 8.4% for nonacute patients (log-rank test, P = .40). The 5-year freedom from dissection-related mortality (including deaths of indeterminate relatedness to dissection repair) was 83.9% ± 5.9% for acute and 90.1% ± 5.9% for nonacute patients (log-rank test, P = .55). Complete false lumen thrombosis in the thoracic aorta increased over time and was observed in 74.1% of acute and in 58.8% of nonacute patients at 5 years. From preprocedure through 5 years, there was an overall increase in true lumen diameter and a concomitant decrease in false lumen diameter in both acute and nonacute patients at the level of the largest diameter in both the thoracic and abdominal aortas. At 5 years, 65.5% of acute and 81.3% of nonacute patients exhibited a stable or shrinking transaortic diameter in the thoracic aorta, and 48.3% of acute and 76.5% of nonacute patients had a stable or shrinking transaortic diameter in the abdominal aorta. Freedom from secondary intervention at 5 years was 65.5% ± 7.5% for acute and 71.2% ± 9.0% for nonacute patients (log-rank test, P = .71)., Conclusions: Endovascular repair of complicated type B aortic dissection with a composite device design demonstrated low all-cause mortality at 30 days, as well as low dissection-related mortality throughout follow-up. Overall, the acute and nonacute cohorts appeared to respond similarly to treatment involving use of the stent-graft and bare metal stent, demonstrating similar clinical outcomes and favorable improvement in aortic remodeling in the thoracic and abdominal aortas., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Aortic intramural hematoma constitutes one of the three classifications of acute aortic syndrome (AAS). Type B intramural hematoma (IMH-B) is localized to the descending thoracic aorta and can be managed through medical, endovascular or surgical means. Data comparing contemporary management with thoracic endovascular aortic repair (TEVAR) versus traditional medical management (MM) is sparse and only moderate strength recommendations for TEVAR are provided in guidelines. This meta-analysis aimed to pool available data from comparative studies between TEVAR and MM and examine differences in outcomes., Methods: Literature search of electronic medical databases was conducted to identify studies comparing TEVAR and MM for management of IMH-B. Data extraction from studies fulfilling the inclusion criteria was performed by two authors and meta-analysis using a random-effects model applied to pool baseline data and examine risk ratios (RR) for management outcomes., Results: Of the initial 2,349 studies, nine studies were identified for analysis. There were 161 TEVAR patients and 166 who were medically managed. The mean age of the cohort was 62.2 years [95% confidence interval (CI): 55.8-68.7 years]. Patients with complicating features of IMH-B at presentation were more likely to appear in the TEVAR group, with more penetrating atheromatous ulcer (PAU) [risk difference (RD), 0.565, 95% CI: 0.240-0.889, P=0.001], ulcer-like projection (ULP) (RD 0.240, 95% CI: 0.965-0.384, P=0.001), and greater IMH size (mean difference, MD 5.47 mm, 95% CI: 0.320-10.6, P=0.037). There was no statistical difference between TEVAR and MM for the primary endpoints of aortic-related death (RR 0.535, 95% CI: 0.191-1.5, P=0.234) or IMH-B regression (RR 1.25, 95% CI: 0.859-1.81, P=0.246). Of the secondary endpoints, TEVAR had both significantly less dissection during follow-up (RR 0.295, 95% CI: 0.0881-0.989, P=0.048) and less rupture during follow-up (RR 0.206, 95% CI: 0.0462-0.921, P=0.039)., Conclusions: A small number of series comparing TEVAR and MM for management of IMH-B are available and random-effects meta-analysis did not reveal any statistically significant difference between treatments for aortic related death or IMH-B regression at a mean follow-up of 37 months. TEVAR was found to be associated with lower risk of dissection and lower risk of rupture during follow-up. Baseline data meta-analysis showed patients with complicating features of PAU, ULP, and larger IMH size were more likely to be managed with TEVAR., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Introduction : Vaccination is a crucial tool for the primary prevention of infectious diseases. Thanks to widespread vaccination, in the last century smallpox has been eradicated, and the Americas and Europe have become polio-free. Areas covered : The aim of our study was to assess vaccination coverage in childhood up to booster preschool age in order to update the analysis on European trends of immunization coverage in the last nine years (2009-2017) and to assess the impact of different national policies on vaccine uptake. Expert opinion : Despite the good results, several EU countries are going through unprecedented outbreaks of vaccine-preventable diseases due to insufficient coverage. The increase of vaccine hesitancy has made it difficult to reach and maintain high rates of vaccination coverage. Enforcing mandatory vaccinations is one of the strategies adopted by some countries, like Italy and France, to increase coverage. However, each country should find the most suitable way to keep up with vaccination coverages according to own cultural and organizational background.

