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Bicuspid aortic valve

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https://www.readbyqxmd.com/read/28913586/evolving-indications-for-transcatheter-aortic-valve-interventions
#1
REVIEW
Anna Franzone, Thomas Pilgrim, Stefan Stortecky, Stephan Windecker
PURPOSE OF REVIEW: The purpose of this review was to summarize recent progress in the field of transcatheter aortic valve replacement (TAVR), discuss expansion of indications, and identify areas of future clinical applications. RECENT FINDINGS: Favorable clinical outcomes as well as continued refinement of transcatheter heart valve technology have prompted the continuous expansion of indications for TAVR. The results of randomized clinical trials comparing the safety and efficacy of TAVR relative to conventional surgical aortic valve replacement (SAVR) in lower- than high-risk patients have recently been published, and trials among lower-risk categories are ongoing...
September 14, 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28906534/management-of-a-complicated-redo-giant-dissecting-aortic-aneurysm
#2
Ibrahim Kara, Alper Erkin, MHalil Ibrahim Erkengel, Kiyasettin Asil
Giant aortic aneurysm is defined as an aneurysm of the aorta of greater than 10 cm in diameter. This rare condition is associated with a high risk of morbidity and mortality and it may lead to fatal complications such as rupture and/or dissection if not managed with proper surgical planning and expertise. Other than atherosclerosis, the main causes of giant ascending aortic aneurysms include Marfan and Ehlers-Danhlos syndromes. Herein we report on a young male patient who had had an aortic valve replacement five years earlier due to a bicuspid aortic valve leading to aortic failure, accompanied by aortic coarctation...
July 23, 2017: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/28903936/aortic-valve-stenosis-alters-expression-of-regional-aortic-wall-shear-stress-new-insights-from-a-4-dimensional-flow-magnetic-resonance-imaging-study-of-571-subjects
#3
Pim van Ooij, Michael Markl, Jeremy D Collins, James C Carr, Cynthia Rigsby, Robert O Bonow, S Chris Malaisrie, Patrick M McCarthy, Paul W M Fedak, Alex J Barker
BACKGROUND: Wall shear stress (WSS) is a stimulus for vessel wall remodeling. Differences in ascending aorta (AAo) hemodynamics have been reported between bicuspid aortic valve (BAV) and tricuspid aortic valve patients with aortic dilatation, but the confounding impact of aortic valve stenosis (AS) is unknown. METHODS AND RESULTS: Five hundred seventy-one subjects underwent 4-dimensional flow magnetic resonance imaging in the thoracic aorta (210 right-left BAV cusp fusions, 60 right-noncoronary BAV cusp fusions, 245 tricuspid aortic valve patients with aortic dilatation, and 56 healthy controls)...
September 13, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28901226/isolated-right-juxtaposition-of-the-atrial-appendages-in-adult-with-bicuspid-aortic-valve-stenosis
#4
Alaae Boutayeb, Fayçal Kenassi, Adil Bensouda, Lahcen Marmade
We report the case of a 30-year-old male who had symptomatic bicuspid aortic valve stenosis. Operative findings unexpectedly revealed right juxtaposition of the atrial appendages. This is a rare association, as juxtaposition of atrial appendages is generally associated with more complex forms of congenital heart disease. The patient underwent successful surgery with uneventful postoperative course.
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28894058/-ruptured-aneurysm-of-the-sinus-of-valsalva-accompanied-with-a-bicuspid-aortic-valve-in-an-elderly-man-report-of-a-case
#5
Toshifumi Murase, Susumu Tamura, Yasuhiro Ohzeki, Kunio Ebine
The combination of ruptured aneurysm of the sinus of Valsalva and a bicuspid aortic valve is very rare in an elderly person. A 71-year-old man with ruptured aneurysm of the sinus of Valsalva and a bicuspid aortic valve had undergone an operation. He was admitted to his other hospital because of heart failure. He was transferred to our hospital to undergo treatment for ruptured aneurysm of sinus of Valsalva. At our hospital, echocardiography findings showed ruptured aneurysm of the sinus of Valsalva, a ventricular septal defect (VSD), and severe aortic regurgitation with moderate stenosis of the bicuspid aortic valve...
September 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28890987/impact-of-patient-specific-inflow-velocity-profile-on-hemodynamics-of-the-thoracic-aorta
#6
Pouya Youssefi, Alberto Gomez, Christopher Arthurs, Rajan Sharma, Marjan Jahangiri, Alberto Figueroa
Background-Computational fluid dynamics (CFD) provides a non-invasive method to functionally assess aortic hemodynamics. The thoracic aorta has an anatomically complex inlet comprising of the aortic valve, which is highly prone to different morphologies and pathologies. We investigated the effect of using patient-specific inflow velocity profiles compared to idealised profiles based on the patient's flow waveform. Methods-A healthy 31yo with a normally functioning tricuspid aortic valve (subject A), and a 52yo with a bicuspid aortic valve, aortic valvular stenosis and dilated ascending aorta (subject B) were studied...
