3,241 results match your criteria Arrhythmogenic Right Ventricular Cardiomyopathy


How ARVC-Related Mutations Destabilize Desmoplakin: An MD Study.

Biophys J 2019 Jan 30. Epub 2019 Jan 30.

Interdisciplinary Center for Scientific Computing, Heidelberg University, Mathematikon, Heidelberg, Germany; Heidelberg Institute for Theoretical Studies, Heidelberg, Germany. Electronic address:

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a familial heart disease linked to mutations in several desmosomal proteins, but the specific effects of these mutations on the molecular level are poorly understood. Among the many documented ARVC-related genetic variants, a striking hotspot of nine mutations has been identified in the plakin domain of desmoplakin. This hotspot can be found at the meeting point of three different subdomains of desmoplakin: two spectrin repeats and a Src homology 3 domain. Read More

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http://dx.doi.org/10.1016/j.bpj.2019.01.023DOI Listing
January 2019

Atrial dysplasia in the atria of humans without cardiovascular disease.

J Investig Med 2019 Feb 14. Epub 2019 Feb 14.

University Heart Center Zürich, Zürich, Switzerland.

Research on atrial histology of humans without cardiovascular disease is scarce. Therefore, our aim was to study human atrial histology in subjects without cardiovascular disease. Histology of the right atrium, left atrium or atrial septum was studied in eight patients (one newborn infant and seven adults) who died of a non-cardiac cause and who were not known to suffer from any cardiovascular pathology. Read More

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http://jim.bmj.com/lookup/doi/10.1136/jim-2018-000916
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http://dx.doi.org/10.1136/jim-2018-000916DOI Listing
February 2019
1 Read

Heart failure in patients with arrhythmogenic right ventricular cardiomyopathy: Genetic characteristics.

Int J Cardiol 2019 Jan 27. Epub 2019 Jan 27.

Cardiomyopathy Service, Royal Brompton Hospital, London, United Kingdom.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disorder. The incidence of heart failure (HF) in ARVC has been reported at 5-13%. We aimed to define the genotype and disease progression of ARVC patients with HF. Read More

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http://dx.doi.org/10.1016/j.ijcard.2019.01.065DOI Listing
January 2019

Distinct molecular signature of phospholamban p.Arg14del arrhythmogenic cardiomyopathy.

Cardiovasc Pathol 2018 Dec 21;40:2-6. Epub 2018 Dec 21.

University of Groningen, University Medical Center Groningen, Department of Pathology, Groningen, The Netherlands.

Phospholamban (PLN) p.Arg14del cardiomyopathy is characterized by a distinct arrhythmogenic biventricular phenotype that can be predominantly left ventricular, right ventricular, or both. Our aim was to further elucidate distinct features of this cardiomyopathy with respect to the distribution of desmosomal proteins observed by immunofluorescence (IF) in comparison to desmosomal arrhythmogenic cardiomyopathy and co-existent genetic variants. Read More

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http://dx.doi.org/10.1016/j.carpath.2018.12.006DOI Listing
December 2018

Prevalence of pathogenic and likely pathogenic variants in the RASopathy genes in patients who have had panel testing for cardiomyopathy.

Am J Med Genet A 2019 Feb 14. Epub 2019 Feb 14.

Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

RASopathies are a group of developmental disorders caused by pathogenic variants in the RAS-MAPK pathway. Cardiomyopathy is a major feature of this group of disorders, specifically hypertrophic cardiomyopathy (HCM). HCM can be the first presenting feature in individuals with RASopathies. Read More

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http://dx.doi.org/10.1002/ajmg.a.61072DOI Listing
February 2019

The sympathetic nervous system and arrhythmogenic right ventricular cardiomyopathy: Further evidence of a strong tie.

Heart Rhythm 2019 Feb 5. Epub 2019 Feb 5.

Department of Molecular Medicine, Section of Cardiology, University of Pavia, Pavia, Italy; Cardiac Intensive Care Unit, Arrhythmia and Electrophysiology and Experimental Cardiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.hrthm.2019.02.004DOI Listing
February 2019
1 Read

Recurrent ventricular tachycardia after catheter ablation in arrhythmogenic right ventricular cardiomyopathy: Scar progression or ineffective ablation?

