8,187 results match your criteria Arrhythmogenic Right Ventricular Cardiomyopathy


Engineered Heart Slice Model of Arrhythmogenic Cardiomyopathy using Plakophilin-2 Mutant Myocytes.

Tissue Eng Part A 2018 Dec 6. Epub 2018 Dec 6.

Johns Hopkins University School of Medicine, Biomedical Engineering, Baltimore, Maryland, United States ;

Arrhythmogenic cardiomyopathy (AC), a cause of sudden cardiac death among young and otherwise healthy individuals, is a heritable disease that can be modeled in vitro using patient-specific cardiac myocytes (CMs) from induced pluripotent stem cells. An understanding of underlying disease mechanisms, particularly in the early concealed stages, could lead to new diagnosis and treatment strategies. However, multicellular syncytial models are needed to understand how genetically-encoded mutations of the desmosomes that interconnect cells lead to aberrant electrical conduction and arrhythmias. Read More

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December 2018

Feasibility and Utility of Intraoperative Epicardial Scar Characterization During Left Ventricular Assist Device Implantation for Destination Therapy.

J Cardiovasc Electrophysiol 2018 Dec 5. Epub 2018 Dec 5.

Center for Arrhythmia Care, University of Chicago Medicine.

Introduction: Ventricular arrhythmias (VA) after left ventricular assist device (LVAD) placement are associated with increased morbidity and mortality. We sought to assess epicardial voltage characteristics at the time of LVAD implantation and investigate relationships between scar burden and post-implant VA.

Methods And Results: Consecutive patients underwent open-chest epicardial electroanatomic mapping immediately prior to LVAD implantation. Read More

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December 2018

K 3.1 Protein is Expressed as a Transmural Gradient across the Rat Left Ventricular Free Wall.

J Cardiovasc Electrophysiol 2018 Dec 5. Epub 2018 Dec 5.

School of Biomedical Sciences, Faculty of Biological Sciences, University of Leeds.

Introduction: K 3.1, also known as TASK-1, is a twin-pore acid-sensitive repolarizing K channel, responsible for a background potassium current that significantly contributes to setting the resting membrane potential of cardiac myocytes. Inhibition of I alters cardiac repolarization and is pro-arrhythmogenic. Read More

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December 2018

Differential pacing from two sites to diagnose risk of ventricular arrhythmia and death.

Pacing Clin Electrophysiol 2018 Dec 4. Epub 2018 Dec 4.

Peter Munk Cardiac Centre, University Health Network, Toronto, ON, Canada.

Background: QRS abnormalities may not be apparent in sinus rhythm in electrically stable cardiomyopathy patients who can have quiescent but highly arrhythmogenic substrate. Here, we test the hypothesis that differential changes in QRS construction during right-ventricular apex pacing (RVP) as opposed to atrial pacing (AP) will identify latent substrate for ventricular arrhythmias (VA) and death.

Methods: Forty-patients with cardiomyopathy free of VA underwent baseline 114-electrode body-surface ECG during AP (100 bpm) and RVP (100 and 120 bpm). Read More

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December 2018
1 Read

Role of autonomic nervous system in atrial fibrillation.

Int J Cardiol 2018 Nov 18. Epub 2018 Nov 18.

Centre for Heart Rhythm Disorders (CHRD), South Australian Health and Medical Research Institute (SAHMRI), University of Adelaide and Royal Adelaide Hospital, Adelaide, Australia.

Atrial fibrillation is the most common sustained arrhythmia and is associated with significant morbidity and mortality. The autonomic nervous system has a significant role in the milieu predisposing to the triggers, perpetuators and substrate for atrial fibrillation. It has direct electrophysiological effects and causes alterations in atrial structure. Read More

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November 2018
3 Reads

The S-wave angle identifies arrhythmogenic right ventricular cardiomyopathy in patients with electrocardiographically concealed disease phenotype.

J Electrocardiol 2018 Nov - Dec;51(6):1003-1008. Epub 2018 Aug 10.

Department of Cardiology, Clinical Sciences, Lund University, Lund, Sweden; Cardiovascular Department, Ospedali Riuniti and University of Trieste, Trieste, Italy; Arrhythmia Clinic, Skåne University Hospital, Lund, Sweden.

