Brugada syndrome
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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
- abrupt
- no prodrome, no orthostatic association
- self-terminating polymorphic ventricular tachycardia
- family history of sudden cardiac death in a relative younger than 45 years
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
Diagnostic procedures
- electrocardiogram*
- precordial ST-segment elevation (downsloping) in leads V1-V3 typically followed by a negative T wave#
- J point elevation
- QRS morphology resembling right bundle branch block
- evidence of ST-segment elevation in family members
- incomplete right bundle branch block[6]
- initial electrocardiogram may be normal
- 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
Complications
- ventricular fibrillation
- suddent cardiac death
Management
- no intervention is required*[6]
- hospital admission with ECG monitoring
- cardiology consult (specialist in electrophysiology)
- consider placement of a cardioverter-defibrillator
- high risk for sudden death[6]
- prior syncope or evidence of ventricular arrhythmia
- evaluation of the patient's family members
More general terms
More specific terms
Additional terms
References
- ↑ Antzelevitch C. The Brugada syndrome. J Cardiovasc Electrophysiol. 1998 May;9(5):513-6. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/9607460
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/9521325
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/9935001
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 16, 17. American College of Physicians, Philadelphia 2012, 2015
- ↑ Mizusawa Y, Wilde AA. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012 Jun 1;5(3):606-16 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22715240
- ↑ 6.0 6.1 6.2 6.3 DeFilippis EM, Vaidya A, Braun D et al A Shocking Turn of Events N Engl J Med. 2018 May 24;378(21):e29. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29791827 https://www.nejm.org/doi/full/10.1056/NEJMimc1710576