J Cardiovasc Electrophysiol 2019 Feb 4. Epub 2019 Feb 4.

Division of Cardiovascular Medicine, Department of Medicine, University of Arizona College of Medicine, Tucson, Arizona.

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http://dx.doi.org/10.1111/jce.13861DOI Listing
February 2019
1 Read

Sudden Death and Left Ventricular Involvement in Arrhythmogenic Cardiomyopathy.

Circulation 2019 Jan 31. Epub 2019 Jan 31.

Cardiovascular pathology, Cardiovascular Pathology Unit, St Georges Hospital Medical School, London, UK, United Kingdom.

Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disorder characterized by myocardial fibro-fatty replacement and an increased risk of sudden cardiac death (SCD). Originally described as a right ventricular (RV) disease, ACM is increasingly recognized as a biventricular entity. We evaluated pathological, genetic, and clinical associations in a large SCD cohort. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.037230DOI Listing
January 2019
1 Read

Characteristics of recurrent ventricular tachyarrhythmia after catheter ablation in patients with arrhythmogenic right ventricular cardiomyopathy.

J Cardiovasc Electrophysiol 2019 Jan 30. Epub 2019 Jan 30.

Heart Rhythm Center, Division of Cardiology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.

Background: The reason for recurrence of ventricular arrhythmia (VA) after catheter ablation in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not clear.

Methods: In this study, 91 ARVC patients (age, 47 ± 13 years; 47 men) who underwent catheter ablation for drug-refractory ventricular arrhythmia (VA) were enrolled. The patients were categorized into single or multiple procedures (n = 28). Read More

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http://doi.wiley.com/10.1111/jce.13853
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http://dx.doi.org/10.1111/jce.13853DOI Listing
January 2019
3 Reads
2.958 Impact Factor

Left axillary active can positioning markedly reduces defibrillation threshold of a transvenous defibrillator failing to defibrillate at maximum output.

Authors:
Raman L Mitra

HeartRhythm Case Rep 2019 Jan 23;5(1):36-39. Epub 2018 Oct 23.

Memorial Advanced Cardiovascular Institute, Beacon Health System, South Bend, Indiana.

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https://linkinghub.elsevier.com/retrieve/pii/S22140271183033
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http://dx.doi.org/10.1016/j.hrcr.2018.10.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342727PMC
January 2019
3 Reads

Ioxynil and diethylstilbestrol disrupt vascular and heart development in zebrafish.

Environ Int 2019 Mar 25;124:511-520. Epub 2019 Jan 25.

Centre of Marine Sciences, University of Algarve, Faro, Portugal. Electronic address:

Background: Endocrine disruption is one of the consequences of industrialization and chemicals released into the environment have a profound impact on organisms. Waterborne micromolar concentrations of ioxynil (IOX) and diethylstilbestrol (DES) in fish affect the development of the heart, vasculature and thyroid gland.

Objectives: The present study aimed to determine how IOX and DES disrupt the crosstalk between the developing thyroid gland and cardio-vascular system in zebrafish. Read More

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http://dx.doi.org/10.1016/j.envint.2019.01.009DOI Listing
March 2019
2 Reads

Arrhythmogenic right ventricular cardiomyopathy (ARVC) mimics: the knot unravelled by cardiovascular MRI.

Clin Radiol 2019 Mar 23;74(3):228-234. Epub 2019 Jan 23.

Bristol Heart Institute, Bristol NIHR Cardiovascular Biomedical Research Unit (BRU), University of Bristol, UK. Electronic address:

Aim: To assess the role of cardiovascular magnetic resonance imaging (CMRI) in patients referred for suspected arrhythmogenic right ventricular cardiomyopathy (ARVC), its ability to identify ARVC mimics, and subsequent clinical impact.

Materials And Methods: The CMRI registry of the year 2014 was analysed to identify all consecutive patients referred for suspected ARVC. A comprehensive CMRI protocol that included anatomy, bi-ventricular function modules, and late gadolinium enhancement (LGE) was performed in all patients. Read More

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http://dx.doi.org/10.1016/j.crad.2018.12.002DOI Listing
March 2019
4 Reads

Assessment of premature ventricular beats in athletes.