Background: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries risk of sudden death. We hypothesize that the S-wave angle differentiates ARVD/C with otherwise normal electrocardiograms from controls.

Materials And Methods: All patients met Task Force 2010 definite ARVD/C criteria. Read More

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August 2018
2 Reads

Unexpected sudden death in pregnancy - arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report.

Forensic Sci Res 2017 23;2(3):161-163. Epub 2017 May 23.

Forensic Unit, Teaching Hospital Kandy, Kandy, Sri Lanka.

Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries. Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive disease, undiagnosed pulmonary hypertension and cardiomyopathies being recognized as major health problems in these settings. This article reported a 27-year-old female who was normal on clinical examination and basic investigations, and on an antenatal visit was found collapsed in the toilet of her house and was pronounced dead on admission to hospital. Read More

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May 2017
2 Reads

Brugada syndrome: a fatal disease with complex genetic etiologies - still a long way to go.

Forensic Sci Res 2017 5;2(3):115-125. Epub 2017 Jul 5.

Department of Forensic Pathology, Zhongshan School of Medicine, Sun Yat-Sen University, Guangzhou, China.

Brugada syndrome (BrS) is an arrhythmogenic disorder which was first described in 1992. This disease is a channelopathy characterized by ST-segment elevations in the right precordial leads and is susceptible to sudden death. BrS is a fatal disease with gender and age preferences. Read More

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Transgenic short-QT syndrome 1 rabbits mimic the human disease phenotype with QT/action potential duration shortening in the atria and ventricles and increased ventricular tachycardia/ventricular fibrillation inducibility.

Eur Heart J 2018 Nov 28. Epub 2018 Nov 28.

Department of Cardiology and Angiology I, Heart Center University of Freiburg, Hugstetter Str. 55, 79106 Freiburg, Germany.

Aims: Short-QT syndrome 1 (SQT1) is an inherited channelopathy with accelerated repolarization due to gain-of-function in HERG/IKr. Patients develop atrial fibrillation, ventricular tachycardia (VT), and sudden cardiac death with pronounced inter-individual variability in phenotype. We generated and characterized transgenic SQT1 rabbits and investigated electrical remodelling. Read More

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November 2018
2 Reads

Arrhythmic Genotypes in Familial Dilated Cardiomyopathy: Implications for Genetic Testing and Clinical Management.

Heart Lung Circ 2018 Oct 11. Epub 2018 Oct 11.

Molecular Cardiology Division, Victor Chang Cardiac Research Institute, Sydney, NSW, Australia; St. Vincent's Clinical School, Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia; Cardiology Department, St. Vincent's Hospital, Sydney, NSW, Australia. Electronic address:

Cardiac arrhythmias are frequently seen in patients with dilated cardiomyopathy (DCM) and can precipitate heart failure and death. In patients with non-ischaemic DCM, evidence for the benefit of an implantable cardioverter-defibrillator (ICD) for primary prevention of sudden cardiac death has recently been questioned. Algorithms devised to identify high-risk individuals who might benefit most from ICD implantation have focussed on clinical criteria with little attention paid to the underlying aetiology of DCM. Read More

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October 2018
1 Read

Bioinformatic analysis of a plakophilin-2-dependent transcription network: implications for the mechanisms of arrhythmogenic right ventricular cardiomyopathy in humans and in boxer dogs.

Europace 2018 Nov;20(suppl_3):iii125-iii132

The Leon H Charney Division of Cardiology, New York University School of Medicine, 435 East 30th Street. SB 707, New York, NY, USA.

Aims: Previous studies in murine hearts and in cell systems have shown that modifications in the expression or sequence integrity of the desmosomal molecule plakophilin-2 (PKP2) can alter the downstream expression of transcripts necessary for the electrical and mechanical function of the heart. These findings have provided support to mechanistic hypotheses that seek to explain arrhythmogenic right ventricular cardiomyopathy (ARVC) in humans. However, the relation between PKP2 expression and the transcriptome of the human heart remains poorly explored. Read More

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November 2018
1 Read

The complex molecular genetics of arrhythmogenic cardiomyopathy.

Int J Cardiol 2018 Nov 6. Epub 2018 Nov 6.

