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Aortopathy

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https://www.readbyqxmd.com/read/28420669/a-possible-early-biomarker-for-bicuspid-aortopathy-circulating-transforming-growth-factor-beta-1-to-soluble-endoglin-ratio
#1
Amalia Forte, Ciro Bancone, Gilda Cobellis, Marianna Buonocore, Giuseppe Santarpino, Theodor Fischlein, Marilena Cipollaro, Marisa De Feo, Alessandro Della Corte
Rationale: The pathogenesis of bicuspid aortic valve (BAV)-associated aortopathy is poorly understood and no prognostic biomarker is currently available. Objective: We aimed to identify putative circulating biomarkers pathogenetically and prognostically linked to bicuspid aortopathy. Methods and Results: By RT-PCR, we evaluated gene expression variations (versus normal aorta) of transforming growth factor-beta1 (TGF-β1), connective tissue growth factor (CTGF), matrix metalloproteinase-2 (MMP-2), MMP-14, endoglin (ENG) and superoxide dismutase 3 (SOD3) in ascending aorta samples from 50 tricuspid and 70 BAV patients undergoing surgery for aortic stenosis (aorta diameter ≤45mm: BAVnon-dil or >45mm: BAVdil)...
April 18, 2017: Circulation Research
https://www.readbyqxmd.com/read/28387797/genetic-abnormalities-in-bicuspid-aortic-valve-root-phenotype-preliminary-results%C3%A2
#2
Evaldas Girdauskas, Lisa Geist, Kushtrim Disha, Iliaz Kazakbaev, Tatiana Groß, Solveig Schulz, Martin Ungelenk, Thomas Kuntze, Hermann Reichenspurner, Ingo Kurth
OBJECTIVES: Genetic defects associated with bicuspid aortopathy have been infrequently analysed. Our goal was to examine the prevalence of rare genetic variants in patients with a bicuspid aortic valve (BAV) with a root phenotype using next-generation sequencing technology. METHODS: We investigated a total of 124 patients with BAV with a root dilatation phenotype who underwent aortic valve ± proximal aortic surgery at a single institution (BAV database, n  = 812) during a 20-year period (1995-2015)...
April 6, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28381694/aortopathy-in-an-adult-with-tricuspid-atresia-and-left-ventricular-non-compaction-after-fontan-procedure
#3
Tomotaka Murakami, Yoshiki Mori, Nao Inoue, Sachie Kaneko, Yasumi Nakashima
No abstract text is available yet for this article.
April 5, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28377231/prevalence-and-impact-of-coexistent-bicuspid-aortic-valve-in-hypertrophic-cardiomyopathy
#4
Ratnasari Padang, Bernard J Gersh, Steve R Ommen, Jeffrey B Geske
BACKGROUND: The association between bicuspid aortic valve (BAV) and hypertrophic cardiomyopathy (HCM) has been reported but its true prevalence is unknown. This study investigated the prevalence and clinical impact of coexistent BAV in a large referral HCM population. METHODS: Retrospective analysis of 3765 echocardiograms between 2004 and 2014 in 2640 consecutive patients with HCM was performed to assess for BAV. Patients with coexistent conditions were studied...
March 8, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28366467/aortic-root-replacement-for-children-with-loeys-dietz-syndrome
#5
Nishant D Patel, Diane Alejo, Todd Crawford, Narutoshi Hibino, Harry C Dietz, Duke E Cameron, Luca A Vricella
BACKGROUND: Loeys-Dietz syndrome (LDS) is an aggressive aortopathy with a proclivity for aortic aneurysm rupture and dissection at smaller diameters than other connective tissue disorders. We reviewed our surgical experience of children with LDS to validate our guidelines for prophylactic aortic root replacement (ARR). METHODS: We reviewed all children (younger than 18 years) with a diagnosis of LDS who underwent ARR at our institution. The primary endpoint was mortality, and secondary endpoints included complications and the need for further interventions...
March 30, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28356339/nicotinamide-phosphoribosyltransferase-in-smooth-muscle-cells-maintains-genome-integrity-resists-aortic-medial-degeneration-and-is-suppressed-in-human-thoracic-aortic-aneurysm-disease
#6
Alanna Watson, Zengxuan Nong, Hao Yin, Caroline O'Neil, Stephanie Fox, Brittany Balint, Ray Guo, Oberdan Leo, Michael W Chu, Robert Gros, J G Pickering
Rationale: The thoracic aortic wall can degenerate over time with catastrophic consequences. Vascular smooth muscle cells (SMCs) can resist and repair artery damage but their capacities decline with age and stress. Recently, cellular production of NAD(+) via nicotinamide phosphoribosyltransferase (Nampt) has emerged as a mediator of cell vitality. However, a role for Nampt in aortic SMCs in vivo is unknown. Objective: To determine if a Nampt-NAD(+) control system exists within the aortic media and is required for aortic health...