Ann Cardiol Angeiol (Paris) 2019 Jan 22. Epub 2019 Jan 22.

Centre de médecine et des sciences de sport, Tunis, Tunisia.

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

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http://dx.doi.org/10.1016/j.ancard.2018.10.013DOI Listing
January 2019
3 Reads

Sudden cardiac death in football players: Towards a new pre-participation algorithm.

Exp Ther Med 2019 Feb 30;17(2):1143-1148. Epub 2018 Nov 30.

Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, 11527 Athens, Greece.

Athletic pre-participation screening is essential for minimizing the risk for sudden cardiac death (SCD) in athletes participating in either competitive or leisure sporting activities. The primary causes of SCD in young athletes (<35 years of age) include hypertrophic cardiomyopathy, congenital anomalies of the coronary artery and arrhythmogenic right ventricular cardiomyopathy. Other abnormalities, such as malignant arrhythmia due to blunt trauma to the chest (commotio cordis), myocarditis, valvular disease, aortic rupture (in Marfan syndrome) and ion channelopathies (catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, long or short QT syndrome), also contribute to a lesser degree to SCD. Read More

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http://www.spandidos-publications.com/10.3892/etm.2018.7041
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http://dx.doi.org/10.3892/etm.2018.7041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327574PMC
February 2019
11 Reads

Primary Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillator Therapy in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.

Am J Cardiol 2019 Jan 8. Epub 2019 Jan 8.

Department of Cardiology, The Heart Center, Rigshospitalet, University of Copenhagen, Denmark & Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.

Implantable cardioverter-defibrillator (ICD) therapy remains a corner stone of sudden cardiac death (SCD) prevention in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We aimed to assess predictors of appropriate ICD therapies in the Scandinavian cohort of ARVC patients who received ICD for primary prevention of SCD. Study group comprised of 79 definite ARVC patients by 2010 Task Force criteria (60% male, age at ICD implant 39 ± 14 years) who were enrolled in the Nordic ARVC Registry and received an ICD for primary SCD prevention. Read More

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http://dx.doi.org/10.1016/j.amjcard.2018.12.049DOI Listing
January 2019
8 Reads

Outcomes of Catheter Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy Without Background Implantable Cardioverter Defibrillator Therapy: A Multicenter International Ventricular Tachycardia Registry.

JACC Clin Electrophysiol 2019 Jan 28;5(1):55-65. Epub 2018 Nov 28.

Texas Cardiac Arrhythmia Institute, St. David's Medical Center, Austin, Texas. Electronic address:

Objectives: This study sought to determine the long-term outcomes of catheter ablation (CA) of ventricular tachycardia (VT) in a series of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) without background implantable cardioverter-defibrillator (ICD) therapy.

Background: Endo-epicardial CA of VT has been demonstrated to be highly effective in reducing recurrent VT in patients with ARVC.

Methods: Thirty-two patients (age 45 ± 13 years, 72% male) with ARVC and VT underwent CA in the absence of ICD therapy. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.09.019DOI Listing
January 2019
1 Read

To Reach or Not to Reach the Whole Arrhythmic Substrate?: A Matter of Accessibility.

JACC Clin Electrophysiol 2019 Jan;5(1):25-27

Arrhythmia Unit, Hospital Universitario Virgen del Rocío, Sevilla, Spain.

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http://dx.doi.org/10.1016/j.jacep.2018.09.003DOI Listing
January 2019
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Combined Endocardial-Epicardial Versus Endocardial Catheter Ablation Alone for Ventricular Tachycardia in Structural Heart Disease: A Systematic Review and Meta-Analysis.

JACC Clin Electrophysiol 2019 Jan 26;5(1):13-24. Epub 2018 Sep 26.

Department of Cardiology, Westmead Applied Research Centre, University of Sydney, Sydney, New South Wales, Australia. Electronic address:

Objectives: This study sought to determine whether combined endocardial-epicardial (endo-epi) ablation was superior to endocardial only ablation in patients with scar-related ventricular tachycardia (VT).

Background: Limited single-center studies suggest that combined endo-epi ablation strategy may be superior to endocardial ablation (endo) alone in patients with nonischemic cardiomyopathy (NICM) and arrhythmogenic right ventricular cardiomyopathy (ARVC), and ischemic cardiomyopathy (ICM).

