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https://www.readbyqxmd.com/read/28102477/potential-new-mechanisms-of-pro-arrhythmia-in-arrhythmogenic-cardiomyopathy-focus-on-calcium-sensitive-pathways
#1
REVIEW
C J M van Opbergen, M Delmar, T A B van Veen
Arrhythmogenic cardiomyopathy, or its most well-known subform arrhythmogenic right ventricular cardiomyopathy (ARVC), is a cardiac disease mainly characterised by a gradual replacement of the myocardial mass by fibrous and fatty tissue, leading to dilatation of the ventricular wall, arrhythmias and progression towards heart failure. ARVC is commonly regarded as a disease of the intercalated disk in which mutations in desmosomal proteins are an important causative factor. Interestingly, the Dutch founder mutation PLN R14Del has been identified to play an additional, and major, role in ARVC patients within the Netherlands...
January 19, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28067540/soluble-st2-is-associated-with-disease-severity-in-arrhythmogenic-right-ventricular-cardiomyopathy
#2
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
#3
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/28040191/left-ventricular-function-in-children-and-adolescents-with-arrhythmogenic-right-ventricular-cardiomyopathy
#4
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
#5
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
#6
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/27992454/the-effects-of-swiprosin-1-on-the-formation-of-pseudopodia-like-structures-and-%C3%AE-adrenoceptor-coupling-in-cultured-adult-rat-ventricular-cardiomyocytes
#7
Franziska Nippert, Rolf Schreckenberg, Antonia Hess, Martin Weber, Klaus-Dieter Schlüter
BACKGROUND: Recent findings suggest that adult terminally differentiated cardiomyocytes adapt to stress by cellular de- and redifferentiation. In the present study we tested the hypothesis that swiprosin-1 is a key player in this process. Furthermore, the relationship between swiprosin-1 and β-adrenoceptor coupling was analyzed. METHODS: In order to study the function of swiprosin-1 in adult rat ventricular cardiomyocytes (ARVC) they were isolated and cultured in a medium containing 20% fetal calf serum (FCS)...
2016: PloS One
https://www.readbyqxmd.com/read/27976647/case-profile-volume-analysis-and-dropout-rate-of-antirabies-vaccination-regimens-among-animal-bite-victims-in-gujarat
#8
Kishor M Dhaduk, Sumit V Unadkat, Pooja R Katharotiya, Ankit R Mer, Monika C Chaudhary, Mrudul M Prajapati
BACKGROUND: Rabies is a preventable neglected public health problem and associated with multiple cultural, religious, and social practices, myths in our country. There is a lack of organized surveillance system to measure the incidence of animal bite and human rabies as well as to evaluate cost-saving of different routes, regimen, and types of antirabies vaccines (ARV)/immunoglobulin available in India. OBJECTIVES: The objective of this study is to know dropout rate in intradermal (i...
October 2016: Indian Journal of Public Health
https://www.readbyqxmd.com/read/27939893/use-of-flecainide-in-combination-antiarrhythmic-therapy-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#9
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/27931611/arrhythmogenic-right-ventricular-cardiomyopathy-clinical-course-and-predictors-of-arrhythmic-risk
#10
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/27860063/a-comparison-of-women-and-men-undergoing-catheter-ablation-for-sustained-monomorphic-ventricular-tachycardia
#11
Samuel H Baldinger, Saurabh Kumar, Jorge Romero, Akira Fujii, Laurence M Epstein, Gregory F Michaud, Roy John, Usha B Tedrow, William G Stevenson
INTRODUCTION: Women are underrepresented in clinical studies on catheter ablation (CA) for ventricular tachycardia (VT). The role of gender as a predictor for VT recurrence after CA is unclear and complication rates have not been compared in a large cohort. METHODS AND RESULTS: We analyzed data of consecutive patients undergoing CA for sustained monomorphic VT at our center between 2005 and 2015. A total of 948 patients underwent 1314 ablation procedures: 114 patients without SHD (48% female), 486 with coronary artery disease (CAD) (9% female), 301 with non-ischemic cardiomyopathy (NICM) (22% female), and 46 with arrhythmogenic right ventricular cardiomyopathy (ARVC) (17% female)...
November 18, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27855808/right-ventricular-imaging-and-computer-simulation-for-electromechanical-substrate-characterization-in-arrhythmogenic-right-ventricular-cardiomyopathy
#12
Thomas P Mast, Arco J Teske, John Walmsley, Jeroen F van der Heijden, René van Es, Frits W Prinzen, Tammo Delhaas, Toon A van Veen, Peter Loh, Pieter A Doevendans, Maarten J Cramer, Joost Lumens
BACKGROUND: Previous studies suggested that electrical abnormalities precede overt structural disease in arrhythmogenic right ventricular cardiomyopathy (ARVC). Abnormal RV deformation has been reported in early ARVC without structural abnormalities. The pathophysiological mechanisms underlying these abnormalities remain unknown. OBJECTIVES: The authors used imaging and computer simulation to differentiate electrical from mechanical tissue substrates among ARVC clinical stages...
