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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/28706583/cardiac-damage-in-athlete-s-heart-when-the-supernormal-heart-fails
#1
REVIEW
Andreina Carbone, Antonello D'Andrea, Lucia Riegler, Raffaella Scarafile, Enrica Pezzullo, Francesca Martone, Raffaella America, Biagio Liccardo, Maurizio Galderisi, Eduardo Bossone, Raffaele Calabrò
Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete's blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias...
June 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28705880/mapping-the-electrical-substrate-in-arrhythmogenic-right-ventricular-cardiomyopathy-there-is-more-than-meets-the-eye
#2
EDITORIAL
Jonathan Chrispin, Fabrizio Assis, Harikrishna Tandri
No abstract text is available yet for this article.
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28705875/electrical-and-structural-substrate-of-arrhythmogenic-right-ventricular-cardiomyopathy-determined-using-noninvasive-electrocardiographic-imaging-and-late-gadolinium-magnetic-resonance-imaging
#3
Christopher M Andrews, Neil T Srinivasan, Stefania Rosmini, Heerajnarain Bulluck, Michele Orini, Sharon Jenkins, Antonis Pantazis, William J McKenna, James C Moon, Pier D Lambiase, Yoram Rudy
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a significant cause of sudden cardiac death in the young. Improved noninvasive assessment of ARVC and better understanding of the disease substrate are important for improving patient outcomes. METHODS AND RESULTS: We studied 20 genotyped ARVC patients with a broad spectrum of disease using electrocardiographic imaging (a method for noninvasive cardiac electrophysiology mapping) and advanced late gadolinium enhancement cardiac magnetic resonance scar imaging...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28705047/galectin-3-correlates-with-arrhythmogenic-right-ventricular-cardiomyopathy-and-predicts-the-risk-of-ventricular-arrhythmias-in-patients-with-implantable-defibrillators
#4
Fahrettin Oz, Imran Onur, Ali Elitok, Evin Ademoglu, Ibrahim Altun, Ahmet Kaya Bilge, Kamil Adalet
Background Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable disorder characterized by fibro-fatty replacement of right ventricular myocytes, increased risk of ventricular arrhythmias, and sudden cardiac death. Galectin-3 (GAL3) is known to play an important role in a number of fibrotic conditions, including cardiac fibrosis. Many studies have focused on the association between GAL3 levels and cardiac fibrosis in heart failure. However, the role of GAL3 in the pathogenesis of ARVD and ventricular arrhythmias has not yet been evaluated thoroughly...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28699125/uptake-of-predictive-genetic-testing-and-cardiac-evaluation-for-children-at-risk-for-an-inherited-arrhythmia-or-cardiomyopathy
#5
Susan Christian, Joseph Atallah, Robin Clegg, Michael Giuffre, Cathleen Huculak, Tara Dzwiniel, Jillian Parboosingh, Sherryl Taylor, Martin Somerville
Predictive genetic testing in minors should be considered when clinical intervention is available. Children who carry a pathogenic variant for an inherited arrhythmia or cardiomyopathy require regular cardiac screening and may be prescribed medication and/or be told to modify their physical activity. Medical genetics and pediatric cardiology charts were reviewed to identify factors associated with uptake of genetic testing and cardiac evaluation for children at risk for long QT syndrome, hypertrophic cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy...
July 11, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28688053/animal-models-of-arrhythmogenic-right-ventricular-cardiomyopathy-what-have-we-learned-and-where-do-we-go-insight-for-therapeutics
#6
REVIEW
Laura Padrón-Barthe, Fernando Domínguez, Pablo Garcia-Pavia, Enrique Lara-Pezzi
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically-determined cardiac heart muscle disorder characterized by fibro-fatty replacement of the myocardium that results in heart failure and sudden cardiac death (SCD), predominantly in young males. The disease is often caused by mutations in genes encoding proteins of the desmosomal complex, with a significant minority caused by mutations in non-desmosomal proteins. Existing treatment options are based on SCD prevention with the implantable cardioverter defibrillator, antiarrhythmic drugs, and anti-heart failure medication...
September 2017: Basic Research in Cardiology
https://www.readbyqxmd.com/read/28670752/sudden-cardiac-death-in-nonischemic-cardiomyopathy-refining-risk-assessment
#7
REVIEW
Matthew M Zipse, Wendy S Tzou
Sudden cardiac death (SCD) risk assessment among patients with nonischemic cardiomyopathy (NICM) has been has been less straightforward than for patients with ischemic cardiomyopathy. The common surrogate that has been associated with highest SCD risk for all cardiomyopathies, and which has been universally used to guide implantation of primary-prevention implantable cardioverter-defibrillators (ICDs), is left ventricular ejection fraction (LVEF) ≤35%. However, this practice has been called into question, especially in light of recent trials suggesting that ICD treatment may not be of additional survival benefit among those with NICM treated with optimal medical therapy...