University of Padua, Department of Cardiac-Thoracic-Vascular Sciences and Public Health, Padua, Italy. Electronic address:

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November 2018

Moderate intensity continuous training reverses the detrimental effects of ovariectomy on RyR1 phosphorylation in rat skeletal muscle.

Mol Cell Endocrinol 2018 Nov 19. Epub 2018 Nov 19.

Department of Internal Medicine, Division of Sports Medicine, Ulm University, Ulm, Germany.

High 17β-Estradiol (E2) concentrations in isolated ventricular myocytes as well as a lack of ovarian hormones in cardiac muscle of ovariectomized (OVX) rodents has been shown to lead to arrhythmogenic effects by inducing post-translational modifications, including phosphorylation of the sarcoplasmic reticulum (SR) Ca release channel ryanodine receptor-2 (RyR2). The effects of estrogens on the phosphorylation status of the RyR1 in skeletal muscle have not been investigated before. Furthermore, while high intensity exercise has been shown to increase RyR phosphorylation, there is no data on the effects of moderate intensity continuous training (MICT). Read More

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November 2018
2 Reads

A founder homozygous DSG2 variant in East Asia results in ARVC with full penetrance and heart failure phenotype.

Int J Cardiol 2019 Jan 28;274:263-270. Epub 2018 Jun 28.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing 100037, PR China. Electronic address:

Background: Variants in the desmoglein-2 (DSG2) gene account for a significant proportion of patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). The aim of this study was to evaluate the genetic epidemiology of DSG2 and the impact of a frequent homozygous DSG2 variant in East Asia.

Methods: Genetic screening of 14 ARVC related genes was performed in 118 unrelated index patients using next-generation sequencing. Read More

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January 2019
2 Reads

A targeted next-generation gene panel reveals a novel heterozygous nonsense variant in TP63 gene in Arrhythmogenic Cardiomyopathy patients.

Heart Rhythm 2018 Nov 16. Epub 2018 Nov 16.

Department of Biology, University of Padua, Padua, Italy.

Background: Arrhythmogenic Cardiomyopathy (ACM) is associated with arrhythmias and risk of sudden death. Mutations in genes encoding proteins of cardiac intercalated discs account for about 60% of ACM cases, but the remaining 40% is still genetically elusive.

Objective: The purpose of this study was to identify the underlying genetic cause in ACM probands. Read More

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November 2018
2 Reads

Arrhythmogenic Cardiomyopathy in 2018: ARVC/ALVC or Both?

Heart Lung Circ 2018 Oct 24. Epub 2018 Oct 24.

Department of Cardiology, Westmead Applied Research Centre, Westmead Hospital, University of Sydney, Sydney, NSW, Australia. Electronic address:

Arrhythmogenic cardiomyopathy (ACM) is now commonly used to describe any form of non-hypertrophic, progressive cardiomyopathy characterised by fibrofatty infiltration of the ventricular myocardium. Right ventricular (RV) involvement refers to the classical arrhythmogenic right ventricular cardiomyopathy, but left ventricular, or bi-ventricular involvement are now recognised. ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Read More

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October 2018
14 Reads

Assessment of premature ventricular beats in athletes.

Tunis Med 2018 Apr;96(4):155-159

Introduction: Premature ventricular complexes (PVC) are generally considered as a benign electrocardiographic abnormality in the athleticpopulation. However it may be indicative of underlying heart disease which may increase the risk of sudden death. This implies the need forcardiological evaluation before indicating the ability to practice competitive sports. Read More

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April 2018
4 Reads

Pro-Arrhythmogenic Effects of Heterogeneous Tissue Curvature - A Suggestion for Role of Left Atrial Appendage in Atrial Fibrillation.

Circ J 2018 Nov 13. Epub 2018 Nov 13.

Yonsei University Health System.

Background: The arrhythmogenic role of complex atrial morphology has not yet been clearly elucidated. We hypothesized that bumpy tissue geometry can induce action potential duration (APD) dispersion and wavebreak in atrial fibrillation (AF). Methods and Results: We simulated a 2D-bumpy atrial model by varying the degree of bumpiness, and 3D-left atrial (LA) models integrated by LA computed tomographic (CT) images taken from 14 patients with persistent AF. Read More

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November 2018
2 Reads

The coronary sinus: Novel arrhythmogenic aspects.