September 11, 2017: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/28890873/diagnostic-accuracy-study-of-routine-echocardiography-for-bicuspid-aortic-valve-a-retrospective-study-and-meta-analysis
#7
Mathias Hillebrand, Dietmar Koschyk, Pia Ter Hark, Helke Schüler, Meike Rybczynski, Jürgen Berger, Amit Gulati, Alexander M Bernhardt, Christian Detter, Evaldas Girdauskas, Stefan Blankenberg, Yskert von Kodolitsch
BACKGROUND: Transthoracic echocardiography (TTE) is the standard procedure to distinguish tricuspid aortic valve (TAV) from bicuspid aortic valve (BAV). Published studies assessed the accuracy of TTE for BAV under ideal conditions. Conversely, we aimed at assessing accuracy of TTE for BAV under routine conditions. METHODS: This retrospective, cross-sectional study of 216 adults included 132 men aged 62±14 years. Of these, 108 had BAV and 108 were age-matched individuals with TAV...
August 2017: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/28886853/comparison-of-efficacy-and-safety-of-transcatheter-aortic-valve-implantation-in-patients-with-bicuspid-versus-tricuspid-aortic-valves
#8
Anna Sannino, Ari Cedars, Robert C Stoler, Molly Szerlip, Michael J Mack, Paul A Grayburn
Bicuspid aortic valve (BAV) stenosis has been considered a contraindication to transcatheter aortic valve implantation (TAVI). The aim of this study is to compare the efficacy and safety of TAVI in patients with BAV with those with tricuspid aortic valve (TAV) using balloon-expandable and self-expanding transcatheter heart valves. This retrospective study included 823 consecutive patients with severe, symptomatic aortic valve stenosis undergoing TAVI in 2 institutions, Baylor Heart and Vascular Hospital (Dallas, TX) and The Heart Hospital Baylor Plano (Plano, TX), from January 2012 to February 2016...
July 31, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28886508/effects-of-clinico-pathological-risk-factors-on-in-vitro-mechanical-properties-of-human-dilated-ascending-aorta
#9
Anna Ferrara, Pasquale Totaro, Simone Morganti, Ferdinando Auricchio
Ascending aorta aneurysms (AsAA) are associated with a degeneration of the aortic wall tissue, which leads to changes in tissue mechanical properties. Risk factors for the development of the AsAA disease are recognized in patient age and gender, valve type, hypertension, diabetes mellitus, smoking history, and a prior diagnosis of Marfan syndrome. The present study aims to assess how such clinico-pathological factors can affect the mechanical properties of human dilated ascending aorta. Specimens of AsAA are excised from 68 patients who underwent elective AsAA surgical repair and stretched until rupture during the execution of uniaxial tensile tests...
August 31, 2017: Journal of the Mechanical Behavior of Biomedical Materials
https://www.readbyqxmd.com/read/28884922/congenital-heart-defects-in-molecularly-proven-kabuki-syndrome-patients
#10
Maria Cristina Digilio, Maria Gnazzo, Francesca Lepri, Maria Lisa Dentici, Elisa Pisaneschi, Anwar Baban, Chiara Passarelli, Rossella Capolino, Adriano Angioni, Antonio Novelli, Bruno Marino, Bruno Dallapiccola
The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%)...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28884233/in-vivo-strain-analysis-of-dilated-ascending-thoracic-aorta-by-ecg-gated-ct-angiographic-imaging
#11
Salvatore Pasta, Valentina Agnese, Marzio Di Giuseppe, Giovanni Gentile, Giuseppe M Raffa, Diego Bellavia, Michele Pilato
Accurate assessment of aortic extensibility is a requisite first step for elucidating the pathophysiology of an ascending thoracic aortic aneurysm (ATAA). This study aimed to develop a framework for the in vivo evaluation of the full-field distribution of the aortic wall strain by imaging analysis of electrocardiographic- (ECG) gated thoracic data of 34 patients with ATAA. Seven healthy controls (i.e., non-aneurysmal aorta) from patients who underwent ECG-gated CT angiography for coronary artery diseases were included for comparison...
September 7, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/28883797/genetic-bases-of-bicuspid-aortic-valve-the-contribution-of-traditional-and-high-throughput-sequencing-approaches-on-research-and-diagnosis
#12
REVIEW
Betti Giusti, Elena Sticchi, Rosina De Cario, Alberto Magi, Stefano Nistri, Guglielmina Pepe
Bicuspid aortic valve (BAV) is a common (0.5-2.0% of general population) congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with syndromic conditions [e.g., Marfan Marfan syndrome or Loeys-Dietz syndrome (MFS, LDS)]. Identification of a syndromic condition in a BAV patient is clinically relevant to personalize aortic surgery indication...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28881023/double-outlet-left-ventricle-with-a-bicuspid-pulmonary-valve-and-aortic-coarctation
#13
Feifei Sun, Miao Fan, Liping Huang, Weidong Ren, Dongyu Li
A double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation. Here, we describe a case of DOLV with bicuspid pulmonary valve, aortic coarctation, and a subpulmonary ventricular septal defect in which both ventricles were well developed. The anatomic features were observed with echocardiography, and the diagnosis was confirmed at surgery. Many variations of DOLV have been described. The features of our case expand the spectrum of this entity and may provide new insight into its complex anatomy...