Niemann-Pick (NP) disease is a rare, autosomal recessive disorder characterized by visceromegaly and neurological alterations due to the excessive storage of lipids, sphingomyelin, and cholesterol. It commonly affects the child population, and only 6% of it occurs in the adult population. Type A is classified as the acute form, type B is the latest and with the best prognosis, and type C is characterized by neurological alteration. The diagnosis is based on enzymatic tests and genetic sequencing, with the latter being the diagnostic confirmation test. No specific treatment exists for this entity, although some patients with NPC type C may benefit from pharmacological treatment with miglustat. The objective of this paper is to describe the clinical characteristics of a grown patient with Niemann-Pick diagnosis type B. This article reports the case of a 55-year-old adult patient with a three-year clinical history consisting of splenomegaly and hematological disorders, without neurological symptoms ruling out frequent pathologies. Type B NP disease is diagnosed by a mutation in the sphingomyelin phosphodiesterase 1 (SMPD1) gene. The patient was receiving multidisciplinary support treatment. Although NP disease is a rare disease according to the literature, it is important to consider this group of disorders as a differential diagnosis, when other more common pathologies have been ruled out in patients with isolated splenomegaly and thrombocytopenia., Competing Interests: The authors have declared that no competing interests exist.

Background: The MDACC group recommends to extend the current borderline classification for pancreatic cancer into three groups: type A patients with resectable/borderline tumor anatomy, type B with resectable/borderline resectable tumor anatomy and clinical findings suspicious for extrapancreatic disease and type C with borderline resectable and marginal performance status/severe pre-existing comorbidity profile or age>80. This study intents to evaluate the proposed borderline classification system in a multicenter patient cohort without neoadjuvant treatment., Methods: Evaluation was based on a multicenter database of pancreatic cancer patients undergoing surgery from 2005 to 2016 (n = 1020). Complications were classified based on the Clavien-Dindo classification. χ 2 -test, Kaplan-Meier estimator and Cox regression hazard model were used for statistical analysis., Results: Most patients (55.1%) were assigned as type A patients, followed by type C (35.8%) and type B patients (9.1%). Neither the complication rate, nor the mortality rate revealed a correlation to any subgroup. Type B patients had a significant worse progression free (p < 0.001) and overall survival (p = 0.005). Type B classification was identified as an independent prognostic marker for progression free survival (p = 0.005, HR 1.47)., Conclusion: The evaluation of the proposed classification in a cohort without neoadjuvant treatment did not justify an additional medical borderline subgroup. A new subgroup based on prognostic borderline patients might be the main target group for neoadjuvant protocols in future., (Copyright © 2018. Published by Elsevier Ltd.)

