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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/28097477/spect-perfusion-abnormality-associated-with-arrhythmogenic-right-ventricular-cardiomyopathy
#1
Charles Hardebeck, Akira Wada, Gbemiga Sofowora
No abstract text is available yet for this article.
January 17, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28097329/quantifying-disease-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-key-to-advancing-therapy
#2
Wojciech Zareba
No abstract text is available yet for this article.
January 11, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28097316/evaluation-of-structural-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#3
Thomas P Mast, Cynthia A James, Hugh Calkins, Arco J Teske, Crystal Tichnell, Brittney Murray, Peter Loh, Stuart D Russell, Birgitta K Velthuis, Daniel P Judge, Dennis Dooijes, Ryan J Tedford, Jeroen F van der Heijden, Harikrishna Tandri, Richard N Hauer, Theodore P Abraham, Pieter A Doevendans, Anneline S J M Te Riele, Maarten J Cramer
Importance: Considerable research has described the arrhythmic course of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, objective data characterizing structural progression, such as ventricular enlargement and cardiac dysfunction, in ARVD/C are relatively scarce. Objectives: To define the extent of structural progression, identify determinants of structural progression, and determine the association between structural progression and electrocardiographic (ECG) changes in patients with ARVD/C...
January 11, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28087747/zurich-international-symposium-on-arrhythmogenic-right-ventricular-cardiomyopathies
#4
Firat Duru, Corinna Brunckhorst
No abstract text is available yet for this article.
December 21, 2016: European Heart Journal
https://www.readbyqxmd.com/read/28076798/neonatal-transplantation-confers-maturation-of-psc-derived-cardiomyocytes-conducive-to-modeling-cardiomyopathy
#5
Gun-Sik Cho, Dong I Lee, Emmanouil Tampakakis, Sean Murphy, Peter Andersen, Hideki Uosaki, Stephen Chelko, Khalid Chakir, Ingie Hong, Kinya Seo, Huei-Sheng Vincent Chen, Xiongwen Chen, Cristina Basso, Steven R Houser, Gordon F Tomaselli, Brian O'Rourke, Daniel P Judge, David A Kass, Chulan Kwon
Pluripotent stem cells (PSCs) offer unprecedented opportunities for disease modeling and personalized medicine. However, PSC-derived cells exhibit fetal-like characteristics and remain immature in a dish. This has emerged as a major obstacle for their application for late-onset diseases. We previously showed that there is a neonatal arrest of long-term cultured PSC-derived cardiomyocytes (PSC-CMs). Here, we demonstrate that PSC-CMs mature into adult CMs when transplanted into neonatal hearts. PSC-CMs became similar to adult CMs in morphology, structure, and function within a month of transplantation into rats...
January 10, 2017: Cell Reports
https://www.readbyqxmd.com/read/28069705/multilevel-analyses-of-scn5a-mutations-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-suggest-non-canonical-mechanisms-for-disease-pathogenesis
#6
Anneline S J M Te Riele, Esperanza Agullo-Pascual, Cynthia A James, Alejandra Leo-Macias, Marina Cerrone, Mingliang Zhang, Xianming Lin, Bin Lin, Nara L Sobreira, Nuria Amat-Alarcon, Roos F Marsman, Brittney Murray, Crystal Tichnell, Jeroen F van der Heijden, Dennis Dooijes, Toon A B van Veen, Harikrishna Tandri, Steven J Fowler, Richard N W Hauer, Gordon Tomaselli, Maarten P van den Berg, Matthew R G Taylor, Francesca Brun, Gianfranco Sinagra, Arthur A M Wilde, Luisa Mestroni, Connie R Bezzina, Hugh Calkins, J Peter van Tintelen, Lei Bu, Mario Delmar, Daniel P Judge
AIMS: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is often associated with desmosomal mutations. Recent studies suggest an interaction between the desmosome and sodium channel protein Nav1.5. We aimed to determine the prevalence and biophysical properties of mutations in SCN5A (the gene encoding Nav1.5) in ARVD/C. METHODS AND RESULTS: We performed whole-exome sequencing in six ARVD/C patients (33% male, 38.2 ± 12.1 years) without a desmosomal mutation...
January 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28067540/soluble-st2-is-associated-with-disease-severity-in-arrhythmogenic-right-ventricular-cardiomyopathy
#7
Kaspar Broch, Ida S Leren, Jørg Saberniak, Thor Ueland, Thor Edvardsen, Lars Gullestad, Kristina H Haugaa
PURPOSE: Diagnostic and prognostic evaluation remains challenging in arrhythmogenic right ventricular cardiomyopathy (ARVC). We measured plasma concentration of soluble ST2 (sST2) and assessed its association with right ventricular (RV) function and ventricular arrhythmias in patients with ARVC. METHODS: We included patients with ARVC and genotype positive relatives. Soluble ST2 was determined by ELISA. We assessed myocardial function by echocardiography including strain by speckle tracking technique...