Authors:
Michael Rubart

J Cardiovasc Electrophysiol 2018 Nov 14. Epub 2018 Nov 14.

Department of Pediatrics, Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, Indiana.

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November 2018

Noninvasive cardiac arrhythmia ablation with particle beams.

Med Phys 2018 Nov;45(11):e1024-e1035

Department of Radiation Oncology, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität, 91054, Erlangen-Nürnberg, Germany.

Cardiac arrhythmias are a major health burden, associated with reduced quality of life and substantial morbidity and mortality. Current therapy includes moderately effective medication and catheter-based ablation of arrhythmogenic substrates in the heart. Catheter interventions frequently have to be repeated due to recurrent arrhythmia, can have rare but severe side-effects and are less suited especially for potentially lethal left ventricular tachycardia. Read More

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November 2018
7 Reads

Altered Ca and Na Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis.

Front Physiol 2018 16;9:1391. Epub 2018 Oct 16.

Department of Neuroscience, Psychology, Drug Sciences and Child Health (NEUROFARBA), University of Florence, Florence, Italy.

Hypertrophic cardiomyopathy (HCM) is the most common mendelian heart disease, with a prevalence of 1/500. HCM is a primary cause of sudden death, due to an heightened risk of ventricular tachyarrhythmias that often occur in young asymptomatic patients. HCM can slowly progress toward heart failure, either with preserved or reduced ejection fraction, due to worsening of diastolic function. Read More

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October 2018

Arrhythmogenic Inflammatory Cardiomyopathy: A Review.

Arrhythm Electrophysiol Rev 2018 Aug;7(3):181-186

UCLA Cardiac Arrhythmia Center, David Geffen School of Medicine at UCLA Los Angeles, USA.

Arrhythmogenic inflammatory cardiomyopathy is a recent clinical description of a subgroup of patients with non-ischaemic cardiomyopathy who are referred to electrophysiologists for evaluation and management of ventricular arrhythmias and are found to have evidence of active cardiac inflammation. The identification of these patients is key, since the aetiology of their arrhythmic burden is likely both related to scar-mediated and direct inflammatory mechanisms, which may have different treatment approaches. Evaluation of these patients starts with a full clinical history and physical examination along with echocardiography, as with most patients with cardiomyopathy, however, additional imaging with fluorodeoxyglucose PET-CT and cardiac MRI is crucial. Read More

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August 2018
3 Reads

Post-Translational Modifications and Diastolic Calcium Leak Associated to the Novel RyR2-D3638A Mutation Lead to CPVT in Patient-Specific hiPSC-Derived Cardiomyocytes.

J Clin Med 2018 Nov 8;7(11). Epub 2018 Nov 8.

PhyMedExp, INSERM, University of Montpellier, CNRS, 371 Avenue du Doyen G. Giraud, 34295 Montpellier CEDEX 5, France.

Background: Sarcoplasmic reticulum Ca leak and post-translational modifications under stress have been implicated in catecholaminergic polymorphic ventricular tachycardia (CPVT), a highly lethal inherited arrhythmogenic disorder. Human induced pluripotent stem cells (hiPSCs) offer a unique opportunity for disease modeling.

Objective: The aims were to obtain functional hiPSC-derived cardiomyocytes from a CPVT patient harboring a novel ryanodine receptor (RyR2) mutation and model the syndrome, drug responses and investigate the molecular mechanisms associated to the CPVT syndrome. Read More

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November 2018
9 Reads

Can abnormal dispersion of ventricular repolarization be a predictor of mortality in arrhythmogenic right ventricular cardiomyopathy: The importance of Tp-e interval.

Ann Noninvasive Electrocardiol 2018 Nov 9:e12619. Epub 2018 Nov 9.

Department of Cardiology, Dr. Suat Gunsel University of Kyrenia Hospital, Kyrenia, Mersin, Turkey.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and specific ventricular pathology. Repolarization abnormalities, the significant contributor to life-threatening arrhythmias and mortality, are frequently observed ECG changes in patients with ARVC. This study aimed to evaluate the changes in Tp-e interval, Tp-e/QT, Tp-e/QTc ratio, and traditional electrocardiographic features of electrical dispersion in patients with ARVC. Read More

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November 2018
2 Reads

Action potential shortening rescues atrial calcium alternans.

