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https://www.readbyqxmd.com/read/29329770/yield-of-family-screening-in-patients-with-isolated-bicuspid-aortic-valve-in-a-general-hospital
#1
Luc Cozijnsen, Hester J van der Zaag-Loonen, Richard L Braam, Mirjam Bakker-de Boo, Jan G Post, Berto J Bouma, Barbara J M Mulder
AIM: To determine the prevalence of unidentified bicuspid aortic valve (BAV) or aortic dilatation (>40mm) in first degree relatives (FDR) of patients with isolated BAV in a general hospital. METHODS AND RESULTS: Patients with isolated BAV received information advising cardiac screening of their FDR. Referred and screened were 134 FDR of 54 adult index patients with isolated BAV (median 2 per index patient). FDR's mean age was 49years (range 16-83years) and 41% were male...
December 22, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29325903/gata4-loss-of-function-mutation-and-the-congenitally-bicuspid-aortic-valve
#2
Ruo-Gu Li, Ying-Jia Xu, Juan Wang, Xing-Yuan Liu, Fang Yuan, Ri-Tai Huang, Song Xue, Li Li, Hua Liu, Yan-Jie Li, Xin-Kai Qu, Hong-Yu Shi, Min Zhang, Xing-Biao Qiu, Yi-Qing Yang
Aggregating evidence suggests that genetic determinants play a pivotal role in the pathogenesis of the congenitally bicuspid aortic valve (BAV). BAV is of pronounced genetic heterogeneity, and the genetic components underlying BAV in an overwhelming majority of patients remain elusive. In the current study, the whole coding exons and adjacent introns, as well as 5' and 3' untranslated regions of the GATA4 gene, which codes for a zinc-finger transcription factor crucial for the normal development of the aortic valve, were screened by direct sequencing in 150 index patients with congenital BAV...
November 23, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29315371/computed-tomography-characteristics-of-the-aortic-valve-and-the-geometry-of-sapien-3-transcatheter-heart-valve-in-patients-with-bicuspid-aortic-valve-disease
#3
Hiroyuki Kawamori, Sung-Han Yoon, Tarun Chakravarty, Yoshio Maeno, Mohammad Kashif, Sharjeel Israr, Yigal Abramowitz, Geeteshwar Mangat, Masaki Miyasaka, Tanya Rami, Yoshio Kazuno, Nobuyuki Takahashi, Hasan Jilaihawi, Mamoo Nakamura, Wen Cheng, John Friedman, Daniel Berman, Rahul Sharma, Raj R Makkar
Aims: We assessed the geometry of transcatheter heart valve (THV) and valve function associated with SAPIEN 3 implantation in patients with bicuspid aortic valve (BAV) stenosis. Methods and results: We included 280 consecutive patients who had a contrast computed tomography (CT) before and after transcatheter aortic valve implantation (TAVI) in our institution. Each THV was assessed by CT at five cross-sectional levels: inflow, annulus, mid, sinus, and outflow. The geometry of THV was assessed for eccentricity (1 - minimum diameter/maximum diameter) and expansion (CT derived external valve area/nominal external valve area)...
January 5, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29305187/cardiac-arrest-and-ventricular-arrhythmia-in-adults-with-ebstein-anomaly-and-left-ventricular-non-compaction
#4
Magdalena Kumor, Magdalena Lipczyńska, Elzbieta Katarzyna Biernacka, Anna Klisiewicz, Anna Wójcik, Marek Konka, Katarzyna Kożuch, Piotr Szymański, Piotr Hoffman
BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features)...
January 2, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29302949/simultaneous-transcatheter-intervention-for-coarctation-of-the-aorta-and-bicuspid-aortic-valve
#5
Yukiko Mizutani, Norio Tada, Takahiko Masuda, Masaki Hata
Coarctation of the aorta (CoA) is a relatively common congenital heart anomaly, and bicuspid aortic valve (BAV) is a common congenital heart disease that coexists with CoA. In larger children and adults with CoA, transcatheter intervention has gained acceptance, but for surgical high-risk patients with aortic stenosis, the use of transcatheter aortic valve implantation (TAVI) has been established. Recently, although favorable data have been reported for TAVI when treating BAV, simultaneous transcatheter intervention for CoA and BAV will prove to be a challenge because of the unique anatomy involved requires multiple procedural steps and also has problems of site access...