September 7, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28877885/bicuspid-aortic-valve-what-to-image-in-patients-considered-for-transcatheter-aortic-valve-replacement
#14
REVIEW
William K F Kong, Victoria Delgado, Jeroen J Bax
No abstract text is available yet for this article.
September 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28871571/acute-aortic-dissection-pathogenesis-risk-factors-and-diagnosis
#15
Joanna Gawinecka, Felix Schönrath, Arnold von Eckardstein
Acute aortic dissection is a rare but life-threatening condition with a lethality rate of 1 to 2% per hour after onset of symptoms in untreated patients. Therefore, its prompt and proper diagnosis is vital to increase a patient's chance of survival and to prevent grievous complications. Typical symptoms of acute aortic dissection include severe chest pain, hypotension or syncope and, hence, mimic acute myocardial infarction or pulmonary embolism. Advanced age, male gender, long-term history of arterial hypertension and the presence of aortic aneurysm confer the greatest population attributable risk...
September 5, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28871348/characterization-of-complex-flow-patterns-in-the-ascending-aorta-in-patients-with-aortic-regurgitation-using-conventional-phase-contrast-velocity-mri
#16
Odd Bech-Hanssen, Frida Svensson, Christian L Polte, Åse A Johnsson, Sinsia A Gao, Kerstin M Lagerstrand
Ascending aorta (AA) flow displacement (FD) is a surrogate for increased wall shear stress. We prospectively studied the flow profile in the AA in patients with aortic regurgitation (AR), to identify predictors of FD and investigate whether magnetic resonance imaging (MRI) phase-contrast flow rate curves (PC-FRC) contain quantitative information related to FD. Forty patients with chronic moderate (n = 14) or severe (n = 26) AR (21 (53%) with bicuspid aortic valve) and 22 controls were investigated. FD was determined from phase-contrast velocity profiles and defined as the distance between the center of the lumen and the "center of velocity" of the peak systolic forward flow or the peak diastolic negative flow, normalized to the lumen radius...
September 4, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28868103/ischemic-stroke-in-a-patient-with-quadricuspid-aortic-valve-and-patent-foramen-ovale
#17
Michael Spartalis, Eleni Tzatzaki, Eleftherios Spartalis, Christos Damaskos, Demetrios Moris, Dimitrios Tsiapras, Vassilis Voudris
Quadricuspid aortic valve (QAV) is a rare congenital aortic valve abnormality. It is less common as compared to bicuspid or unicuspid aortic valve abnormality. QAV causes aortic regurgitation usually in the fifth to sixth decade of life. We present a rare case of a female patient with cryptogenic stroke due to a QAV and a patent foramen ovale (PFO). The patient underwent transcatheter closure of PFO, as there was no clear indication for surgery for her valve. Surgical removal remains the method of choice for the treatment of the QAV before left ventricular decompensation occurs...
August 2017: Cardiology Research
https://www.readbyqxmd.com/read/28864719/anatomical-and-clinical-predictors-of-valve-dysfunction-and-aortic-dilation-in-bicuspid-aortic-valve-disease
#18
Arturo Evangelista, Pastora Gallego, Francisco Calvo-Iglesias, Javier Bermejo, Juan Robledo-Carmona, Violeta Sánchez, Daniel Saura, Roman Arnold, Amelia Carro, Giuliana Maldonado, Augusto Sao-Avilés, Gisela Teixidó, Laura Galian, José Rodríguez-Palomares, David García-Dorado
OBJECTIVE: Bicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications. METHODS: Eight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention...
September 1, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28861430/evolving-treatment-options-for-valve-and-aortic-disease-with-bicuspid-aortic-valve
#19
EDITORIAL
Nicholas S Burris, Michael D Hope
No abstract text is available yet for this article.
August 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28856517/bicuspid-aortic-valve-related-aortopathy
#20
REVIEW
Sina Stock, Salah A Mohamed, Hans-Hinrich Sievers
Bicuspid aortic valve related aortopathy is known to significantly increase the risk for catastrophic aortic events and, therefore, represents a considerable health burden. Albeit of ongoing research in this field including genetic, molecular, hemodynamic and morphologic aspects, bicuspid aortic valve related aortopathy still represents an imperfectly understood disorder. This lack in knowledge results in a lack of consistency considering different therapeutic approaches. Recent studies have provided new insights into the etiology and clinical impacts of bicuspid aortic valve related aortopathy in different clinical settings, leading to a growing body of opinion towards a more individualized surgical approach than currently provided by the guidelines...
August 30, 2017: General Thoracic and Cardiovascular Surgery
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