Combined hexavalent diphtheria-tetanus-acellular pertussis-hepatitis B-inactivated poliomyelitis and Haemophilus influenzae type b vaccine (DTaP-HBV-IPV/Hib) can further reduce the number of injections in pediatric immunization schedules of countries currently using pentavalent DTaP combination vaccines. This open-label, randomized, multicenter study (NCT02096263) conducted in the United States evaluated the immunogenicity and safety of DTaP-HBV-IPV/Hib vaccine compared with concomitant administration of DTaP-HBV-IPV and Hib A or DTaP-IPV/Hib and HBV vaccines. We randomized (1:1:1) infants to receive 3-dose priming with DTaP-HBV-IPV/Hib boosted with DTaP+ Hib B , DTaP-HBV-IPV+ Hib A boosted with DTaP+ Hib A , or DTaP-IPV/Hib+ HBV boosted with DTaP-IPV/Hib, at 2, 4, 6, and 15-18 months of age. We enrolled and vaccinated 585 participants, 486 received a booster, and 476 completed the study. Of these, 466 participants were included in the primary and 408 in the booster according-to-protocol cohorts for immunogenicity. We demonstrated non-inferiority of DTaP-HBV-IPV/Hib vaccine to DTaP-HBV-IPV+ Hib A co-administered vaccines in terms of geometric mean concentrations for pertussis antibodies post-primary vaccination. Post-primary vaccination, seroprotection/seropositivity rates for all vaccine antigens were similarly high between DTaP-HBV-IPV/Hib (≥ 94.8%), DTaP-HBV-IPV+ Hib A (≥ 98.1%) or DTaP-IPV/Hib+ HBV (≥ 97.8%) groups. We observed robust immune responses post-booster, indicating effective priming by the 3 regimens. Reactogenicity was similar in the 3 groups. Twenty-eight serious adverse events were reported during the study; 3 were considered related to vaccination and resolved by the end of the study. These results confirm that DTaP-HBV-IPV/Hib could be a valuable additional source of pediatric DTaP, IPV, HBV, and Hib-containing vaccine in countries that currently use multivalent vaccines.

We assessed the immunogenicity and safety of a three-dose primary vaccination schedule with the combined diphtheria-tetanus-acellular pertussis-inactivated poliovirus/Haemophilus influenzae type b vaccine (DTPa-IPV/Hib) in Korean infants. In this phase III open-label, multicenter study (NCT01309646), healthy infants aged 42-69 days (randomized 1:1) received three doses of either pentavalent DTPa-IPV/Hib (DTPa-IPV/Hib group) or DTPa-IPV and Hib vaccines administered separately (DTPa-IPV+Hib group) at 2, 4, 6 months of age. The primary objective was to demonstrate non-inferiority of DTPa-IPV/Hib compared to DTPa-IPV+Hib vaccines in terms of immune responses to all vaccine antigens, 1 month post-dose 3. Solicited symptoms (local and general) were recorded during 4 days, and unsolicited adverse events (AEs) during 31 days, after each vaccination. Serious AEs (SAEs) were recorded throughout the study duration. The immunogenicity of the pentavalent DTPa-IPV/Hib vaccine was non-inferior compared to concomitant administration of DTPa-IPV+Hib vaccines. One month post-dose 3, nearly all infants had antibody levels above the seroprotective thresholds for anti-diphtheria toxoid, anti-tetanus toxoid, anti-polyribosyl-ribitol phosphate, and anti-poliovirus type 1, 2 and 3, and had antibody levels above the seropositive thresholds for anti-pertussis toxoid (PT), anti-filamentous hemagglutinin (FHA) and anti-pertactin (PRN) antibodies. A vaccine response for PT, FHA and PRN was observed in at least 96.7% of study participants. Anti-PRP geometric mean concentrations appeared lower for the DTPa-IPV/Hib group (8.456 µg/mL) than for the DTPa-IPV+Hib group (18.700 µg/mL). In both groups, the most common solicited symptoms were injection site redness and irritability. Fifty-seven SAEs were reported throughout the study; none were considered to be vaccination related.

