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https://www.readbyqxmd.com/read/28230898/manifestations-and-medicolegal-significance-of-loeys-dietz-syndrome
#1
Roger W Byard
Loeys-Dietz syndrome is a recently described autosomal dominant disorder with underlying vasculopathy characterized by aortic and other vascular aneurysmal dissection/rupture. A 61-year-old man is reported who died suddenly and unexpectedly and at autopsy was found to have a ruptured abdominal aortic aneurysm. Additional findings included dolichostenomelia, high-arched palate, and pectus excavatum. There was a strong family history of Loeys-Dietz syndrome, although the decedent had never been tested. Death was, therefore, due to a ruptured abdominal aortic aneurysm in a case of probable Loeys-Dietz syndrome...
February 23, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28219577/method-of-hemorrhage-control-from-the-aorta-after-repair-of-a-dissected-aortic-aneurysm
#2
Joseph Galea, Alexander Manché
Patients with Marfan syndrome and presenting with acute aortic dissection have fragile aorta, causing bleeding problems in the anastomosis between the graft and the aorta. Hemostatic impairment following circulatory arrest and prolonged cardiopulmonary bypass contribute to these problems. We describe a technique to control persistent bleeding from the anastomosis in the aortopulmonary recess using two kissing inflated balloons at the tip of two Foley catheters to tamponade the bleeding sites.
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28219557/total-endovascular-repair-of-post-dissection-aortic-arch-aneurysm-with-chimney-technique
#3
Wei-Tao Liang, Su Wang, Jian Zhou, Chen-Jun Han, Qiang Liu, Xiao-Yun Wu, Wang-Fu Zang
Open surgery remains the standard procedure for treatment of aortic arch pathologies. However, total endovascular repair can be a safe option for patients who are poor candidates for surgery because of compromised physiology. We report the case of a 63-year-old man with a post-dissection aortic arch aneurysm. We repaired the aneurysm using thoracic endovascular aortic repair (TEVAR) and used the chimney technique to reconstruct the supra-aortic branches. The patient is doing well after 12-month follow-up, demonstrating that TEVAR with the chimney technique can be used successfully for the treatment of post-dissection arch aneurysm...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28217382/pipeline-embolization-device-as-primary-treatment-for-cervical-internal-carotid-artery-pseudoaneurysms
#4
Arthur Wang, Justin Santarelli, Michael F Stiefel
BACKGROUND: Limited data exists on the durability and occlusion rate of treating extracranial cervical internal carotid artery pseudoaneurysms using the pipeline embolization device (PED) flow-diverting stent. METHODS: Three patients presenting with dissecting cervical internal carotid artery pseudoaneurysms were treated with the PED as the sole treatment modality. RESULTS: In all three patients, successful aneurysmal occlusion and parent vessel reconstruction occurred on immediate angiography and continued on 6-month follow-up...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28216363/durable-outcomes-of-thoracic-endovascular-aortic-repair-with-zenith-tx1-and-tx2-devices
#5
Jocelyn M Beach, Yuki Kuramochi, Corey Brier, Eric E Roselli, Matthew J Eagleton
OBJECTIVE: Long-term data regarding the safety and durability of thoracic endovascular aortic repair (TEVAR) are limited. The study objective was to evaluate the long-term outcomes of TEVAR in high-risk patients with descending thoracic aortic pathology. METHODS: High-risk patients were treated with thoracic endografts (2001-2011) under a prospective, physician-sponsored, investigational device exemption trial. Three-dimensional reconstructions and measurements were performed on computed tomography scans acquired before discharge, at 1, 6, and 12 months, and then yearly thereafter...
