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Bicuspid aortic valve

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https://www.readbyqxmd.com/read/27903575/early-severe-coronary-artery-disease-and-aortic-coarctation-in-a-child-with-familial-hypercholesterolaemia
#1
Dina Labib, Haytham Soliman, Kareem Said, Khaled Sorour
An 11-year-old boy presented with easy fatigability, multiple xanthomas, and absent pedal pulsations. Laboratory workup showed severe hypercholesterolaemia and non-invasive imaging revealed 'normally functioning' bicuspid aortic valve and tight aortic coarctation. Coronary angiography showed severe right coronary artery (RCA) stenosis. Medical treatment resulted in significant improvement of dyslipidaemia. We successfully performed balloon dilation and stenting of his coarctation, as well as percutaneous coronary intervention for RCA lesion...
November 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27894865/the-genetic-component-of-bicuspid-aortic-valve-and-aortic-dilation-an-exome-wide-association-study
#2
Marina Gago-Díaz, María Brion, Pastora Gallego, Francisco Calvo, Juan Robledo-Carmona, Daniel Saura, Violeta Sánchez, Javier Bermejo, Teresa Sevilla, Christopher Newton-Cheh, Ángel Carracedo, J Daniel Muehlschlegel, David García-Dorado, Simon C Body, Artur Evangelista
BACKGROUND: Bicuspid aortic valve is the most common cardiovascular congenital malformation affecting 2% of the general population. The incidence of life-threatening complications, the high heritability, and familial clustering rates support the interest in identifying risk or protective genetic factors. The main objective of the present study was to identify population-based genetic variation associated with bicuspid aortic valve and concomitant ascending aortic dilation. MATERIALS AND METHODS: A cross-sectional exome-wide association study was conducted in 565 Spanish cases and 484 controls...
November 25, 2016: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/27893194/incidence-morphology-and-progression-of-bicuspid-aortic-valve-in-pediatric-and-young-adult-subjects-with-coexisting-congenital-heart-defects
#3
Talha Niaz, Joseph T Poterucha, Jonathan N Johnson, Cecilia Craviari, Thomas Nienaber, Jared Palfreeman, Frank Cetta, Donald J Hagler
BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015...
November 28, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27884484/ross-hybrid-arch-and-frozen-elephant-trunk-reconstruction-for-late-complications-of-bicuspid-aortic-valve-and-aortopathy
#4
Matthew Valdis, Gaetano DeRose, Linrui Guo, Michael W A Chu
Young patients with bicuspid aortic valve disease and aortopathy remain a clinical challenge, with many requiring multiple corrective operations throughout their lifetimes. Innovative surgical approaches are often required to address complex aortic pathologic conditions but leave patients at risk for reintervention, lifelong anticoagulation, and suboptimal hemodynamics. We describe an active 44-year-old female triathlete with recurrent bicuspid aortic stenosis, a small aortic root, a hypoplastic aortic arch and complex distal arch, and a descending aortic aneurysm, who underwent a single-stage reconstruction with a combined Ross procedure, hybrid arch, and frozen elephant trunk reconstruction...
December 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27883974/is-undersized-self-expandable-prosthesis-a-valuable-selection-for-transcatheter-aortic-valve-replacement-in-high-risk-bicuspid-aortic-valve-stenosis-report-of-two-successful-cases
#5
EDITORIAL
Francesco Burzotta, Osama Shoeib, Cristina Aurigemma, Piergiorgio Bruno, Carlo Trani
No abstract text is available yet for this article.
