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Aorta bicuspide

James R Thunes, Julie A Phillippi, Thomas G Gleason, David A Vorp, Spandan Maiti
High lethality of aortic dissection necessitates accurate predictive metrics for dissection risk assessment. The not infrequent incidence of dissection at aortic diameters <5.5 cm, the current threshold guideline for surgical intervention (Nishimura et al., 2014), indicates an unmet need for improved evidence-based risk stratification metrics. Meeting this need requires a fundamental understanding of the structural mechanisms responsible for dissection evolution within the vessel wall. We present a structural model of the repeating lamellar structure of the aortic media comprised of elastic lamellae and collagen fiber networks, the primary load-bearing components of the vessel wall...
February 8, 2018: Journal of Biomechanics
Patrizia Presbitero, Loredana Iannetta, Paolo Pagnotta, Bernhard Reimers, Marco L Rossi, Dennis Zavalloni Parenti, Giovanni Bianchi, Francesco Bedogni
BACKGROUND: It is well known that bicuspid valve stenosis can be treated with transcatheter aortic valve implantation (TAVI) even if specific issues can cause problems: dilatation of ascending aorta, possible aorthopathy, eccentricity of the valve and calcium distribution in leaflets and in commissures. We classified Bicuspid aortic valve (BAV) in type 0 (2 cusps and no raphe), and type 1 (2 cusps and one or more raphes). The aim of the present study was to report the results of two types of valve (CoreValve from 2009 to 2016 and Lotus valve from 2014 to 2017) in a consecutive series of BAV patients treated in 2 Italian centers...
April 2018: Minerva Cardioangiologica
Stefan Blunder, Barbara Messner, Bernhard Scharinger, Christian Doppler, Iris Zeller, Andreas Zierer, Günther Laufer, David Bernhard
BACKGROUND AND AIMS: Despite the potential life-threatening consequences of thoracic aortic aneurysms (TAAs), the pathogenesis of these diseases is still poorly understood. While some aspects of TAA formation have been elucidated, the role of vascular smooth muscle cells (SMCs) in both bicuspid aortic valve (BAV)-associated and degenerative tricuspid aortic valve (TAV)-associated TAAs has not yet been fully unravelled. Thus, this work was aimed at uncovering processes in SMC biology that may contribute to TAA formation...
February 5, 2018: Atherosclerosis
Yi-Bin Wang, Yang Li, You-Bin Deng, Ya-Ni Liu, Jun Zhang, Jie Sun, Ying Zhu, Li Li, Qiao-Ying Tang, Wei Zhou
The aim of this study was to test whether enlarged size and impaired elastic properties of the ascending aorta are associated with impaired endothelial function and increases in plasma matrix metalloproteinase (MMP)-2 concentrations in patients with bicuspid aortic valve (BAV) without significant valvular dysfunction. The size and the elasticity of the ascending aorta and the flow-mediated vasodilation (FMD) in the brachial artery in response to hyperemia were evaluated with 2-D echocardiography and high-frequency linear ultrasound in 42 patients with BAV without significant valvular dysfunction and 30 age- and sex-matched healthy controls...
February 20, 2018: Ultrasound in Medicine & Biology
Melissa S W Yamauchi, Michael D Puchalski, Hsin Ti Weng, Nelangi M Pinto, Susan P Etheridge, Angela P Presson, Lloyd Y Tani, L LuAnn Minich, Richard V Williams
BACKGROUND: Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices. METHODS: Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014)...
February 21, 2018: Congenital Heart Disease
Evaldas Girdauskas, Johannes Petersen, Niklas Neumann, Tatiana Groß, Shiho Naito, Mathias Hillebrand, Hermann Reichenspurner, Stefan Blankenberg, Tanja Zeller
OBJECTIVES: We aimed to evaluate the impact of microRNAs to predict the bicuspid aortic valve (BAV)-associated aortopathy. METHODS: Sixty-three BAV patients (mean age 47 ± 11 years, 92% men) with root dilatation, who underwent aortic valve ± proximal aortic surgery (mean post-AVR follow-up 10.3 ± 6.9 years) were included. The BAV aortopathy entities were categorized in the 'less dilated' (aortic root <50 mm) and 'severely dilated' (aortic root ≥50 mm) aorta...