July 3, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28667170/isolated-cardiac-sarcoidosis-masquerading-as-right-ventricular-outflow-tract-ventricular-tachycardia
#8
Auras R Atreya, Mitkumar Patel, Senthil K Sivalingam, Mathias L Stoenescu
A 67-year-old man with coronary artery disease (CAD) and left anterior descending artery (LAD) stent presented with symptomatic monomorphic ventricular tachycardia (VT) at a rate of 190 bpm requiring cardioversion. ECG showed left bundle branch block pattern and inferior axis, suggestive of a right ventricular outflow tract (RVOT) focus rather than left ventricular scar due to LAD territory myocardial infarction (MI). Echocardiography showed normal wall motion. Angiography revealed a patent mid-LAD stent. Cardiac MRI with delayed postcontrast sequence revealed several regions of hyperenhancement abnormality within the basal portion of the interventricular septum...
June 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28665611/comprehensive-myocardial-proteogenomics-profiling-reveals-c-ebp%C3%AE-as-the-key-factor-in-the-lipid-storage-of-arvc
#9
Liang Chen, Fan Yang, Xiao Chen, Man Rao, Ning-Ning Zhang, Kai Chen, HaiTeng Deng, Jiang-Ping Song, Sheng-Shou Hu
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is hereditary cardiomyopathy characterized by the fibro-fatty replacement of the myocardium. A small number of noncomprehensive profiling studies based on human cardiac tissues have been conducted and reported; consequently, ARVC's gene expression pattern characteristics remain largely undocumented. Our study applies large-scaled, quantitative proteomics based on TMT-labeled LC-MS/MS to analyze the left and right ventricular myocardium of four ARVC and four DCM explanted hearts to compare them with normal hearts...
July 13, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28647112/arrhythmogenic-right-ventricular-cardiomyopathy-in-the-boxer-dog-an-update
#10
REVIEW
Kathryn M Meurs
Arrhythmogenic right ventricular cardiomyopathy is an inheritable form of myocardial disease characterized most commonly by ventricular tachycardias, syncope, and sometimes systolic dysfunction and heart failure. A genetic mutation in the striatin gene has been identified in many affected dogs. Dogs with only one copy of the mutation (heterozygous) have a variable prognosis with many dogs remaining asymptomatic or successfully managed on antiarrhythmic drugs for years. Dogs that are homozygous for the mutation seem to have a worse prognosis...
June 21, 2017: Veterinary Clinics of North America. Small Animal Practice
https://www.readbyqxmd.com/read/28645928/molecular-mechanisms-in-cardiomyopathy
#11
REVIEW
Keith Dadson, Ludger Hauck, Filio Billia
Cardiomyopathies represent a heterogeneous group of diseases that negatively affect heart function. Primary cardiomyopathies specifically target the myocardium, and may arise from genetic [hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), mitochondrial cardiomyopathy] or genetic and acquired [dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM)] etiology. Modern genomics has identified mutations that are common in these populations, while in vitro and in vivo experimentation with these mutations have provided invaluable insight into the molecular mechanisms native to these diseases...
July 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28638576/a-classic-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-arvc-and-literature-review
#12
Htun Latt, Thein Tun Aung, Chanwit Roongsritong, David Smith
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28633348/lifelong-arrhythmic-risk-stratification-in-arrhythmogenic-right-ventricular-cardiomyopathy-distribution-of-events-and-impact-of-periodical-reassessment
#13
Chiara Cappelletto, Davide Stolfo, Antonio De Luca, Bruno Pinamonti, Giulia Barbati, Alberto Pivetta, Marco Gobbo, Francesca Brun, Marco Merlo, Gianfranco Sinagra
Aims: The arrhythmic risk stratification of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains controversial. We evaluated the long-term distribution of life-threatening arrhythmic events assessing the impact of periodical risk reassessment. Methods and results: Ninety-eight ARVC patients with no previous major ventricular arrhythmias were retrospectively analysed. Patients were assessed at baseline, at 22 [inter-quartile range (IQR) 16-26], 49 (IQR 41-55) and 97 months (IQR 90-108)...
June 13, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28623036/-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-literature-review-and-case-report
#14
William Alejandro Camargo-Ariza, Silvia Juliana Galvis-Blanco, Tatiana Del Pilar Camacho-Enciso, Carlos Alberto Quiroz-Romero, Juan José Bermudez-Echeverry
Arrhythmogenic right ventricular cardiomyopathy/dysplasia is an inherited autosomal dominant pathology with an estimated prevalence of 1:2,500 to 1:5,000, being higher in males (3:1). It is characterized histologically by replacement of cardiomyocytes by fibro-adipose tissue, which predisposes to ventricular arrhythmias, right ventricular failure, and sudden cardiac death. The main goal of treatment is to reduce the risk of sudden death and improve the quality of life of patients. We present the case of a 23 year old woman whose clinical symptoms started with palpitations, chest pain with physical activity, syncope and headache 6 years ago during her first pregnancy...
