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Brugada syndrome

syncope in the context of a family history of sudden cardiac death Brugada syndrome was originally described in 1992. Epidemiology: 1) commonly affects middle-age men 2) average presentation at 30 years 3) accounts for 40-50% of idiopathic ventricular fibrillation in parts of Southeast Asia Genetics: 1) autosomal dominant 2) mutations in cardiac sodium channel gene SCN5A Clinical manifestations: 1) syncopal episodes - abrupt - no prodrome, no orthostatic association 2) self-terminating polymorphic ventricular tachycardia 3) family history of sudden cardiac death in a relative younger than 45 years Special laboratory: 1) electrocardiogram* a) precordial ST-segment elevation (downsloping) in leads V1-V3 typically followed by a negative T wave# b) J point elevation c) QRS morphology resembling right bundle branch block d) evidence of ST-segment elevation in family members e) incomplete right bundle branch block [6] f) initial electrocardiogram may be normal 2) provocative administration of a class Ia antiarrhythmic, such as procainamide may induce diagnostic electrocardiogram pattern * electrocardiogram plus 1 or more clinical manifestations (or complications) makes diagnosis # benign early repolarization generally has a positive T-wave Diagnostic criteria: - coved-type ST-segment elevation in ECG lead V1 & ECG lead V2 plus one of the following: - ventricular fibrillation or polymorphic ventricular tachycardia - family history of sudden cardiac death < 45 years of age - family members with coved-type ST-segment elevation ECGs - inducibility of ventricular tachycardia with programmed electrical stimulation - syncope - nocturnal agonal respiration Complications: - ventricular fibrillation - suddent cardiac death Management: 1) no intervention is required* [6] 2) hospital admission with ECG monitoring 3) cardiology consult (specialist in electrophysiology) 4) consider placement of a cardioverter-defibrillator - high risk for sudden death [6] - prior syncope or evidence of ventricular arrhythmia 5) evaluation of the patient's family members

Related

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)

Specific

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

General

syndrome

Database Correlations

OMIM 601144

References

  1. Antzelevitch C. The Brugada syndrome. J Cardiovasc Electrophysiol. 1998 May;9(5):513-6. Review. PMID: 9607460
  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: 9521325
  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: 9935001
  4. Medical Knowledge Self Assessment Program (MKSAP) 16, 17. American College of Physicians, Philadelphia 2012, 2015
  5. Mizusawa Y, Wilde AA. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012 Jun 1;5(3):606-16 PMID: 22715240
  6. DeFilippis EM, Vaidya A, Braun D et al A Shocking Turn of Events N Engl J Med. 2018 May 24;378(21):e29. PMID: 29791827 https://www.nejm.org/doi/full/10.1056/NEJMimc1710576