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https://www.readbyqxmd.com/read/29684119/aortic-stiffness-in-adolescent-turner-and-marfan-syndrome-patients
#1
Michal Schäfer, Lorna P Browne, Uyen Truong, James J Jaggers, Max B Mitchell, Ladonna Malone, Gareth Morgan, Kathryn Chatfield, Daniel McLennan, Harma Turbendian, Danial Vargas, Brian Fonseca, Michael DiMaria, Anar Shah, Margaret P Ivy, Alex J Barker, Kendall S Hunter, Neil Wilson, D Dunbar Ivy, David N Campbell
OBJECTIVES: Turner syndrome (TS) and Marfan syndrome (MFS) are partially characterized by aortopathies with a risk of developing severe aortic dilation, stiffness and consequent dissection and aneurysm formation. The incidence of a bicuspid aortic valve (BAV) is also increased in TS. We investigated aortic stiffness in teenage TS and MFS patients and evaluated to what degree stiffness in TS patients is augmented by the presence of a BAV. METHODS: Fifty-seven patients with TS (n = 37) and MFS (n = 20), as well as 22 controls with similar age and size distribution underwent evaluation of thoracic aortic stiffness using phase-contrast magnetic resonance imaging...
April 19, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29680713/bicuspid-aortic-valve-aortopathy-in-the-era-of-fake-news-a-clinician-s-perspective-on-the-truth
#2
EDITORIAL
Hector I Michelena
No abstract text is available yet for this article.
March 29, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29671812/enlightening-the-association-between-bicuspid-aortic-valve-and-aortopathy
#3
REVIEW
Froso Sophocleous, Elena Giulia Milano, Giulia Pontecorboli, Pierpaolo Chivasso, Massimo Caputo, Cha Rajakaruna, Chiara Bucciarelli-Ducci, Costanza Emanueli, Giovanni Biglino
Bicuspid aortic valve (BAV) patients have an increased incidence of developing aortic dilation. Despite its importance, the pathogenesis of aortopathy in BAV is still largely undetermined. Nowadays, intense focus falls both on BAV morphology and progression of valvular dysfunction and on the development of aortic dilation. However, less is known about the relationship between aortic valve morphology and aortic dilation. A better understanding of the molecular pathways involved in the homeostasis of the aortic wall, including the extracellular matrix, the plasticity of the vascular smooth cells, TGFβ signaling, and epigenetic dysregulation, is key to enlighten the mechanisms underpinning BAV-aortopathy development and progression...
April 19, 2018: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/29666014/assessing-wall-stresses-in-bicuspid-aortic-valve-associated-aortopathy-forecasting-the-perfect-storm
#4
EDITORIAL
Alex J Barker, Michael Markl, Paul W M Fedak
No abstract text is available yet for this article.
March 31, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29656820/wall-stress-on-ascending-thoracic-aortic-aneurysms-with-bicuspid-compared-with-tricuspid-aortic-valve
#5
Yue Xuan, Zhongjie Wang, Raymond Liu, Henrik Haraldsson, Michael D Hope, David A Saloner, Julius M Guccione, Liang Ge, Elaine Tseng
OBJECTIVE: Guidelines for repair of bicuspid aortic valve-associated ascending thoracic aortic aneurysms have been changing, most recently to the same criteria as tricuspid aortic valve-ascending thoracic aortic aneurysms. Rupture/dissection occurs when wall stress exceeds wall strength. Recent studies suggest similar strength of bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms; thus, comparative wall stress may better predict dissection in bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms...
March 8, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29656818/the-aortic-root-does-not-dilate-over-time-after-replacement-of-the-aortic-valve-and-ascending-aorta-in-patients-with-bicuspid-or-tricuspid-aortic-valves
#6
Sonya K Hui, Chun-Po Steve Fan, Shakira Christie, Christopher M Feindel, Tirone E David, Maral Ouzounian
OBJECTIVE: Whether the aortopathy associated with bicuspid aortic valve (BAV) disease occurs secondary to genetic or hemodynamic factors remains controversial. In this article we describe the natural history of the aortic root in patients with bicuspid versus tricuspid aortic valves (TAVs) after replacement of the aortic valve and ascending aorta. METHODS: From 1990 to 2010, 406 patients (269 BAV, 137 TAV) underwent aortic valve and ascending aorta replacement at a single institution...
