Clinical cardiologists are encountering an important challenge in the care 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 - for instance hypertrophic or dilated cardiomyopathy - or in the form of arrhythmias, including Brugada syndrome and long QT syndrome. Clinical and genetic research is continuously ongoing not only to identify those at risk but also to better define their level of risk. Deciding on the most appropriate clinical tests is a challenge in inherited diseases. It is in this situation that specialized clinical-genetic clinics have appeared to provide a complete assessment of patients and family members. The clinicians in the community, faced with a growing number of questions and doubts in the caring of these individuals, are increasingly using the expertise of these centers to address the clinical decisions in their patients.

Clinical Approach to Sudden Cardiac Death Syndromes details the main guidelines to clinicians on how to approach the patients and family members with an inherited disease. It also includes an in-depth review of what is currently available to assist in the diagnosis, prevention and risk stratification in sudden cardiac death. This book is therefore an essential reference for all cardiologists and electrophysiologists, but also this will be important reading for general practice physicians who may be responsible for the identification of families at risk.

Zusammenfassung

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.

Inhalt
Sudden Unexplained Death.- Sudden Unexplained Death in the Community.- Sudden Infant Death Syndrome: Gene-Environment Interactions.- Arrhythmias and sudden cardiac death. The initial investigation.- Unexplained Syncope.- Arrhythmias and Sudden Cardiac Death in Adult Congenital Heart Disease.- Endurance Sport Practice and Arrhythmias.- Electrocardiograms Not to Miss.- Sudden Cardiac Death in Forensic Pathology.- Cardiac genetic syndromes.- Genetic Studies.- The Long QT Syndrome.- Brugada Syndrome.- Short QT Syndrome.- Catecholaminergic Polymorphic Ventricular Tachycardia.- Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.- Atrial Fibrillation.- Dilated Cardiomyopathy.- Hypertrophic Cardiomyopathy.- Genetic Lipoprotein Disorders and Cardiovascular Disease.- A Systematic Approach to Marfan Syndrome and Hereditary Forms of Aortic Dilatation and Dissection.- Inherited Metabolic Diseases: Emphasis on Myocardial Disease and Arrhythmogenesis.- Clinical Genetics in Congenital Heart Disease.- Polygenic cardiovascular genetics.- Pharmacogenomics.- Polygenic Studies in the Risk of Arrhythmias.- The Genetic Challenge of Coronary Artery Disease.- Ethical, legal and Social implications.- Psychological Implications of Genetic Investigations.- Participation in Recreational Sports for Young Patients with Genetic Cardiovascular Diseases.- Genetic Counseling in Cardiovascular Conditions.