March 29, 2017: Circulation Research
https://www.readbyqxmd.com/read/28347539/a-novel-murine-model-of-marfan-syndrome-accelerates-aortopathy-and-cardiomyopathy
#7
Nicholas B Cavanaugh, Lan Qian, Nicole M Westergaard, William J Kutschke, Ella J Born, Joseph W Turek
BACKGROUND: Marfan syndrome (MFS) represents a genetic disorder with variable phenotypic expression. The main cardiovascular sequelae of MFS include aortic aneurysm/dissection and cardiomyopathy. Although significant advances in the understanding of transforming growth factor beta signaling have led to promising therapeutic targets for the treatment of aortopathy, clinical studies have tempered this optimism. In particular, these studies suggest additional signaling pathways that play a significant role in disease progression...
March 24, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28325072/aortic-assessment-of-bicuspid-aortic-valve-patients-and-their-first-degree-relatives
#8
Pablo Straneo, Gabriel Parma, Natalia Lluberas, Alvaro Marichal, Gerardo Soca, Leandro Cura, Juan J Paganini, Daniel Brusich, Lucia Florio, Victor Dayan
Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives...
March 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28302143/altered-aortic-3d-hemodynamics-and-geometry-in-pediatric-marfan-syndrome-patients
#9
Roel L F van der Palen, Alex J Barker, Emilie Bollache, Julio Garcia, Michael J Rose, Pim van Ooij, Luciana T Young, Arno A W Roest, Michael Markl, Joshua D Robinson, Cynthia K Rigsby
BACKGROUND: Blood flow dynamics make it possible to better understand the development of aortopathy and cardiovascular events in patients with Marfan syndrome (MFS). Aortic 3D blood flow characteristics were investigated in relation to aortic geometry in children and adolescents with MFS. METHODS: Twenty-five MFS patients (age 15.6 ± 4.0 years; 11 females) and 21 healthy controls (age 16.0 ± 2.6 years; 12 females) underwent magnetic resonance angiography and 4D flow CMR for assessment of thoracic aortic size and 3D blood flow velocities...
March 17, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28299607/comprehensive-4-stage-categorization-of-bicuspid-aortic-valve-leaflet-morphology-by-cardiac-mri-in-386-patients
#10
I G Murphy, J Collins, A Powell, M Markl, P McCarthy, S C Malaisrie, J C Carr, A J Barker
Bicuspid aortic valve (BAV) disease is heterogeneous and related to valve dysfunction and aortopathy. Appropriate follow up and surveillance of patients with BAV may depend on correct phenotypic categorization. There are multiple classification schemes, however a need exists to comprehensively capture commissure fusion, leaflet asymmetry, and valve orifice orientation. Our aim was to develop a BAV classification scheme for use at MRI to ascertain the frequency of different phenotypes and the consistency of BAV classification...
March 15, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28270426/aortopathies-in-adult-congenital-heart-disease-and-genetic-aortopathy-syndromes-management-strategies-and-indications-for-surgery
#11
Joey M Kuijpers, Barbara Jm Mulder
No abstract text is available yet for this article.
March 7, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28268004/aortic-shear-stress-in-patients-with-bicuspid-aortic-valve-with-stenosis-and-insufficiency
#12
Yan Shan, Jun Li, Yongshi Wang, Boting Wu, Alex J Barker, Michael Markl, Chunsheng Wang, Xiaolin Wang, Xianhong Shu
OBJECTIVES: Bicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta. METHODS: Four-dimensional flow magnetic resonance imaging was performed in 50 subjects with right-left bicuspid aortic valve and 15 age- and aortic size-matched controls with tricuspid aortic valve...
February 10, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28254817/deficient-circumferential-growth-is-the-primary-determinant-of-aortic-obstruction-attributable-to-partial-elastin-deficiency
#13
Yang Jiao, Guangxin Li, Arina Korneva, Alexander W Caulk, Lingfeng Qin, Matthew R Bersi, Qingle Li, Wei Li, Robert P Mecham, Jay D Humphrey, George Tellides
OBJECTIVE: Williams syndrome is characterized by obstructive aortopathy attributable to heterozygous loss of ELN, the gene encoding elastin. Lesions are thought to result primarily from excessive smooth muscle cell (SMC) proliferation and consequent medial expansion, although an initially smaller caliber and increased stiffness of the aorta may contribute to luminal narrowing. The relative contributions of such abnormalities to the obstructive phenotype had not been defined. APPROACH AND RESULTS: We quantified determinants of luminal stenosis in thoracic aortas of Eln(-/-) mice incompletely rescued by human ELN...