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27825581/usefulness-of-combined-functional-assessment-by-cardiac-magnetic-resonance-and-tissue-characterization-versus-task-force-criteria-for-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#13
Giovanni Donato Aquaro, Andrea Barison, Giancarlo Todiere, Chrysanthos Grigoratos, Lamia Ait Ali, Gianluca Di Bella, Michele Emdin, Pierluigi Festa
Current task force criteria (TFC) of cardiac magnetic resonance (CMR) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) were generated by comparing probands (mean age of 44 years) to healthy participants of the multi-ethnic study of atherosclerosis (mean age of 60 years). These age differences may be a selection bias because right ventricular end-diastolic volume index decreases 4.6% per decade. Moreover, fat infiltration and late gadolinium enhancement were not included. We evaluated the diagnostic accuracy of TFC using the same methodology used by the task force but comparing probands and age- and gender-matched healthy controls and considering also other morphofunctional and tissue abnormalities detected by CMR...
December 1, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27790640/risk-stratification-in-arrhythmic-right-ventricular-cardiomyopathy-without-implantable-cardioverter-defibrillators
#14
Francesca Brun, Judith A Groeneweg, Kathleen Gear, Gianfranco Sinagra, Jeroen van der Heijden, Luisa Mestroni, Richard N Hauer, Mark Borgstrom, Frank I Marcus, Trina Hughes
OBJECTIVES: The primary objective of this study is risk stratification of patients with arrhythmic right ventricular cardiomyopathy (ARVC). BACKGROUND: There is a need to identify those who need an automatic implantable defibrillator (ICD) to prevent sudden death. METHODS: This is an analysis of 88 patients with ARVC from three centers who were not treated with an ICD. RESULTS: Risk factors for subsequent arrhythmic deaths were pre-enrollment sustained or nonsustained ventricular tachycardia (VT) and decreased left ventricular function...
October 2016: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/27771402/moving-from-multimodality-diagnostic-tests-toward-multimodality-risk-stratification-in-arvc
#15
Arco J Teske, Thomas P Mast
No abstract text is available yet for this article.
October 14, 2016: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27771401/combination-of-ecg-and-echocardiography-for-identification-of-arrhythmic-events-in-early-arvc
#16
Ida S Leren, Jørg Saberniak, Trine F Haland, Thor Edvardsen, Kristina H Haugaa
OBJECTIVES: The aim of this study was to investigate early markers of arrhythmic events (AEs) and improve risk stratification in early arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: AEs are frequent in patients with ARVC, but risk stratification in subjects with early ARVC is challenging. METHODS: Early ARVC disease was defined as possible or borderline ARVC diagnosis according to the ARVC Task Force Criteria 2010. We performed resting and signal averaged electrocardiogram (ECG)...
October 14, 2016: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27769725/structural-and-molecular-pathology-of%C3%A2-the%C3%A2-atrium-in-boxer-arrhythmogenic-right-ventricular-cardiomyopathy
#17
J Vila, R Pariaut, N S Moïse, E M Oxford, P R Fox, C A Reynolds, C Saelinger
OBJECTIVE: To investigate the expression and distribution of desmosomal and gap junction proteins of the intercalated disc in the atria of boxers with arrhythmogenic right ventricular cardiomyopathy (ARVC). ANIMALS: Nineteen control dogs and 13 boxers with histopathologically confirmed ARVC. METHODS: Right and left atrial samples were examined using immunofluorescence and Western blots. The intercalated disc proteins investigated included total and phosphorylated connexin43 (Cx43 and pCx43), connexin45, connexin40, plakoglobin, plakophilin-2, desmoplakin, and N-cadherin...
October 18, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/27761164/the-genetic-background-of-arrhythmogenic-right-ventricular-cardiomyopathy
#18
REVIEW
Seiko Ohno
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by degeneration of the right ventricle and ventricular tachycardia originating from the right ventricle. Additionally, the disease is an inherited cardiomyopathy that mainly follows the autosomal dominant pattern. More than 10 genes have been reported as causative genes for ARVC, and more than half of ARVC patients carry mutations in desmosome related genes. The desmosome is one of the structures involved in cell adhesion and its disruption leads to various diseases, including a skin disease called pemphigus...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27756409/ventricular-structure-in-arvc-going-beyond-volumes-as-a-measure-of-risk
#19
Kristin McLeod, Samuel Wall, Ida Skrinde Leren, Jørg Saberniak, Kristina Hermann Haugaa
BACKGROUND: Altered right ventricular structure is an important feature of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), but is challenging to quantify objectively. The aim of this study was to go beyond ventricular volumes and diameters and to explore if the shape of the right and left ventricles could be assessed and related to clinical measures. We used quantifiable computational methods to automatically identify and analyse malformations in ARVC patients from Cardiovascular Magnetic Resonance (CMR) images...
October 14, 2016: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/27751343/endomyocardial-fibrosis-with-right-ventricular-aneurysm-mimicking-arvc-a-case-report-from-india
#20
Suneesh Kalliath, Rajesh Gopalan Nair, Haridasan Vellani
A 48-year-old man presented with chronic right sided heart failure. 2D echocardiography revealed the classical features of left ventricular endomyocardial fibrosis with a prominent right ventricular apical aneurysm. Right ventriculography further defined the aneurysm. Cardiac magnetic resonance images revealed a thin-walled, apical aneurysm of right ventricle with multiple septations and marked obliteration of left ventricular apex. A delayed-enhancement sequence after the infusion of contrast, demonstrated a hypersignal in the subendocardium, consistent with the right ventricular involvement of endomyocardial fibrosis...
September 2016: Indian Heart Journal
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