July 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29295601/identification-of-immunoreactive-peptides-of-toxins-to-simultaneously-assess-the-neutralization-potency-of-antivenoms-against-neurotoxicity-and-cytotoxicity-of-naja-atra-venom
#6
Bin-Sin Liu, Wen-Guey Wu, Min-Han Lin, Chi-Han Li, Bo-Rong Jiang, Suh-Chin Wu, Chih-Hsiang Leng, Wang-Chou Sung
Assessing the neutralization capability of nonlethal but medically relevant toxins in venom has been a challenging task. Nowadays, neutralization efficacy is evaluated based simply on the survival rates of animals injected with antivenom together with a predefined dose of venom, which can determine potency against neurotoxicity but not validate the capability to neutralize cytotoxin-induced complications. In this study, a high correlation with in-vivo and in-vitro neutralization assays was established using the immunoreactive peptides identified from short-chain neurotoxin and cytotoxin A3...
December 25, 2017: Toxins
https://www.readbyqxmd.com/read/29285803/balloon-aortic-valvuloplasty-to-improve-candidacy-of-patients-evaluated-for-transcatheter-aortic-valve-replacement
#7
Mani Arsalan, Samir Khan, Jake Golman, Molly Szerlip, Cecile Mahoney, Morley Herbert, David Brown, Michael Mack, Elizabeth M Holper
OBJECTIVES: Evaluate the role of balloon aortic valvuloplasty (BAV) in improving candidacy of patients for transcatheter aortic valve replacement (TAVR). BACKGROUND: Patients who are not candidates for TAVR may undergo BAV to improve functional and clinical status. METHODS: 117 inoperable or high-risk patients with critical aortic stenosis underwent BAV as a bridge-to-decision for TAVR. Frailty measures including gait speed, serum albumin, hand grip, activities of daily living (ADL); and NYHA functional class before and after BAV were compared...
December 28, 2017: Journal of Interventional Cardiology
https://www.readbyqxmd.com/read/29273600/deficiency-of-natriuretic-peptide-receptor-2-promotes-bicuspid-aortic-valves-aortic-valve-disease-left-ventricular-dysfunction-and-ascending-aortic-dilatations-in-mice
#8
Mark C Blaser, Kuiru Wei, Rachel L Adams, Yu-Qing Zhou, Laura-Lee Caruso, Zahra Mirzaei, Alan Lam, Richard K Tam, Hangjun Zhang, Scott P Heximer, Mark Henkelman, Craig A Simmons
Rationale: Aortic valve disease is a cell-mediated process without effective pharmacotherapy. C-type natriuretic peptide (CNP) inhibits myofibrogenesis and osteogenesis of cultured valve interstitial cells (VICs), and is downregulated in stenotic aortic valves. However, it is unknown whether CNP signaling regulates aortic valve health in vivo. Objective: To determine whether a deficient CNP signaling axis in mice causes accelerated progression of aortic valve disease. Methods and Results: In cultured porcine VICs, CNP inhibited pathological differentiation via the guanylate cyclase natriuretic peptide receptor 2 (NPR2) and not the G-protein-coupled clearance receptor NPR3...
December 22, 2017: Circulation Research
https://www.readbyqxmd.com/read/29249976/thoracic-aortic-aneurysm-development-in-patients-with-bicuspid-aortic-valve-what-is-the-role-of-endothelial-cells
#9
REVIEW
Vera van de Pol, Kondababu Kurakula, Marco C DeRuiter, Marie-José Goumans
Bicuspid aortic valve (BAV) is the most common type of congenital cardiac malformation. Patients with a BAV have a predisposition for the development of thoracic aortic aneurysm (TAA). This pathological aortic dilation may result in aortic rupture, which is fatal in most cases. The abnormal aortic morphology of TAAs results from a complex series of events that alter the cellular structure and extracellular matrix (ECM) composition of the aortic wall. Because the major degeneration is located in the media of the aorta, most studies aim to unravel impaired smooth muscle cell (SMC) function in BAV TAA...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29246428/comparison-of-procedural-clinical-and-valve-performance-results-of-transcatheter-aortic-valve-replacement-in-patients-with-bicuspid-versus-tricuspid-aortic-stenosis
#10
Yan-Biao Liao, Yi-Jian Li, Tian-Yuan Xiong, Yuan-Weixiang Ou, Wen-Yu Lv, Jia-Ling He, Yi-Ming Li, Zhen-Gang Zhao, Xin Wei, Yuan-Ning Xu, Yuan Feng, Mao Chen
BACKGROUND: Limited date describing the procedural, clinical and valve performance results of transcatheter aortic valve replacement (TAVR) in patients with bicuspid versus tricuspid aortic stenosis (TAV). METHODS: Procedural and clinical results were defined and reported according to VARC-2 criteria. RESULTS: Consecutive 87 patients with BAV and 70 patients with TAV were included. Compared to patients with TAV, patients with BAV had similar incidence of second valve implantation (14...