Objective: The Global Registry for Endovascular Aortic Treatment (GREAT) is a prospective multicenter registry collecting real-world data on the performance of W. L. Gore (Flagstaff, Ariz) aortic endografts. The purpose of the present study was to analyze the implementation and outcomes of thoracic endovascular aortic repair (TEVAR) in GREAT patients with type B aortic dissection (TBAD)., Methods: From 2010 to 2016, >5000 patients were enrolled in the GREAT from 113 centers in 14 countries across 4 continents. The study population comprised those treated for TBAD. The primary outcomes of interest were mortality and freedom from aortic events (AEs)., Results: A total of 264 patients (80% male; mean age, 62 years) underwent TEVAR for the treatment of 170 (64%) acute and 94 (36%) chronic cases of TBAD. Chronic TBAD patients required significantly longer endograft coverage than did acute TBAD patients (P = .05). Early postoperative complications occurred in 9% of patients, with no difference in chronic vs acute dissection (P = .11). The 30-day aortic mortality and all-cause mortality were 1.5% and 2.3%, respectively, with no differences based on chronicity. During a mean follow-up of 26 months, the total aortic mortality was 2.7% and the total all-cause mortality was 12.5%. The all-cause mortality was significantly greater for chronic vs acute TBAD (19.2% vs 8.8%, respectively; P = .02). On multivariate analysis, patients with acute uncomplicated dissections had significantly improved overall survival compared with all other categories of dissections (93% vs 83% at 2 years; P < .05). A proximal landing zone diameter >40 mm was associated with an increased risk of retrograde type A dissection (18% vs 2%; P = .02). Patients undergoing left subclavian artery (LSA) coverage experienced a twofold greater rate of AEs compared with noncoverage patients (P < .01). Patients who underwent LSA revascularization experienced a 1.5-fold greater rate of AEs compared with patients covered without revascularization (P = .04)., Conclusions: TEVAR for TBAD using the conformable GORE TAG thoracic endoprosthesis device can be performed with a low incidence of aortic mortality and complications. Acute uncomplicated TBAD patients had a significantly lower mortality rate than that of other patients. Larger proximal landing zones were associated with more frequent retrograde type A dissection. LSA involvement (coverage and/or revascularization) was associated with an increased risk of AEs during follow-up., (Copyright © 2018. Published by Elsevier Inc.)

Objective: The aim of this study was to evaluate the perioperative and short-term results in a cohort of patients treated during the last year at our institution with the stent-assisted balloon-induced intimal disruption and relamination in aortic dissection repair (STABILISE) technique for acute complicated aortic dissection., Methods: Between June 2016 and June 2017, 10 patients (all male; mean age, 62.6 ± 7.4 years) received treatment for acute complicated aortic dissection with the STABILISE technique. After a standard provisional extension to induce complete attachment procedure using the commercially available endovascular dissection system (Cook Medical, Bloomington, Ind), the distal stent graft area and the bare stent area were ballooned to completely exclude the thoracic false lumen (FL) and to obtain a single-channeled abdominal aorta. Computed tomography was routinely performed within the first postoperative week before discharge and then at 3 months, at 6 months, and yearly thereafter. The technical and clinical success rates were analyzed., Results: The 30-day technical and clinical success rates were 100%, with complete thrombosis of the thoracic FL and no type I endoleak. Malperfusion was resolved in all cases. No aortic ruptures were recorded, and no open conversion was required. One case of delayed spinal cord ischemia fully resolved within the discharge period. Predischarge computed tomography showed complete thrombosis of the thoracic FL in all cases. In two cases, some degree of patency of the abdominal FL was observed. At short-term follow-up, the overall aortic diameters remained stable with no further dilation., Conclusions: The STABILISE technique was safe and feasible in this cohort of patients, with complete thrombosis of the thoracic FL and creation of a single-channeled aorta in most cases. Further studies are needed to ascertain the long-term behavior of the treated aorta., (Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Arachidonate 15-lipoxygenase (ALOX15) and arachidonate 15-lipoxygenase, type B (ALOX15B) catalyze the dioxygenation of polyunsaturated fatty acids and are upregulated in human alternatively activated macrophages (AAMs) induced by Th2 cytokine interleukin-4 (IL-4) and/or interleukin-13. Known primarily for roles in bioactive lipid mediator synthesis, 15-lipoxygenases (15-LOXs) have been implicated in various macrophage functions including efferocytosis and ferroptosis. Using a combination of inhibitors and siRNAs to suppress 15-LOX isoforms, we studied the role of 15-LOXs in cellular cholesterol homeostasis and immune function in naïve and AAMs. Silencing or inhibiting the 15-LOX isoforms impaired sterol regulatory element binding protein (SREBP)-2 signaling by inhibiting SREBP-2 processing into mature transcription factor and reduced SREBP-2 binding to sterol regulatory elements and subsequent target gene expression. Silencing ALOX15B reduced cellular cholesterol and the cholesterol intermediates desmosterol, lanosterol, 24,25-dihydrolanosterol, and lathosterol as well as oxysterols in IL-4-stimulated macrophages. In addition, attenuating both 15-LOX isoforms did not generally affect IL-4 gene expression but rather uniquely impacted IL-4-induced CCL17 production in an SREBP-2-dependent manner resulting in reduced T cell migration to macrophage conditioned media. In conclusion, we identified a novel role for ALOX15B, and to a lesser extent ALOX15, in cholesterol homeostasis and CCL17 production in human macrophages.