Methods: A systematic review of Medline, Cochrane, and Embase databases was performed for studies that reported outcomes comparing endo-epi with endo VT ablation alone. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.08.010DOI Listing
January 2019
2 Reads

Plakophilin-2 Truncation Variants in Patients Clinically Diagnosed With Catecholaminergic Polymorphic Ventricular Tachycardia and Decedents With Exercise-Associated Autopsy Negative Sudden Unexplained Death in the Young.

JACC Clin Electrophysiol 2019 Jan 1;5(1):120-127. Epub 2018 Nov 1.

Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota; Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Objectives: This study determined if radical plakophilin-2 (PKP2) variants might underlie some cases of clinically diagnosed catecholaminergic polymorphic ventricular tachycardia (CPVT) and exercise-associated, autopsy-negative sudden unexplained death in the young (SUDY).

Background: Pathogenic variants in PKP2 cause arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a cardiomyocyte-specific PKP2 knockout mouse model revealed that loss of PKP2 markedly reduced expression of genes critical in intracellular calcium handling. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.09.010DOI Listing
January 2019

Cardiac Sympathectomy For Refractory Ventricular Tachycardia in Arrhythmogenic Right Ventricular Cardiomyopathy.

Heart Rhythm 2019 Jan 21. Epub 2019 Jan 21.

Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University School of Medicine.

Background: The sympathetic nervous system plays an important role in arrhythmogenesis in arrhythmogenic right ventricular cardiomyopathy (ARVC). Sudden cardiac death commonly occurs during exertion and beta-blockers are associated with reduction in arrhythmia burden. Bilateral cardiac sympathetic denervation (BCSD) has been shown to reduce implantable cardioverter-defibrillator (ICD) shocks in patients with structural heart disease and refractory ventricular tachycardia (VT), however data in ARVC are sparse. Read More

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http://dx.doi.org/10.1016/j.hrthm.2019.01.019DOI Listing
January 2019
2 Reads

Fifty Years of Ventricular Tachycardia in a Single Patient.

Ulster Med J 2019 Jan 22;88(1):15-16. Epub 2019 Jan 22.

Cardiology Department, Belfast HSC Trust.

We report a patient who first presented during childhood in the early 1960's with several episodes of ventricular tachycardia (VT) and we describe her management which reflected the best medical knowledge at the time. She then presented more than 50 years later, again with VT, at which time a definitive diagnosis of the underlying cause was made. Her case illustrates the evolution in the understanding and management of VT over the past 50 years. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342044PMC
January 2019
2 Reads

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) - What We Have Learned after 40 Years of the Diagnosis of This Clinical Entity.

Arq Bras Cardiol 2019 Jan;112(1):91-103

'Unité de Rythmologie de l'Institut de Cardiologie de l'Hôpital Pitié-Salpêtrière, Paris - France.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) was initially recognized as a clinical entity by Fontaine and Marcus, who evaluated a group of patients with ventricular tachyarrhythmia from a structurally impaired right ventricle (RV). Since then, there have been significant advances in the understanding of the pathophysiology, manifestation and clinical progression, and prognosis of the pathology. The identification of genetic mutations impairing cardiac desmosomes led to the inclusion of this entity in the classification of cardiomyopathies. Read More

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http://dx.doi.org/10.5935/abc.20180266DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317628PMC
January 2019
1 Read

Sudden arrhythmia death syndrome in young victims: a five-year retrospective review and two-year prospective molecular autopsy study by next-generation sequencing and clinical evaluation of their first-degree relatives.

Hong Kong Med J 2019 Feb 23;25(1):21-9. Epub 2019 Jan 23.

Department of Pathology, Princess Margaret Hospital, Kwai Chung, Hong Kong.

Objective: Sudden arrhythmia death syndrome (SADS) accounts for about 30% of causes of sudden cardiac death (SCD) in young people. In Hong Kong, there are scarce data on SADS and a lack of experience in molecular autopsy. We aimed to investigate the value of molecular autopsy techniques for detecting SADS in an East Asian population. Read More

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https://www.hkmj.org/earlyrelease/hkmj187256.html
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http://dx.doi.org/10.12809/hkmj187256DOI Listing
February 2019
4 Reads

Bioinformatics analysis of sex differences in arrhythmogenic right ventricular cardiomyopathy.