J Physiol 2018 Nov 9. Epub 2018 Nov 9.

Department of Physiology & Biophysics, Rush University Medical Center, Chicago, IL, 60612, USA.

Key Points: Cardiac alternans refers to a beat-to-beat alternation in contraction, action potential (AP) morphology and Ca transient (CaT) amplitude, and represents a risk factor for cardiac arrhythmia, including atrial fibrillation. We developed strategies to pharmacologically manipulate the AP waveform with the goal to reduce or eliminate the occurrence of CaT and contraction alternans in atrial tissue. With combined patch-clamp and intracellular Ca measurements we investigated the effect of specific ion channel inhibitors and activators on alternans. Read More

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November 2018
4 Reads

Sex-related differences in cardiomyopathies.

Int J Cardiol 2018 Oct 30. Epub 2018 Oct 30.

Institute of Cardiovascular Science, University College London and St. Bartholomew's Hospital, London, United Kingdom.

Cardiomyopathies (CMPs) are a heterogeneous group of heart muscle diseases with several different phenotypes defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular heart disease and congenital heart disease sufficient to explain the observed myocardial abnormality. CMPs can be classified into one of the following, i.e. Read More

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October 2018
5 Reads

Prevalence of F-fluorodeoxyglucose positron emission tomography abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy.

Int J Cardiol 2018 Oct 26. Epub 2018 Oct 26.

Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK; NIHR University College London Hospitals Biomedical Research Centre, UK.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease that causes sudden cardiac death in the young. Inflammatory myocardial infiltrates have been described at autopsy and on biopsy, but there are few data on the presence of myocarditis in living patients with ARVC using non-invasive imaging techniques. FDG-PET is a validated technique for detecting myocardial inflammation in clinically suspected myocarditis. Read More

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October 2018
1 Read

Arrhythmogenic Substrates for Atrial Fibrillation in Obesity.

Front Physiol 2018 22;9:1482. Epub 2018 Oct 22.

The Dorothy M. Davis Heart & Lung Research Institute, The Ohio State University Wexner Medical Center, Columbus, OH, United States.

Global obesity rates have nearly tripled since 1975. This obesity rate increase is mirrored by increases in atrial fibrillation (AF) that now impacts nearly 10% of Americans over the age of 65. Numerous epidemiologic studies have linked incidence of AF and obesity and other obesity-related diseases, including hypertension and diabetes. Read More

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October 2018
2 Reads

Age as a Critical Determinant of Atrial Fibrillation: A Two-sided Relationship.

Can J Cardiol 2018 Nov 8;34(11):1396-1406. Epub 2018 Aug 8.

Montreal Heart Institute, Université de Montréal, Montreal, Québec, Canada; Department of Pharmacology and Therapeutics, McGill University, Montreal, Québec, Canada; Institute of Pharmacology, University Duisburg-Essen, Essen, Germany; LIRYC Center, Bordeaux, France. Electronic address:

The incidence of atrial fibrillation (AF), the most common sustained arrhythmia and a major public health burden, increases exponentially with age. However, mechanisms underlying this long-recognized association remain incompletely understood. Experimental and human studies have demonstrated the involvement of aging in several arrhythmogenic processes, including atrial electrical and structural remodelling, disturbed calcium homeostasis, and enhanced atrial ectopic activity/increased vulnerability to re-entry induction. Read More

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November 2018
3 Reads

Cardiovascular magnetic resonance imaging pattern in patients with autoimmune rheumatic diseases and ventricular tachycardia with preserved ejection fraction.

Int J Cardiol 2018 Oct 25. Epub 2018 Oct 25.

Arthritis Research UK Epidemiology Unit, University of Manchester, Manchester, UK. Electronic address:

Background: Ventricular tachycardia/fibrillation (VT/VF) may occur in autoimmune rheumatic diseases (ARDs). We hypothesized that cardiovascular magnetic resonance (CMR) can identify arrhythmogenic substrates in ARD patients.

Patients - Methods: Using a 1. Read More

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October 2018
5 Reads

The arrhythmogenic cardiotoxicity of the quinoline and structurally related antimalarial drugs: a systematic review.

BMC Med 2018 Nov 7;16(1):200. Epub 2018 Nov 7.

Centre for Tropical Medicine and Global Health, Nuffield Department of Medicine, University of Oxford, Oxford, UK.