Background: Postaxial polydactyly, type B is the most common type of polydactyly. The vestigial sixth finger is attached by a narrow neurovascular pedicle to the lateral aspect of the hand or foot at the level of the metacarpal-phalangeal joint or the metatarsal-phalangeal joint. The occurrence of this type of polydactyly varies among racial groups, by sex and sidedness. Postaxial polydactyly, type A is a fully developed extra digit on the lateral aspect of the hand or foot with a bifid fifth or sixth metacarpal/metatarsal and is much less common., Methods: In a malformations surveillance program, the frequency in racial groups, sex ratio and the frequency of other anomalies can be established., Results: Five hundred forty-five affected infants were identified from 1972 to 2012 in the surveillance of 289,365 liveborn and stillborn infants and elective terminations because of fetal anomalies detected prenatally. Postaxial polydactyly, type B was an isolated anomaly in 95% of the affected newborns. There were more affected males than females. Black infants were affected more often than White infants: 0.91/100 vs. 0.035/100 infants. The dangling extra digit was much more common in the hands than in the feet., Conclusions: Postaxial polydactyly, type B is almost always an isolated, mild malformation with no medical significance. Postaxial polydactyly, types B and A occurred in several infants, suggesting that either the underlying mutation(s) can cause both types of postaxial polydactyly or that some affected infants have more than one mutation. Autosomal dominant inheritance with variable expressivity is postulated., (© 2018 Wiley Periodicals, Inc.)

This report provides a rare histological example and the appropriate management of spontaneous aortic dissection secondary to giant cell arteritis.

Thoracic endovascular repair (TEVAR) is an effective treatment for type B aortic dissection (TBAD). Here, we evaluated the early-midterm effectiveness and safety of TEVAR for treating TBAD patients after renal transplantation. Six patients with TBAD treated with TEVAR after renal transplantation were recruited between February 2012 and December 2016. They were then followed up with clinical examinations and computed tomography angiography (CTA). TEVAR was successfully performed in all patients (100%), and the primary tear sites were well covered by stents with or without coverage of the left subclavian artery. No severe complications occurred in any patient during perioperative period. The one-year survival rate was 100%, one patient died of renal graft failure and heart failure four years after TEVAR; the remaining five patients (83.3%) survived and exhibited no severe complications. Our findings show that TEVAR provides satisfactory short-midterm results for TBAD patients after renal transplantation. Moreover, our experience shows that it need relative longer proximal landing zone to prevent the endoleak and recurrence. However, regular hematodialysis, long-term immunosuppressive therapy, and blood pressure control remain crucial factors to prolong survival. Long-term follow-up studies are needed to evaluate the long-term prognosis in these patients., Competing Interests: CONFLICTS OF INTEREST The authors report that there are no conflicts of interest.

