Current Concepts in Arrhythmogenic Cardiomyopathy, Second Edition presents essential insights into all aspects of this complex disease and will serve as a valuable guide to help readers provide the best possible care for their patients.
Discussion by internationally recognized authorities includes:
- Increasing evidence that arrhythmogenic cardiomyopathy is not necessarily confined to the RV, but often involves both ventricles
- Basic and clinical science of arrhythmogenic cardiomyopathy
- Pathophysiology, molecular mechanisms, and genetic background
- The mechanisms of disease progression leading to a diversity of disease phenotypes
- Guidance in the clinical setting for diagnosis, risk stratification, and therapy
The first edition of this book is entitled Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.
Author(s): Corinna Brunckhorst; Ardan M. Saguner; Firat Duru
Edition: 2
Publisher: Cardiotext Publishing, LLC
Year: 2021
Language: English
Pages: 216
City: Minneapolis
Cover
Title Page
Copyright
Dedication
Contents
Contributors
Preface
Abbreviations
Introduction
Chapter 1: The History of Naxos Disease: Landmarks in the History of the Disorder
Clinical Observations
Description of a New Syndrome
Nomenclature and Genetic Studies
Clinical Projects and Collaborations
References
Chapter 2: Metabolic Deregulation in Arrhythmogenic Cardiomyopathy: Novel Pathogenic Insights and Their Clinical Application
Introduction
Pathologies andPathogenic Mechanisms of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
Establishing iPSCs from Patients with Clinical ARVC and Desmosome Gene Mutations
Metabolic Maturation-based Pathogenic Induction of ARVC Pathologies
Conclusions and Future Perspective
References
Chapter 3: Pleiotropic Functions of Plakophilin-2 and Arrhythmia Mechanisms
Introduction
Conclusions
References
Chapter 4: Immune Signaling in Arrhythmogenic Cardiomyopathy
Introduction
Inhibition of NFκB Signaling Reverses ACM Disease Features In Vitro
Inhibition of NFκB Signaling Prevents Development of ACM Disease Features In Vivo
The Heart Contains Abundant Cytokines in ACM
Discussion
References
Chapter 5: Studying Arrhythmogenic Cardiomyopathy Under the Microscope
Introduction
Pathological Anatomy of ARVC
From ARVC to AC, Recognizing the Wider Phenotypic Spectrum
Nonischemic LV Scar
Role of Transvenous Endomyocardial Biopsy
References
Chapter 6: Genetic Contributions to Arrhythmogenic Cardiomyopathy
Introduction
Syndromic ACM
Nonsyndromic Desmosomal ACM
Nondesmosomal ACM
Whole Exome Sequencing Studies
Reduced Penetrance
Relevance of Genetic Testing
Summary and Future Directions
References
Chapter 7: Current Challenges in the Diagnosis of Arrhythmogenic Cardiomyopathies
Introduction
Diagnosis in Biventricular and Predominant LV Disease
Electrocardiographic Challenges in ACM Diagnosis
Genetic Challenges in ACM Diagnosis
Conclusion
References
Chapter 8: Differential Diagnosis of Phenotypic Variants of Arrhythmogenic Cardiomyopathy
Introduction
Differential Diagnosis of ARVC
Differential Diagnosis of Left-Dominant and Biventricular ALVC
The 2019 HRS Expert Consensus Statement on Arrhythmogenic Cardiomyopathy
Summary
References
Chapter 9: Life-Threatening Arrhythmias Without Overt Clinical Disease: “Concealed”Arrhythmogenic Right Ventricular Cardiomyopathy
Introduction
Clinical Phases of ARVC
Concealed Phase of ARVC
Representative Cases of Concealed ARVC
Molecular Insights of Concealed ARVC and Sudden Death
Clinical Implications of “Concealed Phase” ARVC
Take-Home Messages
References
Chapter 10: Genotype-Phenotype Correlations in ACM: The Chinese Experience
Introduction
Genotype-Clinical Phenotype in Fuwai ACM Cohort
Genotype-Based Clinicopathology: The Fuwai Classification of ACM
Conclusion
References
Chapter 11: Biomarkers in Inherited Arrhythmias: Arrhythmogenic Right Ventricular Cardiomyopathy and Beyond
Introduction
Novel Biomarker for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
Novel Biomarker Profile for Brugada Syndrome
Other Biomarkers in Inherited Arrhythmias
Autoimmunity as a Target for Therapy in Inherited Arrhythmias (ACM)
References
Chapter 12: Atrial Fibrillation and ECG Markers of Atrial Involvement in Arrhythmogenic Right Ventricular Cardiomyopathy
Introduction
Prevalence of Atrial Fibrillation in ARVC
Pathophysiologic Mechanisms of AF in ARVC Patients
P-Wave Indices as Indicators of Atrial Involvement in ARVC
Conclusion
References
Chapter 13: Medical Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy
Introduction
Available Medical Therapies
Developing Medical Therapy to Slow or Prevent Disease Progression
Medical Therapy for ARVC: Does One Size Fit All?
References
Chapter 14: Role of Catheter Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy
Introduction
Catheter Ablation in ARVC
References
Chapter 15: Arrhythmogenic Right Ventricular Cardiomyopathy Research: Better Together
Introduction
Challenges and Achievements After 20 Years of a Large Registry: The North American Experience
Using Infrastructure for a Successful National Collaboration: The DutchExperience
Joint Successes
Predicting Arrhythmic Risk
Toward the Future
References
Chapter 16: The Role of Protein Redistribution in the Diagnosis of Arrhythmogenic Cardiomyopathy: A Translational Perspective
Introduction
Protein Remodeling in the ACM Myocardium
The Molecular Signature of ACM
Protein Remodeling in the ACM Buccal Epithelium
References
Chapter 17: The Zurich ARVC Program
Introduction
ARVC Registry and Biobank
Research Projects
Clinical Research
Translational Research
Zurich International Symposium on Arrhythmogenic Cardiomyopathies
Conclusions
References
Impressions from the Zurich International Symposium on Arrhythmogenic Cardiomyopathies 2019
Index
