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https://www.readbyqxmd.com/read/29433109/pathogenesis-of-aortic-wall-complications-in-marfan-syndrome
#1
Nimrat Grewal, Adriana C Gittenberger-de Groot
BACKGROUND: Patients with Marfan (MFS) syndrome and patients with a bicuspid aortic valve (BAV) are more prone to develop aortic dilation and dissection compared to persons with a tricuspid aortic valve (TAV). To elucidate potential common as well as distinct pathways of clinical relevance, we compared the histopathological substrates of aortic pathology. PATIENT AND METHODS: Ascending aortic wall specimen were divided in five groups: BAV (n=36) and TAV (n=23) without and with dilation and non-dilated MFS (n=8)...
February 2, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/29426841/altered-dna-methylation-indicates-an-oscillatory-flow-mediated-epithelial-to-mesenchymal-transition-signature-in-ascending-aorta-of-patients-with-bicuspid-aortic-valve
#2
Hanna M Björck, Lei Du, Silvia Pulignani, Valentina Paloschi, Karin Lundströmer, Alexandra S Kostina, Cecilia Österholm, Anna Malashicheva, Anna Kostareva, Arturo Evangelista, Gisela Teixidó-Tura, Shohreh Maleki, Anders Franco-Cereceda, Per Eriksson
Disturbed flow has been suggested to contribute to aneurysm susceptibility in bicuspid aortic valve (BAV) patients. Lately, flow has emerged as an important modulator of DNA methylation. Hear we combined global methylation analysis with in vitro studies of flow-sensitive methylation to identify biological processes associated with BAV-aortopathy and the potential contribution of flow. Biopsies from non-dilated and dilated ascending aortas were collected from BAV (n = 21) and tricuspid aortic valve (TAV) patients (n = 23)...
February 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29362194/coronary-button-pseudoaneurysms-after-aortic-root-replacement-in-a-child-with-loeys-deitz-syndrome
#3
Charles D Fraser, Xun Zhou, Rui Han Liu, Cecillia Lui, Duke E Cameron, Marshall L Jacobs, Luca A Vricella, Narutoshi Hibino
Loeys-Deitz syndrome (LDS) is a connective tissue disorder characterized by aggressive aortopathy with a proclivity for aortic aneurysmal rupture and dissection. Prophylactic surgical intervention is often indicated to ameliorate risk of aneurysm rupture. Aortic root replacement involves excision of the coronary arteries from the aortic root with a button of surrounding aortic tissue and subsequent anastomosis of these buttons to the synthetic aortic graft. We report the case of a 16-year-old girl with LDS who developed pseudoaneurysms at the sites of previous coronary button implantation...
February 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29362193/ascending-aortic-aneurysm-in-a-child-with-fibulin-4-deficiency
#4
Makoto Hibino, Yoshimasa Sakai, Wataru Kato, Keisuke Tanaka, Kazuyoshi Tajima, Takehiko Yokoyama, Mitsuji Iwasa, Hiroko Morisaki, Toyonori Tsuzuki, Akihiko Usui
EFEMP2 (alias FBLN4) encodes extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B and leads to severe aortopathy with aneurysm formation and vascular tortuosity. A 4-month-old child presented with a large ascending aortic aneurysm, and genetic testing revealed an EFEMP2 mutation. We achieved successful repair of the ascending aortic aneurysm at 33 months of age and report the macroscopic and microscopic findings.
February 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29357934/the-importance-of-genotype-phenotype-correlation-in-the-clinical-management-of-marfan-syndrome
#5
Víctor Manuel Becerra-Muñoz, Juan José Gómez-Doblas, Carlos Porras-Martín, Miguel Such-Martínez, María Generosa Crespo-Leiro, Roberto Barriales-Villa, Eduardo de Teresa-Galván, Manuel Jiménez-Navarro, Fernando Cabrera-Bueno
BACKGROUND: Marfan syndrome (MFS) is a disorder of autosomal dominant inheritance, in which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) gene mutations are found in more than 90% of MFS cases. The aim of our study was to summarise variants in FBN-1 and establish the genotype-phenotype correlation, with particular interest in the onset of aortic events, in a broad population of patients with an initial clinical suspicion of MFS. MATERIAL AND METHODS: This single centre prospective cohort study included all patients presenting variants in the FBN-1 gene who visited a Hereditary Aortopathy clinic between September 2010 and October 2016...
