Brugada, Brugada syndrome, Syndrome,Sudden cardiac death. Death. drhasanmahmud@gmail.com -for further clarification.

1. BRUGADA SYNDROME
Presenter:
Dr Hasan Mahmud Iqbal
NHFH&RI.BD.

2. DEFINITION
Brugada syndrome(BrS) is an heritable
arrhythmia syndrome characterized by an ECG pattern
consisting of coved-type ST-segment elevation (≥2 mm)
followed by a negative T wave in the right precordial leads
V1 through V3 and increased risk for sudden death
resulting from episodes of polymorphic ventricular
tachyarrhythmias.
Ref:Braunwald’s Heart Disease: A textbook of cardiovascular medicine,10th Edition, 2015.

3. • Brugada syndrome- initially described by Josep and
Pedro Brugada in 1992.
• It is an autosomal dominant disease but mutation can
occur in 30% of cases.
• Genes have been identified as causing Brugada
syndrome are as follows:
- CACNA1C, CACNB2, GPD1L, HCN4,KCNE3, SCN1B,
SCN3B, and SCN5A.
• The SCN5A gene is a voltage-gated sodium channel
gene most commonly implicated and there is loss of
function of sodium channels.

4. CLINICAL PRESENTATION

5. Clinical Presentation
• The penetrance and expressivity of the Brugada
syndrome are highly variable and range from life-long
asymptomatic individuals to SCD during the first year of
life.
• It is a disorder of young male adults in Southeast Asian
regions with arrhythmogenic manifestations, first
arising at an average age of 40 years and sudden death
typically occur during sleep.
Ref: Braunwald’s Heart Disease: A textbook of cardiovascular
medicine,10th Edition, 2015.

6. Clinical Presentation
• Sudden unexplained nocturnal death in young males
is now considered phenotypically, genetically and
functionally the same disorder as Brugada Syndrome.
• A 2007 population study of 30 children (<16 years
of age) affected by Brugada, fever was the most
common precipitating factor for arrhythmic cardiac
events, including syncope and SCD.
Ref: Braunwald’s Heart Disease: A textbook of cardiovascular
medicine,10th Edition, 2015.

7. Type 1- ST segment elevated (>2mm), upward convexity + inverted T wave (“coved type”)
Type 2 - ST segment elevated (>2mm) ,saddle back configuration
Type 3- Morphology either Type 1 or Type 2 but ST elevation (< 2mm)

8. GUIDELINE FOR DX:
2013 expert consensus
statement:
Heart Rhythm
Society/European Heart
Rhythm Association/Asia
Pacific Heart Rhythm
Society
(HRS/EHRA/APHRS)
recommended a diagnosis
of Brugada syndrome
when the following criteria
are met:
1. ST-segment elevation with type I
morphology ≥2 mm in ≥1 lead(V1,V2)
2.Spontaneous ECG change or after
provocative drug test with IV
administration of Class I antiarrhythmic
drugs.( flecainide, procainamide)
3.Type 2 or type 3 ST-segment elevation
≥1(V1,V2) when a provocative drug test
with IV administration of class I
antiarrhythmic drugs induces a type I
ECG morphology.

9. 2011, the Heart Rhythm Society
(HRS) and the European Heart
Rhythm Association (EHRA) issued
a joint expert consensus statement
on genetic testing for channelopathies and
cardiomyopathies with the following
recommendations for BrS testing
Class I
Mutation specific genetic testing
should be done for family
members following identification
of BrS mutation.
GUIDELINE FOR DX:
Genetic Testing

10. ELECTROPHYSIOLOGIC STUDY
• Some investigators use an electrophysiologic study
(EPS) to determine the inducibility of arrhythmias, in
an effort to risk-stratify patients with Brugada
syndrome.
• However, the predictive value of this approach is
debated.
Priori S G, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, et al. Natural
history of Brugada syndrome: insights for risk stratification and management. Circulation.
2002 Mar 19. 105(11):1342-7.

11. MANAGEMENT

12. 2015 ESC GUIDELINES FOR MANAGEMENT
AND PREVENTIONS OF SCD:
Class I
Lifestyle changes:
 Avoidance of drugs that induce ST-segment
elevation in rt precordial leads.
 Avoidance of excessive alcohol and meals
 Prompt treatment of fever with antipyretic drugs.
ICD implantation should be done in patients who
are survivors of an aborted cardiac arrest and/or
have documented sustained VT.

13. 2013 HRS/EHRA/APHRS guidelines-
Class III
ICD implantation is not indicated in asymptomatic
BrS patients with a drug induced type I ECG and on the
basis of a family history of sudden cardiac death alone.

14. CLASS II
Quinidine due to its inhibitory effect on transient outward
current (Ito)may be beneficial in BrS with reduction in
inducible ventricular arrhythmias and clinical events.

15. Catheter Ablation
• Recent data suggested that manifestations of BrS
arise from electrophysiological abnormalities in the rt
ventricular outflow tract epicardium and catheter
ablation of abnormal signals in this area can
normalize ECG manifestation and prevent future
ventricular arrythmias.
Ref: Nademanne K, Veerakul G, ChandanamattaP, et at. Prevention of Ventricular Fibrillation
Episodes in Brugada Syndrome by Catheter Ablation Over the Anterior Right Ventricular Outflow
Tract Epicardium. Circulation. 2011;123:1270-1279

16. Thank You All