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Thoracic aortic aneurysms and dissection

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https://www.readbyqxmd.com/read/28715511/endovascular-treatment-of-genetically-linked-aortic-diseases
#1
D Böckler, K Meisenbacher, A S Peters, C Grond-Ginsbach, M S Bischoff
BACKGROUND: The most important structural proteins of the vascular wall are collagen and elastin. Genetically linked connective tissue diseases lead to degeneration, aneurysm formation and spontaneous dissection or rupture of arteries. The most well-known are Marfan syndrome, vascular Ehlers-Danlos syndrome (type IV), Loeys-Dietz syndrome and familial aortic aneurysms and dissections. OBJECTIVE: This review article addresses the current status of endovascular treatment options for important connective tissue diseases...
2017: Gefässchirurgie: Zeitschrift Für Vaskuläre und Endovaskuläre Chirurgie
https://www.readbyqxmd.com/read/28701309/arhgap18-protects-against-thoracic-aortic-aneurysm-formation-by-mitigating-the-synthetic-and-pro-inflammatory-smooth-muscle-cell-phenotype
#2
Renjing Liu, Lisa Lo, Angelina J Lay, Yang Zhao, Ka Ka Ting, Elizabeth N Robertson, Andrew G Sherrah, Sorour R Jarrah, Haibo Li, Zhaoxiong Zhou, Brett D Hambly, David R Richmond, Richmond W Jeremy, Paul G Bannon, Matthew A Vadas, Jennifer Gamble
Rationale: Thoracic aortic aneurysm (TAA) is a potentially lethal condition which can affect individuals of all ages. TAA may be complicated by the sudden onset of life threatening dissection or rupture. The underlying mechanisms leading to TAA formation, particularly in the non-syndromal idiopathic group of patients, are not well understood. Thus, identification of new genes and targets that are involved in TAA pathogenesis are required to help prevent and/or reverse the disease phenotype. Objective: Here we explore the role of ARHGAP18, a novel Rho GAP expressed by smooth muscle cells (SMC), in the pathogenesis of TAA...
July 12, 2017: Circulation Research
https://www.readbyqxmd.com/read/28696036/cervical-artery-dissection-expands-the-cardiovascular-phenotype-in-fbn1-related-weill-marchesani-syndrome
#3
Kelsey Newell, Wendy Smith, Brian Ghoshhajra, Eric Isselbacher, Angela Lin, Mark E Lindsay
Weill-Marchesani syndrome (WMS) is a rare form of acromelic dysplasia that is characterized by distinctive skeletal, ocular, and cardiovascular abnormalities. Previously described cardiac manifestations of WMS include aortic and pulmonary valve stenosis, mitral valve prolapse, mitral stenosis, and QTc prolongation. Autosomal dominant forms of WMS result from heterozygous pathogenic variants in FBN1, a gene with a well characterized role in the pathogenesis of thoracic aortic aneurysm (TAA) in the context of Marfan syndrome...
July 11, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28693381/a-meta-analysis-of-weekend-admission-and-surgery-for-aortic-rupture-and-dissection
#4
Hisato Takagi, Tomo Ando, Takuya Umemoto
We performed a meta-analysis to determine whether weekend admission and surgery for ruptured abdominal/thoracic aortic aneurysm (RAAA/RTAA) and acute aortic dissection (AAD) is associated with increased mortality. MEDLINE and EMBASE were searched from January 1946 to December 2016 using PubMed and OVID. Eligible studies were prospective or retrospective, comparative or cohort studies enrolling patients admitting or undergoing surgery for RAAA/RTAA/AAD and reporting mortality after weekend (including holiday) versus weekday admission/surgery...
July 1, 2017: Vascular Medicine
https://www.readbyqxmd.com/read/28693049/hybrid-repair-techniques-for-complex-aneurysms-and-dissections-involving-the-aortic-arch-and-thoracic-aorta
#5
Rami Tadros, Scott R Safir, Peter L Faries, Daniel K Han, Rajiv K Chander, James F McKinsey, Michael L Marin, Allan S Stewart, Sharif Ellozy
Aortic aneurysms involving the ascending aorta, aortic arch, and descending thoracic aorta have been a challenging entity to surgically treat for over 60 years. Despite the mortality of the disease, early open surgical procedures also had significant morbidity and mortality. The inherent risk in treating multiple anatomic segments simultaneously led to the innovation of the staged elephant trunk (ET) approach by Borst in 1983. To avoid the thoracotomy and associated complications related to the second stage of the procedure, an endovascular completion paradigm was begun by Volodos in 1991...