Influenza virus is epidemiologically very important due to the antigenic variations occurring in it. In present study isolation and identification of influenza virus was attempted from Kasauli and adjoining areas. 71 clinical samples i.e. throat washings were processed and inoculated into the eggs for virus isolation. The isolated strains of H3N2, H1N1 and Influenza B were identified, purified and passaged in eggs. The harvested virus was inactivated by 0.03 % formalin and three formulations of crude, inactivated, whole virus monovalent vaccine for H3N2, H1N1 and influenza B as well as a trivalent vaccine was prepared. The immunogenicity study of the vaccine was performed in the mouse model by using varying dilutions of four types of vaccines for a period of 40 days. The impact of booster dose was also analyzed in the study. A significant seroconversion was observed in the mice sera verifying that the monovalent and trivalent lots of influenza vaccine were antigenically effective based on the serological tests.

Background: Distal stent graft-induced new entry (DSINE) has been increasingly observed following thoracic endovascular aortic repair (TEVAR) for type B aortic dissection (TBAD). We seek to identify the risk factors for DSINE following TEVAR in patients with TBAD., Methods: Between January 2009 and January 2013, we performed TEVAR for 579 patients with TBAD. The clinical data were retrospectively analyzed with univariate and multivariate analyses to identify the risk factors for DSINE., Results: Two patients (0.3%) died after the initial TEVAR. Morbidity included spinal cord injury in 2 (0.3%), stroke in 3 (0.5%) and endoleak in 12 (2.1%) patients. Clinical and radiological follow-up was complete in 100% (577/577) averaging 47±16 months. Late death occurred in 6 patients. DSINE occurred in 39 patients (6.7%) at mean 22±17 months after the initial TEVAR, which was managed with re-TEVAR in 25 and medically in 14. At 33±18 months after DSINE, 11 of patients managed medically (11/14) and all patients managed with re-TEVAR (25/25) survived (P=0.048). Freedom from DSINE was 92.7% at 5 years (95% CI: 90.0-94.7%). Using tapered stent grafts with a proximal end 4-8 mm larger than the distal end, TEVAR performed in the acute phase (≤14 days from onset) was associated with a significantly lower incidence of DSINE than TEVAR performed in the chronic phase (4.3%, 7/185 vs. 13.9%, 15/108; P=0.003). Risk factors for DSINE were stent grafts less than 145 mm in length [odds ratio (OR) 2.268; 95% CI: 1.121-4.587; P=0.023] and TEVAR performed in the chronic phase (OR 1.935; 95% CI: 1.004-3.731; P=0.049)., Conclusions: Our results show that TEVAR performed during the acute phase and using stent grafts longer than 145 mm could decrease the incidence of DSINE in patients with TBAD. Tapered stent grafts with a proximal end 4-8 mm larger than the distal end may be helpful in preventing DSINE after TEVAR performed in the acute phase than TEVAR performed in the chronic phase, due to the difference in mobility of the dissected flap. Expedite repeat TEVAR is recommended to improve the clinical prognosis for patients with DSINE.

A novel combined Haemophilus influenzae type b-Neisseria meningitidis serogroups A and C-tetanus-toxoid conjugate vaccine (Hib-MenAC vaccine) has been developed to protect children against diseases caused by Hib, MenA, and MenC. This study investigated the safety and immunogenicity of the Hib-MenAC vaccine administered in 2-dose series to children aged 6-23 months and in a single dose to children aged 2-5 y. A randomized, positive-controlled, non-inferiority clinical trial was conducted for 1200 healthy participants in each age group. Within each age group, participants were randomly allocated to the Hib-MenAC group or the control group at a ratio of 1:1. Adverse reactions were recorded within 28 d after each dose. Blood samples were obtained to assess immunogenicity on day 0 and at 28 d after a complete vaccination course. For the investigational vaccine, the incidence of total adverse reactions in vaccinees aged 6-23 months was 46.8% and that in vaccinees aged 2-5 y was 29.8%. Most adverse reactions were mild or moderate. One non-fatal serious adverse event occurred in the Hib-MenAC group, but was unrelated to vaccination. The seroconversion rate to the 3 components reached 94.0%, and the proportion of vaccinees with rSBA titers ≥ 1:8 and PRP ≥ 0.15 g/mL reached 97.0% in both age groups. The safety and immunogenicity of the Hib-MenAC vaccine were non-inferior when compared to the licensed vaccines. It was concluded that the novel vaccine would be expected to protect children against all of the targeted diseases.

