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BACKGROUND: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. METHODS: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. RESULTS: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). CONCLUSION: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.

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The Brazilian Society of Angiology and Vascular Surgery, through the Guidelines Project, presents new Abdominal Aortic Aneurysm Guidelines, on the subject of care for abdominal aortic aneurysm patients. Its development prioritized descriptive guidelines, using the EMBASE, LILACS, and PubMed databases. References include randomized controlled trials, systematic reviews, meta-analyses, and cohort studies. Quality of evidence was evaluated by a pair of coordinators, aided by the RoB 2 Cochrane tool and the Newcastle Ottawa Scale forms. The subjects include juxtarenal aneurysms, infected aneurysms, and new therapeutic techniques, especially endovascular procedures. The current version of the guidelines include important recommendations for the primary topics involving diagnosis, treatment, and follow-up for abdominal aortic aneurysm patients, providing an objective guide for medical practice, based on scientific evidence and widely available throughout Brazil.

A Sociedade Brasileira de Angiologia e Cirurgia Vascular, por meio do projeto Diretrizes, apresenta as novas Diretrizes de Aorta Abdominal, referentes aos cuidados de pacientes com aneurisma de aorta abdominal. Para sua elaboração, foram priorizadas diretrizes descritivas, utilizando as bases EMBASE, LILACS e PubMed. As referências incluem ensaios clínicos randomizados, revisões sistemáticas, metanálises e estudos de coorte. A qualidade das evidências foi examinada por uma dupla de coordenadores, com auxílio da ferramenta RoB 2 da Colaboração Cochrane e dos formulários da Newcastle Ottawa Scale. Aneurismas justarrenais, infectados e novas técnicas terapêuticas, principalmente no âmbito endovascular, estão entre os temas estudados. A versão atual das Diretrizes apresenta importantes recomendações para os principais itens que envolvem o diagnóstico, tratamento e acompanhamento de pacientes com aneurisma de aorta abdominal, oferecendo um guia objetivo para prática médica, construído a partir de evidências científicas e amplamente acessível em todo o território nacional.

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Abstract The Brazilian Society of Angiology and Vascular Surgery, through the Guidelines Project, presents new Abdominal Aortic Aneurysm Guidelines, on the subject of care for abdominal aortic aneurysm patients. Its development prioritized descriptive guidelines, using the EMBASE, LILACS, and PubMed databases. References include randomized controlled trials, systematic reviews, meta-analyses, and cohort studies. Quality of evidence was evaluated by a pair of coordinators, aided by the RoB 2 Cochrane tool and the Newcastle Ottawa Scale forms. The subjects include juxtarenal aneurysms, infected aneurysms, and new therapeutic techniques, especially endovascular procedures. The current version of the guidelines include important recommendations for the primary topics involving diagnosis, treatment, and follow-up for abdominal aortic aneurysm patients, providing an objective guide for medical practice, based on scientific evidence and widely available throughout Brazil.

Resumo A Sociedade Brasileira de Angiologia e Cirurgia Vascular, por meio do projeto Diretrizes, apresenta as novas Diretrizes de Aorta Abdominal, referentes aos cuidados de pacientes com aneurisma de aorta abdominal. Para sua elaboração, foram priorizadas diretrizes descritivas, utilizando as bases EMBASE, LILACS e PubMed. As referências incluem ensaios clínicos randomizados, revisões sistemáticas, metanálises e estudos de coorte. A qualidade das evidências foi examinada por uma dupla de coordenadores, com auxílio da ferramenta RoB 2 da Colaboração Cochrane e dos formulários da Newcastle Ottawa Scale. Aneurismas justarrenais, infectados e novas técnicas terapêuticas, principalmente no âmbito endovascular, estão entre os temas estudados. A versão atual das Diretrizes apresenta importantes recomendações para os principais itens que envolvem o diagnóstico, tratamento e acompanhamento de pacientes com aneurisma de aorta abdominal, oferecendo um guia objetivo para prática médica, construído a partir de evidências científicas e amplamente acessível em todo o território nacional.

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Treatment of ascending aorta disease is surgical; however, some series have evaluated the effectiveness of endovascular treatment. We report the case of a patient with a ruptured pseudoaneurysm who underwent endovascular repair via the left common carotid artery. The clinical and neurological evolution was satisfactory during the in-hospital follow-up. (Level of Difficulty: Intermediate.).

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INTRODUCTION: Approximately 40 cases of spontaneous rupture of the aorta have been described in the literature. Diagnosis can be challenging, and a high index of clinical suspicion enabling prompt treatment can be lifesaving. REPORT: This report presents the case of a 73 year old man who had three multilevel ruptures of the aorta at different times, all treated endovascularly. The patient had a good post-operative course, with no complaints during regular follow up. DISCUSSION: Clinical presentation was non-specific because the ruptures were contained, but spontaneous rupture of the aorta can manifest with a catastrophic presentation and hypovolaemic shock, requiring immediate treatment. The endovascular technique used was safe and resolved the patient's symptoms.

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Abstract Sickle cell anemia is a haematological disorder characterized by multiple vaso-occlusive complications, resulting in a reduced life expectancy. These patients are exposed to several triggering factors for sickle cell crises when they are submitted to cardiovascular surgeries with extracorporeal circulation. Therefore, meticulous care and perioperative management are required. This paper reports a successful case of combined cardiovascular surgery - aortic valve replacement and ascending aortic aneurysmectomy - with no serious post-operative complications. In this report, we emphasize the peculiarities of perioperative care in patients with sickle cell anemia.