Background: Several quinoline and structurally related antimalarial drugs are associated with cardiovascular side effects, particularly hypotension and electrocardiographic QT interval prolongation. A prolonged QT interval is a sensitive but not specific risk marker for the development of Torsade de Pointes-a potentially lethal polymorphic ventricular tachyarrhythmia. The increasing use of quinoline and structurally related antimalarials in mass treatments to eliminate malaria rapidly highlights the need to review their cardiovascular safety profiles. Read More

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November 2018
5 Reads

Right atrial pathology in arrhythmogenic right ventricular dysplasia.

Cardiol J 2018 Nov 5. Epub 2018 Nov 5.

Pitié-Salpêtrière Hospital.

Background: Atrial fibrillation (AF) is the most common atrial arrhythmia in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD). Considering the histologic changes known in the right ventricular (RV) in ARVD, the aim of the present study was to examine right atrial (RA) pathology in patients with ARVD.

Methods: Histology of RA and RV was assessed from autopsy material in 3 patients with ARVD without persistent atrial arrhythmia. Read More

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November 2018
1 Read

Brugada Syndrome: anaesthetic considerations and management algorithm.

Minerva Anestesiol 2018 Oct 30. Epub 2018 Oct 30.

Pediatric Arrhythmia Unit, Department of Cardiology, Sant Joan de Déu Hospital, Barcelona, Spain.

Brugada syndrome is characterized by arrhythmogenic risk that may be exacerbated by different metabolic and pharmacological factors. Since its first description, knowledge of this syndrome and its detection by physicians belonging to different specialties have gradually increased. The risk of arrhythmias is well known to increase in the postoperative period, and this risk is particularly accentuated in patients with Brugada syndrome. Read More

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October 2018
6 Reads
2.130 Impact Factor

PKP2 and DSG2 genetic variations in Latvian arrhythmogenic right ventricular dysplasia/cardiomyopathy registry patients.

Anatol J Cardiol 2018 Nov;20(5):296-302

Scientific Laboratory of Molecular Genetics, Riga Stradins University; Riga-Latvia.

Objective: The Latvian arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD-C) registry was established to determine the genetic background of ARVD-C for analyzing discovered genetic variation frequencies in the European and Latvian populations.

Methods: In total, 38 patients with suspected ARVD-C were selected. The clinical parameters were defined according to the ARVD-C guidelines, PKP2 and DSG2 gene analysis was performed using the Sanger sequencing. Read More

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November 2018
4 Reads

Sarcomere variants in arrhythmogenic cardiomyopathy: Pathogenic factor or bystander?

Gene 2018 Oct 30;687:82-89. Epub 2018 Oct 30.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167A Beilishi Road, Xi Cheng District, Beijing 100037, China.. Electronic address:

Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease, which is mainly caused by desmosomal mutations. Sarcomere variants were the primary genetic basis of hypertrophic cardiomyopathy (HCM) and were recently detected in arrhythmogenic cardiomyopathy (ACM). Our aim is to seek potential pathogenic variants of sarcomere genes in our ACM cohort and describe their characteristics. Read More

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October 2018
1 Read

Loss of function desmoplakin I and II mutations underlie dominant arrhythmogenic cardiomyopathy with a hair and skin phenotype.

Br J Dermatol 2018 Nov 1. Epub 2018 Nov 1.

Blizard Institute, Queen Mary University of London, London, UK.

Aims: Arrhythmogenic Cardiomyopathy (AC) is an inherited, frequently under diagnosed disorder, predisposing to sudden cardiac death. Rare, recessive forms of AC can be associated with woolly hair and palmoplantar keratoderma, but most autosomal dominant AC forms have been reported as cardiac specific. Causative mutations frequently occur in desmosomal genes including desmoplakin (DSP) In this study, we have systematically investigated the presence of a skin and hair phenotype in heterozygous desmoplakin (DSP) mutation carriers with AC. Read More

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November 2018
1 Read

Cardiac magnetic resonance based deformation imaging: role of feature tracking in athletes with suspected arrhythmogenic right ventricular cardiomyopathy.

Int J Cardiovasc Imaging 2018 Oct 31. Epub 2018 Oct 31.