March 2, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28251911/sex-differences-in-phenotypes-of-bicuspid-aortic-valve-and-aortopathy-insights-from-a-large-multicenter-international-registry
#14
William K F Kong, Madelien V Regeer, Arnold C T Ng, Louise McCormack, Kian Keong Poh, Tiong Cheng Yeo, Miriam Shanks, Sarah Parent, Roxana Enache, Bogdan A Popescu, James W Yip, Lawrence Ma, Vasileios Kamperidis, Enno T van der Velde, Bart Mertens, Nina Ajmone Marsan, Victoria Delgado, Jeroen J Bax
BACKGROUND: This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). METHODS AND RESULTS: Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta...
March 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28240702/utility-of-genetic-testing-in-elite-volleyball-players-with-aortic-root-dilation
#15
Nicole Herrick, Christopher Davis, Lisa Vargas, Hal Dietz, Paul Grossfeld
Basketball and volleyball attract individuals with a characteristic biophysical profile, mimicking features of Marfan Syndrome (MFS). Consequently, identification of these abnormalities can be lifesaving. PURPOSE: To determine how physical examination, echocardiography, and genetic screening can identify elite volleyball players with a previously undiagnosed aortopathy. METHODS: We have performed cardiac screening on 90 US Volleyball National Team members and identified four individuals with dilated sinuses of Valsalva...
February 25, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28238234/markers-of-lipid-metabolism-do-not-correlate-with-the-expression-of-aortopathy-in-patients-with-bicuspid-aortic-valve-disease
#16
Tatiana M Sequeira Gross, Thomas Kuntze, Alexander Bernhardt, Hermann Reichenspurner, Yskert von Kodolitsch, Evaldas Girdauskas
BACKGROUND: Controversy exists regarding the potential effects of lipid metabolism on the expression of proximal aortopathy. The study aim was to compare the association between markers of lipid metabolism and the expression of proximal aortopathy in patients with either bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV) disease. METHODS: A total of 458 consecutive patients (68% males) underwent aortic valve replacement (AVR) with or without concomitant aortic surgery between January 2008 and December 2014 at the authors' institution...
September 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28209770/are-patients-with-loeys-dietz-syndrome-misdiagnosed-with-beals-syndrome
#17
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/28203207/morphotype-dependent-flow-characteristics-in-bicuspid-aortic-valve-ascending-aortas-a-benchtop-particle-image-velocimetry-study
#18
Andrew McNally, Ashish Madan, Philippe Sucosky
The bicuspid aortic valve (BAV) is a major risk factor for secondary aortopathy such as aortic dilation. The heterogeneous BAV morphotypes [left-right-coronary cusp fusion (LR), right-non-coronary cusp fusion (RN), and left-non-coronary cusp fusion (LN)] are associated with different dilation patterns, suggesting a role for hemodynamics in BAV aortopathogenesis. However, assessment of this theory is still hampered by the limited knowledge of the hemodynamic abnormalities generated by the distinct BAV morphotypes...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28194732/late-post-avr-progression-of-bicuspid-aortopathy-link-to-hemodynamics
#19
Shiho Naito, Tatiana Gross, Kushtrim Disha, Yskert von Kodolitsch, Hermann Reichenspurner, Evaldas Girdauskas
BACKGROUND AND AIM OF THE STUDY: The ascending aortic dilatation may progress after aortic valve replacement (AVR) in bicuspid aortic valve (BAV) patients. Our aim was to evaluate rheological flow patterns and histological characteristics of the aneurysmal aorta in BAV patients at the time of reoperative aortic surgery. MATERIALS AND METHODS: 13 patients (mean age: 42 ± 9 years, 10 (77%) male) with significant progression of proximal aortopathy after isolated AVR surgery for BAV disease (i...
February 13, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28190607/patient-specific-assessment-of-hemodynamics-by-computational-fluid-dynamics-in-patients-with-bicuspid-aortopathy
#20
Naoyuki Kimura, Masanori Nakamura, Kenji Komiya, Satoshi Nishi, Atsushi Yamaguchi, Osamu Tanaka, Yoshio Misawa, Hideo Adachi, Koji Kawahito
OBJECTIVE: Hemodynamics related to eccentric blood flow may factor into the development of bicuspid aortic valve aortopathy. We investigated wall shear stress distribution by means of magnetic resonance imaging-based computational fluid dynamics in patients with a bicuspid aortic valve. METHODS: Included were 12 patients with a bicuspid aortic valve (aortic stenosis, n = 11; root enlargement, n = 1). Three patients with a normal tricuspid aortic valve (arch aneurysm, n = 1; descending aortic aneurysm, n = 2) were included for comparison...
January 11, 2017: Journal of Thoracic and Cardiovascular Surgery
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