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Aortopathy

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https://www.readbyqxmd.com/read/28209770/are-patients-with-loeys-dietz-syndrome-misdiagnosed-with-beals-syndrome
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28185644/bicuspid-aortic-valve-morphotype-correlates-with-regional-antioxidant-gene%C3%A2-expression-profiles-in-the-proximal%C3%A2-ascending-aorta
#5
Julie A Phillippi, Jennifer C Hill, Marie Billaud, Benjamin R Green, Mary P Kotlarczyk, Thomas G Gleason
BACKGROUND: Bicuspid aortic valve (BAV) is associated with asymmetric dilatation of the proximal ascending aorta. We previously demonstrated increased susceptibility of smooth muscle cells to oxidative stress in the BAV-aneurysmal aorta and hypothesized that antioxidant expression is regionally defined and influenced by the BAV morphotype. METHODS: BAV valve morphology was defined according to number of raphes: type 0 (0 raphes), type 1 (1 raphe), or type 2 (2 raphes) and by the raphe location among the left (L), right (R) or non (N) coronary cusps...
February 6, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28185277/three-dimensional-parametric-modeling-of-bicuspid-aortopathy-and-comparison-with-computational-flow-predictions
#6
Salvatore Pasta, Giovanni Gentile, Giuseppe M Raffa, Francesco Scardulla, Diego Bellavia, Angelo Luca, Michele Pilato, Cesare Scardulla
Bicuspid aortic valve (BAV)-associated ascending aneurysmal aortopathy (namely "bicuspid aortopathy") is a heterogeneous disease making surgeon predictions particularly challenging. Computational flow analysis can be used to evaluate the BAV-related hemodynamic disturbances, which likely lead to aneurysm enlargement and progression. However, the anatomic reconstruction process is time consuming so that predicting hemodynamic and structural evolution by computational modeling is unfeasible in routine clinical practice...
February 10, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28182589/bicuspid-aortic-valve-aortopathy-mechanistic-and-clinical-insights-from-recent-studies
#7
David G Guzzardi, Subodh Verma, Paul W M Fedak
PURPOSE OF REVIEW: This focused review summarizes key insights from the past 12 months of basic science and clinical research on bicuspid aortic valve (BAV)-associated aortopathy. RECENT FINDINGS: Recent studies in BAV-associated aortopathy support a heterogeneous spectrum of disease with distinct phenotypes. Basic science studies provide further support for the concept of regional differences in the severity of aortopathy within the aorta of BAV patients. Clinical studies compared outcomes of BAV patients after isolated aortic valve replacement and showed that those with primarily valvular insufficiency as compared with stenosis may be at greater risk for important aortic events over time...
March 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28151876/late-onset-radiation-induced-constrictive-pericarditis-and-cardiomyopathy-after-radiotherapy-a-case-report
#8
Xiao-Feng Zhuang, Yan-Min Yang, Xiao-Lu Sun, Zhong-Kai Liao, Jie Huang
INTRODUCTION: Radiation-induced heart disease (RIHD) is a serious side effect of cancer treatment, including coronary artery disease, valvular cardiac dysfunction, cardiomyopathy, aortopathy, and chronic constrictive pericarditis. Herein, this case we present was diagnosed as radiation-induced constrictive pericarditis and cardiomyopathy by means of cardiac magnetic resonance (CMR) and transthoracic echocardiogram, finally confirmed by pathology after performing heart transplant operation...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28149605/the-timing-of-elective-ascending-aortic-aneurysm-replacement-for-non-syndromic-patients-and-the-implication-of-bicuspid-aortic-valve-related-aortopathy
#9
EDITORIAL
Ali Khoynezhad
No abstract text is available yet for this article.