December 9, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29244161/the-impact-of-coronary-anomalies-on-the-outcome-in-aortic-valve-surgery-comparison-of-bicuspid-aortic-valve-versus-tricuspid-aortic-valve-morphotype
#11
Shiho Naito, Johannes Petersen, Hermann Reichenspurner, Evaldas Girdauskas
OBJECTIVES: The association of anomalous anatomy of coronary arteries and bicuspid aortic valve (BAV) has been sporadically reported. Our aim was to evaluate the prevalence of coronary anomalies between BAV and tricuspid aortic valve (TAV) and to analyse their impact on major adverse cardiac events during and after aortic valve surgery. METHODS: A total of 1099 consecutive patients who received preoperative coronary evaluation and elective aortic valve replacement/repair between January 2013 and July 2016 at our institution were involved...
December 12, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29226889/assessment-of-bicuspid-aortic-valve-phenotypes-and-associated-pathologies-a-transesophageal-echocardiographic-study
#12
Selcen Yakar Tülüce, Kamil Tülüce, Ersin Çağrı Şimşek, Özgen Şafak, Mehmet Şefa Ökten, Zeynep Yapan Emren, Sadık Volkan Emren, Uğur Kocabaş, Serdar Bayata, Cem Nazlı
OBJECTIVE: We investigated the frequency of different bicuspid aortic valve disease (BAV) phenotypes,the associated valvular pathologies, and the aortopathy phenotypes, using 2-dimensional (2D) transthoracic, 2D transesophageal echocardiography (TEE) and 3-dimensional (3D) TEE. METHODS: A total of 154 patients with BAV were included. Five BAV phenotypes were detected. To better define valvular pathologies, binary classifications of BAV were used: BAV with antero-posterior commisural line (BAV-AP) and right-left commissural line (BAV-RL)...
December 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29223500/impact-of-chronic-kidney-disease-on-mortality-in-adults-undergoing-balloon-aortic-valvuloplasty
#13
Puja B Parikh, Samantha Novotny, Allen Jeremias, Javed Butler, Jie Yang, Donglei Yin, Luis Gruberg
BACKGROUND: Balloon aortic valvuloplasty (BAV) is often utilized as a bridge prior to surgical or transcatheter aortic valve replacement. Chronic kidney disease (CKD) is commonly present in patients with aortic stenosis, however, its association with outcomes following BAV has not been well studied. Accordingly, we sought to assess the impact of CKD on mortality in adults undergoing BAV. METHODS: The Nationwide Inpatient Sample was screened for hospitalizations involving adults undergoing BAV from 2006 to 2012...
October 26, 2017: Cardiovascular Revascularization Medicine: Including Molecular Interventions
https://www.readbyqxmd.com/read/29221290/should-sinus-of-valsalva-be-preserved-in-patients-with-bicuspid-aortic-valve-and-aortic-dilation
#14
Yulin Wang, Yi Lin, Kanhua Yin, Kai Zhu, Zhaohua Yang, Yongxin Sun, Hao Lai, Chunsheng Wang
Background: It is recommended that dilated ascending aortas (≥45 mm) should be replaced at the time of aortic valve replacement (AVR) for bicuspid aortic valve (BAV). The risk of progressive sinus of Valsalva dilatation after AVR and ascending aorta replacement is less clear. Methods: We identified 156 patients (age 56.2±10.8 years, 46 females) who underwent AVR and ascending aorta replacement in our institution from 2010-2014, 124 (79%) of whom had BAV. Aortic root and ascending aorta sizes were determined from preoperative and the most recent echocardiograms...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29207363/improvement-in-aortic-valve-area-in-patients-with-aortic-stenosis-through-use-of-a-new-hourglass-shaped-valvuloplasty-balloon
#15
Elizabeth Goldenberg, Wesley Pedersen, Emmanouil S Brilakis, Michael Mooney, Mario Gössl, Anil Poulose, Bilal Murad, James Kolbeck, Paul Sorajja
OBJECTIVES: The study aim was to assess the effect of hourglass-shaped V8 and TAV8 balloons (InterValve, Inc) on aortic valve area (AVA) and other outcomes in patients with severe aortic stenosis undergoing balloon aortic valvuloplasty (BAV). BAV has re-emerged with transcatheter therapy. Cylindrical balloons have been the device of choice despite limitations. The hourglass-shaped balloons, with shorter, broader segments separated by a narrowed waist, permit enhanced fixation and better leaflet opening without annular or sinotubular compromise...