Mol Med Rep 2019 Mar 17;19(3):2238-2244. Epub 2019 Jan 17.

Department of Cardiology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310000, P.R. China.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease that exhibits sex differences on clinical presentation. The present study aimed to investigate the sex differences associated with ARVC by conducting an integrated bioinformatics analysis. The GSE29819 gene expression dataset was downloaded from the Gene Expression Omnibus database. Read More

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http://dx.doi.org/10.3892/mmr.2019.9873DOI Listing
March 2019
3 Reads

Subcutaneous implantable cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy: Results from an Italian multicenter registry.

Int J Cardiol 2019 Apr 12;280:74-79. Epub 2019 Jan 12.

Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Italy. Electronic address:

Background: Despite expanding indication of the subcutaneous implantable cardioverter defibrillator (S-ICD) in clinical practice, limited data exists on safety and efficacy of S-ICD in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. The aim of this multicenter study was to evaluate the safety and efficacy of S-ICD in ARVC patients.

Methods: The study population included 44 consecutive patients with definite ARVC diagnosis according to the 2010 ITF criteria (57% male, mean age 37 ± 17 years [range 10-75 years]) who received an S-ICD. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01675273183619
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http://dx.doi.org/10.1016/j.ijcard.2019.01.041DOI Listing
April 2019
4 Reads

Desmoglein 2 mutation provokes skeletal muscle actin expression and accumulation at intercalated discs in murine hearts.

J Cell Sci 2019 Jan 18. Epub 2019 Jan 18.

Institute of Molecular and Cellular Anatomy, RWTH Aachen University, 52074 Aachen, Germany

Arrhythmogenic cardiomyopathy (AC) is an incurable progressive disease that is linked to mutations in genes coding for components of desmosomal adhesions that are localized to the intercalated disc region, which electromechanically couples adjacent cardiomyocytes. To date, the underlying molecular dysfunctions are not well characterized. In two murine AC models, we find an upregulation of the skeletal muscle actin gene () known to be a compensatory reaction to compromised heart function. Read More

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http://dx.doi.org/10.1242/jcs.199612DOI Listing
January 2019
2 Reads

Hemodynamic Changes in the Right Ventricle Induced by Variations of Cardiac Output: A Possible Mechanism for Arrhythmia Occurrence in the Outflow Tract.

Sci Rep 2019 Jan 14;9(1):100. Epub 2019 Jan 14.

University Heart Center, Department of Cardiology, Zurich, 8091, Switzerland.

The rationale of this paper is to investigate right ventricular (RV) hemodynamics in relation to changes in cardiac output, and in particular to study exercise-induced stresses at the RV outflow tract (RVOT), which is a common site of ventricular arrhythmias in the athlete's heart. We hypothesize that the thin-walled RVOT is exposed to high wall shear stresses (WSS) during physiological states associated with high cardiac output such as exercise, and therefore, may be particularly prone to substrate formation leading to ventricular tachyarrhythmias. 3D Particle Tracking Velocimetry (3D-PTV), an optical imaging method, has been performed in a novel anatomically accurate compliant silicone right heart model derived from a high resolution MRI heart scan of a healthy male proband. Read More

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http://dx.doi.org/10.1038/s41598-018-36614-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331649PMC
January 2019
3 Reads

[The 'Dangerous' ECG].

Authors:
Gian Flury

Praxis (Bern 1994) 2019 Jan;108(1):45-52

1 Innere Medizin und Kardiologie, Ospidal, Gesundheitszentrum Unterengadin, Scuol.

The 'Dangerous' ECG Abstract. This review aims to draw the attention of physicians confronted with cardiac emergencies to some specific ECG pathomorphologies in acute coronary syndrome and pulmonary embolism, as well as to malignant arrhythmias in hyperkalemia, drug-induced QTc prolongation, WPW, and arrhythmogenic right ventricular cardiomyopathy. If they are not detected the resultant failure to treat or incorrect treatment can have serious consequences for the patient and the doctor (liability consequences). Read More

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http://dx.doi.org/10.1024/1661-8157/a003155DOI Listing
January 2019
1 Read

Three-Dimensional Myocardial Scar Characterization from the Endocardium: Usefulness of Endocardial Unipolar Electroanatomic Mapping.

