Clinical cardiologists are encountering an important challenge in the caring of families with inherited cardiac diseases. The majority of the inherited cardiac diseases causing sudden death express themselves at variable ages in the form of altered muscle function (i.e hypertrophic or dilated cardiomyopathy) or in the form of arrhythmias (i.e. Brugada syndrome, long QT syndrome). However, it is not uncommon that the first sign of the disease may actually be sudden cardiac death, even before the identification of clear clinical abnormalities. In this last decade, with more than 50 new disease-associated genes identified, the possibility of genetic testing has opened a new opportunity to disease diagnosis and prevention. Clinical and genetic research is continuously on-going not only to identify those at risk, but to better define their level or risk still with limited success.

Until recently, the cellular basis for sudden death, the BrugadaSyndrome, has largely remained an unknown to modernarrhythmologists and cardiologists, particularly in the absence ofany structural heart disease. Detailed observations of age-groups,especially the young, families and populations where sudden deathfrequently occurs, and improved understanding of its contributoryfactors and mechanisms are, however, showing the way forward. This addition to the Clinical Approaches to Tachyarrhythmias(CATA) Series, written by the investigators who discovered andprobed the Brugada Syndrome, discusses the history, etiology,pathology and clinical manifestations of sudden death. Fromdiagnosis, prognosis, to therapeutic approaches using the latest incathater ablation techniques, electrophysiological surgery, andgenetic appraisal, the work is a testimony to the author'sinvestigation. Using clinical cases in Thailand and Laos, theyfurther unravel the syndrome's molecular mechanisms, studyingrelated syndromes, such as the long-QT syndrome, infant death, andarryhthmogenic right ventricular cardiomyopathy. By being informed of the electrophysiological abnormalities thatcontribute to familial and genetic diseases, physicians,cardiologists and all those who care for patients with cardiacarrhythmias will be better able to identify and treat patients inwhom the Brugada Syndrome may strike next.

This book draws on the established European guidelines from the ESCthat address the key issues in sudden cardiac death, such asidentifying individuals at risk prior to an episode of aventricular tachyarrhythmia or a sudden cardiac arrest, andresponding in a timely fashion to the person suffering the eventout-of-the-hospital. It presents an update on what is known aboutsudden cardiac arrest, from basic experimental studies to clinicaltrials, and serves as a complement to the ESC Core Syllabus on thissubject. Topics include epidemiology, genetics, arrhythmogenicmechanisms, risk stratification, autonomic nervous system andphenotypes. Disease states and special populations are alsocovered, as well as drug, device and ablation treatments, and costeffectiveness. All chapters are co-authored by experts from bothEurope and the US. The ESC Education Series This book is part of the ESC Education Series. The series isdesigned to provide medical professionals with the latestinformation about the understanding, diagnosis and management ofcardiovascular diseases. Where available, managementrecommendations are based on the established European Guidelines,which encompass the best techniques to use with each cardiacdisease. Throughout the series, the leading international opinionleaders have been chosen to edit and contribute to the books. Theinformation is presented in a succinct and accessible format with aclinical focus.

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.

This text atlas focuses on the pathology and molecular genetics of sudden cardiac death in the young and in athletes, presenting the state of the art in the field as the basis for development and implementation of more effective prevention strategies, including, ultimately, molecular therapy that will cure the underlying biological defect. A wealth of high-resolution color images, accompanied by clear supporting text, are presented to document the anatomic pathology of the cardiac diseases most frequently responsible for sudden cardiac death in this population, including coronary artery diseases, cardiomyopathies, myocarditis, valve diseases, conduction system abnormalities, congenital heart diseases, and ion channel diseases. The role of the molecular autopsy in overcoming the limitations of morphological investigations and offering new insights and avenues for prevention is explained. The approach is, however, interdisciplinary, with close attention also to epidemiologic and clinical aspects. The authors draw throughout on their experience gained over 30 years in the course of a prospective study carried out in the Veneto Region, North East Italy. This text atlas will be of great value not only for cardiologists but also for geneticists, sports physicians, and residents in cardiology and pathology.

Sudden death in athletes is a global problem. Although it is a relatively rare phenomenon (1/100,000 persons), when it does occur, it is often as an incomprehensible event. In fact, it strikes subjects who presumably should be much healthier than the general population. In the previous 20 years, many authors have studied this problem in an attempt to understand the causes and prevent these events, and it has been determined that, in the vast majority of cases, athletes who die suddenly have an underlying heart disease (arrhythmogenic cardiomyopathy, hypertrophic cardiomyopathy, coronary anomalies, channelopathies, etc.). In most cases these diseases do not produce major symptoms and do not preclude sports activity even at the highest levels, although they do increase the incidence of sudden death. How to discover these diseases in asymptomatic athletes is a hotly debated issue. In particular, there is controversy as to whether all athletes should undergo detailed medical screening, including electrocardiogram, or whether the costs of this screening are too high in relation to the event incidence. The purpose of this book is to accurately analyze the causes of sudden death in athletes and to provide cardiologists and sports physicians with useful tips on how to identify at-risk individuals.

The aim of Molecular Cardiology: Methods and Protocols is to document state-of-the-art molecular and genetic techniques in the area of cardiology. These modern approaches enable researchers to readily study heart diseases at the molecular level and will promote the development of new therapeutic str- egies. Methods for genetic dissection, signal transduction, and microarray analysis are excellent tools for the study of the molecular mechanisms of cardiovascular diseases. Protocols for transgenesis take advantage of recent advances in many areas of molecular and cell biology. Transgenic models of heart diseases (cardiac hypertrophy, cardiac dysfunction, and so on. ) are powerful tools for the study of heart disease pathogenesis. Methods for gene transfer to heart tissue using viral and nonviral vectors form the basis of gene therapy for heart diseases. Heart-specific promoters containing a hypox- inducible cardioprotective gene switch are key for protection of the heart from ischemia. Gene and stem cell therapies open novel and exciting avenues for the prevention and treatment of heart diseases. Molecular Cardiology: Methods and Protocols consists of 26 chapters de- ing with various aspects of molecular cardiology, including gene transfer and gene therapy for cardiovascular disease, stem cell therapy for cardiovascular disease, gene analysis in the injured and hypertrophied heart, and transgenesis in cardiovascular research. This book provides step-by-step methods for the successful completion of experimental procedures, and would be useful for both experienced and new investigators in the field of molecular cardiology.