June 13, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28596827/erratum-to-arrhythmogenic-right-ventricular-cardiomyopathy-secondary-to-adipose-infiltration-as-a-cause-of-episodic-collapse-in-a-horse
#15
Alexandra G Raftery, Nerea Cuesta-Garcia, Hal Thompson, David G M Sutton
[This corrects the article DOI: 10.1186/s13620-015-0052-3.].
2017: Irish Veterinary Journal
https://www.readbyqxmd.com/read/28595345/supraventricular-arrhythmias-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-associate-with-long-term-outcome-after-catheter-ablation-of-ventricular-tachycardias
#16
Andreas Müssigbrodt, Helge Knopp, Elena Efimova, Alexander Weber, Livio Bertagnolli, Sebastian Hilbert, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
Aims: This study aimed to assess the impact of supraventricular tachycardia (SVT) on long-term results of radiofrequency catheter ablation therapy of ventricular tachycardia (VT) in a large cohort of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Methods and results: Supraventricular tachycardia occurrence has been studied in patients from our ARVD/C registry (70 patients, 48 male, age 53.2 ± 14.0, 45 patients (64.3%) with previous VT ablation)...
June 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28588093/implantable-cardioverter-defibrillator-therapy-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-predictors-of-appropriate-therapy-outcomes-and-complications
#17
Gabriela M Orgeron, Cynthia A James, Anneline Te Riele, Crystal Tichnell, Brittney Murray, Aditya Bhonsale, Ihab R Kamel, Stephan L Zimmerman, Daniel P Judge, Jane Crosson, Harikrishna Tandri, Hugh Calkins
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. Once the diagnosis is established, risk stratification to determine whether implantable cardioverter-defibrillator (ICD) placement is warranted is critical. METHODS AND RESULTS: The cohort included 312 patients (163 men, age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an ICD...
June 6, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28578331/whole-genome-sequence-identified-a-rare-homozygous-pathogenic-mutation-of-the-dsg2-gene-in-a-familial-arrhythmogenic-cardiomyopathy-involving-both-ventricles
#18
Yubi Lin, Qianhuan Zhang, Zhi An Zhong, Zhe Xu, Siqi He, Fang Rao, Yang Liu, Jiaojiao Tang, Feng Wang, Hui Liu, Jiajun Xie, Hongmei Wu, Shuxia Wang, Xin Li, Zhixin Shan, Chunyu Deng, Zili Liao, Hai Deng, Hongtao Liao, Yumei Xue, Wanqun Chen, Xianzhang Zhan, Bin Zhang, Shulin Wu
BACKGROUND: This study was designed to identify the pathogenic mutation in a Chinese family with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) using whole genome sequencing (WGS). METHODS AND RESULTS: Probands II:1 and II:2 underwent routine examinations for diagnosis. Genomic DNA was extracted from the peripheral blood of family members and analyzed using WGS. A total of 60,285 single-nucleotide polymorphisms (SNP) and 13,918 insertions/deletions (InDel) occurring in the exonic regions of genes and predisposing to cardiomyopathies and arrhythmias were identified...
June 3, 2017: Cardiology
https://www.readbyqxmd.com/read/28575228/right-ventricular-outflow-tract-dimensions-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-a-multicentre-study-comparing-echocardiography-and-cardiovascular-magnetic-resonance
#19
Alexander Gotschy, Ardan M Saguner, Markus Niemann, Sandra Hamada, Deniz Akdis, Ji-Na Yoon, Elena V Parmon, Victoria Delgado, Jeroen J Bax, Sebastian Kozerke, Corinna Brunckhorst, Firat Duru, Felix C Tanner, Robert Manka
Aims: Right ventricular outflow tract (RVOT) dilation is one of the echocardiographic criteria in the 2010 revised Task Force Criteria (TFC) of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). However, studies comparing cardiac magnetic resonance (CMR) and transthoracic echocardiography (TTE) suggest a lower diagnostic accuracy of TTE due to its operator dependence and limited reproducibility. The goal of this study was to compare the 2010 TFC measures of RVOT dilation with three alternative measures for improving the echocardiographic assessment of RVOT in patients with ARVC/D...
May 26, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28536891/myocardial-voltage-ratio-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#20
REVIEW
Andreas Müssigbrodt, Livio Bertagnolli, Elena Efimova, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Michael Döring, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
AIMS: This study aimed to analyze the influence of scar distribution between the endocardium and the epicardium in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: Electroanatomical mapping data were derived from our ARVD/C registry. Myocardial voltage distribution between the endocardium and the epicardium was analyzed in 28 patients (18 men, 49.9 ± 13.0 years) with previous ventricular tachycardia (VT) ablation and complete right ventricular maps...
June 2017: Herzschrittmachertherapie & Elektrophysiologie
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