J Cardiovasc Electrophysiol 2019 Jan 4. Epub 2019 Jan 4.

Electrophysiology Section, Hospital of the University of Pennsylvania, Philadelphia, PA, U.S.A.

Epicardial ablation may be required to eliminate ventricular tachycardia (VT) in patients with underlying structural heart disease. The decision to gain epicardial access is frequently based on the suspicion of an epicardial origin for the VT and/or presence of an arrhythmogenic substrate. Epicardial pathology and VT is frequently present in patients with non-ischemic right and/or left cardiomyopathies even in the setting of modest or no endocardial bipolar voltage substrate. Read More

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http://dx.doi.org/10.1111/jce.13842DOI Listing
January 2019
1 Read
2.958 Impact Factor

Subcutaneous Implantable Cardioverter-Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Transatlantic Experience.

J Am Heart Assoc 2018 Nov;7(21):e008782

1 Division of Cardiology Department of Medicine Johns Hopkins Hospital Baltimore MD.

Background Despite growing use of the subcutaneous implantable cardioverter-defibrillator (S- ICD ), its clinical role in arrhythmogenic right ventricular cardiomyopathy/dysplasia ( ARVC /D) patients remains undefined. We aim to elucidate the cardiac phenotype, implant characteristics, and long-term efficacy regarding appropriate therapy and complications in ARVC /D patients with an S- ICD implant. Methods and Results A transatlantic cohort of ARVC /D patients who underwent S- ICD implantation was analyzed for clinical characteristics, S- ICD therapy, and long-term outcome including device-related complications. Read More

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http://dx.doi.org/10.1161/JAHA.118.008782DOI Listing
November 2018
9 Reads

Mechanisms and management of inappropriate therapy in subcutaneous implantable cardioverter defibrillators.

J Cardiovasc Electrophysiol 2018 Dec 21. Epub 2018 Dec 21.

Electrophysiology and Arrhythmia Service, Cardiology Division, Department of Medicine, Boston Medical Center, Boston, Massachusetts.

Subcutaneous implantable cardioverter defibrillators (S-ICDs) provide reliable defibrillation and have enhanced supraventricular tachycardia discrimination and fewer infection rates compared with traditional transvenous systems. However, inappropriate shocks remain a frequent problem. Herein, we review the various mechanisms of these inappropriate therapies, some of which are unique to S-ICDs, and propose an algorithm for preventing recurrences. Read More

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http://dx.doi.org/10.1111/jce.13831DOI Listing
December 2018
3 Reads

Utility of ripple mapping for identification of slow conduction channels during ventricular tachycardia ablation in the setting of arrhythmogenic right ventricular cardiomyopathy.

J Cardiovasc Electrophysiol 2018 Dec 21. Epub 2018 Dec 21.

Department of Medicine, Section of Cardiac Electrophysiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Ripple mapping displays every deflection of a bipolar electrogram and enables the visualization of conduction channels (RMCC) within postinfarction ventricular scar to guide ventricular tachycardia (VT) ablation. The utility of RMCC identification for facilitation of VT ablation in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVC) has not been described.

Objective: We sought to (a) identify the slow conduction channels in the endocardial/epicardial scar by ripple mapping and (b) retrospectively analyze whether the elimination of RMCC is associated with improved VT-free survival, in ARVC patients. Read More

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http://dx.doi.org/10.1111/jce.13819DOI Listing
December 2018
2 Reads

Identification of Crucial Genes and Pathways in Human Arrhythmogenic Right Ventricular Cardiomyopathy by Coexpression Analysis.

Front Physiol 2018 6;9:1778. Epub 2018 Dec 6.

Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

As one common disease causing young people to die suddenly due to cardiac arrest, arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder of heart muscle whose progression covers one complicated gene interaction network that influence the diagnosis and prognosis of it. In our research, differentially expressed genes (DEGs) were screened, and we established a weighted gene coexpression network analysis (WGCNA) and gene set net correlations analysis (GSNCA) for identifying crucial genes as well as pathways related to ARVC pathogenic mechanism ( = 12). In the research, the results demonstrated that there were 619 DEGs in total between non-failing donor myocardial samples and ARVC tissues (FDR < 0. Read More

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http://dx.doi.org/10.3389/fphys.2018.01778DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291487PMC
December 2018
1 Read

12-Lead Electrocardiography Parameters for Differentiating Athlete's Heart From Arrhythmogenic Right Ventricular Cardiomyopathy.