February 14, 2018: Interactive Cardiovascular and Thoracic Surgery
M N Hasan, M K Ahmed, M M Rahman, T Parvin, M B Rashid, F I Khaled, S S Shakil
Coarctation of the aorta is a congenital cardiac malformation that can go undiagnosed until old age with only hypertension as a marker of its presence because clinical signs can be subtle and overlooked if a complete physical exam is not performed. Long-term survival is exceptional in patients with untreated aortic coarctation. In this case report, we present a late diagnosis of aortic coarctation in a 45-year-old male. Our patient was relatively asymptomatic until he presented with exertional dyspnea and fatigue in his fourth decade of life in Bangabandhu Sheikh Mujib Medical University (BSMMU), on the month of August, 2016...
January 2018: Mymensingh Medical Journal: MMJ
Emile S Farag, Pim van Ooij, R Nils Planken, Kayleigh C P Dukker, Frederiek de Heer, Berto J Bouma, Danielle Robbers-Visser, Maarten Groenink, Aart J Nederveen, Bas A J M de Mol, Jolanda Kluin, S Matthijs Boekholdt
BACKGROUND: Use of 4-dimensional flow magnetic resonance imaging (4D-flow MRI) derived wall shear stress (WSS) heat maps enables identification of regions in the ascending aorta with increased WSS. These regions are subject to dysregulation of the extracellular matrix and elastic fiber degeneration, which is associated with aortic dilatation and dissection. PURPOSE: To evaluate the effect of the presence of aortic valve stenosis and the aortic diameter on the peak WSS and surface area of increased WSS in the ascending aorta...
February 16, 2018: Journal of Magnetic Resonance Imaging: JMRI
Oliver Reuthebuch, Luca Koechlin, Ulrich Schurr, Martin Grapow, Jens Fassl, Friedrich S Eckstein
OBJECTIVE: To assess the clinical implementation and report preliminary results of a novel technique called the Ozaki procedure for stentless aortic valve replacement through reconstruction of the valve leaflets from autologous pericardium. METHOD: Between September 2015 and May 2017 30 patients (20 males, mean ± standard deviation age 66.83 ± 10.55 years) suffering from aortic stenosis (AS, n = 7), aortic regurgitation (AR, n = 12), or a combination of both (AS/AR, n = 11) were assigned for an Ozaki procedure...
February 14, 2018: Swiss Medical Weekly
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
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
Harendra Arora, Priya Ajit Kumar
A 37-year-old male presented to the Emergency Department with acute worsening of back pain and new onset dyspnea. Transthoracic echocardiography revealed moderate left ventricular dysfunction and a bicuspid aortic valve (BAV). In addition, he was noted to have a dilated thoracic aorta concerning for a dissection, severe aortic insufficiency (AI), and both a pericardial and pleural effusion. Magnetic resonance imaging revealed a Type A ascending aortic dissection. He was taken emergently to the operating room for repair...
October 2017: Journal of Anaesthesiology, Clinical Pharmacology
Dawid Miśkowiec, Piotr Lipiec, Ewa Szymczyk, Paulina Wejner-Mik, Błażej Michalski, Karolina Kupczyńska, Karina Wierzbowska-Drabik, Jarosław D Kasprzak
AIM: To evaluate the impact of the 2 most common bicuspid aortic valve (BAV) morphology patterns [right-left (RL) vs right-noncoronary (RN) cusp fusion] on the aortic diameters and the impact of gender, aortic stenosis (AS), aortic regurgitation (AR), and age on the observed effects. METHODS: The PubMed databases was searched up to December 31, 2016 to identify studies investigating the morphology of BAV and aortic diameters. Inclusion criteria were as follows: the data on diameter of sinuses of Valsalva (SVD) and/or ascending aorta (AAD) and BAV morphology...
