J Wave Syndromes

1. Front Matter

   Pages i-xv

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2. History of the J Wave and J Wave Syndromes

   • Ihor Gussak, Georg Gussak

   Pages 1-14

3. J Wave Syndromes: From Bench to Bedside

   • Gan-Xin Yan, Jian-Zeng Dong, Chang-Cong Cui

   Pages 15-32

4. Ionic and Cellular Mechanisms Underlying J Wave Syndromes

   • Charles Antzelevitch, Bence Patocskai

   Pages 33-76

5. Genetic Basis of Early Repolarization Syndrome

   • Minoru Horie, Keiko Sonoda, Seiko Ohno

   Pages 77-90

6. J Wave Syndrome-Susceptibility Mutations Versus Benign Rare Variants: How Do We Decide?

   • Jamie D. Kapplinger, David J. Tester, Michael J. Ackerman

   Pages 91-120

7. Prevalence and Clinical Characteristics of Brugada Syndrome

   • Arthur A. M. Wilde, Pieter G. Postema

   Pages 121-138

8. Update on the Differential Diagnosis and Treatment of Brugada Syndrome

   • Elena Arbelo, Josep Brugada Terradellas

   Pages 139-172

9. Risk Stratification in Brugada Syndrome: Clinical Characteristics, Electrocardiographic Parameters and Auxiliary Testing

   • Arnon Adler, Sami Viskin

   Pages 173-191

10. Prevalence and Risk Stratification of Patients with Electrocardiographic Pattern of Early Repolarization

    • Heikki V. Huikuri

    Pages 193-206

11. Prognostic Value of Early Repolarization Pattern in Development of VT/VF in Ischemic Heart Disease, Hypothermia, Etc.

    • Hiroshi Morita, Hiroki Sugiyama

    Pages 207-232

12. Similarities and Differences in the Electrocardiographic and Clinical Features Between Early Repolarization Syndrome and Brugada Syndrome

    • Wataru Shimizu

    Pages 233-244

13. Atrial Arrhythmias Associated with J Wave Syndromes

    • Can Hasdemir

    Pages 245-258

14. Phenotypic Expression and Genetics of J Wave Syndrome in the Early Stage of Arrhythmogenic Right Ventricular Cardiomyopathy

    • Domenico Corrado, Marina Cerrone, Alessandro Zorzi, Mario Delmar

    Pages 259-280

15. Clinical Features of Electrical Storms Associated with J Wave Syndromes and Acquired Forms of J Wave Syndrome

    • Gi-Byoung Nam

    Pages 281-300

16. Therapy for J Wave Syndromes

    • Antonio Frontera, Michael E. Field, Arnaud Denis, Nicolas Derval, Caroline Thambo, Pierre Jais et al.

    Pages 301-318

17. J Wave Syndromes in China

    • Jihong Guo, Changsheng Ma

    Pages 319-324

18. Back Matter

    Pages 325-327

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This book delineates the state of the art of the diagnosis and treatment of J wave syndromes, as well as where future research needs to be directed. It covers basic science, translational and clinical aspects of these syndromes. The authors are leading experts in their respective fields, who have contributed prominently to the literature concerning these topics.

J wave syndromes are one of the hottest topics in cardiology today. Cardiac arrhythmias associated with Brugada syndrome (BrS) or an early repolarization (ER) pattern in the inferior or infero-lateral ECG leads are thought to be mechanistically linked to accentuation of transient outward current (Ito)-mediated J waves. Although BrS and ER syndrome (ERS) differ with respect to magnitude and lead location of abnormal J waves, they are thought to represent a continuous spectrum of phenotypic expression termed J wave syndromes. ERS is divided into three subtypes with the most severe, Type 3, displaying an ER pattern globally in the inferior, lateral and right precordial leads. BrS has been linked to mutations in 19 different genes, whereas ERS has been associated with mutations in 7 different genes.

There is a great deal of confusion as to how to properly diagnose and treat the J wave syndromes as well as confusion about the underlying mechanisms. The demonstration of successful epicardial ablation of BrS has provided new therapeutic options for the management of this syndrome for which treatment alternatives are currently very limited, particularly in the case of electrical storms caused by otherwise uncontrollable recurrent VT/VF. An early repolarization pattern is observed in 2-5% of the US population. While it is clear that the vast majority of individuals exhibiting an ER pattern are not at risk for sudden cardiac death, the challenge moving forward is to identify those individuals who truly are at risk and to design safe and effective treatments.

• Brugada Syndrome
• Early Repolarization Syndrome
• Electrical Diseases of the Heart
• Electrophysiology
• J wave syndromes
• Ventricular tachycardia

• Cardiovascular Research, Lankenau Institute for Medical Research, Wynnewood, USA

  Charles Antzelevitch

• Lankenau Institute for Medical Research, Lankenau Medical Center, Wynnewood, USA

  Gan-Xin Yan

Dr Antzelevitch is Professor and Executive Director of Cardiovascular Research at the Lankenau Institute for Medical Research and Director of Research at Lankenau Heart Institute in Wynnewood, PA.  Awards include the Distinguished Scientist Award from the North American Society of Pacing and Electrophysiology (Heart Rhythm Society), Carl J. Wiggers Award of the American Physiological Society, the Distinguished Scientist Award of the American College of Cardiology and the Distinguished Service Award of the Cardiac Electrophysiology Society.  He has published over 510 original papers and reviews and seven books.  He is currently Associate Editor of the Heart Rhythm journal and Secretary-Treasurer of the Cardiac Electrophysiology Society.

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