February 16, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28216358/outcomes-of-thoracic-endovascular-aortic-repair-using-aortic-arch-chimney-stents-in-high-risk-patients
#6
Igor Voskresensky, Salvatore T Scali, Robert J Feezor, Javairiah Fatima, Kristina A Giles, Rosamaria Tricarico, Scott A Berceli, Adam W Beck
BACKGROUND: Aortic arch disease is a challenging clinical problem, especially in high-risk patients, in whom open repair can have morbidity and mortality rates of 30% to 40% and 2% to 20%, respectively. Aortic arch chimney (AAC) stents used during thoracic endovascular aortic repair (TEVAR) are a less invasive treatment strategy than open repair, but the current literature is inconclusive about the role of this technology. The focus of this analysis is on our experience with TEVAR and AAC stents...
February 16, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28216353/left-subclavian-artery-revascularization-in-zone-2-thoracic-endovascular-aortic-repair-is-associated-with-lower-stroke-risk-across-all-aortic-diseases
#7
Rhiannon J Bradshaw, S Sadie Ahanchi, Obie Powell, Sebastian Larion, Colin Brandt, Michael C Soult, Jean M Panneton
BACKGROUND: The best management strategy for the left subclavian artery (LSA) in pathologic processes of the aorta requiring zone 2 thoracic endovascular aortic repair (TEVAR) remains controversial. We compared LSA coverage with or without revascularization as well as the different means of LSA revascularization. METHODS: A retrospective chart review was conducted of patients with any aortic diseases who underwent zone 2 TEVAR deployment from 2007 to 2014. Primary end points included 30-day stroke and 30-day spinal cord injury (SCI)...
February 16, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28216343/treatment-strategy-based-on-the-natural-course-for-patients-with-spontaneous-isolated-superior-mesenteric-artery-dissection
#8
Seon-Hee Heo, Young-Wook Kim, Shin-Young Woo, Yang-Jin Park, Kwang-Bo Park, Duk-Kyung Kim
OBJECTIVE: The optimal treatment of spontaneous isolated superior mesenteric artery dissection (SISMAD) is still not known, and we sought to determine an optimal treatment strategy for patients with SISMAD based on its natural clinical course. METHODS: We retrospectively reviewed consecutive patients with SISMAD treated from 2001 through 2016. Diagnosis and angiographic type of SISMAD were determined with contrast-enhanced computed tomography (CT) scan, and the clinical features were obtained using a fixed form questionnaire...
February 16, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28212053/transthoracic-echocardiography-pitfalls-and-limitations-as-delineated-at-cardiac-ct-and-mr-imaging
#9
Sachin B Malik, Natalie Chen, Rex A Parker, Joe Y Hsu
Transthoracic echocardiography ( TTE transthoracic echocardiography ) is a critical tool in the field of clinical cardiology. It often serves as one of the first-line imaging modalities in the evaluation of cardiac disease owing to its low cost, portability, widespread availability, lack of ionizing radiation, and ability to evaluate both anatomy and function of the heart. Consequently, a large majority of patients undergoing a cardiac computed tomography (CT) or magnetic resonance (MR) imaging examination will have a TTE transthoracic echocardiography available for review...
February 17, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28209770/are-patients-with-loeys-dietz-syndrome-misdiagnosed-with-beals-syndrome
#10
Rebecca Woolnough, Andrew Dhawan, Kimberly Dow, Jagdeep S Walia
Beals syndrome, also known as congenital contractural arachnodactyly (Online Mendelian Inheritance in Man: 121050), is an autosomal dominant disorder caused by a mutation in FBN2 that is typically characterized by congenital contractures and arachnodactyly. It shares a number of phenotypic features with Loeys-Dietz syndrome (Online Mendelian Inheritance in Man: 609192). Loeys-Dietz syndrome, initially described in 2005, is associated with mutations for the transforming growth factor β receptor and is characterized by findings of cerebral, thoracic, and abdominal arterial aneurysms...