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AIM: To summarize the current evidence on psychological issues in thoracic aortic disease (TAD) and infective endocarditis (IE) setting. METHODS: We performed a narrative review about psychological issues in adults with IE and TAD. Through the electronic databases, PubMed and PsycINFO, we searched full manuscripts in English and published until September 1, 2014. RESULTS: We found sixteen studies exploring psychological issues in patients with IE (six studies) and in TAD (ten papers). Psychological issues assessed were quality of life, depression, anxiety and posttraumatic stress disorder. Quality of life was explored in IE (four papers) and in TAD (eight papers). Depression and anxiety were analyzed in TAD only (five papers). Post-traumatic stress disorder was assessed in IE (one study). Quality of life was found impaired in three of four studies about IE and in three of eight studies about TAD. Posttraumatic stress disorder was present in 11% and was associated with lower levels of quality of life in IE patients. In TAD patients, anxiety and depression levels after different invasive interventions did not differ. CONCLUSION: Sixteen studies report about psychological issues in IE and TAD. Most of them explore quality of life and to a less extent anxiety and depression.

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The aim of this work is to report a probable first case of a thoracic aortic aneurysm in the remains of a pre-Columbian individual from South America and to explore the relationship of this case to the only other paleopathological case previously described. We also consider the implications of both cases for the origins of syphilis. This study is based on the macroscopical analysis of human remains recovered during excavation of the Chiu Chiu 273 prehistoric cemetery, in the Antofagasta Region of Northern Chile. Ceramic sherds from the grave have a thermoluminescence date of 2160±100 A.P. or 210 B.C. The skeletal remains of an adult individual display resorptive lesions in both the sternum and the first two thoracic vertebrae, which are suggestive of a thoracic aortic aneurysm. The lesions observed in the case described are clearly compatible with the development of an aneurysm of the thoracic aorta (ascending portion and arch). We suggest that this aneurysm has a syphilitic etiology, considering the vascular segments compromised, the type of lesions observed, and the prevalent etiology of this kind of cardiovascular pathology in pre-penicillin times. Since the only two cases of thoracic aortic aneurysms reported to date have been found in the Americas and are clearly pre-Columbian, it can be suggested that venereal syphilis was present in the Americas in times before European contact.

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Introducción: El reparo endovascular de la aorta torácica (REVAT) se ha incrementado en el mundo en los últimos años con relación al abierto. Objetivo: Comparar los resultados clínicos del REVAT frente al reparo abierto en la Fundación Cardio Infantil (Bogotá, Colombia) entre 2002 y 2011. Metodología: Análisis retrospectivo de la cohorte de pacientes sometidos a reparo abierto (grupo 1) en comparación de REVAT (grupo 2). En cada uno de los grupos se evaluó tiempo quirúrgico, tasa de morbilidad, mortalidad, reintervención y estancia hospitalaria. Resultados: Se incluyeron 57 pacientes en el análisis (26 % reparo abierto; 74 % REVAT). Se reintervinieron dos pacientes por endofugas tipo 1 en el grupo 2 y un caso por sangrado en el grupo 1. La mortalidad operatoria fue para el grupo 1 de 20 %, y para el grupo 2, de 2,3 %. El tiempo quirúrgico fue de 398 ± 180 min (grupo 1) versus 85,5 ± 35 min (grupo 2 (p = 0,0001) y el tiempo de estancia hospitalaria promedio fue de 9,8 días (grupo 1) y 5,3 días (grupo 2) [p = 0,01]). El tiempo promedio de seguimiento fue 4,8 ± 3,1 años. Conclusiones: El REVAT parece ofrecer menor morbilidad, mortalidad, tiempo quirúrgico y estancia hospitalaria respecto al reparo abierto, aunque las poblaciones de pacientes incluidos no fueron estrictamente comparables. Se requieren nuevos análisis en un diseño prospectivo, idealmente aleatorizado para documentar los beneficios a largo plazo de este tipo de reparo...

Introduction: Thoracic Endovascular Aortic Repair(TEVAR) has increased worldwide morethan open repair in the last few years. Objective:To compare clinical outcomes of TEVARversus open surgery at Fundación Cardio Infantil(Bogota, Colombia), between 2002 and 2011.Methods: Retrospective analysis of the cohort ofpatients with open repair (group 1) in comparisonto TEVAR (group 2). In each group, surgicaltime, morbidity rates (infection, hemorrhage,medular ischemia, cerebrovascular event, andpostoperative renal failure), mortality, reintervention,and hospital stay were evaluated. For comparisonsa univariate analysis was used, being a p< 0.05 statistically significant. Results: 57 patientswere included (26 % open repair; 74 % TEVAR).Two type 1 endoleaks on group 2 and 1 bleedingpatient in group 1 required a second surgery. Surgicalmortality was 20 % in group 1, and 2.3 % ingroup 2); surgical time was 398 ± 180 (group 1)versus 85.5 ± 35 min (group 2) (p = 0.0001); andhospital stay was 9,8 days (group 1) and 5.3 days(group 2). Average follow-up time was 4.8 ± 3.1years. Conclusions: TEVAR may be associatedwith less morbidity, mortality, surgical time, andhospital stay than open repair, although the populationsincluded were not strictly comparable.New, prospective studies, ideally randomized,are needed to support the long term benefits ofthis type of repair...

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