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https://www.readbyqxmd.com/read/28802510/aortic-dilatation-associated-with-bicuspid-aortic-valve-relation-to-sex-hemodynamics-and-valve-morphology-the-national-heart-lung-and-blood-institute-sponsored-national-registry-of-genetically-triggered-thoracic-aortic-aneurysms-and-cardiovascular-conditions
#1
Mary J Roman, Norma L Pugh, Richard B Devereux, Kim A Eagle, Kathryn Holmes, Scott A LeMaire, Rita K Milewski, Shaine A Morris, Siddharth K Prakash, Reed E Pyeritz, William J Ravekes, Ralph V Shohet, Howard K Song, Federico M Asch
This study analyzed the impact of sex, hemodynamic profile, and valve fusion pattern on aortopathy associated with bicuspid aortic valve (BAV). The National Heart Lung and Blood Institute-sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) provided comprehensive information on a large population of well-characterized patients with BAV. Of 969 enrolled patients with BAV, 551 (57%, 77% male) had already undergone valvular and/or aortic surgery...
July 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28795978/update-on-bicuspid-aortic-valve-aortopathy
#2
Evaldas Girdauskas, Johannes Petersen
PURPOSE OF REVIEW: The appropriate treatment of bicuspid aortic valve (BAV)-associated aortopathy is still controversial. We aimed to summarize recent evidence from the literature that focused on the prediction of aortopathy progression and adverse aortic events. RECENT FINDINGS: Following the detailed description of valvulo-aortic phenotypes in BAV disease in the previous imaging studies, the most current research focused on the definition of hemodynamic markers that may affect the progression of BAV-associated aortopathy and search for blood-born biomarkers to improve the prediction of adverse aortic events...
August 8, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28790933/mechanisms-of-smooth-muscle-cell-differentiation-are-distinctly-altered-in-thoracic-aortic-aneurysms-associated-with-bicuspid-or-tricuspid-aortic-valves
#3
Elena Ignatieva, Daria Kostina, Olga Irtyuga, Vladimir Uspensky, Alexey Golovkin, Natalia Gavriliuk, Olga Moiseeva, Anna Kostareva, Anna Malashicheva
Cellular and molecular mechanisms of thoracic aortic aneurysm are not clear and therapeutic approaches are mostly absent. Thoracic aortic aneurysm is associated with defective differentiation of smooth muscle cells (SMC) of aortic wall. Bicuspid aortic valve (BAV) comparing to tricuspid aortic valve (TAV) significantly predisposes to a risk of thoracic aortic aneurysms. It has been suggested recently that BAV-associated aortopathies represent a separate pathology comparing to TAV-associated dilations. The only proven candidate gene that has been associated with BAV remains NOTCH1...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28780662/bicuspid-aortic-valvulopathy-and-associated-aortopathy-a-review-of-contemporary-studies-relevant-to-clinical-decision-making
#4
REVIEW
Michael H Kwon, Thoralf M Sundt
The bicuspid aortic valve (BAV) phenotype is becoming increasingly recognized as a complex and heterogeneous clinical entity, with some but not all patients developing accelerated degrees of both aortic insufficiency (AI) and aortic stenosis (AS) in comparison to patients with tricuspid aortic valves (TAV). In addition, there remains a well-established association between the BAV phenotype and aortic enlargement independent of valve function as well as progression among some to ascending aortic aneurysm and the attendant concern over risk of aortic dissection...
September 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28751568/mtor-mechanistic-target-of-rapamycin-inhibition-decreases-mechanosignaling-collagen-accumulation-and-stiffening-of-the-thoracic-aorta-in-elastin-deficient-mice
#5
Yang Jiao, Guangxin Li, Qingle Li, Rahmat Ali, Lingfeng Qin, Wei Li, Yibing Qyang, Daniel M Greif, Arnar Geirsson, Jay D Humphrey, George Tellides
OBJECTIVE: Elastin deficiency because of heterozygous loss of an ELN allele in Williams syndrome causes obstructive aortopathy characterized by medial thickening and fibrosis and consequent aortic stiffening. Previous work in Eln-null mice with a severe arterial phenotype showed that inhibition of mTOR (mechanistic target of rapamycin), a key regulator of cell growth, lessened the aortic obstruction but did not prevent early postnatal death. We investigated the effects of mTOR inhibition in Eln-null mice partially rescued by human ELN that manifest a less severe arterial phenotype and survive long term...
July 27, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28740678/bicuspid-aortic-root-spared-during-ascending-aorta-surgery-an-update-of-long-term-results
#6
EDITORIAL
Marco Russo, Guglielmo Saitto, Paolo Nardi, Fabio Bertoldo, Carlo Bassano, Antonio Scafuri, Antonio Pellegrino, Giovanni Ruvolo
BACKGROUND: Bicuspid aortic valve (BAV) aortopathy is well known in literature even if only few data exist regarding isolated supra-coronary aneurysm with normally functioning valve and root. Aim of this study is to clarify the long-term fate of bicuspid aortic root spared at the time of ascending aorta surgery. METHODS: We identified forty-seven patients (mean age, 57±11 y; range, 35-81 y, 31 males) who were treated by means of supracoronary aortic replacement in presence of normally functioning BAV and not significantly enlarged sinues of Valsalva...
June 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28740468/evolution-of-precision-medicine-and-surgical-strategies-for-bicuspid-aortic-valve-associated-aortopathy
#7
Ali Fatehi Hassanabad, Alex J Barker, David Guzzardi, Michael Markl, Chris Malaisrie, Patrick M McCarthy, Paul W M Fedak
Bicuspid aortic valve (BAV) is a common congenital cardiac malformation affecting 1-2% of people. BAV results from fusion of two adjacent aortic valve cusps, and is associated with dilatation of the aorta, known as bicuspid valve associated aortopathy. Bicuspid valve aortopathy is progressive and associated with catastrophic clinical events, such as aortic dissection and rupture. Therefore, frequent monitoring and early intervention with prophylactic surgical resection of the proximal aorta is often recommended...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28696852/imaging-of-pregnancy-related-vascular-complications
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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