Brugada Syndrome

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Contents 1 More Specific Terms 2 Introduction 3 Epidemiology 4 Genetics 5 Clinical-manifestations 6 Diagnostic-procedures 7 Complications 8 Management 9 More General Terms 10 Additional Terms 11 Internet Database 12 References

More Specific Terms

• Brugada syndrome type 1
• Brugada syndrome type 3
• Brugada syndrome type 4
• Brugada syndrome type 8

Introduction

• syncope in the context of a family history of sudden cardiac death
  
• Brugada syndrome was originally described in 1992.

Epidemiology

• commonly affects middle-age men
• average presentation at 30 years
• accounts for 40-50% of idiopathic ventricular fibrillation in parts of Southeast Asia

Genetics

• autosomal dominant
• mutations in cardiac sodium channel gene SCN5A

Clinical-manifestations

• syncopal episodes
• ventricular fibrillation
• self-terminating polymorphic ventricular tachycardia
• family history of sudden cardiac death in a relative younger than 45 years

Diagnostic-procedures

• electrocardiogram*

• precordial ST segment elevation (downsloping) in leads V1- V3 typically followed by a negative T wave#
• QRS morphology resembling right bundle branch block
• evidence of ST segment elevation in family members
• initial electrocardiogram may be normal

• provocative administration of a class Ia antiarrhythmic, such as procainamide may induce daignostic electocardiogram pattern

• * electrocardiogram plus 1 or more clinical manifestations makes diagnosis
• # benign early repolarization generally has a positive T-wave

Complications

• suddent cardiac death

Management

• hospital admission with ECG monitoring
• cardiology consult (specialist in electrophysiology)
• consider placement of a cardioverter-defibrillator
• evaluation of the patient's family members

More General Terms

• syndrome

Additional Terms

• Na+ channel protein type 5 subunit alpha; Na+ channel protein type V subunit alpha; voltage-gated Na+ channel subunit alpha Nav1.5; Na+ channel protein cardiac muscle subunit alpha; HH1 (SCN5A)

Internet Database

OMIM: 601144

References

1. Antzelevitch C. The Brugada syndrome. J Cardiovasc Electrophysiol. 1998 May;9(5):513-6. Review. PMID: [1]
2. Chen Q, Kirsch GE, Zhang D, Brugada R, Brugada J, Brugada P, Potenza D, Moya A, Borggrefe M, Breithardt G, Ortiz-Lopez R, Wang Z, Antzelevitch C, O'Brien RE, Schulze-Bahr E, Keating MT, Towbin JA, Wang Q. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature. 1998 Mar 19;392(6673):293-6. PMID: [2]
3. Gussak I, Antzelevitch C, Bjerregaard P, Towbin JA, Chaitman BR. The Brugada syndrome: clinical, electrophysiologic and genetic aspects. J Am Coll Cardiol. 1999 Jan;33(1):5-15. Review. PMID: [3]

Brugada syndrome