Brugada Syndrome

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.

Brugada Phenocopy: The Art of Recognizing the Brugada ECG Pattern details all aspects associated with alternative diagnosis to Brugada Syndrome (BrS). Coverage includes how to identify the proper ECG pattern, what to do to investigate for BrP, and how to avoid misinterpretations and the use of unnecessary and expensive treatments. Chapters are written by experienced professionals, many of whom are colleagues that initially described this condition. This easy to use volume is a must have reference for researchers of cardiology, cardiologists, electrocardiologists, internists, emergency care doctors and students, residents and fellows. Assists in the proper recognition of the Brugada ECG patterns and how to distinguish true BrS from other conditions with identical ECG Expands understanding on how to properly recognize the ECG of Brugada patterns Contains access to a companion website with video to enhance understanding of proper measurement of the beta angle (Chevallier) and the base of the triangle (Serra)

Brugada syndrome (BrS) is a sudden cardiac death disease characterised by right bundle-branch block with ST segment elevation in the lead V1 and V2 in the absence of any structural diseases. This syndrome is highly associated with sudden cardiac death, especially in young adults. It is associated with a history of SCD in up to four first-degree family members. The authors of this book examine Brugada Syndrome (BS), and its possible link to arrhythmogenic cardiomyopathy. Furthermore, Type 1 ECG is considered a diagnostic sign for the Brugada Syndrome and its clinical significance is explored in this book. Lastly, the book presents the readers with a case report of a patient with Brugada syndrome characterised by an abnormal electrocardiogram (ECG).

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.

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.

The volume includes more than seventy articles written by well-recognized world leaders in the field of clinical electrophysiology and arrhythmology. It represents an important update on the most recent advances in the diagnosis, prognosis and treatment of cardiac arrhythmias.

Brugada Syndrome: New Insights for the Healthcare Professional: 2011 Edition is a ScholarlyPaper™ that delivers timely, authoritative, and intensively focused information about Brugada Syndrome in a compact format. The editors have built Brugada Syndrome: New Insights for the Healthcare Professional: 2011 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Brugada Syndrome in this eBook to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Brugada Syndrome: New Insights for the Healthcare Professional: 2011 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.

Electrocardiographically characterised by a distinct ST-segment elevation in the right precordial leads, Brugada syndrome (BrS) is an arrhythmogenic disease reported to be responsible for at least 4% of all sudden deaths (at least 20% of sudden deaths in those without structural heart disease), and is a leading cause of death in subjects under the age of forty years. Global prevalence of the BrS varies from 5% to 20% in every 10,000 inhabitants worldwide, and it is considered endemic in Asian and Southeast Asian countries. The diagnosis is mainly based on electrocardiographic features and may be hampered by incomplete penetrance, particularly because of dynamic ECG manifestations. As far as we know, inheritance occurs via an autosomal dominant mode of transmission, and about eighteen genes have been associated with BrS; thus far, genetic abnormalities are found in 30-50% of genotyped BrS patients. Mutations in the cardiac sodium channel gene SCN5A are identified in 1128% of patients with BrS, with over 300 different mutations of SCN5A having been identified as the list still grows. Because a genotype remains lacking for at least half of BrS probands, a negative genetic test does not rule out BrS. Febrile states, dysionia and many drugs such as vagotonic agents, sodium channel blockers, α-adrenergic agonists, β-adrenergic blockers, antidepressants, and alcohol and cocaine toxicity may unmask the electrocardiographic concealed ECG manifestations of BrS. Identifying patients with BrS at risk of malignant arrhythmias and sudden cardiac death remains the most important objective and therefore the fundamental question remains on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients. The therapeutic approach for patients with BrS is centered on the prevention of cardiac arrest and syncopal events due to ventricular arrhythmias. Lifestyle changes, such as never using drugs that may potentially induce ventricular arrhythmias and an immediate treatment of fever, should be recommended in all patients with diagnosis of BrS. Some drugs have been tested in BrS patients, but controlled data and/or randomised studies proving their real effective have not been performed. Currently, the cornerstone of therapy for the prevention of sudden cardiac death (SCD) is an implantable cardioverter defibrillator (ICD). An accurate individual risk stratification to select patients for ICD implantation should be accurately performed, particularly in asymptomatic patients. Lastly, catheter ablation of the substrate located in the anterior epicardial region of the right ventricular outflow tract may be considered in patients with a history of arrhythmic storms or repeated appropriate ICD shocks. The study of the areas of delayed depolarisation in patients with BrS and the procedure of catheter ablation of the substrate located in the anterior epicardial region therefore opens new horizons in regards to the management of patients with extremely frequent ventricular tachycardia/ventricular fibrillation (VT/VF), for patients at high risk who refuse the implantation of an ICD, and for a better prognostic stratification of patients with BrS not classified as high risk for SCD.