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27866029/understanding-the-structural-features-of-symptomatic-calcific-aortic-valve-stenosis-a-broad-spectrum-clinico-pathologic-study-in-236-consecutive-surgical-cases
#6
Daniela Galli, Roberta Manuguerra, Rodolfo Monaco, Laura Manotti, Matteo Goldoni, Gabriella Becchi, Cecilia Carubbi, Giulia Vignali, Nicola Cucurachi, Tiziano Gherli, Francesco Nicolini, Roberto Lorusso, Marco Vitale, Domenico Corradi
BACKGROUND: With age, aortic valve cusps undergo varying degrees of sclerosis which, sometimes, can progress to calcific aortic valve stenosis (AVS). To perform a retrospective clinico-pathologic investigation in patients with calcific AVS. METHODS: We characterized and graded the structural remodeling in 236 aortic valves (200 tricuspid and 36 bicuspid) from patients with calcific AVS (148 males; average 72years); possible relationships between general/clinical/echocardiographic characteristics and the histopathologic changes were explored...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27866026/survival-and-cardiovascular-events-after-coarctation-repair-in-long-term-follow-up-coafu-predictive-value-of-clinical-variables
#7
P Bambul Heck, J Pabst von Ohain, H Kaemmerer, P Ewert, A Hager
OBJECTIVE: Long-term sequelae and events after coarctation repair are well described. However, the predictive value of variables from clinical follow-up investigation for late events and survival has rarely been investigated. METHODS: All patients who participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for reevaluation of survival, current clinical status and major cardiovascular events...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27863744/towards-the-improved-quantification-of-in-vivo-abnormal-wall-shear-stresses-in-bav-affected-patients-from-4d-flow-imaging-benchmarking-and-application-to-real-data
#8
F Piatti, S Pirola, M Bissell, I Nesteruk, F Sturla, A Della Corte, A Redaelli, E Votta
Bicuspid aortic valve (BAV), i.e. the fusion of two aortic valve cusps, is the most frequent congenital cardiac malformation. Its progression is often characterized by accelerated leaflet calcification and aortic wall dilation. These processes are likely enhanced by altered biomechanical stimuli, including fluid-dynamic wall shear stresses (WSS) acting on both the aortic wall and the aortic valve. Several studies have proposed the exploitation of 4D-flow magnetic resonance imaging sequences to characterize abnormal in vivo WSS in BAV-affected patients, to support prognosis and timing of intervention...
November 11, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27861233/aortic-volumetry-at-contrast-enhanced-magnetic-resonance-angiography-feasibility-as-a-sensitive-method-for-monitoring-bicuspid-aortic-valve-aortopathy
#9
Brian Trinh, Iram Dubin, Ozair Rahman, Marcos P Ferreira Botelho, Nicholas Naro, James C Carr, Jeremy D Collins, Alex J Barker
OBJECTIVES: Bicuspid aortic valve patients can develop thoracic aortic aneurysms and therefore require serial imaging to monitor aortic growth. This study investigates the reliability of contrast-enhanced magnetic resonance angiography (CEMRA) volumetry compared with 2-dimensional diameter measurements to identify thoracic aortic aneurysm growth. MATERIALS AND METHODS: A retrospective, institutional review board-approved, and Health Insurance Portability and Accountability Act-compliant study was conducted on 20 bicuspid aortic valve patients (45 ± 8...
November 17, 2016: Investigative Radiology
https://www.readbyqxmd.com/read/27860546/transcatheter-aortic-valve-replacement-state-of-the-art-and-future-directions
#10
Mackram F Eleid, David R Holmes
Transcatheter aortic valve replacement (TAVR) is a transformational and rapidly evolving treatment for patients with aortic stenosis who require valve replacement. Novel technological advancements have made this percutaneous minimally invasive therapy a first-line treatment for many patients at extreme risk for conventional cardiac surgery. New devices and improvements in existing devices have reduced procedural complications, and scientific trials are investigating the role of TAVR in lower-risk aortic stenosis populations, in patients with aortic regurgitation, and in patients with bicuspid aortic valve disease...
November 14, 2016: Annual Review of Medicine
https://www.readbyqxmd.com/read/27855987/simulations-of-morphotype-dependent-hemodynamics-in-non-dilated-bicuspid-aortic-valve-aortas
#11
Kai Cao, Samantha K Atkins, Andrew McNally, Janet Liu, Philippe Sucosky
Bicuspid aortic valves (BAVs) generate flow abnormalities that may promote aortopathy. While positive helix fraction (PHF) index, flow angle (θ), flow displacement (d) and wall shear stress (WSS) exhibit abnormalities in dilated BAV aortas, it is unclear whether those anomalies stem from the abnormal valve anatomy or the dilated aorta. Therefore, the objective of this study was to quantify the early impact of different BAV morphotypes on aorta hemodynamics prior to dilation. Fluid-structure interaction models were designed to quantify standard peak-systolic flow metrics and temporal WSS characteristics in a realistic non-dilated aorta connected to functional tricuspid aortic valve (TAV) and type-I BAVs...
