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https://www.readbyqxmd.com/read/28696852/imaging-of-pregnancy-related-vascular-complications
#1
R Scooter Plowman, Cylen Javidan-Nejad, Constantine A Raptis, Douglas S Katz, Vincent M Mellnick, Sanjeev Bhalla, Patricia Cornejo, Christine O Menias
Pregnancy results in substantial hemodynamic and prothrombotic changes that form the foundation for downstream vascular complications, both during pregnancy and in the postpartum period. In addition, several important risk factors, including older patient age, diabetes, and smoking, can increase the risk for vascular-related pregnancy complications. Because radiologists often play an important role in evaluation of the pregnant patient, understanding the pathophysiology of vascular-related complications in pregnancy and their imaging appearances is essential for diagnostic accuracy...
July 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28694784/4d-flow-analysis-of-bav-related-fluid-dynamic-alterations-evidences-of-wall-shear-stress-alterations-in-absence-of-clinically-relevant-aortic-anatomical-remodeling
#2
Filippo Piatti, Francesco Sturla, Malenka M Bissell, Selene Pirola, Massimo Lombardi, Igor Nesteruk, Alessandro Della Corte, Alberto C L Redaelli, Emiliano Votta
Bicuspid aortic valve (BAV) is the most common congenital cardiac disease and is a foremost risk factor for aortopathies. Despite the genetic basis of BAV and of the associated aortopathies, BAV-related alterations in aortic fluid-dynamics, and particularly in wall shear stresses (WSSs), likely play a role in the progression of aortopathy, and may contribute to its pathogenesis. To test whether WSS may trigger aortopathy, in this study we used 4D Flow sequences of phase-contrast cardiac magnetic resonance imaging (CMR) to quantitatively compare the in vivo fluid dynamics in the thoracic aorta of two groups of subjects: (i) five prospectively enrolled young patients with normo-functional BAV and with no aortic dilation and (ii) ten age-matched healthy volunteers...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28689786/oxidative-stress-in-bicuspid-aortic-valve-related-aortopathy-hand-me-downs-and-yoga-pants
#3
EDITORIAL
Jeffrey A Jones
No abstract text is available yet for this article.
June 17, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28683903/genetic-disorders-of-the-thoracic-aorta-and-indications-for-surgery
#4
REVIEW
Jack C J Sun
Genetic disorders of the aorta are rare but can lead to life-threatening thoracic aortic aneurysms. Although the genetic causes of many of these connective tissue diseases are well defined, others such as familial thoracic aortic aneurysm and bicuspid aortic valve aortopathy are not. The natural history of genetic thoracic aortic aneurysms is not well understood or predictable, and surgical guidelines for treatment remain imprecise. Future research should strive to provide in-depth and detailed genetic profiling to drive clinical management, including medical and surgical therapies...
August 2017: Cardiology Clinics
https://www.readbyqxmd.com/read/28673110/valve-sparing-root-and-total-arch-replacement-for-cutis-laxa-aortopathy
#5
Anji T Yetman, James Hammel, Jennifer N Sanmann, Lois J Starr
Aortic aneurysms requiring surgery in early childhood are rare. Herein we describe the case of a three-year-old with massive aneurysmal aortic dilation secondary to the rare and often lethal genetic disorder, cutis laxa. Initial thoracic aortic aneurysm gene panel was negative. Parents of the child were not known to be consanguineous, but high-density SNP array revealed several regions of homozygosity. This prompted targeted sequence analysis that identified a novel homozygous missense mutation in the gene for cutis laxa, EFEMP2...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28663257/smooth-muscle-cells-derived-from-second-heart-field-and-cardiac-neural-crest-reside-in-spatially-distinct-domains-in-the-media-of-the-ascending-aorta
#6
Hisashi Sawada, Debra L Rateri, Jessica J Moorleghen, Mark W Majesky, Alan Daugherty
OBJECTIVE: Smooth muscle cells (SMCs) of the proximal thoracic aorta are embryonically derived from the second heart field (SHF) and cardiac neural crest (CNC). However, distributions of these embryonic origins are not fully defined. The regional distribution of SMCs of different origins is speculated to cause region-specific aortopathies. Therefore, the aim of this study was to determine the distribution of SMCs of SHF and CNC origins in the proximal thoracic aorta. APPROACH AND RESULTS: Mice with repressed LacZ in the ROSA26 locus were bred to those expressing Cre controlled by either the Wnt1 or Mef2c (myocyte-specific enhancer factor 2c) promoter to trace CNC- and SHF-derived SMCs, respectively...
