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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/29154691/optimal-screening-methods-to-detect-cardiac-disorders-in-athletes-an-evidence-based-review
#1
Zachary K Winkelmann, Ashley K Crossway
Reference/Citation:  Harmon KG, Zigman M, Drezner JA. The effectiveness of screening history, physical exam, and ECG to detect potentially lethal cardiac disorders in athletes: a systematic review/meta-analysis. J Electrocardiol. 2015;48(3):329-338. CLINICAL QUESTION:   Which screening method should be considered best practice to detect potentially lethal cardiac disorders during the preparticipation physical examination (PE) of athletes? DATA SOURCES:   The authors completed a comprehensive literature search of MEDLINE, CINAHL, Cochrane Library, Embase, Physiotherapy Evidence Database (PEDro), and SPORTDiscus from January 1996 to November 2014...
November 20, 2017: Journal of Athletic Training
https://www.readbyqxmd.com/read/29141175/sudden-cardiac-arrest-during-participation-in-competitive-sports
#2
Cameron H Landry, Katherine S Allan, Kim A Connelly, Kris Cunningham, Laurie J Morrison, Paul Dorian
BACKGROUND: The incidence of sudden cardiac arrest during participation in sports activities remains unknown. Preparticipation screening programs aimed at preventing sudden cardiac arrest during sports activities are thought to be able to identify at-risk athletes; however, the efficacy of these programs remains controversial. We sought to identify all sudden cardiac arrests that occurred during participation in sports activities within a specific region of Canada and to determine their causes...
November 16, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29107359/heart-transplantation-in-arrhythmogenic-right-ventricular-cardiomyopathy-experience-from-the-nordic-arvc-registry
#3
Thomas Gilljam, Kristina H Haugaa, Henrik K Jensen, Anneli Svensson, Henning Bundgaard, Jim Hansen, Göran Dellgren, Finn Gustafsson, Hans Eiskjær, Arne K Andreassen, Johan Sjögren, Thor Edvardsen, Anders G Holst, Jesper Hastrup Svendsen, Pyotr G Platonov
OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. METHODS: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry...
October 21, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29105955/right-ventricular-systolic-function-and-mechanical-dispersion-identify-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#4
Meriam Åström Aneq, Eva Maret, Lars Brudin, Anneli Svensson, Jan Engvall
PURPOSE: To assess right ventricular (RV) regional and global systolic function using feature tracking (FT) in patients with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) and to investigate if changes in strain amplitude and mechanical dispersion indicate a propensity for arrhythmia. MATERIALS AND METHODS: Twenty-seven patients fulfilling Task Force Criteria for ARVC and 24 healthy volunteers underwent MR at 1·5 Tesla. Steady-state free precession cine of long-axis slices and a short-axis stack of the RV was acquired...
November 6, 2017: Clinical Physiology and Functional Imaging
https://www.readbyqxmd.com/read/29102365/pregnancy-in-women-with-a-cardiomyopathy-outcomes-and-predictors-from-a-retrospective-cohort
#5
Gilles Billebeau, Martin Etienne, Riadh Cheikh-Khelifa, Daniele Vauthier-Brouzes, Estelle Gandjbakhch, Richard Isnard, Jacky Nizard, Michel Komajda, Marc Dommergues, Philippe Charron
BACKGROUND: Pregnancies in women with pre-existing cardiomyopathies are considered at high risk for complications. However, few data are available to characterize their natural history and predict the outcome. AIMS: Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetric events in women with a cardiomyopathy during pregnancy, excluding peripartum cardiomyopathy. METHODS: In this retrospective study in a referral centre for cardiomyopathies, we included 43 consecutive pregnancies in 36 women with dilated, hypertrophic, arrhythmogenic right ventricular or tachycardia-induced cardiomyopathy, or left ventricular non-compaction...
October 25, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/29069369/high-resolution-three-dimensional-late-gadolinium-enhanced-cardiac-magnetic-resonance-imaging-to-identify-the-underlying-substrate-of-ventricular-arrhythmia
#6
Alexia Hennig, Marjorie Salel, Frederic Sacher, Claudia Camaioni, Soumaya Sridi, Arnaud Denis, Michel Montaudon, François Laurent, Pierre Jais, Hubert Cochet
Aims: Cardiac magnetic resonance (CMR) is recommended as a second-line method to diagnose ventricular arrhythmia (VA) substrate. We assessed the diagnostic yield of CMR including high-resolution late gadolinium-enhanced (LGE) imaging. Methods and results: Consecutive patients with sustained ventricular tachycardia (VT), non-sustained VT (NSVT), or ventricular fibrillation/aborted sudden death (VF/SCD) underwent a non-CMR diagnostic workup according to current guidelines, and CMR including LGE imaging with both a conventional breath-held and a free-breathing method enabling higher spatial resolution (HR-LGE)...