Objectives: Fenestrated/branched thoracic endovascular repair (F/Br-TEVAR) is increasingly applied for atherosclerotic thoracoabdominal aortic aneurysm (TAAA); however, use in post-dissection TAAAs is still very limited. Experience with F/Br-TEVAR in the treatment of post-dissection TAAA is presented., Methods: Data were analysed from prospectively maintained databases including all patients with post-dissection TAAAs that underwent F/Br-TEVAR within the period January 2010 to July 2013 in two vascular institutions. Evaluated outcomes included initial technical success, operative mortality and morbidity, late survival, endoleak, aneurysm diameter regression, renal function, and reintervention during follow-up (FU)., Results: A total of 31 patients (25 male, mean age 65 ± 11.4 years) were treated. Technical success was 93.5% and 30-day mortality 9.6%. Temporary spinal cord ischaemia occurred in four (12.6%) patients, with no case of permanent paraplegia. Mean FU was 17.0 ± 10 months. There were seven late deaths, all aneurysm unrelated. Estimated overall survival rates were 83.9 ± 6.7, 76.4 ± 7.9 and 71.6 ± 8.7% at 6, 12, and 18 months, respectively. Impairment of renal function occurred in two (6.4%) patients. Endoleaks were diagnosed in 12 patients during FU, including six type IB endoleaks and six type II endoleaks. Reintervention was required in seven (22.5%) patients. Mean aneurysm sac regression was 9.3 ± 8.7 mm, with a false lumen thrombosis rate of 66.7% and 88.2% for patients with a FU longer than 6 and 12 months respectively., Conclusions: F/Br-EVAR is feasible for patients with a post-dissection TAAA. Although associated with additional technical challenges, and a significant need for reintervention, it leads to favourable aneurysm morphologic changes, and may play a more prominent role in the future for this type of pathology if long-term results confirm the good initial outcome., (Copyright © 2014 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.)

