Clinical Approach To Sudden Cardiac Death Syndromes

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.

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.

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 dedicates the first seven chapters to cardiac electrophysiological dysfunction that can lead to ventricular fibrillation and sudden death. The next six chapters expand the topics of sudden death to other causes. Brugada Syndrome is the top cause of non-traumatic sudden death in young males of Southeastern Asian and southern European origins. Chapters One and Two review extensively the history and current status, mechanisms of arrhythmias, and related gene mutations in Brugada Syndrome, and discuss gaps in the current knowledge. Chapter Three presents clinical studies on the A1180V and R1193Q mutations of the cardiac sodium channel gene, SCN5A. The authors identified R1193Q mutation in 12.8%-16% of a healthy Chinese population. This result prompted the question of whether SCN5A mutations are still the top genetic bases of Brugada and long QT syndromes. Chapters Four, Five and Seven discuss the pathophysiology causes, risk factors, predictors, prevention, and possible therapeutical strategies of sudden cardiac death. Chapter Six reviews the scope and causes of sudden cardiac death in athletics.Chapter Seven is dedicated to the roles of electrical cardiac systole in sudden death. Chapter Eight reviews sudden death related to cardiac and brain tumours, pulmonary tumour embolism, asphyxia, massive exsanguinations, and their pathophysiology. Chapter Nine summarises the pathophysiology of sudden death caused by myocardial tuberculosis. Chapter Ten introduces pathophysiology of sudden death during hot baths in deep bathtubs, which occurs frequently in elderly people in Japan. Chapter Eleven discusses air pollution as a trigger of sudden death. Chapter Twelve presents biochemical, cytological and histopathological examination of sudden unexpected death in infancy. The last chapter of the book, Chapter Thirteen, reviews post-mortem cardiac markers in different body fluids and their application in forensic pathology diagnosis of sudden cardiac death. In combination, these chapters cover a broad range of topics about sudden death.

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.

Sudden cardiac death is the leading cause of non-traumatic mortality in young (

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.

This book dedicates the first seven chapters to cardiac electrophysiological dysfunction that can lead to ventricular fibrillation and sudden death. The next six chapters expand the topics of sudden death to other causes. Brugada Syndrome is the top cause of non-traumatic sudden death in young males of Southeastern Asian and southern European origins. Chapters One and Two review extensively the history and current status, mechanisms of arrhythmias, and related gene mutations in Brugada Syndrome, and discuss gaps in the current knowledge. Chapter Three presents clinical studies on the A1180V and R1193Q mutations of the cardiac sodium channel gene, SCN5A. The authors identified R1193Q mutation in 12.8%-16% of a healthy Chinese population. This result prompted the question of whether SCN5A mutations are still the top genetic bases of Brugada and long QT syndromes. Chapters Four, Five and Seven discuss the pathophysiology causes, risk factors, predictors, prevention, and possible therapeutical strategies of sudden cardiac death. Chapter Six reviews the scope and causes of sudden cardiac death in athletics. Chapter Seven is dedicated to the roles of electrical cardiac systole in sudden death. Chapter Eight reviews sudden death related to cardiac and brain tumors, pulmonary tumor embolism, asphyxia, massive exsanguinations, and their pathophysiology. Chapter Nine summarizes the pathophysiology of sudden death caused by myocardial tuberculosis. Chapter Ten introduces pathophysiology of sudden death during hot baths in deep bathtubs, which occurs frequently in elderly people in Japan. Chapter Eleven discusses air pollution as a trigger of sudden death. Chapter Twleve presents biochemical, cytological and histopathological examination of sudden unexpected death in infancy. The last chapter of the book, Chapter Thirteen, reviews postmortem cardiac markers in different body fluids and their application in forensic pathology diagnosis of sudden cardiac death. In combination, these chapters cover a broad range of topics about sudden death.