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Bicuspid aortic valve

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https://www.readbyqxmd.com/read/28817398/three-dimensional-echocardiography-diagnosis-of-double-perforation-of-bicuspid-aortic-valve
#1
Luca Longobardo, Valentina Coluccia, Alessia Azzu, Iside S Scarfò, Ottavio Alfieri, Giovanni La Canna
: Leaflet perforation is a frequently underdiagnosed mechanism of aortic regurgitation, often underdiagnosed by two-dimensional echocardiography. Three-dimensional echocardiography allows a detailed assessment of cusp anatomy, improving diagnosis compared with two-dimensional echo.
August 16, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28815188/painless-aortic-dissection-diagnostic-dilemma-with-fatal-outcomes-what-do-we-learn
#2
Saeeda Fatima, Konika Sharma
Aortic dissection is the most catastrophic clinical condition that involves the aorta. It has a high mortality as well as high rate of misdiagnosis due to frequent unusual presentation. Typically, it presents with acute chest, back, and tearing abdominal pain. However, it can present atypically with minimal or no pain, making diagnosis difficult. Physicians should always suspect acute aortic dissection in patients with certain clinical conditions like difficult-to-control hypertension, giant cell arteritis, bicuspid aortic valve, intracranial aneurysms, simple renal cysts, family history of aortic disease, and Marfan syndrome, especially when a patient presents with ischemic symptoms involving multiple organ without an obvious cause...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28805011/bicuspid-and-unicuspid-aortic-valves-different-phenotypes-of-the-same-disease-insight-from-the-gentac-registry
#3
Joseph M Krepp, Mary J Roman, Richard B Devereux, Adrienne Bruce, Siddharth K Prakash, Shaine A Morris, Dianna M Milewicz, Kathryn W Holmes, William Ravekes, Ralph V Shohet, Reed E Pyeritz, Cheryl L Maslen, Barbara L Kroner, Kim A Eagle, Liliana Preiss, Federico M Asch
BACKGROUND: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed. METHODS: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions...
August 14, 2017: Congenital Heart Disease
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
#4
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/28802508/prognostic-significance-of-left-ventricular-fibrosis-in-patients-with-congenital-bicuspid-aortic-valve
#5
Gentian Lluri, Pierangelo Renella, J Paul Finn, Gabriel Vorobiof, Jamil Aboulhosn, Arjun Deb
This study sought to evaluate the prognostic value of left ventricular (LV) fibrosis assessed by late gadolinium enhancement (LGE) of the myocardium during cardiac magnetic resonance (CMR) imaging in patients with bicuspid aortic valve (BAV), which is associated with early aortic valve fibrosis and calcification. To what degree the LV myocardial wall is affected by fibrosis and its prognostic value is currently unknown. This is a retrospective, single-center study evaluating all adult patients with BAV who had CMR and followed from March 2002 to March 2016...
July 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28795978/update-on-bicuspid-aortic-valve-aortopathy
#6
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
#7
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/28790051/severe-aortic-complications-in-a-patient-with-variant-shone-s-complex-and-bicuspid-aortic-valve
#8
Mashrafi Ahmed, Hany Aziz, Leng Jiang
No abstract text is available yet for this article.
August 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28780662/bicuspid-aortic-valvulopathy-and-associated-aortopathy-a-review-of-contemporary-studies-relevant-to-clinical-decision-making
#9
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/28777155/a-splenic-igg4-sclerosing-angiomatoid-nodular-transformation-sant-treated-by-hemisplenectomy-a-radiologic-histochemical-and-immunohistochemical-study
#10
Raffaele Gaeta, Francescamaria Donati, Emanuele F Kauffmann, Daniela Campani
INTRODUCTION: Sclerosing angiomatoid nodular transformation (SANT) is a rare benign lesion of unknown origin for which total splenectomy is the standard treatment. CASE PRESENTATION: A 54-year-old man with a history of recurrent pancreatitis, bicuspid aortic valve, and aortic dissection underwent abdominal ultrasound, Computed tomography and magnetic resonance imaging, which revealed a 6-cm hypoechoic splenic mass diagnosed as cavernous hemangioma. Owing to his relevant past history, he was considered eligible for emisplenectomy and not for total excision, which is associated with long-term risks, especially infections...