Purpose: To present a single case that clearly demonstrates the progression through the whole spectrum of an acute aortic syndrome, from a solitary penetrating aortic ulcer (PAU) through to multiple PAUs with intra-mural haematoma (IMH) progression. The index images show that despite the clinical presentation of an acute type B aortic dissection, a classical dissection flap is never demonstrated in this patient and thus highlights the need for early input from vascular surgery for all potential acute aortic syndromes., Case Report: We present the case of a 71-year-old lady who attended the emergency department with hypertension and inter-scapular chest pain. Upon investigation with computed tomography aortography (CTA), she was found to have a solitary PAU only with minimal IMH. She did not demonstrate any classical radiological signs of acute type B aortic dissection. She was admitted under a medical team and her chest pain abated with no treatment. The treating medical team adjusted her anti-hypertensive medication and discharged her home with an urgent vascular surgical outpatient appointment for further follow-up and surveillance. We acknowledge that a preferred approach would have been, at least admission and close blood pressure monitoring, with a repeat CTA the next day. Two days later, she represented with further chest pain and on repeat CTA was found to have multiple PAUs and progression of the small IMH. She was then admitted under vascular surgery and subsequently transferred to the parent tertiary referral vascular surgical unit. Despite aggressive anti-hypertensive management, she had persistent intractable chest pain and was treated with a single thoracic stent graft. Completion angiography demonstrated total resolution of the PAUs., Conclusion: This case along with index images demonstrates the whole spectrum of the acute aortic syndrome from a solitary PAU to multiple PAUs with IMH extension. Despite an eventual clinical picture of an acute type B aortic dissection, there was a notable absence of a classical dissection flap on any imaging. We recommend that all aortic pathologies should be at least discussed with and preferably managed by vascular surgeons. The absence of a classical dissection flap on imaging is not a contra-indication to emergent treatment with thoracic stent grafting and in select patients is actually the only therapy that will treat this pathology., (© The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Objective: The VIRTUE Registry describes the mid-term clinical and morphological results of thoracic endovascular repair (TEVR) in patients with type B aortic dissection., Methods: This was a prospective cohort study. The VIRTUE Registry is a prospective, multicentre clinical trial that enrolled patients with complicated acute (<15 days), subacute (15-92 days), and chronic (>92 days) type B aortic dissections treated with the Valiant endograft. One hundred patients were enrolled and the clinical outcomes described at the 3-year follow-up. Analysis of the aortic area and false lumen thrombosis rates defined the morphological response to TEVR in the three clinical groups., Results: Three-year all-cause mortality (18%, 4%, and 24%), dissection related mortality (12%, 4%, and 9%), aortic rupture (2%, 0%, and 4%), retrograde type A dissection (5%, 0%, and 0%), and aortic reintervention rates (20%, 22%, and 39%) were, respectively, defined for patients with acute (n = 50), subacute (n = 24), and chronic (n = 26) dissections. Analysis of aortic morphology observed that patients with subacute dissection demonstrated a similar degree of aortic remodelling to patients with acute dissection. Patients with acute and subacute dissection exhibited greater aortic plasticity than patients with chronic dissection., Conclusions: The principle clinical findings suggest that TEVR is able to provide good protection from aortic-related death in the mid-term, but with a high rate of aortic reintervention. Analysis of aortic morphology suggested that aortic remodelling in subacute patients is similar to the acute group. Retention of aortic plasticity in the subacute group lengthens the therapeutic window for the treatment of uncomplicated type B dissection., (Copyright © 2014 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.)

Patients with chronic aortic dissections are at high risk of catheter-induced complications. We report a 41-year-old patient with a type B aortic dissection (Stanford) who underwent successful three-vessel percutaneous coronary intervention via the right radial artery approach following a non-ST elevation myocardial infarction. The patient remained asymptomatic at 6 mo follow-up. Trans-radial approach for coronary interventions can be used safely in patients with Stanford type B aortic dissection without increasing the risk of procedure- related complications in this high-risk group of patients.

Haemophilus influenzae type b was once the most common cause of invasive H. influenzae infection, but the incidence of this disease has decreased markedly with introduction of conjugate vaccines to prevent the disease. In contrast, the incidence of invasive infection caused by nontypable H. influenzae has increased in the US and in European countries. Neutrophil extracellular traps (NETs) are fibrous structures released extracellularly from activated neutrophils during inflammation, including in pneumonia, and rapidly trap and kill pathogens as a first line of immunological defense. However, their function and pathological role have not been fully investigated. Here, we report a case of fatal nontypable H. influenzae infection with severe pneumonia and bacteremia in an adult found to have a vast amount of NETs in his sputum. The patient had a two-day history of common cold-like symptoms and was taken to the emergency room as a cardiopulmonary arrest. He recovered temporarily, but died soon afterwards, although appropriate antibiotic therapy and general management had been instituted. Massive lobular pneumonia and sepsis due to nontypable H. influenzae was found, in spite of H. influenzae type b vaccine being available. His sputum showed numerous bacteria phagocytosed by neutrophils, and immunohistological staining indicated a number of NETs containing DNA, histone H3, and neutrophil elastase. This case highlights an association between formation of NETs and severe respiratory and septic infection. An increase in severe nontypable H. influenzae disease can be expected as a result of "pathogen shift" due to increased use of the H. influenzae type b vaccine in Japan.