January 9, 2017: Biomarkers: Biochemical Indicators of Exposure, Response, and Susceptibility to Chemicals
https://www.readbyqxmd.com/read/28057231/anterior-t-wave-inversion-in-young-white%C3%A2-athletes-and-nonathletes-prevalence-and-significance
#8
Aneil Malhotra, Harshil Dhutia, Sabiha Gati, Tee-Joo Yeo, Helder Dores, Rachel Bastiaenen, Rajay Narain, Ahmed Merghani, Gherardo Finocchiaro, Nabeel Sheikh, Alexandros Steriotis, Abbas Zaidi, Lynne Millar, Elijah Behr, Maite Tome, Michael Papadakis, Sanjay Sharma
BACKGROUND: Anterior T-wave inversion (ATWI) on electrocardiography (ECG) in young white adults raises the possibility of cardiomyopathy, specifically arrhythmogenic right ventricular cardiomyopathy (ARVC). Whereas the 2010 European consensus recommendations for ECG interpretation in young athletes state that ATWI beyond lead V1 warrants further investigation, the prevalence and significance of ATWI have never been reported in a large population of asymptomatic whites. OBJECTIVES: This study investigated the prevalence and significance of ATWI in a large cohort of young, white adults including athletes...
January 3, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28052233/arrhythmogenic-right-ventricular-cardiomyopathy
#9
Domenico Corrado, Mark S Link, Hugh Calkins
No abstract text is available yet for this article.
January 5, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28040191/left-ventricular-function-in-children-and-adolescents-with-arrhythmogenic-right-ventricular-cardiomyopathy
#10
Paweena Chungsomprasong, Robert Hamilton, Wietske Luining, Meena Fatah, Shi-Joon Yoo, Lars Grosse-Wortmann
The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC...
December 2, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/28017936/catheter-ablation-reduces-ventricular-tachycardia-burden-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-insights-from-a-north-western-french-multicentre-registry
#11
Zouheir Souissi, Stéphane Boulé, Jean-Sylvain Hermida, Alexandre Doucy, Philippe Mabo, Dominique Pavin, Frédéric Anselme, Nathanaël Auquier, Sandro Ninni, Augustin Coisne, François Brigadeau, Valérie Deken-Delannoy, Didier Klug, Dominique Lacroix
AIMS: Studies assessing radiofrequency ablation (RFA) of ventricular tachycardia (VT) in arrhythmogenic right ventricular cardiomyopathy (ARVC) report VT recurrences, but have not evaluated the impact of RFA on relevant clinical events during follow-up. We aimed to investigate relevant RFA outcomes in a multicentric registry. METHODS AND RESULTS: This study included 49 patients with ARVC (46 with definite diagnosis, 3 with borderline diagnosis according to revised Task Force Criteria) who underwent 92 RFA procedures (83 endocardial, 9 combined endo-epicardial) between 1999-2015...
December 25, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28012557/exercise-testing-for-long-term-follow-up-in-arrhythmogenic-right-ventricular-cardiomyopathy
#12
Daniel Karlsson, Jan Engvall, Agota Alfoldine Ando, Meriam Åström Aneq
OBJECTIVES: We investigated arrhythmia, electrocardiography and physical work capacity (PWC) in the follow-up of ARVC. DESIGN: Twenty-three patients (13 men; age 41±12years) fulfilling diagnostic criteria were re-investigated after at least five years. RESULTS: Ventricular arrhythmia during exercise testing (ET) was present in 14 patients (61%) and showed variation between examinations. In eleven (48%), complex ventricular ectopic activity was observed at peak exercise or immediately thereafter...
October 28, 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27939893/use-of-flecainide-in-combination-antiarrhythmic-therapy-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#13
Simon Ermakov, Edward P Gerstenfeld, Yana Svetlichnaya, Melvin M Scheinman
BACKGROUND: Antiarrhythmic therapy is commonly used for suppression of arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) in conjunction with implantable cardioverter-defibrillators and catheter ablation. The efficacy of combination flecainide and sotalol/metoprolol therapy for patients refractory to single agents and/or catheter ablation has not been well established. OBJECTIVE: The purpose of this study was to describe our experience with the addition of flecainide in combination with sotalol/metoprolol for treatment of arrhythmias in patients with ARVC...