October 23, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29062102/arrhythmogenic-cardiomyopathy-related-dsg2-mutations-affect-desmosomal-cadherin-binding-kinetics
#7
Mareike Dieding, Jana Davina Debus, Raimund Kerkhoff, Anna Gaertner-Rommel, Volker Walhorn, Hendrik Milting, Dario Anselmetti
Cadherins are calcium dependent adhesion proteins that establish the intercellular mechanical contact by bridging the gap to adjacent cells. Desmoglein-2 (Dsg2) is a specific cadherin of the cell-cell contact in cardiac desmosomes. Mutations in the DSG2-gene are regarded to cause arrhythmogenic (right ventricular) cardiomyopathy (ARVC) which is a rare but severe heart muscle disease. The molecular pathomechanisms of the vast majority of DSG2 mutations, however, are unknown. Here, we investigated the homophilic binding of wildtype Dsg2 and two mutations which are associated with ARVC...
October 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29059343/arrhythmogenic-right-ventricular-cardiomyopathy-vs-dilated-cardiomyopathy-implications-for-next-generation-sequencing-and-microrna-regulation-in-appropriate-diagnosis-authors-reply
#8
Argelia Medeiros-Domingo
No abstract text is available yet for this article.
October 20, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29042423/low-prevalence-of-inappropriate-shocks-in-patients-with-inherited-arrhythmia-syndromes-with-the-subcutaneous-implantable-defibrillator-single-center-experience-and-long-term-follow-up
#9
Boris Rudic, Erol Tülümen, Veronika Berlin, Susanne Röger, Ksenija Stach, Volker Liebe, Ibrahim El-Battrawy, Christina Dösch, Theano Papavassiliu, Ibrahim Akin, Martin Borggrefe, Jürgen Kuschyk
BACKGROUND: Up to 40% of patients with transvenous implantable cardioverter-defibrillator (ICD) experience lead-associated complications and may suffer from high complication rates when lead extraction is indicated. Subcutaneous ICD may represent a feasible alternative; however, the efficacy of the subcutaneous ICD in the detection and treatment of ventricular arrhythmias in patients with hereditary arrhythmia syndromes has not been fully evaluated. METHODS AND RESULTS: Patients with primary hereditary arrhythmia syndromes who fulfilled indication for defibrillator placement were eligible for enrollment...
October 17, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29040463/total-pericardium-agenesis-mistaken-for-arrhythmogenic-right-ventricular-cardiomyopathy
#10
Mikael Laredo, Guillaume Duthoit, Estelle Gandjbakhch, Alban Redheuil, Jean-Louis Hébert
No abstract text is available yet for this article.
October 13, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29036601/arrhythmogenic-right-ventricular-cardiomyopathy-implications-of-next-generation-sequencing-and-of-mirnas-regulation-in-appropriate-understanding-and-treatment
#11
Salvatore Patanè, Francesco Patanè
No abstract text is available yet for this article.
October 4, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29036525/circulating-micrornas-in-arrhythmogenic-right-ventricular-cardiomyopathy-with-ventricular-arrhythmia
#12
Shinya Yamada, Ya-Wen Hsiao, Shih-Lin Chang, Yenn-Jiang Lin, Li-Wei Lo, Fa-Po Chung, Shuo-Ju Chiang, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Chin-Yu Lin, Yao-Ting Chang, Abigail Louise D Te, Yung-Nan Tsai, Shih-Ann Chen
Aims: MicroRNAs (miRNAs) have been implicated in cardiac diseases. This study aimed to characterize the circulating miRNAs in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and correlate the miRNAs with the clinical outcomes of ARVC. Methods and results: This study included 62 patients with ventricular arrhythmia (VA): 28 patients (45%) had definite ARVC, 11 (18%) had borderline or possible ARVC, and 23 (37%) had idiopathic ventricular tachycardia (VT)...
September 25, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29034900/generation-of-ipsc-line-from-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-carrying-mutations-in-pkp2-gene
#13
Aleksandr Khudiakov, Daria Kostina, Anna Zlotina, Natalia Yany, Alexey Sergushichev, Tatiana Pervunina, Alexey Tomilin, Anna Kostareva, Anna Malashicheva
Human iPSC line was generated from patient-specific adipose tissue-derived mesenchymal multipotent stromal cells carrying two mutations in plakophilin-2 (PKP2) gene using non-integrative reprogramming method. Reprogramming factors OCT4, KLF4, SOX2, CMYC were delivered using Sendai viruses. Pluripotency was confirmed in vitro using immunofluorescence and RT-PCR analysis and in vivo by teratoma assay. The reported iPSC line could be useful tool for in vitro modeling of arrhythmogenic right ventricular cardiomyopathy...