December 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28148315/assessment-of-the-structure-and-function-of-the-aorta-by-echocardiography
#10
REVIEW
Anitha Parthiban, Girish Shirali
Echocardiography is the primary modality for imaging the aorta for the diagnosis and serial evaluation of pathological conditions. In this article, we review the methodology for optimal echocardiographic imaging of the various segments of the aorta and discuss abnormalities of the aorta including stenosis, dilation including aortopathy and sinus of Valsalva aneurysms, and fistulous communications involving the ascending aorta including aortoventricular tunnel and ruptured sinus of Valsalva aneurysm. We review novel echocardiographic measurements of aortic functional properties of the aorta such as elasticity and stiffness, and review the literature on the potential additive value of such measurements for structural assessment alone...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28119285/aortopathy-in-a-mouse-model-of-marfan-syndrome-is-not-mediated-by-altered-transforming-growth-factor-%C3%AE-signaling
#11
Hao Wei, Jie Hong Hu, Stoyan N Angelov, Kate Fox, James Yan, Rachel Enstrom, Alexandra Smith, David A Dichek
BACKGROUND: Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin-1 (FBN1); however, the mechanisms through which fibrillin-1 deficiency causes MFS-associated aortopathy are uncertain. Recently, attention was focused on the hypothesis that MFS-associated aortopathy is caused by increased transforming growth factor-β (TGF-β) signaling in aortic medial smooth muscle cells (SMC). However, there are many reasons to doubt that TGF-β signaling drives MFS-associated aortopathy...
January 24, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28102444/patients-with-bicuspid-and-tricuspid-aortic-valve-exhibit-distinct-regional-microrna-signatures-in-mildly-dilated-ascending-aorta
#12
Sebastian Albinsson, Alessandro Della Corte, Azra Alajbegovic, Katarzyna K Krawczyk, Ciro Bancone, Umberto Galderisi, Marilena Cipollaro, Marisa De Feo, Amalia Forte
MicroRNAs are able to modulate gene expression in a range of diseases. We focused on microRNAs as potential contributors to the pathogenesis of ascending aorta (AA) dilatation in patients with stenotic tricuspid (TAV) or bicuspid aortic valve (BAV). Aortic specimens were collected from the 'concavity' and the 'convexity' of mildly dilated AAs and of normal AAs from heart transplant donors. Aortic RNA was analyzed through PCR arrays, profiling the expression of 84 microRNAs involved in cardiovascular disease...
January 19, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28102232/aetiology-and-management-of-hereditary-aortopathy
#13
REVIEW
Aline Verstraeten, Ilse Luyckx, Bart Loeys
Aortic aneurysms are a major health problem because they account for 1-2% of all deaths in the Western population. Although abdominal aortic aneurysms (AAAs) are more prevalent than thoracic aortic aneurysms (TAAs), TAAs have been more exhaustively studied over the past 2 decades because they have a higher heritability and affect younger individuals. Gene identification in both syndromic and nonsyndromic TAA is proceeding at a rapid pace and has already pinpointed >20 genes associated with familial TAA risk...
January 19, 2017: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/28099082/therapeutics-targeting-drivers-of-thoracic-aortic-aneurysms-and-acute-aortic-dissections-insights-from-predisposing-genes-and-mouse-models
#14
Dianna M Milewicz, Siddharth K Prakash, Francesco Ramirez
Thoracic aortic diseases, including aneurysms and dissections of the thoracic aorta, are a major cause of morbidity and mortality. Risk factors for thoracic aortic disease include increased hemodynamic forces on the ascending aorta, typically due to poorly controlled hypertension, and heritable genetic variants. The altered genes predisposing to thoracic aortic disease either disrupt smooth muscle cell (SMC) contraction or adherence to an impaired extracellular matrix, or decrease canonical transforming growth factor beta (TGF-β) signaling...
January 14, 2017: Annual Review of Medicine
https://www.readbyqxmd.com/read/28065612/vertebral-tortuosity-index-in-patients-with-non-connective-tissue-disorder-related-aneurysm-disease
#15
F Virgilio, B Maurel, M Davis, G Hamilton, T M Mastracci
OBJECTIVE: The vertebral tortuosity index (VTI) predicts increased risk of acute aortic events in patients with known genetic aortopathies. This study describes the VTI in a cohort of patients with non-connective tissue disorder-related large aneurysms. METHODS: Hospital imaging records from July 2012 to March 2016 were interrogated to identify patients with aneurysmal disease who had undergone computed tomographic angiography that included imaging of vertebral arteries...
