The Brugada Syndrome

Until recently, the cellular basis for sudden death, the Brugada Syndrome, has largely remained an unknown to modern arrhythmologists and cardiologists, particularly in the absence of any structural heart disease. Detailed observations of age-groups, especially the young, families and populations where sudden death frequently occurs, and improved understanding of its contributory factors and mechanisms are, however, showing the way forward.

This addition to the Clinical Approaches to Tachyarrhythmias (CATA) Series, written by the investigators who discovered and probed the Brugada Syndrome, discusses the history, etiology, pathology and clinical manifestations of sudden death. From diagnosis, prognosis, to therapeutic approaches using the latest in cathater ablation techniques, electrophysiological surgery, and genetic appraisal, the work is a testimony to the author's investigation. Using clinical cases in Thailand and Laos, they further unravel the syndrome's molecular mechanisms, studying related syndromes, such as the long-QT syndrome, infant death, and arryhthmogenic right ventricular cardiomyopathy.

By being informed of the electrophysiological abnormalities that contribute to familial and genetic diseases, physicians, cardiologists and all those who care for patients with cardiac arrhythmias will be better able to identify and treat patients in whom the Brugada Syndrome may strike next.

Contributors.

Preface.

Dedication.

1 Brugada Syndrome: Overview.

2 History of the Brugada Syndrome.

3 Biophysical Analysis of Mutant Sodium Channels in Brugada Syndrome.

4 Molecular Genetics of the Brugada Syndrome.

5 Cellular Mechanisms Underlying the Brugada Syndrome.

6 Brugada Syndrome: Diagnostic Criteria.

7 Value of 12 Lead ECG and Derived Methodologies in the Diagnosis of Brugada Disease.

8 Brugada Syndrome: Relationship to Other Arrhythmogenic Syndromes.

9 ST Segment Elevation and Sudden Death in the Athlete.

10 Brugada Syndrome Genetics. Genetic Screening- Role in Diagnosis.

11 Genotype-Phenotype Relationship in Brugada Syndrome.

12 Gender Differences in Brugada Syndrome.

13 Predisposing Factors.

14 Acquired Forms of Brugada Syndrome.

15 Brugada Syndrome and supraventricular tacharrhythmias.

16 Prognosis in Individuals with the Brugada Syndrome.

17 Treatment of the Brugada Syndrome with the Implantable Cardioverter Defibrillator.

18 Pharmacologic Approach to Therapy of the Brugada Syndrome: Quinidine as an Alterative to ICD Therapy?.

19 Potential for Ablation Therapy in Patients with the Brugada Syndrome.

Index

Charles Antzelevitch, PhD, is Executive Director/Director of Research; Gordon K. Moe Scholar; Professor of Pharmacology at the Masonic Medical Research Laboratory; Utica, New York.

Pedro Brugada, MD, PhD, is Professor of Cardiology at the Cardiovascular Center OLV Hospital in Aalst, Belgium.

Since its introduction as a new clinical entity in 1992, the Brugada syndrome has attracted great interest because of its high incidence in many parts of the world and its association with high risk for sudden death in infants, children and young adults. Recent years have witnessed an exponential rise in the number of reported cases and a striking proliferation of papers serving to define the clinical, genetic, cellular, ionic and molecular aspects of the disease.

This text delineates the diagnostic criteria for the syndrome and deals with risk stratification and approaches to therapy. The book handles this topic in a very comprehensive manner with the clinical, molecular, genetic, and cellular aspects of the Brugada syndrome authored by the leading experts in the field.