The Brugada Syndrome From Bench To Bedside

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.

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.

Contributors,

vii

Preface,

xi

Dedication,

xiii

1 Brugada syndrome: overview,

1

(22)

C. Antzelevitch, PhD,

P. Brugada, MD, PhD,

M. Borggrefe, MD, PhD,

J. Brugada, MD,

R. Brugada, MD,

D. Corrado, MD, PhD,

I. Gussak, MD, PhD,

H. Le Marec, MD, PhD,

K. Nademanee, MD,

A.R. Perez Riera, MD,

H. Tan, MD, PhD,

W. Shimizu, MD, PhD,

E. Schulze-Bahr, MD,

A.A.M. Wilde, MD, PhD

2 History of the Brugada syndrome,

23

(3)

I. Gussak, MD, PhD,

P. Bjerregaard, MD

3 Biophysical analysis of mutant sodium channels in Brugada syndrome,

26

(16)

H. Tan, MD, PhD

4 Molecular genetics of the Brugada syndrome,

42

(10)

E. Schulze-Bahr, MD,

L. Eckardt, MD,

M. Paul, MD,

T. Wichter, MD,

G. Breithardt, MD

5 Cellular mechanisms underlying the Brugada syndrome,

52

(26)

C. Antzelevitch, PhD,

J.M. Fish, DVM,

J.M. Di Diego, MD

6 Brugada syndrome: diagnostic criteria,

78

(9)

A.A M. Wilde, MD, PhD

7 Value of 12 lead electrocardiogram and derived methodologies in the diagnosis of Brugada disease,

87

(24)

A.R. Perez Riera, MD,

C. Ferreira, MD, PhD,

E. Schapachnik, MD

8 Brugada syndrome: relationship to other arrhythmogenic syndromes,

111

(8)

D. Corrado, MD, PhD,

C. Basso, MD, PhD,

G. Buja, MD,

A. Nava, MD,

G. Thiene, MD

9 ST segment elevation and sudden death in the athlete,

119

(11)

D. Corrado, MD, PhD,

A. Pelliccia, MD,

C. Antzelevitch, PhD,

L. Leoni, MD,

M. Schiavon, MD,

G. Buja, MD,

B. Maron, MD,

G. Thiene, MD,

C. Basso, MD, PhD

10 Brugada syndrome: role of genetics in clinical practice,

130

(10)

R. Brugada, MD

11 Genotype-phenotype relationship in the Brugada syndrome,

140

(9)

J.P.P. Smits, MD,

A.A.M. Wilde, MD, PhD

12 Gender differences in Brugada syndrome,

149

(8)

V. Probst, MD,

S. Pattier, J-J. Schott, PhD,

H. Le Marec, MD, PhD

13 Predisposing factors,

157

(9)

K. Nademanee, MD,

G. Veerakul, MD,

M. Schwab, MD

14 Acquired forms of Brugada syndrome,

166

(12)

W. Shimizu, MD, PhD

15 Atrial tachyarrhythmias in Brugada syndrome,

178

(6)

M. Borggrefe, MD, PhD,

R. Schimpf, MD,

F. Gaita, MD,

L. Eckardt, MD,

C. Wolpert, MD

16 Prognosis in individuals with Brugada syndrome,

184

(10)

J. Brugada, MD,

R. Brugada, MD,

P. Brugada, MD, PhD

17 Treatment of Brugada syndrome with an implantable cardioverter defibrillator,

194

(8)

P. Brugada, MD, PhD,

E. Bartholomay, MD,

L. Mont, MD,

R. Brugada, MD,

J. Brugada, MD

18 Pharmacologic approach to therapy of Brugada syndrome: quinidine as an alternative to ICD therapy?,

202

(10)

B. Belhassen, MD,

S. Viskin, MD

19 Potential for ablation therapy in patients with Brugada syndrome,

212

(9)

L-F. Hsu, MBBS,

B. Cauchemez, MD,

P. Jais, MD,

P. Sanders, MBBS, PhD,

M. Hocini, MD,

P. Maury, MD,

J. Angel Cabrera, MD,

F. Extramiana, MD,

C. Scavée, MD,

Y. Takahashi, MD,

M. Rotter, MD,

J-L. Pasquie, MD, PhD,

J. Victor, MD,

S. Garrigue, MD,

J. Clémenty, MD,

M. Haissaguerre, MD

Index,

221

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The Brugada Syndrome From Bench To Bedside

Summary

Author Biography

Table of Contents

Supplemental Materials