Sudden Cardiac Death And Channelopathies

This book provides an expert overview on ion channel-related arrhythmia mechanisms, and describes important advances in our understanding of how ion channel dysfunction causes cardiac disease. Both, scientific findings and clinical implications are presented and discussed by scientists who have considerably contributed to the field. The book is organized in three parts: part I treats the molecular and electrophysiological mechanisms of function and dysfunction of ion channels, part II focuses on genetics and clinical findings, whereas part III describes novel research techniques, the use of stem cells and animal models and provides an outlook on future investigations and applications. The book is written for scientists in Cardiovascular Biology and Neuroscience and will be of general interest to Medical Doctors in Cardiology, Cardiac Electrophysiology and related disciplines.

This issue of Cardiac Electrophysiology Clinics, edited by Drs. Mohammad Shenasa, N. A. Mark Estes III, and Gordon F. Tomaselli, will cover Contemporary Challenges in Sudden Cardiac Death. Topics covered in this issue include Pathophysiology; Basic electrophysiological mechanism; Channelopathy and Myopathy as causes of sudden cardiac death; Public access to defibrillation; Sudden cardiac death in children adolescence; Sudden cardiac death in specific cardiomyopathies; Ventricular arrhythmias and sudden cardiac death; lessons learned from cardiac implantable rhythm devices; future directions, and more.

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.

Ion channel dysfunction in humans leads to impairment of the excitable processes necessary for the normal function of several tissues, such as muscle and brain. It follows that an increasing number of human diseases have been associated with malfunctioning ion channels, many of which have a genetic component. This volume of Advances in Genetics presents a broad and comprehensive overview of the inherited channelopathies in humans, including clinical, genetic and molecular aspects of these conditions. Keeping true to the scope of the serial, novel genomic and modeling research approaches and a review of potential therapeutic approaches for each of these conditions are also incorporated.

Sudden cardiac death is a global health threat for which we have only partial answers. With growing elucidation of the underlying pathophysiological mechanisms of sudden cardiac death, better patient identification and treatment options are being developed. These include risk stratification paradigms, ICD therapy, pharmacological options, ablative procedures, and other treatments. This book covers many of these options, including defibrillator technology and clinical applications. It also examines pathophysiological pathways and etiologies as well as highlights risk-stratification in ion channel diseases and structural heart disease such as dilated cardiomyopathy.

Clearly presents the pathology of heart disease from fetus to adolescence, integrating histology and macroscopy with effects of treatment.