July 11, 2017: Surgical Technology International
https://www.readbyqxmd.com/read/28691213/central-cannulation-strategy-for-extent-i-thoracoabdominal-aneurysm-repair-of-chronic-type-b-aortic-dissection
#6
Reilly D Hobbs, Tyler J Wallen, Caroline M Komlo, Patrick J Moeller, Alberto Pochettino, Joseph E Bavaria, Prashanth Vallabhajosyula
INTRODUCTION: We evaluated the safety profile of a central cardiopulmonary bypass (CPB) cannulation strategy for repair of extent I thoracoabdominal aortic aneurysms (TAAA) with chronic type B dissection in comparison to traditional peripheral CPB cannulation strategies. METHODS: Patients undergoing extent I TAAA repair for chronic type B dissection from 2002 to 2011 were retrospectively reviewed. Patients were grouped by their CPB cannulation strategy. Patients in Group I underwent central aortic cannulation (n = 28) through a left thoracotomy incision...
July 10, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28689484/transcatheter-aortic-valve-implantation-as-a-bailout-procedure-for-acute-aortic-valve-regurgitation-during-endovascular-arch-repair
#7
Adrien Hertault, Rachel E Clough, Thomas Modine, Jean-Luc Auffray, Mohamad Koussa, Stéphan Haulon
PURPOSE: To report emergent transcatheter aortic valve implantation (TAVI) to treat acute severe aortic regurgitation caused by valve cusp dysfunction following proximal migration of an endograft implanted in the ascending aorta during endovascular arch repair. CASE REPORT: A 65-year-old man had been previously treated with thoracic and fenestrated endografts in a 2-stage procedure for a chronic type B dissection. At 2-year follow-up, aneurysmal evolution of the distal arch led to development of a proximal type Ia endoleak...
July 1, 2017: Journal of Endovascular Therapy
https://www.readbyqxmd.com/read/28678310/the-expression-of-the-brm-and-mmp2-genes-in-thoracic-aortic-aneurysm-and-aortic-dissection
#8
Y-H Li, X-M Li, M-S Lu, M-F Lv, X Jin
OBJECTIVE: To study the expression, roles, and clinical significance of Brahma (BRM) and matrix metalloproteinase 2 (MMP2) in the thoracic aortic aneurysm and aortic dissection. PATIENTS ND METHODS: Arterial specimens from 20 cases of thoracic aortic dissection and 38 cases of thoracic aortic aneurysm, as well as normal tissue were collected, paraffin-embedded, sectioned, and stained with anti-BRM and MMP2 monoclonal antibodies. Sections were analyzed by immunofluorescence, and the distribution and expression of BRM and MMP2 in the aortic wall were determined...
June 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28666732/a-functional-variant-of-smad4-enhances-thoracic-aortic-aneurysm-and-dissection-risk-through-promoting-smooth-muscle-cell-apoptosis-and-proteoglycan-degradation
#9
Ying Wang, Hao-Yue Huang, Guang-Liang Bian, Yun-Sheng Yu, Wen-Xue Ye, Fei Hua, Yi-Huan Chen, Zhen-Ya Shen
Recent studies indicate important roles for SMAD4 in SMCs proliferation, extracellular matrix maintenance, and blood vessel remodeling. However, the genetic effects of SMAD4 in the pathogenesis of thoracic aortic aneurysm and dissection (TAAD) are still largely unknown. Here we identified a functional variant of SMAD4 which might be involved in the pathological progression of TAAD. Five tagging SNPs of SMAD4 were genotyped in 202 TAAD cases and 400 controls using MALDI-TOF. rs12455792 CT or TT variant genotypes was associated with an significantly elevated TAAD risk (adjusted OR=1...