February 16, 2017: Pediatrics
https://www.readbyqxmd.com/read/28207465/two-rare-variants-of-left-vertebral-artery
#11
Rajani Singh
Though the variations of vertebral artery are clinically asymptomatic yet abnormalities are of diagnostic importance either prior to vascular surgery in the neck region or in patients of intravascular diseases such as arteriovenous malformations or cerebral aneurysms. Therefore, the aim of the study is to bring out 2 variations in the configuration of vertebral artery and their clinical implication. During dissection of thorax of 2 female cadavers, 2 different variants of configurations of left vertebral arteries were observed...
February 15, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28190713/transient-atrial-fibrillation-after-open-abdominal-aortic-revascularization-surgery-is-associated-with-increased-length-of-stay-mortality-and-readmission-rates
#12
Barbara A Blanco, Anai N Kothari, Pegge M Halandras, Robert H Blackwell, Dawn M Graunke, Paul C Kuo, Jae S Cho
BACKGROUND: It is well established that transient postoperative atrial fibrillation (TPAF) is associated with adverse postoperative outcomes after major cardiac and noncardiac operations. The purpose of this study was to elucidate the incidence, impact, and risk factors associated with the development of TPAF in patients undergoing revascularization surgery for occlusive diseases of the abdominal aorta and its branches (AAB). METHODS: By use of the Healthcare Cost and Utilization Project State Inpatient Database from Florida and California, patients who underwent open revascularization of AAB between 2006 and 2011 were identified...
February 9, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28190547/surgical-outcomes-of-chronic-descending-dissections-type-i-versus-iii-debakey
#13
Ivancarmine Gambardella, Mario Gaudino, Christopher Lau, Monica Munjal, Mohamed Elsayed, Leonard N Girardi
BACKGROUND: We evaluated the results of open operation for the treatment of descending thoracic aneurysm (DTA) and thoracoabdominal aortic aneurysm (TAAA) in patients with DeBakey type I versus type III chronic aortic dissection. METHODS: We reviewed our institutional aortic database and compared the results of open repair in patients with type I versus type III chronic aortic dissection. Between 1997 and 2015, 726 patients underwent open DTA or TAAA repair at our institution...
February 9, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28186882/multi-modality-imaging-enables-detailed-hemodynamic-simulations-in-dissecting-aneurysms-in-mice
#14
Evan Phillips, Paolo Di Achille, Matthew Bersi, Jay Humphrey, Craig Goergen
A multi-modality imaging based modeling approach was used to study complex unsteady hemodynamics and lesion growth in a dissecting abdominal aortic aneurysm model. We combined in vivo ultrasound (geometry and flow) and in vitro optical coherence tomography (geometry) to obtain the high resolution needed to construct detailed hemodynamic simulations over large portions of the murine vasculature, which include fine geometric complexities. We illustrate this approach for a spectrum of dissecting abdominal aortic aneurysms induced in male apolipoprotein E-null mice by high-dose angiotensin II infusion...
February 6, 2017: IEEE Transactions on Medical Imaging
https://www.readbyqxmd.com/read/28185953/a-novel-smad3-mutation-caused-multiple-aneurysms-in-a-patient-without-osteoarthritis-symptoms
#15
Audrey Courtois, Wouter Coppieters, Vincent Bours, Jean-Olivier Defraigne, Alain Colige, Natzi Sakalihasan
Heterozygous mutations in the SMAD3 gene were recently described as the cause of a form of non-syndromic familial aortic thoracic aneurysm and dissection (FTAAD) transmitted as an autosomal dominant disorder and often associated with early-onset osteoarthritis. This new clinical entity, called aneurysms-osteoarthritis syndrome (AOS) or Loeys-Dietz syndrome 3 (LDS3), is characterized by aggressive arterial damages such as aneurysms, dissections and tortuosity throughout the arterial tree. We report, here, the case of a 45 year-old man presenting multiple visceral arteries and abdominal aortic aneurysms but without dissection of the thoracic aorta and without any sign of osteoarthritis...