January 22, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29346529/echocardiographic-criteria-to-detect-unicuspid-aortic-valve-morphology
#6
Sebastian Ewen, Irem Karliova, Petra Weber, Stephan H Schirmer, Hashim Abdul-Khaliq, Jakob Schöpe, Felix Mahfoud, Hans-Joachim Schäfers
Aims: Unicuspid aortic valve (UAV) is a rare congenital malformation associated with severe aortic stenosis or regurgitation. This study aimed to systematically determine echocardiographic criteria to identify UAV. Methods and results: All patients underwent a preoperative baseline examination, including echocardiography. A total of 69 patients with intraoperatively confirmed UAV underwent an aortic valve repair procedure between August 2001 and May 2011. To compare the findings of UAV cases with those of other valve morphologies, we examined 99 consecutive patients with a bicuspid aortic valve (BAV) and 103 consecutive patients with a tricuspid aortic valve (TAV) undergoing isolated aortic valve surgery before May 2016...
January 15, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29334594/thoracic-aortic-aneurysm-in-patients-with-loss-of-function-filamin-a-mutations-clinical-characterization-genetics-and-recommendations
#7
Ming Hui Chen, Sangita Choudhury, Mami Hirata, Siri Khalsa, Bernard Chang, Christopher A Walsh
The frequency and gender distribution of thoracic aortic aneurysm as a cardiovascular manifestation of loss-of-function (LOF) X-linked FilaminA (FLNA) mutations are not known. Furthermore, there is very limited cardiovascular morbidity or mortality data in children and adults. We analyzed cardiac data on the largest series of 114 patients with LOF FLNA mutations, both children and adults, with periventricular nodular heterotopia (PVNH), including 48 study patients and 66 literature patients, median age of 22...
February 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29332374/transcatheter-aortic-valve-replacement-in-bicuspid-aortic-valve-stenosis-where-do-we-stand
#8
Sung-Han Yoon, Rahul Sharma, Tarun Chakravarty, Masaki Miyasaka, Tomoki Ochiai, Takahiro Nomura, Norman Gellada, Shadi Nemanpour, Mamoo Nakamura, Wen Chen, Raj Makkar
Bicuspid aortic valve is the most common congenital cardiac defect in adults, and symptom typically develops in adulthood. In the majority of cases, bicuspid aortic valve disease progress with ages and surgical aortic valve replacement is performed with excellent operative outcomes. However, with the relatively slow progression of disease, surgical aortic valve replacement is required in elderly patients but the surgical risk often deemed extremely high due to old age and multiple comorbidities. Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for intermediate- and high-risk patients with symptomatic severe aortic valve stenosis (AS)...
January 11, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29329990/transcatheter-aortic-valve-implantation-in-morphologically-complex-root-aneurysms
#9
Markus Mach, Bernhard Winkler, David Santer, Harald Pisarik, Sandra Folkmann, Marieluise Harrer, Gabriel Weiss, Franz Veit, Christoph Adlbrecht, Andreas Strouhal, Georg Delle-Karth, Martin Grabenwöger
Performing transcatheter aortic valve implantation in the presence of aneurysmatic aortopathy is widely contraindicated; however, it needs to be taken into account as bailout strategy in selected patients. Deliberate preoperative assessing measurements become the crucial key element in this context. After meticulous valve selection, retrograde access is obtained via the right subclavian artery additionally serving as backup arterial cannulation site in case of conversion. TAVI is then performed via the transapical route...
January 9, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29320330/cardiovascular-manifestations-and-complications-of-loeys-dietz-syndrome-ct-and-mr-imaging-findings
#10
William W Loughborough, Kishore S Minhas, Jonathan C L Rodrigues, Stephen M Lyen, Helen E Burt, Nathan E Manghat, Marcus J Brooks, Graham Stuart, Mark C K Hamilton
Loeys-Dietz syndrome (LDS) is a recently described genetic connective tissue disorder with a wide spectrum of multisystem involvement. LDS is characterized by rapidly progressive aortic and peripheral arterial aneurysmal disease. LDS and the other inherited aortopathies such as Marfan syndrome have overlapping phenotypic features. However, LDS is characterized by a more aggressive vascular course; patient morbidity and mortality occur at an early age, with complications developing at relatively smaller aortic dimensions...
January 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29286269/landmark-lecture-perloff-lecture-tribute-to-professor-joseph-kayle-perloff-and-lessons-learned-from-him-aortopathy-in-adults-with-chd
#11
Koichiro Niwa
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery...
December 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29251110/aortic-graft-at-coronary-artery-bypass-surgery-as-a-source-of-human-aortic-smooth-muscle-cells
#12
Daria Kostina, Dmitry Zverev, Vadim Grebennik, Mikhail Gordeev, Elena Ignatieva, Irina Voronkina, Anna Kostareva, Anna Malashicheva
One of the serious obstacles of the aortopathies research is a considerable shortage of human aortic smooth muscle cells (SMCs), which can be used to model the disease. SMC in most cases come from the whole aorta of transplant donors, which are rather difficult to access. In the course of coronary artery bypass graft (CABG) surgery, a fragment of aortic tissue is excised to make a bypass root. In this study, we show a possibility to use CABG leftover fragments of thoracic aorta as a source of human SMC for in vitro research...