December 9, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27932238/gender-differences-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-clinical-manifestations-electrophysiological-properties-substrate-characteristics-and-prognosis-of-radiofrequency-catheter-ablation
#14
Chin-Yu Lin, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Yao-Ting Chang, Yun-Yu Chen, Rohit Walia, Abigail Louise D Te, Shinya Yamada, Shih-Ann Chen
BACKGROUND: Gender differences in the penetrance and clinical expression of genetic mutations have been reported in patients with arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C). Our study aimed at clarifying the impact of gender on ventricular substrates and clinical outcomes after radiofrequency catheter ablation (RFCA). METHODS: Patients with ARVD/C underwent RFCA for drug-refractory ventricular arrhythmias (VAs) were consecutively enrolled...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27931612/further-progress-in-predicting-life-threatening-arrhythmias-in-patients%C3%A2-with-arrhythmogenic-right%C3%A2-ventricular-cardiomyopathy
#15
EDITORIAL
Cynthia A James, Hugh Calkins
No abstract text is available yet for this article.
December 13, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27931611/arrhythmogenic-right-ventricular-cardiomyopathy-clinical-course-and-predictors-of-arrhythmic-risk
#16
Andrea Mazzanti, Kevin Ng, Alessandro Faragli, Riccardo Maragna, Elena Chiodaroli, Nicoletta Orphanou, Nicola Monteforte, Mirella Memmi, Patrick Gambelli, Valeria Novelli, Raffaella Bloise, Oronzo Catalano, Guido Moro, Valentina Tibollo, Massimo Morini, Riccardo Bellazzi, Carlo Napolitano, Vincenzo Bagnardi, Silvia G Priori
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its progression over time and predictors of arrhythmias are still being defined. OBJECTIVES: This study sought to describe the clinical course of ARVC and occurrence of life-threatening arrhythmic events (LAE) and cardiovascular mortality; identify risk factors associated with increased LAE risk; and define the response to therapy. METHODS: We determined the clinical course of 301 consecutive patients with ARVC using the Kaplan-Meier method adjusted to avoid the bias of delayed entry...
December 13, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27920450/catheter-ablation-for-ventricular-tachycardia-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-a-systematic-review-and-meta-analysis
#17
H Jiang, X -L Zhang, Q -L Yang, S -H Zhu, H -S Yu, B Xu, W Huang
No abstract text is available yet for this article.
2016: Acta Cardiologica
https://www.readbyqxmd.com/read/27919765/late-gadolinium-enhancement-in-brugada-syndrome-a-marker-for-subtle-underlying-cardiomyopathy
#18
Rachel Bastiaenen, Andrew T Cox, Silvia Castelletti, Yanushi D Wijeyeratne, Nicholas Colbeck, Nadia Pakroo, Hammad Ahmed, Nick Bunce, Lisa Anderson, James C Moon, Sanjay Prasad, Sanjay Sharma, Elijah R Behr
BACKGROUND: There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. OBJECTIVE: We characterised Brugada syndrome (BrS) patients using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). METHODS: BrS was diagnosed according to international guidelines. 26% BrS patients carried SCN5A mutations. CMR data from 78 BrS patients were compared with 78 healthy controls (44±15 vs 42±14 years; p=0...
December 2, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27909515/myocardial-biopsy-in-idiopathic%C3%A2-atrial-fibrillation-and-other-arrhythmias-nosological-diagnosis-clinical-and-morphological-parallels-and-treatment
#19
O V Blagova, A V Nedostup, E A Kogan, V A Sulimov, S A Abugov, A G Kupriyanova, V A Zaydenov, A E Donnikov, E V Zaklyazminskaya, E A Okisheva
BACKGROUND: The nosological nature of "idiopathic" arrhythmias and the effect of etiotropic and pathogenetic treatment are often unknown. METHODS AND RESULTS: 19 patients (42.6±11.3 years, 9 women) with atrial fibrillation (n = 16), supraventricular (n = 10) and ventricular (n = 4) premature beats, supraventricular (n = 2) and ventricular tachycardia (n = 1), left bundle branch block (n= 2), AV block (n = 2) without structural heart changes. Viruses were identified (polymerase chain reaction, PCR) along with measurement of anti-heart antibodies (AHA) and endomyocardial biopsy (EMB)...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27900866/-the-benefit-of-magnetic-resonance-for-diagnosing-cardiomyopathy-and-myocarditis
#20
Michal Fikrle, Petr Kuchynka, Martin Mašek, Jana Podzimková, Jan Kuchař, Aleš Linhart, Tomáš Paleček
Magnetic resonance is becoming an increasingly used examination in cardiology, since it greatly improves the accuracy of diagnosing of many heart diseases. At present magnetic resonance is the gold standard in assessing the volumes of the heart chambers and the systolic function of both ventricles. The possibility of detecting tissue characteristics to refine the diagnostics of different types of myocardial pathology is of essential importance. The authors summarize in the article the present knowledge about the use of magnetic resonance of the heart in the field of myocardial disease, i...
2016: Vnitr̆ní Lékar̆ství
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