August 2, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28773988/investigation-on-the-regional-loss-factor-and-its-anisotropy-for-aortic-aneurysms
#11
Nastaran Shahmansouri, Mohammed Alreshidan, Alexander Emmott, Kevin Lachapelle, Ismaïl El-Hamamsy, Raymond Cartier, Richard L Leask, Rosaire Mongrain
An aortic aneurysm is a lethal arterial disease that mainly occurs in the thoracic and abdominal regions of the aorta. Thoracic aortic aneurysms are prevalent in the root/ascending parts of the aorta and can lead to aortic rupture resulting in the sudden death of patients. Understanding the biomechanical and histopathological changes associated with ascending thoracic aortic aneurysms (ATAAs), this study investigates the mechanical properties of the aorta during strip-biaxial tensile cycles. The loss factor-defined as the ratio of dissipated energy to the energy absorbed during a tensile cycle-the incremental modulus, and their anisotropy indexes were compared with the media fiber compositions for aneurysmal (n = 26) and control (n = 4) human ascending aortas...
October 26, 2016: Materials
https://www.readbyqxmd.com/read/28751538/coronary-anatomy-in-children-with-bicuspid-aortic-valves-and-associated-congenital-heart-disease
#12
Wilke M C Koenraadt, Margot M Bartelings, Regina Bökenkamp, Adriana C Gittenberger-de Groot, Marco C DeRuiter, Martin J Schalij, Monique Rm Jongbloed
OBJECTIVE: In patients with bicuspid aortic valve (BAV), coronary anatomy is variable. High take-off coronary arteries have been described, but data are scarce, especially when associated with complex congenital heart disease (CHD). The purpose of this study was to describe coronary patterns in these patients. METHODS: In 84 postmortem heart specimens with BAV and associated CHD, position and height of the coronary ostia were studied and related to BAV morphology...
July 27, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28740678/bicuspid-aortic-root-spared-during-ascending-aorta-surgery-an-update-of-long-term-results
#13
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
#14
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/28730163/brain-strokes-related-to-aortic-aneurysma-the-analysis-of-three-cases
#15
Żanna Pastuszak, Adam Stępień, Joanna Kordowska, Agnieszka Rolewska, Dariusz Galbarczyk
Brain stroke connected with aortic blood flow disturbances is a rare disease and its incidence is difficult to assume. Nevertheless, 10-50% of patients with aortic dissection may not experience any pain. In case of 18-30% patients with aortic dissection neurological signs are first disease presentation and among them ischemic stroke is the most common. The most popular aortic dissection classification is with use of Stanford system. Type A involves the ascending aorta and type B is occurring distal to the subclavian artery...
2017: Open Medicine (Warsaw, Poland)
https://www.readbyqxmd.com/read/28712585/conduction-disturbance-after-isolated-surgical-aortic-valve-replacement-in-degenerative-aortic-stenosis
#16
You Mi Hwang, Jun Kim, Ji Hyun Lee, Minsu Kim, Jongmin Hwang, Joon Bum Kim, Sung-Ho Jung, Suk Jung Choo, Gi Byoung Nam, Kee Joon Choi, Cheol Hyun Chung, Jae Won Lee, You Ho Kim
OBJECTIVE: Conduction disturbances are common in patients with aortic stenosis. We investigated the incidence, reversibility, and prognosis of conduction disorders requiring permanent pacemaker implantation in patients with degenerative aortic stenosis after isolated aortic valve replacement. METHODS: This was a retrospective study conducted at a tertiary care center. We evaluated the incidence of conduction disturbances in patients who underwent isolated surgical aortic valve replacement for aortic stenosis between January 2005 and May 2015...
June 15, 2017: Journal of Thoracic and Cardiovascular Surgery
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
#17
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
#18
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
#19
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/28682324/matching-patients-with-the-ever-expanding-range-of-tavi-devices
#20
REVIEW
Brandon M Jones, Amar Krishnaswamy, E Murat Tuzcu, Stephanie Mick, Wael A Jaber, Lars G Svensson, Samir R Kapadia
Transcatheter aortic valve implantation (TAVI) has become a widely accepted strategy for the treatment of aortic stenosis in patients at intermediate, high, or prohibitive surgical risk. After >1 decade of innovation and clinical trial experience, the available technology for TAVI has grown enormously, and now includes a myriad of vascular access approaches and innovative valve designs. As a result, the range of patients who can benefit from these advances continues to grow rapidly. Furthermore, given the improved safety profile and clinical success of current-generation devices in randomized trials, the use of TAVI among even low-risk populations is justified in current trials...
July 6, 2017: Nature Reviews. Cardiology
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