October 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29029613/right-precordial-directed-electrocardiographical-markers-identify-arrhythmogenic-right-ventricular-cardiomyopathy-in-the-absence-of-conventional-depolarization-or-repolarization-abnormalities
#14
Daniel Cortez, Anneli Svensson, Jonas Carlson, Sharon Graw, Nandita Sharma, Francesca Brun, Anita Spezzacatene, Luisa Mestroni, Pyotr G Platonov
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. METHODS: Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects...
October 13, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29024504/high-validity-of-cardiomyopathy-diagnoses-in-western-sweden-1989-2009
#15
Carmen Basic, Annika Rosengren, Sandra Lindström, Maria Schaufelberger
AIM: Hospital discharges with a diagnosis of cardiomyopathy have more than doubled in Sweden since 1987. We validated the cardiomyopathy diagnoses over this time period to investigate that the increase was real and not a result of improved recognition of the diagnosis and better diagnostic methods. METHODS AND RESULTS: Every fifth year from 1989 to 2009, records for all patients with a cardiomyopathy diagnosis were identified by searching the local registers in three hospitals in Västra Götaland, Sweden...
October 11, 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28960618/ventricular-tachycardia-ablation-in-arrhythmogenic-right-ventricular-cardiomyopathy-patients-with-tmem43-gene-mutations
#16
Amir Abdelwahab, Martin Gardner, Ratika Parkash, Christopher Gray, John Sapp
INTRODUCTION: Catheter ablation of VT in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging, frequently requiring multiple or epicardial ablation procedures; TMEM43 gene mutations typically cause aggressive disease. We sought to compare VT ablation outcomes for ARVC patients with and without TMEM43 mutations. METHODS: Patients with prior ablation for ARVC-related VT were reviewed. Demographic, procedural and follow-up data were reviewed retrospectively...
September 27, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28957535/exercise-and-the-right-ventricle-a-potential-achilles-heel
#17
Andre La Gerche, Dhrubo J Rakhit, Guido Claessen
Exercise is associated with unequivocal health benefits and results in many structural and functional changes of the myocardium that enhance performance and prevent heart failure. However, intense exercise also presents a significant hemodynamic challenge in which the right-sided heart chambers are exposed to a disproportionate increase in afterload and wall stress that can manifest as myocardial fatigue or even damage if intense exercise is sustained for prolonged periods. This review focuses on the physiological factors that result in a disproportionate load on the right ventricle during exercise and the long-term consequences...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28957534/combining-computer-modelling-and-cardiac-imaging-to-understand-right-ventricular-pump-function
#18
John Walmsley, Wouter van Everdingen, Maarten J Cramer, Frits W Prinzen, Tammo Delhaas, Joost Lumens
Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. We begin by highlighting the complexity of interactions that make modelling the RV both challenging and necessary, and then summarize the multiscale modelling approaches used to date to simulate RV pump function in the context of these interactions...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28957531/fibroblasts-and-the-extracellular-matrix-in-right-ventricular-disease
#19
Nikolaos G Frangogiannis
Right ventricular failure predicts adverse outcome in patients with pulmonary hypertension (PH), and in subjects with left ventricular heart failure and is associated with interstitial fibrosis. This review manuscript discusses the cellular effectors and molecular mechanisms implicated in right ventricular fibrosis. The right ventricular interstitium contains vascular cells, fibroblasts, and immune cells, enmeshed in a collagen-based matrix. Right ventricular pressure overload in PH is associated with the expansion of the fibroblast population, myofibroblast activation, and secretion of extracellular matrix proteins...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28940781/right-ventricular-scar-related-ventricular-tachycardia-in-non-ischemic-cardiomyopathy-electrophysiological-characteristics-mapping-and-ablation-of-underlying-heart-disease
#20
Saurabh Kumar, Samuel H Baldinger, Sunil Kapur, Jorge Romero, Nishaki K Mehta, Saagar Mahida, Akira Fujii, Usha B Tedrow, William G Stevenson
BACKGROUND: Right ventricular (RV)-scar related ventricular tachycardia (VT) is often due to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) or cardiac sarcoidosis (CS), but some patients whose clinical course has not been described do not fulfill diagnostic criteria for these diseases. We sought to characterize the electrophysiologic substrate and catheter ablation outcomes of such patients, termed RV cardiomyopathy of unknown source (RCUS). METHODS AND RESULTS: Data of 100 consecutive patients who presented with RV cardiomyopathy and/or RV-related VT for ablation was reviewed (51 ARVC/D, 22 CS; 27 RCUS)...
September 21, 2017: Journal of Cardiovascular Electrophysiology
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