Heart and Vascular Center, Semmelweis University, Budapest, Hungary.

Both, arrhythmogenic right ventricular cardiomyopathy (ARVC) and regular training are associated with right ventricular (RV) remodelling. Cardiac magnetic resonance (CMR) is given an important role in the diagnosis of ARVC in current task force criteria (TFC), however, they contain no cut-off values for athletes. We aimed to confirm the added value of feature tracking and to provide new cut-off values to differentiate between ARVC and athlete's heart. Read More

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October 2018
1 Read

Inhibition of Advanced Glycation End Products Formation Attenuates Cardiac Electrical and Mechanical Remodeling and Vulnerability to Tachyarrhythmias in Diabetic Rats.

J Pharmacol Exp Ther 2018 Oct 31. Epub 2018 Oct 31.

College of Medicine, Chang Gung University.

Diabetic patients with cardiomyopathy show a higher incidence of arrhythmias and sudden death. Chronic hyperglycemia induces the formation of advanced glycation end products (AGEs), which contribute to the pathogenesis of diabetic cardiomyopathy. This study investigated whether inhibition of AGEs formation by aminoguanidine (AG) could prevent the cardiac electromechanical and arrhythmogenic remodeling in diabetes mellitus. Read More

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October 2018
2 Reads

Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Cardiol Young 2018 Oct 30:1-3. Epub 2018 Oct 30.

1Department of Pediatric Cardiology,Okinawa Nambu and Children's Medical Center,Okinawa,Japan.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery. Read More

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October 2018

Arrhythmogenic Mechanisms in Heart Failure: Linking β-Adrenergic Stimulation, Stretch, and Calcium.

Front Physiol 2018 16;9:1453. Epub 2018 Oct 16.

Department of Physiology, Cardiovascular Research Institute Maastricht, Maastricht University, Maastricht, Netherlands.

Heart failure (HF) is associated with elevated sympathetic tone and mechanical load. Both systems activate signaling transduction pathways that increase cardiac output, but eventually become part of the disease process itself leading to further worsening of cardiac function. These alterations can adversely contribute to electrical instability, at least in part due to the modulation of Ca handling at the level of the single cardiac myocyte. Read More

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October 2018

Thrombogenic and Arrhythmogenic Roles of the Left Atrial Appendage in Atrial Fibrillation.

Circulation 2018 Oct;138(18):2036-2050

Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY.

Understanding the anatomy, physiology, and arrhythmogenic and thrombogenic roles of the left atrial appendage (LAA) has become very important. The potential deleterious effects of this chamber in patients with atrial fibrillation have led to the development of specific treatments for this structure. It has been established that the LAA is the area where the vast majority of thrombi in nonvalvular atrial fibrillation are formed and that some LAA morphologies may actually facilitate thrombi formation and risk stratification for thromboembolic events in patients with low CHADS-VASc scores. Read More

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October 2018
4 Reads

A novel desmin mutation causing severe left ventricular arrhythmogenic cardiomyopathy/dysplasia.

J Thorac Dis 2018 Sep;10(Suppl 26):S3100-S3102

Department of Medicine and Therapeutics, Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, China.

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September 2018

Non-β-Blocking Carvedilol Analog, VK-II-86, Prevents Ouabain-Induced Cardiotoxicity.

Circ J 2018 Oct 23. Epub 2018 Oct 23.

Centro de Investigaciones Cardiovasculares, CONICET La Plata, Facultad de Ciencias Médicas, Universidad Nacional de La Plata.

Background: It has been shown that carvedilol and its non β-blocking analog, VK-II-86, inhibit spontaneous Ca release from the sarcoplasmic reticulum (SR). The aim of this study is to determine whether carvedilol and VK-II-86 suppress ouabain-induced arrhythmogenic Ca waves and apoptosis in cardiac myocytes. Methods and Results: Rat cardiac myocytes were exposed to toxic doses of ouabain (50 µmol/L). Read More

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October 2018
6 Reads

Activin Receptor-Like Kinase 4 Haplodeficiency Mitigates Arrhythmogenic Atrial Remodeling and Vulnerability to Atrial Fibrillation in Cardiac Pathological Hypertrophy.