June 22, 2017: EBioMedicine
https://www.readbyqxmd.com/read/28659818/managing-thoracic-aortic-aneurysm-in-patients-with-bicuspid-aortic-valve-based-on-aortic-root-involvement
#10
REVIEW
Elizabeth Norton, Bo Yang
Bicuspid aortic valve (BAV) can be both sporadic and hereditary, is phenotypically variable, and genetically heterogeneous. The clinical presentation of BAV is diverse and commonly associated with a high prevalence of valvular dysfunction producing altered hemodynamics and aortic abnormalities (e.g., aneurysm and dissection). The thoracic aortic aneurysm (TAA) in BAV frequently involves the proximal aorta, including the aortic root, ascending aorta, and aortic arch, but spares the aorta distal to the aortic arch...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28652363/vascular-disease-causing-mutation-smooth-muscle-%C3%AE-actin-r258c-dominantly-suppresses-functions-of-%C3%AE-actin-in-human-patient-fibroblasts
#11
Zhenan Liu, Audrey N Chang, Frederick Grinnell, Kathleen M Trybus, Dianna M Milewicz, James T Stull, Kristine E Kamm
The most common genetic alterations for familial thoracic aortic aneurysms and dissections (TAAD) are missense mutations in vascular smooth muscle (SM) α-actin encoded by ACTA2 We focus here on ACTA2-R258C, a recurrent mutation associated with early onset of TAAD and occlusive moyamoya-like cerebrovascular disease. Recent biochemical results with SM α-actin-R258C predicted that this variant will compromise multiple actin-dependent functions in intact cells and tissues, but a model system to measure R258C-induced effects was lacking...
July 11, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28649221/notch-1-mutation-in-a-patient-with-spontaneous-and-recurrent-dissections-of-extracranial-arteries
#12
Carlos Guevara, Gonzalo Farias, Kateryna Bulatova, Pablo Alarcón, Wendy Soruco, Carlos Robles, Marcelo Morales
Dissections of extracranial arteries are estimated to account for only 2% of all ischemic strokes but for approximately 20% of strokes in patients younger than 45 years old. Most dissections of extracranial arteries involve some trauma stretch, mechanical stress, or connective tissue abnormalities. In the absence of these disorders, determining the etiology of recurrent extracranial dissections is quite challenging because the underlying nature of these cases is poorly understood. We report the case of a 44-year-old female with recurrent dissections of the vertebral and carotid arteries associated with a heterozygous mutation p...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28648480/a-10-year-institutional-experience-with-open-branched-graft-reconstruction-of-aortic-aneurysms-in-connective-tissue-disorders-versus-degenerative-disease
#13
Caitlin W Hicks, Jennifer Lue, Natalia O Glebova, Bryan A Ehlert, James H Black
OBJECTIVE: Aortic reconstruction for complex thoracoabdominal aortic aneurysms (TAAAs) can be challenging, especially in patients with connective tissue disorders (CTDs) in whom tissue fragility is a major concern. Branched graft reconstruction is a more complex operation compared with inclusion patch repair of the aorta but is frequently necessary in patients with CTDs or other pathologies because of anatomic reasons. We describe our institutional experience with open branched graft reconstruction of aortic aneurysms and compare outcomes for patients with CTDs vs degenerative pathologies...
June 22, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28638687/aneurysm-of-the-ascending-aorta-in-systemic-lupus-erythematosus-case-report-and-review-of-the-literature
#14
Hector Corominas, Maria Tsokos, Martha Quezado, George C Tsokos
Cardiovascular manifestations in patients with systemic lupus erythematosus (SLE) are common, but aortic aneurysm formation is rare. We present a 63-year-old male SLE patient with a two-year history of skin lesions, leucopenia, pericarditis, mitral valve vegetations consistent with Liebman-Sacks endocarditis, and an aneurysm of the ascending aorta, which was successfully repaired surgically. Histologic examination of the aneurysm showed medial cystic degeneration, smooth muscle necrosis, and mild adventitial perivascular lymphocytic aggregates...