October 2017: Cell Transplantation
https://www.readbyqxmd.com/read/29248286/perioperative-evaluation-of-regional-aortic-wall-shear-stress-patterns-in-patients-undergoing-aortic-valve-and-or-proximal-thoracic-aortic-replacement
#13
Emilie Bollache, Paul W M Fedak, Pim van Ooij, Ozair Rahman, S Chris Malaisrie, Patrick M McCarthy, James C Carr, Alex Powell, Jeremy D Collins, Michael Markl, Alex J Barker
OBJECTIVES: To assess in patients with aortopathy perioperative changes in thoracic aortic wall shear stress (WSS), which is known to affect arterial remodeling, and the effects of specific surgical interventions. METHODS: Presurgical and postsurgical aortic 4D flow MRI were performed in 33 patients with aortopathy (54 ± 14 years; 5 women; sinus of Valsalva (d_SOV)/midascending aortic (d_MAA) diameters = 44 ± 5/45 ± 6 mm) scheduled for aortic valve (AVR) and/or root (ARR) replacement...
November 16, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29226889/assessment-of-bicuspid-aortic-valve-phenotypes-and-associated-pathologies-a-transesophageal-echocardiographic-study
#14
Selcen Yakar Tülüce, Kamil Tülüce, Ersin Çağrı Şimşek, Özgen Şafak, Mehmet Şefa Ökten, Zeynep Yapan Emren, Sadık Volkan Emren, Uğur Kocabaş, Serdar Bayata, Cem Nazlı
OBJECTIVE: We investigated the frequency of different bicuspid aortic valve disease (BAV) phenotypes,the associated valvular pathologies, and the aortopathy phenotypes, using 2-dimensional (2D) transthoracic, 2D transesophageal echocardiography (TEE) and 3-dimensional (3D) TEE. METHODS: A total of 154 patients with BAV were included. Five BAV phenotypes were detected. To better define valvular pathologies, binary classifications of BAV were used: BAV with antero-posterior commisural line (BAV-AP) and right-left commissural line (BAV-RL)...
December 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29223844/bicuspid-aortopathy-seeing-the-forest-for-the-trees
#15
EDITORIAL
Kevin L Greason
No abstract text is available yet for this article.
November 7, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29223836/everybody-is-different-a-plea-for-individualizing-treatment-of-aortopathy
#16
EDITORIAL
Hans-Hinrich Sievers
No abstract text is available yet for this article.
November 20, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29198801/bicuspid-aortic-valve-associated-aortopathy-a-slowly-evolving-picture
#17
EDITORIAL
Christian D Etz, Michael A Borger
No abstract text is available yet for this article.
November 29, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29169750/bicuspid-aortic-valve-a-complex-aortopathy-rather-than-a-simple-valvulopathy
#18
EDITORIAL
Guido Gelpi, Claudia Romagnoni
No abstract text is available yet for this article.
January 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29169748/ascending-aortic-size-in-aortic-coarctation-depends-on-aortic-valve-morphology-understanding-the-bicuspid-valve-phenotype
#19
Erik L Frandsen, Luke J Burchill, Abigail M Khan, Craig S Broberg
BACKGROUND: In roughly half of patients with coarctation of the aorta (CoA), the aorta may be enlarged. It is uncertain whether enlargement is independent of aortic valve morphology. We sought to compare aortic size in CoA with a tricuspid valve (TAV) to those with bicuspid aortic valve (BAV). METHODS: Sixty-eight CoA patients and 20 healthy controls with prior cardiac magnetic resonance (CMR) imaging were included. CMR was retrospectively reanalyzed to measure aortic root and mid-ascending aorta...
January 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29165337/mirnome-profiling-in-bicuspid-aortic-valve-associated-aortopathy-by-next-generation-sequencing
#20
Andrea Borghini, Ilenia Foffa, Silvia Pulignani, Cecilia Vecoli, Lamia Ait-Ali, Maria Grazia Andreassi
The molecular mechanisms underlying thoracic aortic aneurysm (TAA) in patients with bicuspid aortic valve (BAV) are incompletely characterized. MicroRNAs (miRNAs) may play a major role in the different pathogenesis of aortopathy. We sought to employ next-generation sequencing to analyze the entire miRNome in TAA tissue from patients with BAV and tricuspid aortic valve (TAV). In the discovery stage, small RNA sequencing was performed using the Illumina MiSeq platform in 13 TAA tissue samples (seven patients with BAV and six with TAV)...
November 22, 2017: International Journal of Molecular Sciences
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