J Am Heart Assoc 2018 Aug;7(16):e008842

1 Department of Cardiology Xinhua Hospital School of Medicine Shanghai Jiao Tong University Shanghai China.

Background Activin receptor-like kinase 4 ( ALK 4) is highly expressed in mammal heart. Atrial fibrillation ( AF ) is closely related to ventricular pressure overload. Because pressure overload increases atrial pressure and leads to atrial remodeling, it would be informative to know whether ALK 4 exerts potential effects on atrial remodeling and AF vulnerability in a pressure-overload model. Read More

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August 2018
2.880 Impact Factor

Ablation compared with drug therapy for recurrent ventricular tachycardia in arrhythmogenic right ventricular cardiomyopathy: Results from a multicenter study.

Heart Rhythm 2018 Oct 24. Epub 2018 Oct 24.

Arrhythmia Unit, Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:

Background: The comparative efficacy of antiarrhythmic drug (AAD) therapy vs ventricular tachycardia (VT) ablation in arrhythmogenic right ventricular cardiomyopathy (ARVC) is unknown.

Objective: We compared outcomes of AAD and/or β-blocker (BB) therapy with those of VT ablation (with AAD/BB) in patients with ARVC who had recurrent VT.

Methods: In a multicenter retrospective study, 110 patients with ARVC (mean age 38 ± 17 years; 83% men) with a minimum of 3 VT episodes were included; 77 (70%) were initially treated with AAD/BB and 32 (29%) underwent ablation. Read More

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October 2018
8 Reads

A large familial pathogenic Plakophilin-2 gene () deletion manifesting with sudden cardiac death and lone atrial fibrillation: Evidence for alternating atrial and ventricular phenotypes.

HeartRhythm Case Rep 2018 Oct 25;4(10):486-489. Epub 2018 Jul 25.

Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, London, Canada.

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October 2018
1 Read

Arrhythmogenic Right Ventricular Cardiomyopathy: Progress Toward Personalized Management.

Annu Rev Med 2018 Oct 24. Epub 2018 Oct 24.

Division of Cardiology, Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA; email:

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease characterized by fibrofatty replacement of the ventricular myocardium, a high risk of ventricular arrhythmias, and progressive ventricular dysfunction. The clinical course is highly variable, and optimal approaches to management remain undefined. ARVC is associated with pathogenic variants in genes encoding the cardiac desmosome. Read More

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October 2018
1 Read

Form Follows Function?

Circ Cardiovasc Imaging 2018 Sep;11(9):e008271

The Labatt Family Heart Centre, Department of Paediatrics (M.d.C.V.A., L.G.-W.), The Hospital for Sick Children, University of Toronto, Ontario, Canada.

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September 2018
1 Read

Regional Strain by Cardiac Magnetic Resonance Imaging Improves Detection of Right Ventricular Scar Compared With Late Gadolinium Enhancement on a Multimodality Scar Evaluation in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.

Circ Cardiovasc Imaging 2018 Sep;11(9):e007546

Precision Medicine Center of Excellence for ARVC and Complex Ventricular Arrhythmias, Johns Hopkins University School of Medicine, Baltimore, MD (T.Z., F.R.A., J.C., A.K., S.M., R.B., H.C., H.T.).

Background: Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy characterized by fibrofatty replacement of right ventricular myocardium resulting in reentrant ventricular tachycardia (VT). Cardiac magnetic resonance imaging (CMR) can noninvasively measure regional abnormalities using tissue-tracking strain as well as late gadolinium enhancement (LGE). In this study, we examine arrhythmogenic substrate using regional CMR strain, LGE, and electroanatomic mapping (EAM) in arrhythmogenic right ventricular cardiomyopathy patients presenting for VT ablation. Read More

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September 2018
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5.320 Impact Factor

Atrial Dysfunction in Arrhythmogenic Right Ventricular Cardiomyopathy.

Circ Cardiovasc Imaging 2018 Sep;11(9):e007344

Department of Radiology, University Medical Center Utrecht, The Netherlands (B.K.V.).

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that is predominantly known to affect the ventricles. Evidence for atrial involvement remains limited. Therefore, we aimed to characterize atrial involvement in ARVC using functional cardiac magnetic resonance, define the extent of atrial size and function variation attributable to ventricular variables, and identify cardiac magnetic resonance-based predictors of atrial arrhythmias (AA) in ARVC. Read More

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September 2018
5 Reads