December 2017: Journal of Invasive Cardiology
https://www.readbyqxmd.com/read/29206322/4d-flow-mri-cardiac-function-and-t1-mapping-association-of-valve-mediated-changes-in-aortic-hemodynamics-with-left-ventricular-remodeling
#16
Julia Geiger, Amir A Rahsepar, Kenichiro Suwa, Alex Powell, Ahmadreza Ghasemiesfe, Alex J Barker, Jeremy D Collins, James C Carr, Michael Markl
BACKGROUND: Patients with bicuspid aortic valve (BAV) show altered hemodynamics in the ascending aorta that can be assessed by 4D flow MRI. PURPOSE: Comprehensive cardiac MRI was applied to test the hypothesis that BAV-mediated changes in aortic hemodynamics (wall shear stress [WSS] and velocity) are associated with parameters of left ventricular (LV) remodeling. STUDY TYPE: Retrospective data analysis. POPULATION: Forty-nine BAV patients (mean age = 50...
December 5, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29196848/left-sided-congenital-heart-lesions-in-mosaic-turner-syndrome
#17
Nouha Bouayed Abdelmoula, Balkiss Abdelmoula, Walid Smaoui, Imen Trabelsi, Rim Louati, Samir Aloulou, Wafa Aloulou, Fatma Abid, Senda Kammoun, Khaled Trigui, Olfa Bedoui, Hichem Denguir, Souad Mallek, Mustapha Ben Aziza, Jamila Dammak, Oldez Kaabi, Nawel Abdellaoui, Fatma Turki, Asma Kaabi, Wafa Kamoun, Jihen Jabeur, Wided Ltaif, Kays Chaker, Haytham Fourati, Samir M'rabet, Hedi Ben Ameur, Naourez Gouia, Mohamed Nabil Mhiri, Tarek Rebai
In the era of the diseasomes and interactome networks, linking genetics with phenotypic traits in Turner syndrome should be studied thoroughly. As a part of this stratagem, mosaicism of both X and Y chromosome which is a common finding in TS and an evaluation of congenital heart diseases in the different situations of mosaic TS types, can be helpful in the identification of disturbed sex chromosomes, genes and signaling pathway actors. Here we report the case of a mosaic TS associated to four left-sided CHD, including BAV, COA, aortic aneurysms and dissections at an early age...
December 1, 2017: Molecular Genetics and Genomics: MGG
https://www.readbyqxmd.com/read/29191731/characteristic-morphologies-of-the-bicuspid-aortic-valve-in-patients-with-genetic-syndromes
#18
Talha Niaz, Joseph T Poterucha, Timothy M Olson, Jonathan N Johnson, Cecilia Craviari, Thomas Nienaber, Jared Palfreeman, Frank Cetta, Donald J Hagler
BACKGROUND: In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. METHODS: The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016...
November 28, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29188316/impact-of-bicuspid-aortic-valve-morphology-on-aortic-valve-disease-and-aortic-dilation-in-pediatric-patients
#19
Rebekah M Ward, Jordan M Marsh, Jeffrey M Gossett, Mallikarjuna R Rettiganti, R Thomas Collins
Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is associated with aortic stenosis and insufficiency, and aortic dilation in adult groups, but data in pediatric groups are limited. We sought to assess the impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients. We performed a retrospective review of all echocardiograms in patients with isolated BAV who were followed at our institution from July 2002 to July 2012. BAV morphology, aortic valve stenosis and/or insufficiency, and aortic dimensions were measured manually...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29183746/assessment-of-the-anti-snakebite-properties-of-extracts-of-aniba-fragrans-ducke-lauraceae-used-in-folk-medicine-as-complementary-treatment-in-cases-of-envenomation-by-bothrops-atrox
#20
Valéria Mourão de Moura, Noranathan da Costa Guimarães, Luana Travassos Batista, Luciana A Freitas-de-Sousa, Joanderson de Sousa Martins, Maria Carolina Scheffer de Souza, Patrícia D Oliveira de Almeida, Wuelton Marcelo Monteiro, Ricardo Bezerra de Oliveira, Maria Cristina Dos-Santos, Rosa Helena Veras Mourão
ETHNOPHARMACOLOGICAL RELEVANCE: Extracts of leaves and bark of Aniba fragrans are used as tea (decoction) to treat snakebites in communities in the Brazilian Amazon. The aqueous extract of the leaves of A. fragrans has been proven to be effective against Bothrops venom, but only when pre-incubated with the venom. This study sought to assess the potential of different types of extract of this species to inhibit the biological activities of Bothrops atrox venom (BaV) when used the same way as in folk medicine...
November 25, 2017: Journal of Ethnopharmacology
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