June 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28631201/seven-esophageal-perforation-cases-after-aortic-replacement-stenting-for-thoracic-aortic-dissection-or-aneurysm
#15
Yoshihisa Yaguchi, Yoshimasa Kumata, Masahiro Horikawa, Takashi Kiyokawa, Tsuyoshi Inaba, Ryoji Fukushima
BACKGROUND: Esophageal perforation after aortic replacement/stenting for aortic dissection or aneurysm is a rare but severe complication. However, its cause, standard treatment, and prognosis are unclear. We analyzed the treatment and outcome retrospectively from seven cases experienced at our hospital. CASE PRESENTATION: The median age of the patients was 70 years (range, 41-86), and six of the seven cases were male. As the first treatment, aortic replacement techniques were performed in five, and thoracic endovascular aortic repair (TEVAR) procedure was performed in two...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28607545/the-genetics-of-aortopathies-in-clinical-cardiology
#16
REVIEW
Amit Goyal, Ali R Keramati, Matthew J Czarny, Jon R Resar, Arya Mani
Aortopathies pose a significant healthcare burden due to excess early mortality, increasing incidence, and underdiagnosis. Understanding the underlying genetic causes, early diagnosis, timely surveillance, prophylactic repair, and family screening are keys to addressing these diseases. Next-generation sequencing continues to expand our understanding of the genetic causes of heritable aortopathies, rapidly clarifying their underlying molecular pathophysiology and suggesting new potential therapeutic targets...
2017: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/28606160/consumption-coagulopathy-in-acute-aortic-dissection-principles-of-management
#17
Yuyong Liu, Lu Han, Jiachen Li, Ming Gong, Hongjia Zhang, Xinliang Guan
BACKGROUND: The effect of acute aortic dissection itself on coagulopathy or surgery-related coagulopathy has never been specifically studied. The aim of the present study was to perioperatively describe consumption coagulopathy in patients with acute aortic dissection. METHODS: Sixty-six patients with acute type A aortic dissection were enrolled in this study from January 2015 to September 2016. Thirty-six patients with thoracic aortic aneurysms were used as a control group during the same period...
June 12, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28605479/collapsed-stent-graft-and-severe-malperfusion-2%C3%A2-years-after-endovascular-aortic-repair
#18
Yoshihiro Goto, Soh Hosoba, Shinji Ogawa, Yoshihisa Kinoshita
Late collapsing of a stent graft is an extremely rare event, with one existing report describing this phenomenon. A 65-year-old man with a history of endovascular aortic repair for an abdominal aortic aneurysm presented with paraplegia and bilateral lower limb ischaemia. Contrast-enhanced computed tomography showed a dissection of the descending thoracic aorta and a collapsed stent graft, resulting in bilateral lower limb ischaemia. Subsequent axillo-bifemoral bypass resolved his lower extremity functions. The patient required haemodialysis due to acute kidney failure...
June 10, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28600386/associations-of-age-and-sex-with-marfan-phenotype-the-national-heart-lung-and-blood-institute-gentac-genetically-triggered-thoracic-aortic-aneurysms-and-cardiovascular-conditions-registry
#19
Mary J Roman, Richard B Devereux, Liliana R Preiss, Federico M Asch, Kim A Eagle, Kathryn W Holmes, Scott A LeMaire, Cheryl L Maslen, Dianna M Milewicz, Shaine A Morris, Siddharth K Prakash, Reed E Pyeritz, William J Ravekes, Ralph V Shohet, Howard K Song, Jonathan W Weinsaft
BACKGROUND: The associations of age and sex with phenotypic features of Marfan syndrome have not been systematically examined in a large cohort of both children and adults. METHODS AND RESULTS: We evaluated 789 Marfan patients enrolled in the National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry (53% male; mean age 31 [range: 1-86 years]). Females aged ≥15 and males aged ≥16 years were considered adults based on average age of skeletal maturity...
June 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28597717/physician-modified-thoracic-stent-grafts-for-the-treatment-of-aortic-arch-lesions
#20
Ludovic Canaud, Toshio Baba, Thomas Gandet, Kouhei Narayama, Baris Ata Ozdemir, Tsuyoshi Shibata, Pierre Alric, Kiyofumi Morishita
PURPOSE: To evaluate outcomes of physician-modified thoracic stent-grafts for the treatment of aortic arch aneurysms. METHODS: A retrospective dual-center analysis was performed involving 36 patients (mean age 74.7±9 years, range 58-91; 27 men) with an aortic arch lesion who were treated between November 2013 and June 2016 using physician-modified thoracic stent-grafts. Half of the patients had a degenerative aneurysm; the remainder had type B dissection (n=9), traumatic transection (n=3), type Ia endoleak after previous endografting (n=5), or aortoesophageal fistula (n=1)...
June 1, 2017: Journal of Endovascular Therapy
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