November 11, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27847162/patient-specific-computational-fluid-dynamics-assessment-of-aortic-hemodynamics-in-a-spectrum-of-aortic-valve-pathologies
#12
Pouya Youssefi, Alberto Gomez, Taigang He, Lisa Anderson, Nick Bunce, Rajan Sharma, C Alberto Figueroa, Marjan Jahangiri
OBJECTIVES: The complexity of aortic disease is not fully exposed by aortic dimensions alone, and morbidity or mortality can occur before intervention thresholds are met. Patient-specific computational fluid dynamics (CFD) were used to assess the effect of different aortic valve morphologies on velocity profiles, flow patterns, helicity, wall shear stress (WSS), and oscillatory shear index (OSI) in the thoracic aorta. METHODS: A total of 45 subjects were divided into 5 groups: volunteers, aortic regurgitation-tricuspid aortic valve (AR-TAV), aortic stenosis-tricuspid aortic valve (AS-TAV), aortic stenosis-bicuspid aortic valve right-left cusp fusion (BAV[RL]), and aortic stenosis-right-non cusp fusion (AS-BAV[RN])...
September 23, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27845433/analysis-of-serum-metabolites-to-diagnose-bicuspid-aortic-valve
#13
Wenshuo Wang, Aikebaier Maimaiti, Yun Zhao, Lingfei Zhang, Hongyue Tao, Hui Nian, Limin Xia, Biao Kong, Chunsheng Wang, Mofang Liu, Lai Wei
Bicuspid aortic valve (BAV) is the most common congenital heart disease. The current study aims to construct a diagnostic model based on metabolic profiling as a non-invasive tool for BAV screening. Blood serum samples were prepared from an estimation group and a validation group, each consisting of 30 BAV patients and 20 healthy individuals, and analyzed by liquid chromatography-mass spectrometry (LC-MS). In total, 2213 metabolites were detected and 41 were considered different. A model for predicting BAV in the estimation group was constructed using the concentration levels of monoglyceride (MG) (18:2) and glycerophospho-N-oleoyl ethanolamine (GNOE)...
November 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27843769/a-case-of-infective-endocarditis-along-with-a-ruptured-valve-caused-by-streptococcus-agalactiae-in-an-immunocompetent-man
#14
Kiyozumi Suzuki, Yuji Hirai, Fujiko Morita, Yuki Uehara, Hiroko Oshima, Kazunori Mitsuhashi, Atsushi Amano, Toshio Naito
Streptococcus agalactiae (S. agalactiae) is a major cause of invasive disease in neonates and pregnant women, but has also recently been observed among non-pregnant adults, especially elderly persons or persons with underlying chronic disease. S. agalactiae is also a rare cause of infective endocarditis, and most cases require early surgery. We report the case of a 43-year-old previously healthy man who experienced rapid progressive culture-negative infective endocarditis with aortic valve vegetation and severe aortic regurgitation, which was complicated by lumbar spondylodiscitis...
