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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/28804588/first-reported-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-oman
#1
Hatim Al Lawati, Humoud Al Dhuhli
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder characterized by fatty degeneration of the right ventricular myocardium with variable involvement of the left ventricle. The condition is associated with exercise-mediated ventricular tachycardia and is one of the recognized causes of sudden cardiac death in the young and in athletes. Here, we report the first confirmed case of ARVC in Oman and present its electrocardiographic, echocardiographic features, and radiological findings on gated, contrast-enhanced cardiac computed tomography...
July 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#2
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28771538/a-novel-noninvasive-surface-ecg-analysis-using-interlead-qrs-dispersion-in-arrhythmogenic-right-ventricular-cardiomyopathy
#3
Wan-Hsin Hsieh, Chin-Yu Lin, Abigail Louise D Te, Men-Tzung Lo, Cheng-I Wu, Fa-Po Chung, Yi-Chung Chang, Shih-Lin Chang, Chen Lin, Li-Wei Lo, Yu-Feng Hu, Jo-Nan Liao, Yun-Yu Chen, Shih-Jie Jhuo, Sunu Budhi Raharjo, Yenn-Jiang Lin, Shih-Ann Chen
BACKGROUND: This study investigated the feasibility of using the precordial surface ECG lead interlead QRS dispersion (IQRSD) in the identification of abnormal ventricular substrate in arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: Seventy-one consecutive patients were enrolled and reclassified into 4 groups: definite ARVC with epicardial ablation (Group 1), ARVC with ventricular tachycardia (VT, Group 2), idiopathic right ventricular outflow tract VT without ARVC (Group 3), and controls without VT (Group 4)...
2017: PloS One
https://www.readbyqxmd.com/read/28767663/contribution-of-exome-sequencing-for-genetic-diagnostic-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#4
Joel Fedida, Veronique Fressart, Philippe Charron, Elodie Surget, Tiphaine Hery, Pascale Richard, Erwan Donal, Boris Keren, Guillaume Duthoit, Françoise Hidden-Lucet, Eric Villard, Estelle Gandjbakhch
BACKGROUND: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy mainly caused by heterozygous desmosomal gene mutations, the major gene being PKP2. The genetic cause remains unknown in ~50% of probands with routine desmosomal gene screening. The aim of this study was to assess the diagnostic accuracy of whole exome sequencing (WES) in ARVC/D with negative genetic testing. METHODS: WES was performed in 22 patients, all without a mutation identified in desmosomal genes...
2017: PloS One
https://www.readbyqxmd.com/read/28765973/differential-expression-of-b-type-natriuretic-peptide-between-left-and-right-ventricles-with-particular-regard-to-sudden-cardiac-death
#5
Zhi-Peng Cao, Jia-Jia Xue, Yuan Zhang, Mei-Hui Tian, Ying Xiao, Yu-Qing Jia, Bao-Li Zhu
The aim of the present study was to investigate the differential expression of B‑type natriuretic peptide (BNP) between the left and right ventricle (RV) in sudden cardiac death (SCD). A total of 26 forensic autopsy cases of sudden death (survival time <30 min, postmortem interval <48 h or frozen within 6 h following death) in the present institute were examined. The cases consisted of acute ischemic heart disease (AIHD, n=15) with/without apparent myocardial necrosis as a sign of infarction (acute myocardial infarction, n=6; ischemic heart disease, IHD, n=9), and arrhythmogenic right ventricular cardiomyopathy (ARVC/D, n=5), in addition to traffic accidents and high falls without any pre existing heart disease as control (C, total n=6)...
August 2, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28750076/targeted-next-generation-sequencing-detects-novel-gene-phenotype-associations-and-expands-the-mutational-spectrum-in-cardiomyopathies
#6
Cinzia Forleo, Anna Maria D'Erchia, Sandro Sorrentino, Caterina Manzari, Matteo Chiara, Massimo Iacoviello, Andrea Igoren Guaricci, Delia De Santis, Rita Leonarda Musci, Antonino La Spada, Vito Marangelli, Graziano Pesole, Stefano Favale
Cardiomyopathies are a heterogeneous group of primary diseases of the myocardium, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC), with higher morbidity and mortality. These diseases are genetically diverse and associated with rare mutations in a large number of genes, many of which overlap among the phenotypes. To better investigate the genetic overlap between these three phenotypes and to identify new genotype-phenotype correlations, we designed a custom gene panel consisting of 115 genes known to be associated with cardiomyopathic phenotypes and channelopathies...
2017: PloS One
https://www.readbyqxmd.com/read/28740174/plakophilin-2-is-required-for-transcription-of-genes-that-control-calcium-cycling-and-cardiac-rhythm
#7
Marina Cerrone, Jerome Montnach, Xianming Lin, Yan-Ting Zhao, Mingliang Zhang, Esperanza Agullo-Pascual, Alejandra Leo-Macias, Francisco J Alvarado, Igor Dolgalev, Thomas V Karathanos, Kabir Malkani, Chantal J M Van Opbergen, Joanne J A van Bavel, Hua-Qian Yang, Carolina Vasquez, David Tester, Steven Fowler, Fengxia Liang, Eli Rothenberg, Adriana Heguy, Gregory E Morley, William A Coetzee, Natalia A Trayanova, Michael J Ackerman, Toon A B van Veen, Hector H Valdivia, Mario Delmar
Plakophilin-2 (PKP2) is a component of the desmosome and known for its role in cell-cell adhesion. Mutations in human PKP2 associate with a life-threatening arrhythmogenic cardiomyopathy, often of right ventricular predominance. Here, we use a range of state-of-the-art methods and a cardiomyocyte-specific, tamoxifen-activated, PKP2 knockout mouse to demonstrate that in addition to its role in cell adhesion, PKP2 is necessary to maintain transcription of genes that control intracellular calcium cycling. Lack of PKP2 reduces expression of Ryr2 (coding for Ryanodine Receptor 2), Ank2 (coding for Ankyrin-B), Cacna1c (coding for CaV1...
July 24, 2017: Nature Communications
https://www.readbyqxmd.com/read/28706583/cardiac-damage-in-athlete-s-heart-when-the-supernormal-heart-fails
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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