Brugada syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The Brugada syndrome is a genetic disease that is characterized by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death in young adults, and occasionally in children and infants.
Historical Perspective
The Brugada brothers were the first to describe the characteristic ECG findings and link them to sudden death.
Before that the characteristic ECG findings were often mistaken for a right ventricular myocardial infarction. In 1953 a publication by Oscher mentioned that despite being mistaken for right ventricular myocardial infarction, the ECG findings were not associated with myocardial ischemia.[1]
Although the ECG findings of Brugada syndrome were first reported[2] among survivors of cardiac arrest in 1989, it was only in 1992 that the Brugada brothers[3] recognized it as a distinct clinical entity, causing sudden death by causing ventricular fibrillation.
Epidemiology and Demographics
Insofar as Brugada syndrome is a relatively newly recognized syndrome, its incidence and prevalence continues to increase. Brugada syndrome is quite common in Southeast Asia where it is endemic, and affects 50 out of every 10,000 individuals. It is the second leading cause of death after car accidents among young people in these countries. It has been estimated that Brugada syndrome accounts for 4% of all sudden cardiac deaths and 20% of sudden cardiac deaths among patients with structurally normal hearts. It is 8-10 times more common in men.
EKG Characteristics
As shown by the racing below, The EKG characteristics of Bugada syndrome include:
- a) A broad P-wave with some PQ prolongation
- b) J point elevation in the right precordial leads (V1-V3)
- c) Coved ST segment elevation
- d) An inverted T wave
Provocation of EKG Changes
The findings of Brugada syndrome are often concealed, but can be unmasked or modulated by a number of drugs and pathophysiological states including sodium channel blockers, a febrile state, vagotonic agents that mimic sleep, tricyclic antidepressants, as well as cocaine and Propranolol intoxication.
References
- ↑ OSHER HL, WOLFF L (1953). "Electrocardiographic pattern simulating acute myocardial injury". The American Journal of the Medical Sciences. 226 (5): 541–5. PMID 13104407. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, Daliento L, Dalla Volta S. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J 1989 Dec;118(6):1203-9 PMID 2589161
- ↑ Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992 Nov 15;20(6):1391-6. PMID 1309182