2016: IDCases
https://www.readbyqxmd.com/read/27833985/common-and-unusual-dental-development-abnormalities-in-a-patient-with-bicuspid-aortic-valve
#15
Anca Maria Răducanu, Ion Victor Feraru, Ioana Suciu, Elina Teodorescu, Andreea Cristiana Didilescu, Ileana Ionescu, Ecaterina Ionescu
Bicuspid aortic valve (BAV) is the most common congenital abnormality of the heart. In this condition, instead of three cusps, the aortic valve has two cusps. Children with congenital heart diseases are at increased risk of developing oral diseases, such as: higher number of decayed teeth, developmental anomalies, periodontal disease, malocclusion, dental crowding, as well as susceptibility to develop infective endocarditis from bacteremia caused by chronic poor oral health. However, little information is available regarding oral manifestations and their management in patients with congenital heart defects, despite the importance of these diseases...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27802285/mmp-2-isoforms-in-aortic-tissue-and-serum-of-patients-with-ascending-aortic-aneurysms-and-aortic-root-aneurysms
#16
Anke Tscheuschler, Philipp Meffert, Friedhelm Beyersdorf, Claudia Heilmann, Nadja Kocher, Xenia Uffelmann, Philipp Discher, Matthias Siepe, Fabian A Kari
OBJECTIVE: The need for biological markers of aortic wall stress and risk of rupture or dissection of ascending aortic aneurysms is obvious. To date, wall stress cannot be related to a certain biological marker. We analyzed aortic tissue and serum for the presence of different MMP-2 isoforms to find a connection between serum and tissue MMP-2 and to evaluate the potential of different MMP-2 isoforms as markers of high wall stress. METHODS: Serum and aortic tissue from n = 24 patients and serum from n = 19 healthy controls was analyzed by ELISA and gelatin zymography...
2016: PloS One
https://www.readbyqxmd.com/read/27793403/long-term-results-following-pericardial-patch-augmentation-for-incompetent-bicuspid-aortic-valves-a-single-center%C3%A2-experience
#17
Marlene Thudt, Nestoras Papadopoulos, Nadejda Monsefi, Aleksandra Miskovic, Afsaneh Karimian-Tabrizi, Andreas Zierer, Anton Moritz
BACKGROUND: Many techniques for repair of bicuspid aortic valves have been described and long-term results differ considerably. The current study evaluates our institutional results using the pericardial patch augmentation technique with the aim of increasing coaptation height. METHODS: From November 2002 through April 2015, 103 consecutive patients underwent aortic valve repair using pericardial patch augmentation for incompetent bicuspid aortic valve. Of them 26 were referred with an aortic valve regurgitation grade 1+ or 2+ and were excluded from the current report...
October 25, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27779199/mesenchymal-state-of-intimal-cells-may-explain-higher-propensity-to-ascending-aortic-aneurysm-in-bicuspid-aortic-valves
#18
Shohreh Maleki, Sanela Kjellqvist, Valentina Paloschi, Joelle Magné, Rui Miguel Mamede Branca, Lei Du, Kjell Hultenby, Johan Petrini, Jonas Fuxe, Janne Lehtiö, Anders Franco-Cereceda, Per Eriksson, Hanna M Björck
Individuals with a bicuspid aortic valve (BAV) are at significantly higher risk of developing aortic complications than individuals with tricuspid aortic valves (TAV) and defective signaling during the embryonic development and/or life time exposure to abnormal hemodynamic have been proposed as underlying factors. However, an explanation for the molecular mechanisms of aortopathy in BAV has not yet been provided. We combined proteomics, RNA analyses, immunohistochemistry, and electron microscopy to identify molecular differences in samples of non-dilated ascending aortas from BAV (N = 62) and TAV (N = 54) patients...
October 25, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27777532/asymptomatic-interrupted-aortic-arch-severe-tricuspid-regurgitation-and-bicuspid-aortic-valve-in-a-76-year-old-woman
#19
Masih Tajdini, Akram Sardari, Seyed Khalil Forouzannia, Abdolvahab Baradaran, Seyed Mohammad Reza Hosseini, Seyed Ebrahim Kassaian
Interrupted aortic arch is a rare congenital abnormality with a high infancy mortality rate. The principal finding is loss of luminal continuity between the ascending and descending portions of the aorta. Because of the high mortality rate in infancy, interrupted aortic arch is very rare among adults. In this report, we describe the case of a 76-year-old woman with asymptomatic interrupted aortic arch, severe tricuspid regurgitation, and bicuspid aortic valve. To our knowledge, she is the oldest patient ever reported with this possibly unique combination of pathologic conditions...
October 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/27777368/mycotic-aneurysm-of-the-ascending-aorta-in-a-child-with-bicuspid-aortic-valve
#20
Shotaro Higa, Tai Fuchigami, Nobuhiro Nagata
No abstract text is available yet for this article.
October 24, 2016: Asian Cardiovascular & Thoracic Annals
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