March 13, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29653232/gata6-loss-of-function-mutation-contributes-to-congenital-bicuspid-aortic-valve
#7
Ying-Jia Xu, Ruo-Min Di, Qi Qiao, Xiu-Mei Li, Ri-Tai Huang, Song Xue, Xing-Yuan Liu, Juan Wang, Yi-Qing Yang
Congenital bicuspid aortic valve (BAV), the most common form of birth defect in humans, is associated with substantial morbidity and mortality. Increasing evidence demonstrates that genetic risk factors play a key role in the pathogenesis of BAV. However, BAV is a genetically heterogeneous disease and the genetic determinants underpinning BAV in an overwhelming majority of patients remain unknown. In the present study, the coding exons and flanking introns of the GATA6 gene, which encodes a zinc-finger transcription factor essential for the normal development of the aortic valves, were sequenced in 152 unrelated patients with congenital BAV...
April 10, 2018: Gene
https://www.readbyqxmd.com/read/29652290/absent-right-superior-vena-cava-and-persistent-left-superior-vena-cava-in-a-patient-with-bicuspid-aortic-valve-with-aortic-stenosis
#8
Kushant Gupta, Vijayakanth Bhuvana, Varun Bansal, Ruma Ray, Arkalgud Sampath Kumar
Persistent left superior vena cava (LSVC) with absent right SVC (RSVC) is a rare congenital anomaly. If undetected, the condition may pose difficulties in central venous catheter insertion, pacemaker electrode insertion, and cannulation during cardiopulmonary bypass. We describe a case of persistent LSVC with absent RSVC, who was diagnosed to have bicuspid aortic valve with aortic stenosis.
April 2018: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/29643145/single-coronary-artery-with-bicuspid-aortic-valve
#9
Dominika M Zoltowska, Yashwant Agrawal, Guramrinder Singh Thind, Jagadeesh K Kalavakunta
No abstract text is available yet for this article.
April 11, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29625103/aortic-valve-reoperation-following-stentless-bioprosthesis-short-and-long-term-outcomes
#10
Bo Yang, Himanshu J Patel, Elizabeth L Norton, Christina Debenedictus, Linda Farhat, Xiaoting Wu, Kevin He, Whitney E Hornsby, Donald S Likosky, G Michael Deeb
BACKGROUND: Limited data are available regarding outcomes for stentless aortic valve reoperation. The reported reoperative mortality has been unacceptably high. METHODS: Between 1997-2017, a retrospective analysis was performed on 143 patients that underwent open aortic valve reoperations for failed stentless aortic valve bioprostheses. We evaluated both short- and long-term outcomes on this cohort of patients. RESULTS: Bicuspid aortic valve was present in 107/143 (75%) of patients at the time of the initial Freestyle procedure and 84% (120/143) patients underwent a modified inclusion aortic root replacement procedure...
April 3, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29622096/management-of-patients-with-aortic-valve-stenosis
#11
REVIEW
Amrit Kanwar, Jeremy J Thaden, Vuyisile T Nkomo
With increased life expectancy and aging of the population, aortic stenosis is now one of the most common valvular heart diseases. Early recognition and management of aortic stenosis are of paramount importance because untreated symptomatic severe disease is universally fatal. The advent of transcather aortic valve replacement technologies provides exciting avenues of care to patients with this disease in whom traditional surgical procedures could not be performed or were associated with high risk. This review for clinicians offers an overview of aortic stenosis and updated information on the current status of various treatment strategies...
April 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29615572/the-role-of-hedgehog-signalling-in-the-formation-of-the-ventricular-septum
#12
REVIEW
Antonia Wiegering, Ulrich Rüther, Christoph Gerhardt
An incomplete septation of the ventricles in the vertebrate heart that disturbes the strict separation between the contents of the two ventricles is termed a ventricular septal defect (VSD). Together with bicuspid aortic valves, it is the most frequent congenital heart disease in humans. Until now, life-threatening VSDs are usually treated surgically. To avoid surgery and to develop an alternative therapy (e.g., a small molecule therapy), it is necessary to understand the molecular mechanisms underlying ventricular septum (VS) development...
December 12, 2017: Journal of Developmental Biology
https://www.readbyqxmd.com/read/29606322/valve-performance-classification-in-630-subcoronary-ross-patients-over-22-years
#13
Hans-Hinrich Sievers, Ulrich Stierle, Michael Petersen, Stefan Klotz, Doreen Richardt, Michael Diwoky, Efstratios I Charitos
OBJECTIVE: To define the function of the "Ross valves" and its clinical meaning in a practical valve performance classification as part of the outcome analysis. METHODS: From 1994 to 2017, 630 consecutive patients underwent the subcoronary Ross procedure at our institution. The valve performance classification combines hemodynamics, symptoms, and management criteria. Median follow-up was 12.5 years (maximum 22.3 years, 7404 patient-years, 99.4% completeness)...
March 12, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29598880/unusual-prebypass-echocardiographic-findings-before-bicuspid-aortic-valve-surgery
#14
Denis Lemesle, Clarisse Berroëta, Paola Mascitti, Nizar Khelil, Marie-Christine Malergue, Ivan Philip
No abstract text is available yet for this article.