June 29, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28659818/managing-thoracic-aortic-aneurysm-in-patients-with-bicuspid-aortic-valve-based-on-aortic-root-involvement
#7
REVIEW
Elizabeth Norton, Bo Yang
Bicuspid aortic valve (BAV) can be both sporadic and hereditary, is phenotypically variable, and genetically heterogeneous. The clinical presentation of BAV is diverse and commonly associated with a high prevalence of valvular dysfunction producing altered hemodynamics and aortic abnormalities (e.g., aneurysm and dissection). The thoracic aortic aneurysm (TAA) in BAV frequently involves the proximal aorta, including the aortic root, ascending aorta, and aortic arch, but spares the aorta distal to the aortic arch...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28655506/in-silico-shear-and-intramural-stresses-are-linked-to-aortic-valve-morphology-in-dilated-ascending-aorta
#8
S Pasta, G Gentile, G M Raffa, D Bellavia, G Chiarello, R Liotta, A Luca, C Scardulla, M Pilato
OBJECTIVE/BACKGROUND: The development of ascending aortic dilatation in patients with bicuspid aortic valve (BAV) is highly variable, and this makes surgical decision strategies particularly challenging. The purpose of this study was to identify new predictors, other than the well established aortic size, that may help to stratify the risk of aortic dilatation in BAV patients. METHODS: Using fluid-structure interaction analysis, both haemodynamic and structural parameters exerted on the ascending aortic wall of patients with either BAV (n = 21) or tricuspid aortic valve (TAV; n = 13) with comparable age and aortic diameter (42...
June 24, 2017: European Journal of Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28651938/elevated-oxidative-stress-in-the-aortic-media-of-patients-with-bicuspid-aortic-valve
#9
Marie Billaud, Julie A Phillippi, Mary P Kotlarczyk, Jennifer C Hill, Bradley W Ellis, Claudette M St Croix, Nadiezhda Cantu-Medéllin, Eric E Kelley, Thomas G Gleason
OBJECTIVE: Congenital bicuspid aortic valve (BAV) is distinctly associated with the development of ascending aortopathy in adulthood, portending risk of both ascending aortic aneurysm and dissection. Our previous work implicated deficiency in oxidative stress response as a mediator of the BAV-associated aortopathy. We hypothesize that reactive oxygen species generation invokes elevated local oxidative tissue damage in ascending aorta of patients with BAV. METHODS: Ascending aortic specimens were obtained from patients undergoing elective aortic replacement and/or aortic valve replacement and during heart transplant operations...
May 25, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28622940/feasibility-of-performing-non-contrast-magnetic-resonance-angiography-in-pregnant-subjects-with-familial-aortopathies
#10
William E Moody, Luke Pickup, Emma Plunkett, John Fryearson, Paul F Clift, R Katie Morris, Peter J Thompson, Sara Thorne, Lucy E Hudsmith
BACKGROUND: Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic resonance angiography (MRA) in pregnant subjects with inherited aortopathy. METHODS: Fifteen pregnant subjects (age 27±4yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D-SSFP MRA at 1...
June 2, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28616345/wrapping-of-the-ascending-aorta-revisited-is-there-any-role-left-for-conservative-treatment-of-ascending-aortic-aneurysm
#11
REVIEW
José María González-Santos, María Elena Arnáiz-García
Dilatation of the ascending aorta (AA) is a common finding in patients with aortic valve disease. The clinical practice guidelines recommend replacing the AA whenever the diameter exceeds 45 mm. However, no consensus has been reached regarding the approach when the aorta is only moderately dilated. Although the risk in aorta replacement is generally low, it may be higher when associated with other complex surgical procedures or it is carried out in elderly patients or patients with significant comorbidity. This would justify the use of alternative surgical techniques, which reduce surgical risk and guarantee a durable correction of the aortic pathology...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28616342/bicuspid-aortic-valve-syndrome-a-multidisciplinary-approach-for-a-complex-entity
#12
REVIEW
María Martín, Rebeca Lorca, José Rozado, Rubén Alvarez-Cabo, Juan Calvo, Isaac Pascual, Helena Cigarrán, Isabel Rodríguez, César Morís
Bicuspid aortic valve (BAV) or bicuspid aortopathy is the most common congenital heart disease. It can be clinically silent and it is often identified as an incidental finding in otherwise healthy, asymptomatic patients. However, it can be dysfunctioning at birth, even requiring neonatal intervention, or, in time, lead to aortic stenosis, aortic insufficiency, and endocarditis, and also be associated with aortic aneurysm and aortic dissection. Given its prevalence and significant complications, it is estimated that BAV is responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28607545/the-genetics-of-aortopathies-in-clinical-cardiology
#13
REVIEW
Amit Goyal, Ali R Keramati, Matthew J Czarny, Jon R Resar, Arya Mani
Aortopathies pose a significant healthcare burden due to excess early mortality, increasing incidence, and underdiagnosis. Understanding the underlying genetic causes, early diagnosis, timely surveillance, prophylactic repair, and family screening are keys to addressing these diseases. Next-generation sequencing continues to expand our understanding of the genetic causes of heritable aortopathies, rapidly clarifying their underlying molecular pathophysiology and suggesting new potential therapeutic targets...
2017: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/28584589/computerized-tomography-use-in-williams-beuren-syndrome-aortopathy
#14
Neale Nicola Kalis, Leena Khalifa Sulaibikh, Saud Rashid Al Amer, Haya Yousif Al Amer
Williams-Beuren syndrome is a multisystem genetic disorder caused by hemizygous deletion on chromosome 7q11.23, encompassing about 28 genes including the elastin gene, ELN. Cardiovascular abnormalities are frequent and are related to elastin insufficiency. These abnormalities include supravalvular aortic stenosis (SVAS) in 70% of case, pulmonic valve stenosis, and renal artery stenosis. Definitive therapy for supravalvar aortic stenosis consists of surgical correction of the arteriopathies. Outcomes after surgical correction of SVAS depend on the extent of the arteriopathy and the presence of other associated lesions...