February 4, 2018: Echocardiography
Yuan Wang, Hasan Jilaihawi, Guanyuan Song, Moyang Wang, Bin Lv, Hao Wang, Guannan Niu, Zheng Zhou, Yongjian Wu
To characterize the anatomy of aorto-iliofemoral arterial tree according to aortic valve phenotype by CT in patients referred for transcatheter aortic valve replacement (TAVR). We retrospectively enrolled 215 patients screened for TAVR who underwent CT. Dimensions, calcification, vascular tortuosity index score and other putative risk features of 13 different regions were evaluated for bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) morphology. The study consisted of 44% BAVs with younger age than TAVs...
February 2, 2018: International Journal of Cardiovascular Imaging
Adrian Kolesar, Tomas Toporcer, Milan Bajmoczi, Jan Luczy, Peter Candik, Frantisek Sabol
BACKGROUND: The unicuspid aortic valve (UAV) is a well-described pediatric congenital abnormality, with incidence of 0.02% in the general population. Bicuspidization has been described as a potential surgical option to repair this defect. METHODS: Seventeen symptomatic young patients with a unicuspid valve combined with either valve insufficiency or valve stenosis underwent aortic valve bicuspidization procedure using either an equine pericardium. In addition to bicuspidization, eight patients underwent aortic ring implantation and five patients underwent supracoronary replacement of the aorta...
January 29, 2018: Annals of Thoracic Surgery
Rahul K Bhukar, Deepak Gowda, Jinaga N Rao, Neelam Desai
Ascending aortic aneurysms are rare in children and may lead to aortic insufficiency, dissection, and/or rupture. We present a 5-year-old child diagnosed with an ascending aortic aneurysm and severe aortic insufficiency, successfully treated with ascending aortic replacement with an aortic valve repair using a bicuspidation technique.
January 30, 2018: Journal of Cardiac Surgery
Goki Inno, Yosuke Takahashi, Yasuyuki Kato, Yasuyuki Sasaki
We report the successful surgical treatment of aortic regurgitation in a 27-year-old woman with Turner syndrome (TS) who was admitted with exacerbation of dyspnea on exertion. Echocardiography showed a bicuspid aortic valve with severe aortic regurgitation and computed tomography showed dilatation of the ascending aorta and aortic root. Due to the patient's low body surface area (due to TS), standard determination of aortic size was not possible; therefore, we used the reference curves of aortic diameters in children...
January 25, 2018: General Thoracic and Cardiovascular Surgery
Jean-Baptiste Michel, Guillaume Jondeau, Dianna M Milewicz
Vascular Smooth Muscle Cells (vSMCs) play a crucial role in both the pathogenesis of Aneurysms and Dissections of the ascending thoracic aorta (TAAD) in humans and in the associated adaptive compensatory responses, since thrombosis and inflammatory processes are absent in the majority of cases. Aneurysms and dissections share numerous characteristics, including aetiologies and histopathological alterations: vSMC disappearance, medial areas of mucoid degeneration, and ExtraCellular Matrix (ECM) breakdown. Three aetiologies predominate in TAAD in humans: i) genetic causes in heritable familial forms, ii) an association with bicuspid aortic valves and iii) a sporadic degenerative form linked to the aortic aging process...
January 17, 2018: Cardiovascular Research
Ümit Yaşar Sinan, Burcu Dirlik Serim, Rukiye Yıldırım, Özge Çetinarslan, Mehmet Serdar Küçükoğlu
Infective endocarditis (IE) is an infectious disease that affects the endothelium of the large intrathoracic vessels, heart valves, and intra-cardiac foreign body material. A 20-year-old woman was admitted to the cardiology department with complaints of fever and palpitations. Transthoracic echocardiography revealed a bicuspid aortic valve, aortic root enlargement, and aortic coarctation. Transesophageal echocardiography revealed a bicuspid aortic valve, but there was no vegetation. Methicillin-sensitive Staphylococcus aureus was identified on a blood culture...
January 2018: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
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
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