March 1, 2018: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29590334/a-rare-missense-mutation-in-myh6-associates-with-non-syndromic-coarctation-of-the-aorta
#15
Thorsteinn Bjornsson, Rosa B Thorolfsdottir, Gardar Sveinbjornsson, Patrick Sulem, Gudmundur L Norddahl, Anna Helgadottir, Solveig Gretarsdottir, Audur Magnusdottir, Ragnar Danielsen, Emil L Sigurdsson, Berglind Adalsteinsdottir, Sverrir I Gunnarsson, Ingileif Jonsdottir, David O Arnar, Hrodmar Helgason, Tomas Gudbjartsson, Daniel F Gudbjartsson, Unnur Thorsteinsdottir, Hilma Holm, Kari Stefansson
Aims: Coarctation of the aorta (CoA) accounts for 4-8% of congenital heart defects (CHDs) and confers substantial morbidity despite treatment. It is increasingly recognized as a highly heritable condition. The aim of the study was to search for sequence variants that affect the risk of CoA. Methods and results: We performed a genome-wide association study of CoA among Icelanders (120 cases and 355 166 controls) based on imputed variants identified through whole-genome sequencing...
March 24, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29579303/impact-of-the-bicuspid-aortic-valve-on-aortic-root-haemodynamics-three-dimensional-computed-fluid-dynamics-simulation
#16
Denis Berdajs, Selim Mosbahi, Friedrich S Eckstein, Dominique Charbonnier, Enrico Ferrari, Ludwig K von Segesser
OBJECTIVES: The aim was to evaluate the impact of a bicuspid aortic valve (BAV) on local shear stress and on the pressure profile on the elements of the aortic root (AoR). METHODS: The experiment setup included a BAV with aortic valve stenosis (n = 5 pigs, 67 ± 3.5 kg) and insufficiency (n = 5 pigs, 66.7 ± 4.4 kg). By implanting 6 high-fidelity microsonometric crystals in each AoR, we determined the 3-dimensional (3D) geometry of the AoR. Experimental and geometric data were used to create a 3D time- and pressure-dependent computed fluid dynamic model of the AoR with the BAV...
March 22, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29572020/the-genetics-of-bicuspid-aortic-valve-disease-shall-we-take-it-down-a-notch
#17
EDITORIAL
Leora B Balsam
No abstract text is available yet for this article.
March 2, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29567669/gata6-regulates-aortic-valve-remodeling-and-its-haploinsufficiency-leads-to-rl-type-bicuspid-aortic-valve
#18
Lara Gharibeh, Hiba Komati, Yohan Bossé, Munir Boodhwani, Mahyar Heydarpour, Megan Fortier, Romina Hassandazeh, Janet Ngu, Patrick Mathieu, Simon Body, Mona Nemer
Background -Bicuspid aortic valve (BAV), the most common congenital heart defect affecting 1-2% of the population, is a major risk factor for premature aortic valve disease and accounts for the majority of valve replacement. The genetic basis and the mechanisms of BAV etiology and pathogenesis remain largely undefined. Methods -Cardiac structure and function was assessed in mice lacking a Gata6 allele. Human GATA6 gene variants were analyzed in 452 BAV cases from the BAV consortium and 1849 controls from the Framingham GWAS study...
March 22, 2018: Circulation
https://www.readbyqxmd.com/read/29567131/midterm-outcomes-after-aortic-valve-neocuspidization-with-glutaraldehyde-treated-autologous-pericardium
#19
Shigeyuki Ozaki, Isamu Kawase, Hiromasa Yamashita, Shin Uchida, Mikio Takatoh, Nagaki Kiyohara
OBJECTIVE: We had previously reported the short-term results of the aortic valve neocuspidization (AVNeo) procedure. We have now evaluated the midterm results with the longest follow-up of 118 months. METHODS: From April 2007 through December 2015, 850 patients were treated with AVNeo using autologous pericardium. Medical records of these patients were retrospectively reviewed. The procedure was on the basis of independent tricuspid replacement using autologous pericardium...
February 15, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29563378/infective-endocarditis-associated-with-atopic-dermatitis
#20
Shigeaki Aoyagi, Takeshi Oda, Kumiko Wada, Eiji Nakamura, Tomokazu Kosuga, Hiroshi Yasunaga
We report a case of aortic valve infective endocarditis (IE) in a 24-year-old man with atopic dermatitis (AD). He had a history of balloon valvuloplasty for a stenotic bicuspid aortic valve, and had dental caries but no invasive dental procedure before the onset of IE. On admission, skin lesions of AD with itching and scratches were found on the neck, trunk, and extremities. Echocardiography showed a vegetation on the aortic valve with mild steno-regurgitation, but extension of IE to the annulus was not detected...
March 20, 2018: International Heart Journal
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