January 5, 2017: European Journal of Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28060855/determinants-and-prognostic-significance-of-symptomatic-status-in-patients-with-moderately-dysfunctional-bicuspid-aortic-valves
#16
Soo Youn Lee, Chi Young Shim, Geu-Ru Hong, In Jeong Cho, Hyuk-Jae Chang, Jong-Won Ha, Namsik Chung
BACKGROUND: We aimed to identify the clinical and echocardiographic determinants of symptoms and their prognostic implications in patients with moderately dysfunctional bicuspid aortic valves (BAVs). METHODS: Among 1,019 subjects in the BAV registry treated in a single tertiary care center, the records of 127 patients (85 men, age 58±13 years) with moderately dysfunctional BAVs were comprehensively reviewed. The patients were divided into two groups based on symptom status: asymptomatic (n = 80) vs...
2017: PloS One
https://www.readbyqxmd.com/read/28052146/prognostic-implications-of-raphe-in-bicuspid-aortic-valve-anatomy
#17
William K F Kong, Victoria Delgado, Kian Keong Poh, Madelien V Regeer, Arnold C T Ng, Louise McCormack, Tiong Cheng Yeo, Miriam Shanks, Sarah Parent, Roxana Enache, Bogdan A Popescu, Michael Liang, James W Yip, Lawrence C W Ma, Vasileios Kamperidis, Philippe J van Rosendael, Enno T van der Velde, Nina Ajmone Marsan, Jeroen J Bax
Importance: Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. Objective: To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality)...
January 4, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28035354/identification-of-a-missense-mutation-of-col3a1-in-a-chinese-family-with-atypical-ehlers-danlos-syndrome-using-targeted-next-generation-sequencing
#18
Wenwen Zhang, Qian Han, Min Zhou, Feng Ran, Tong Qiao, Long Yi, Changjian Liu, Zhao Liu
Aortopathy represents an important cause of mortality in industrialized countries, with a number of genes identified as predispose factors. It can be difficult to identify the genetic lesions underlying this disorder, particularly when the phenotype is atypical. The present study performed targeted next‑generation sequencing of 428 genes associated with cardiovascular diseases in a family with aortopathy, the proband of which presented with abdominal aortic aneurysm rupture only, with tissue fragility noted in surgery...
February 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27989036/ascending-aortic-stiffness-with-bicuspid-aortic-valve-is-variable-and-not-predicted-by-conventional-parameters-in-young-patients
#19
Nicholas S Burris, Petter Dyverfeldt, Michael D Hope
BACKGROUND: Bicuspid aortic valve (BAV)-related aortopathy is characterized by histological abnormalities that result in aortic wall stiffening and aortic dilation. The study aim was to determine the range of ascending aortic stiffness seen in a clinical cohort of patients with BAV, and to identify the association of aortic stiffness with standard clinical and imaging parameters. METHODS: Patients with BAV (n = 65) and normal subjects (n = 10) were studied using conventional phase-contrast magnetic resonance imaging through the ascending aorta...
May 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/27989035/aortopathy-in-bicuspid-aortic-valve-stenosis-with-fusion-of-right-left-versus-right-non-coronary-cusps-are-these-different-diseases
#20
Evaldas Girdauskas, Mina Rouman, Kushtrim Disha, Georg Dubslaff, Beatrix Fey, Bernhard Theis, Iver Petersen, Michael A Borger, Yskert von Kodolitsch, Thomas Kuntze, Hermann Reichenspurner
BACKGROUND: The study aim was to prospectively analyze the impact of cusp fusion pattern on aortopathy severity in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. METHODS: A total of 137 consecutive patients with BAV stenosis (93 males, 44 females; mean age 61 ± 9 years) underwent aortic valve replacement with or without concomitant aortic surgery between January 2012 and March 2015. All patients underwent preoperative cardiac magnetic resonance imaging (cMRI) in order to evaluate morphological/functional parameters of the aortic root...
May 2016: Journal of Heart Valve Disease
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