JACC Clin Electrophysiol 2018 Dec;4(12):1626-1628

Center for Cardiological Innovation, Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.

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http://dx.doi.org/10.1016/j.jacep.2018.10.002DOI Listing
December 2018
1 Read

Electrocardiographic Features Differentiating Arrhythmogenic Right Ventricular Cardiomyopathy From an Athlete's Heart.

JACC Clin Electrophysiol 2018 Dec 1;4(12):1613-1625. Epub 2018 Nov 1.

Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.

Objectives: This study sought to compare electrocardiogram (ECG) variants in athletic and arrhythmogenic right ventricular cardiomyopathy (ARVC) cohorts matched for the confounders of age, sex, and ethnicity.

Background: Anterior T-wave inversion (TWI) is a common electrocardiographic finding in both athletes and patients with ARVC, and is a frequent conundrum in the setting of pre-participation screening. J-point elevation (JPE) has been proposed as an accurate means of identifying athletes, whereas disease markers, including premature ventricular contractions (PVCs) and low-voltage signals, have been associated with ARVC. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.09.008DOI Listing
December 2018
1 Read

Relationship Between Electrocardiographic Findings and Cardiac Magnetic Resonance Phenotypes in Arrhythmogenic Cardiomyopathy.

J Am Heart Assoc 2018 Nov;7(22):e009855

1 Department of Cardiac, Thoracic and Vascular Sciences University of Padova Italy.

Background The new designation of arrhythmogenic cardiomyopathy defines a broader spectrum of disease phenotypes, which include right dominant, biventricular, and left dominant variants. We evaluated the relationship between electrocardiographic findings and contrast-enhanced cardiac magnetic resonance phenotypes in arrhythmogenic cardiomyopathy. Methods and Results We studied a consecutive cohort of patients with a definite diagnosis of arrhythmogenic cardiomyopathy, according to 2010 International Task Force criteria, who underwent electrocardiography and contrast-enhanced cardiac magnetic resonance. Read More

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https://www.ahajournals.org/doi/10.1161/JAHA.118.009855
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http://dx.doi.org/10.1161/JAHA.118.009855DOI Listing
November 2018
10 Reads

Concealed Arrhythmogenic Right Ventricular Cardiomyopathy in Sudden Unexplained Cardiac Death Events.

Circ Genom Precis Med 2018 Nov;11(11):e002355

Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Australia (J.I., R.D.B., L.Y., C.S.).

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http://dx.doi.org/10.1161/CIRCGEN.118.002355DOI Listing
November 2018
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Blockade of the Adenosine 2A Receptor Mitigates the Cardiomyopathy Induced by Loss of Plakophilin-2 Expression.

Front Physiol 2018 5;9:1750. Epub 2018 Dec 5.

Leon H. Charney Division of Cardiology, NYU School of Medicine, New York, NY, United States.

Mutations in plakophilin-2 (PKP2) are the most common cause of familial Arrhythmogenic Right Ventricular Cardiomyopathy, a disease characterized by ventricular arrhythmias, sudden death, and progressive fibrofatty cardiomyopathy. The relation between loss of PKP2 expression and structural cardiomyopathy remains under study, though paracrine activation of pro-fibrotic intracellular signaling cascades is a likely event. Previous studies have indicated that ATP release into the intracellular space, and activation of adenosine receptors, can regulate fibrosis in various tissues. Read More

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https://www.frontiersin.org/article/10.3389/fphys.2018.01750
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http://dx.doi.org/10.3389/fphys.2018.01750DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290386PMC
December 2018
7 Reads

Assessment of right ventricular sympathetic dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy: An I-metaiodobenzylguanidine SPECT/CT study.

J Nucl Cardiol 2018 Dec 17. Epub 2018 Dec 17.

Department of Nuclear Medicine, University of Munich, Munich, Germany.