January 2017: Heart Views: the Official Journal of the Gulf Heart Association
https://www.readbyqxmd.com/read/28544830/assessment-of-contributors-of-aortopathy-and-subclinical-left-ventricular-dysfunction-in-normally-functioning-bicuspid-aortic-valves
#15
Kamil Tuluce, Selcen Yakar Tuluce, Ersin Cagri Simsek, Serdar Bayata, Cem Nazli
BACKGROUND: Left ventricular (LV) function and the dimensions of aortic valves from normally functioning bicuspid aortic valve (BAV) patients were compared with those of healthy control patients. A comparison between patients with antero-posterior BAV (BAV-AP) or right-left BAV (BAV-RL) was also performed, and the determinants of aortopathy and LV function were investigated. METHODS: Sixty-eight patients with aortic velocities <2 m/s and trivial or mild aortic regurgitation were included in the study...
January 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28541271/protein-altering-and-regulatory-genetic-variants-near-gata4-implicated-in-bicuspid-aortic-valve
#16
Bo Yang, Wei Zhou, Jiao Jiao, Jonas B Nielsen, Michael R Mathis, Mahyar Heydarpour, Guillaume Lettre, Lasse Folkersen, Siddharth Prakash, Claudia Schurmann, Lars Fritsche, Gregory A Farnum, Maoxuan Lin, Mohammad Othman, Whitney Hornsby, Anisa Driscoll, Alexandra Levasseur, Marc Thomas, Linda Farhat, Marie-Pierre Dubé, Eric M Isselbacher, Anders Franco-Cereceda, Dong-Chuan Guo, Erwin P Bottinger, G Michael Deeb, Anna Booher, Sachin Kheterpal, Y Eugene Chen, Hyun Min Kang, Jacob Kitzman, Heather J Cordell, Bernard D Keavney, Judith A Goodship, Santhi K Ganesh, Gonçalo Abecasis, Kim A Eagle, Alan P Boyle, Ruth J F Loos, Per Eriksson, Jean-Claude Tardif, Chad M Brummett, Dianna M Milewicz, Simon C Body, Cristen J Willer
Bicuspid aortic valve (BAV) is a heritable congenital heart defect and an important risk factor for valvulopathy and aortopathy. Here we report a genome-wide association scan of 466 BAV cases and 4,660 age, sex and ethnicity-matched controls with replication in up to 1,326 cases and 8,103 controls. We identify association with a noncoding variant 151 kb from the gene encoding the cardiac-specific transcription factor, GATA4, and near-significance for p.Ser377Gly in GATA4. GATA4 was interrupted by CRISPR-Cas9 in induced pluripotent stem cells from healthy donors...
May 25, 2017: Nature Communications
https://www.readbyqxmd.com/read/28514465/bicuspid-aortopathy-reply
#17
Victoria Delgado, William K F Kong, Jeroen J Bax
No abstract text is available yet for this article.
May 17, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28511923/igg4-aortopathy-an-underappreciated-cause-of-non-infectious-thoracic-aortitis
#18
Victor Aguirre, Catherine Connolly, Robert Stuklis
IgG4 related thoracic aortitis is a recent addition to the differential diagnosis for inflammatory aortic disease - a condition which is often underappreciated until complications arise such as aneurysmal formation or aortic dissection. Currently, IgG4 aortitis remains a post-surgical diagnosis reliant on positive immunohistochemistry findings. Management is guided by the extent of disease involvement, which can be gauged by serum IgG4 levels and radiological findings. Options include surgical resection, corticosteroid therapy and steroid-sparing agents to prevent relapses...
April 19, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28501375/transthoracic-echocardiography-versus-computed-tomography-for-ascending-aortic-measurements-in-patients-with-bicuspid-aortic%C3%A2-valve
#19
Jae Yoon Park, Thomas A Foley, Crystal R Bonnichsen, Matthew J Maurer, Krista M Goergen, Vuyisile T Nkomo, Maurice Enriquez-Sarano, Eric E Williamson, Hector I Michelena
BACKGROUND: Ascending aorta dilatation is common in bicuspid aortic valve (BAV). The aim of this study was to investigate agreement of transthoracic echocardiographic (TTE) measurement of the sinuses of Valsalva and the tubular mid-ascending aorta (Asc-Ao) compared with electrocardiographically gated computed tomographic angiographic (CTA) assessment in patients with BAV. METHODS: Fifty-three patients with BAV (mean age, 54 ± 14 years; 74% men) who underwent both TTE and CTA imaging for ascending aortic assessment were retrospectively identified...
May 10, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28495059/bicuspid-aortic-valve-aortopathy-is-not-cancer
#20
EDITORIAL
Thoralf M Sundt
No abstract text is available yet for this article.
April 7, 2017: Journal of Thoracic and Cardiovascular Surgery
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