Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review
Jorge Romero,Eeliany Mejia-Lopez,Carlos Manrique,Richard J. Lucariello +3 more
- Vol. 7, Iss: 7, pp 97-114
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TLDR
Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations, therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy.Abstract:
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for 11%–22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation.
Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.read more
Citations
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Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Proposed Modification of the Task Force Criteria
TL;DR: The original 1994 International Task Force criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC / D) were based on structural,histological,ECG,arrhythmic,and familial features of the disease.
Journal ArticleDOI
The MOGE(S) Classification of Cardiomyopathy for Clinicians
Eloisa Arbustini,Navneet Narula,Luigi Tavazzi,Alessandra Serio,Maurizia Grasso,Valentina Favalli,Riccardo Bellazzi,Jamil A. Tajik,Robert O. Bonow,Valentin Fuster,Jagat Narula +10 more
TL;DR: The recently proposed MOGE(S) nosology system embodies all of these characteristics, and describes the morphofunctional phenotype, organ involvement, genetic inheritance pattern, etiological annotation, and the functional status of the disease using both the American College of Cardiology/American Heart Association stage and New York Heart Association functional class.
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Establishment of specialized clinical cardiovascular genetics programs: Recognizing the need and meeting standards a scientific statement from the American Heart Association
Ferhaan Ahmad,Elizabeth M. McNally,Michael J. Ackerman,Linda Baty,Sharlene M. Day,Iftikhar J. Kullo,Peace C. Madueme,Martin S. Maron,Matthew W. Martinez,Lisa Salberg,Matthew R.G. Taylor,Janel E. Wilcox +11 more
TL;DR: This scientific statement outlines current best practices for delivering cardiovascular genetic evaluation and care in both the pediatric and the adult settings, with a focus on team member expertise and conditions that most benefit from genetic evaluation.
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Desmoglein 2–Dependent Arrhythmogenic Cardiomyopathy Is Caused by a Loss of Adhesive Function
TL;DR: It is proposed that loss of Dsg2 compromises adhesion, and that this is a major pathogenic mechanism in DSG2-related and probably other desmosome-related ACs.
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Desmoglein-2 interaction is crucial for cardiomyocyte cohesion and function
Angela Schlipp,Camilla Schinner,Volker Spindler,Franziska Vielmuth,Katja Gehmlich,Petros Syrris,William J. McKenna,Andreas Dendorfer,Eva Hartlieb,Jens Waschke +9 more
TL;DR: The data demonstrate that desmoglein-2 plays a critical role in cardiomyocyte cohesion and function and indicates that single and tandem peptide can be used to specifically target desmquirein-1-2-mediated adhesion.
References
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Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies.
P Richardson,William J. McKenna,Michael R. Bristow,Bernhard Maisch,B Mautner,John B. O'Connell,E Olsen,Gaetano Thiene,J F Goodwin,I Gyarfas,I Martin,P Nordet +11 more
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A comparison of antiarrhythmic-drug therapy with implantable defibrillators in patients resuscitated from near-fatal ventricular arrhythmias
TL;DR: Among survivors of ventricular fibrillation or sustained ventricular tachycardia causing severe symptoms, the implantable cardioverter-defibrillator is superior to antiarrhythmic drugs for increasing overall survival.
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ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons
Andrew E. Epstein,John P. DiMarco,Kenneth A. Ellenbogen,N.A. Mark Estes,Roger A. Freedman,Leonard S. Gettes,A. Marc Gillinov,Gabriel Gregoratos,Stephen C. Hammill,David L. Hayes,Mark A. Hlatky,L. Kristin Newby,Richard L. Page,Mark H. Schoenfeld,Michael J. Silka,Lynne W. Stevenson,Michael O. Sweeney +16 more
TL;DR: Sidney C. Smith, Jr,MD, FACC, FAHA, Chair Alice K. Jacobs, MD, F ACC,FAHA, Vice-Chair Cynthia D. Adams, RN, PhD, FAH.
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ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death
Douglas P. Zipes,A. John Camm,Martin Borggrefe,Alfred E. Buxton,Bernard R. Chaitman,Martin Fromer,Gabriel Gregoratos,George J. Klein,Arthur J. Moss,Robert J. Myerburg,Silvia G. Priori,Miguel A. Quinones,Dan M. Roden,Michael J. Silka,Cynthia M. Tracy,Jean-Jacques Blanc,Andrzej Budaj,Veronica Dean,Jaap W. Deckers,Catherine Despres,Kenneth Dickstein,John Lekakis,Keith McGregor,Marco Metra,João Morais,Ady Osterspey,Juan Tamargo,José Luis Zamorano,Sidney C. Smith,Alice K. Jacobs,Cynthia D. Adams,Elliott M. Antman,Jeffrey L. Anderson,Sharon A. Hunt,Jonathan L. Halperin,Rick A. Nishimura,Joseph P. Ornato,Richard L. Page,Barbara Riegel +38 more
TL;DR: This guideline is pleased to have this guideline developed in conjunction with the European Society of Cardiology (ESC) and to have been selected from all 3 organizations to examine subject-specific data and write guidelines.
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Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Proposed Modification of the Task Force Criteria
Frank I. Marcus,William J. McKenna,Duane L. Sherrill,Cristina Basso,Barbara Bauce,David A. Bluemke,Hugh Calkins,Domenico Corrado,Moniek G.P.J. Cox,James P. Daubert,Guy Fontaine,Kathleen Gear,Richard N.W. Hauer,Andrea Nava,Michael H. Picard,Nikos Protonotarios,Jeffrey E. Saffitz,Danita M. Yoerger Sanborn,Jonathan S. Steinberg,Harikrishna Tandri,Gaetano Thiene,Jeffrey A. Towbin,Adalena Tsatsopoulou,Thomas Wichter,Wojciech Zareba +24 more
TL;DR: Modifications of the Task Force Criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia represent a working framework to improve the diagnosis and management of this condition.
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