Purpose: The purpose of the study was to evaluate a novel approach for the quantification of right ventricular sympathetic dysfunction in patients diagnosed with ARVC/D through state-of-the-art functional SPECT/CT hybrid imaging.

Methods: Sympathetic innervation of the heart was assessed using I-MIBG-SPECT/CT in 17 patients diagnosed with ARVC according to the modified task force criteria, and in 10 patients diagnosed with idiopathic ventricular fibrillation (IVF). The I-MIBG-uptake in the left (LV) and right ventricle (RV) was evaluated separately based on anatomic information derived from the CT scan, and compared to the uptake in the mediastinum (M). Read More

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http://dx.doi.org/10.1007/s12350-018-01545-3DOI Listing
December 2018
2 Reads

Right ventricular sympathetic dysfunction in arrhythmogenic right ventricular cardiomyopathy: News or noise?

Authors:
Vineet Kumar

J Nucl Cardiol 2018 Dec 17. Epub 2018 Dec 17.

Division of Cardiovascular Diseases, University of Alabama at Birmingham, 930 Faculty Office Towers, 510 20th Street South, Birmingham, AL, 35294-0006, USA.

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http://dx.doi.org/10.1007/s12350-018-01549-zDOI Listing
December 2018
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Axon guidance pathway genes are associated with schizophrenia risk.

Exp Ther Med 2018 Dec 19;16(6):4519-4526. Epub 2018 Sep 19.

Department of Psychiatry and Mental Health, Qiqihar Medical University, Qiqihar, Heilongjiang 161006, P.R. China.

In the present study, we analyzed schizophrenia (SCZ)-related genome-wide association studies (GWAS) to identify genes and pathways associated with SCZ. We identified 1,098 common genes (1,098/9,468) and 20 shared KEGG pathways (both P<0.01) by integrating candidate genes from the European and American SCZ-related GWAS. Read More

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http://dx.doi.org/10.3892/etm.2018.6781DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257106PMC
December 2018

A Rare Desmoglein-2 Gene Mutation in Arrhythmogenic Right Ventricular Cardiomyopathy Inciting Incessant Ventricular Fibrillation.

Cureus 2018 Sep 29;10(9):e3388. Epub 2018 Sep 29.

Internal Medicine, Overlook Medical Center, Summit, USA.

A case of a 51-year-old female with history of hypertension and a significant family history of premature coronary artery disease presented to the hospital after cardiac arrest. She successfully completed a targeted temperature management therapy with full neurologic recovery. Her hospital course was complicated by several bouts of ventricular fibrillation (VF) arrest which was rescued by timely defibrillation, high quality cardiorespiratory resuscitation, and administration of antiarrhythmic medications and inotropic agents. Read More

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https://www.cureus.com/articles/14174-a-rare-desmoglein-2-ge
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http://dx.doi.org/10.7759/cureus.3388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6267707PMC
September 2018
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Differential pacing from two sites to diagnose risk of ventricular arrhythmia and death.

Pacing Clin Electrophysiol 2019 Feb 18;42(2):189-200. Epub 2018 Dec 18.

Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, 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 electrocardiogram during AP (100 beats per minute [bpm]) and RVP (100 and 120 bpm). Read More

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http://doi.wiley.com/10.1111/pace.13566
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http://dx.doi.org/10.1111/pace.13566DOI Listing
February 2019
7 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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http://dx.doi.org/10.1016/j.jelectrocard.2018.08.009DOI Listing
August 2018
5 Reads

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

Heart Lung Circ 2019 Jan 11;28(1):31-38. 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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http://dx.doi.org/10.1016/j.hlc.2018.09.010DOI Listing
January 2019
4 Reads

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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http://dx.doi.org/10.1093/europace/euy238DOI Listing
November 2018
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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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http://dx.doi.org/10.1016/j.ijcard.2018.06.105DOI Listing
January 2019
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Arrhythmogenic Cardiomyopathy in 2018-2019: ARVC/ALVC or Both?

Heart Lung Circ 2019 Jan 24;28(1):164-177. 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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https://linkinghub.elsevier.com/retrieve/pii/S14439506183195
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http://dx.doi.org/10.1016/j.hlc.2018.10.013DOI Listing
January 2019
32 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
13 Reads