February 6, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28180096/the-frozen-elephant-trunk-technique-european-association-for-cardio-thoracic-surgery-position-and-bologna-experience
#16
REVIEW
Luca Di Marco, Antonio Pantaleo, Alessandro Leone, Giacomo Murana, Roberto Di Bartolomeo, Davide Pacini
Complex lesions of the thoracic aorta are traditionally treated in 2 surgical steps with the elephant trunk technique. A relatively new approach is the frozen elephant trunk (FET) technique, which potentially allows combined lesions of the thoracic aorta to be treated in a 1-stage procedure combining endovascular treatment with conventional surgery using a hybrid prosthesis. These are very complex and time-consuming operations, and good results can be obtained only if appropriate strategies for myocardial, cerebral, and visceral protection are adopted...
February 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28174405/-thoracic-endovascular-aortic-repair-for-a-descending-thoracic-aneurysm-associated-with-pseudocoarctation-of-the-aorta-report-of-a-case
#17
Hiroaki Aida, Satoru Kagaya, Kazuo Abe
A 77-year-old man with a descending thoracic aneurysm associated with pseudocoarctation of the aorta underwent thoracic endovascular aortic repair (TEVAR). Computed tomography demonstrated kinking of the descending thoracic aorta. The aneurysm was located distal to a kinking and constricting aortic isthmus. TEVAR was performed because of the patient's comorbidity such as valvular heart diseases and postradiation status for the thoracic Hodgkin disease. TEVAR was successful but small aortic dissection was occurred distal to the stentgraft...
February 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28173744/glutathione-system-participation-in-thoracic-aneurysms-from-patients-with-marfan-syndrome
#18
Alejandra María Zúñiga-Muñoz, Israel Pérez-Torres, Verónica Guarner-Lans, Elías Núñez-Garrido, Rodrigo Velázquez Espejel, Claudia Huesca-Gómez, Ricardo Gamboa-Ávila, María Elena Soto
Aortic dilatation in Marfan syndrome (MFS) is progressive. It is associated with oxidative stress and endothelial dysfunction that contribute to the early acute dissection of the vessel and can result in rupture of the aorta and sudden death. We evaluated the participation of the glutathione (GSH) system, which could be involved in the mechanisms that promote the formation and progression of the aortic aneurysms in MFS patients. Patients and methods: Aortic aneurysm tissue was obtained during chest surgery from eight control subjects and 14 MFS patients...
February 8, 2017: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/28168982/two-decades-of-experience-with-root-remodeling-and-valve-repair-for-bicuspid-aortic-valves
#19
Ulrich Schneider, Susanne K Feldner, Christopher Hofmann, Jakob Schöpe, Stefan Wagenpfeil, Christian Giebels, Hans-Joachim Schäfers
OBJECTIVE: Bicuspid aortic valve anatomy is associated with ascending aortic aneurysm in approximately 50% of individuals and may lead to severe aortic regurgitation with aortic dilatation. Both entities may be treated by valve repair and root remodeling. The objective was to review the cumulative experience of 20 years. METHODS: Between November 1995 and December 2015, 357 patients (324 male; age 10-80 years; mean, 49 ± 13 years) underwent combined bicuspid aortic valve repair and root remodeling...
January 10, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28168690/postmortem-serum-tryptase-levels-with-special-regard-to-acute-cardiac-deaths
#20
Ning Xiao, Dong-Ri Li, Qi Wang, Fu Zhang, Yan-Geng Yu, Hui-Jun Wang
An elevated serum tryptase concentration is considered a specific marker for systemic mast cell activation, a central feature of anaphylaxis. However, in some cases of acute cardiovascular death, high concentrations of serum tryptase are also observed. We compared the postmortem serum tryptase concentrations in 74 cases assigned to the following four groups: anaphylactic deaths (Group A, n = 20), acute cardiac deaths (Group ACD, n = 30), acute dissecting aneurysm ruptures (Group ADA, n = 10), and controls (Group C, n = 14)...
February 7, 2017: Journal of Forensic Sciences
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