This document discusses Brugada syndrome, a condition characterized by abnormal ECG findings and increased risk of sudden cardiac death. It presents information on the epidemiology, pathogenesis, diagnosis, and treatment of Brugada syndrome. Key points include that it is more common in males and Asians, and symptoms can include palpitations or syncope. Diagnosis involves specific ECG patterns and drug challenge may be used. Treatment focuses on managing arrhythmias using medications like quinidine or implantable cardioverter-defibrillators, with the latter being the only proven effective treatment.

2.  Importance of Brugada Syndrome
 ECG Changes in Brugada Syndrome
 Epidemiology
 Pathogenesis
 Diagnosis
 Treatment

3.  Brugada syndrome (BrS) is a
genetically inherited condition
that is characterised by
abnormal electrocardiogram
(ECG) findings and an
increased risk of sudden
cardiac death
 also known as sudden adult
death syndrome (SADS)
 this is often referred to as a
sodium channelopathy

4.  Often patients are Asian
 Prevalence:
› Japan 1.0%, Type 1 is common up to 0.16%
› Finland 0.6%, Type 1 is rare
› USA 0.4%
 Gender:
› Male (Up to 9x more common!)
 Children
› consider fever, syncope
 Age
› average age of diagnosis is 41

5.  Genetics
› Autosomal dominant inheritance with variable
expression
› Cardiac sodium channel gene
› No structural abnormalities

7. Cardiac Arrest in a Structurally Normal Heart
› 4 ~ 12% of all sudden death and 20% of sudden
death in patients with structurally normal heart
› Long QT Syndrome
› Preexcitation Syndrome
› Commotio cordis
› Brugada Syndrome
However Brugada Syndrome may be responsible for 20% of these sudden cardiac
arrest deaths

8.  First described in 1992 by J & P Brugada
brothers
What is It?
 Pseudo-RBBB
 ST Elevation V1-V3

9. Mortality rate up to 10% / year in untreated
patients with typical ECG changes!
polymorphic VT

10.  Normal
 RBBB
› QRS ≥120ms
› Terminal R wave in V1 (RSR1
)
› Slurred S wave in I and V6
 Brugada Syndrome

11. Type 1 “Coved Type”
 J wave ≥ 2mm convex
 ST segment descends
 Inverted T wave
Type 2 “Saddle back”
 J wave ≥ 2mm
 ST segment ≥1mm
 Upright or biphasic T
Type 3 “Saddle back”
 J wave ≥2mm
 ST segment <1mm
 Positive T wave

12. A B C

13.  Pseudo-
RBBB
(but no
slurred S
in V6)
 ST
Elevation
V1-V3
 T wave
inversion
= Brugada
Syndrom
e ECG

15.  Ventricular Arrhythmias
› Localized reentry -> Premature Ventricular
Contractions (PVCs)-> VT or VF
 Atrial Fibrillation
› More common!
Due to the impaired sodium channels that
cause a local conduction block and shorter
refractory period there is localized reentry
causing coupled PVC that can precipitate
ventricular arrhythmias.

16. Sodium Imbalances
 Drugs: Cocaine
 TCAs(Tricyclic
antidepressants) and
Neuroleptics in overdose
 Sodium channel blockers:
procainamide
 Electrolyte Imbalances:
Sodium, Calcium
 Lithium
Drugs
 B-blockers
 Local anesthetics
Autonomic Tone
 Fever
 Night
 Valsalva
 Pacing(anxiety)

17.  Male
 Family history
 Abnormal ECG
 Inducible VT or VF
 Previous syncope
› 19% arrhythmia in 33 months
 Previous arrest
› 62% arrhythmia in 33 months

18. Type 1 ECG changes +
 Documented VF, VT
 Family hx of sudden cardiac death
 Family members with ECG changes
 Inducible VT
 Unexplained syncope probable VT/VF
 Nocturnal agonal respiration
Type 2 and 3
 Type 1 ECG induced with sodium channel blocker
 And criteria above

20.  Ajmaline 1mg/kg over 5min. IV
 Flecainide 2mg/kg over 10 min. IV
(max 150mg)
 Procainamide 10mg/kg over 10min. IV
 Pilsicainide 1mg/kg over 10min. IV

21. Quinidine
 Less inducible VT
Amiodarone
 For patients with frequent discharges
Implantable Cardioverter-Defibrillator (ICD)
 Only treatment with proven efficacy

23.  Brugada syndrome can be identified by ECG
and successfully treated by ICD

24.  This study of 334 patients with diagnosed Brugada
syndrome shows the outcome depending on their
presentation. Those who presented asymptomatically
with ECG changes had the best outcome, while those
with syncope did not do as well, and those with cardiac
arrest were likely

25.  Think of Brugada syndrome in a patient with
palpitations or syncope!
› Pseudo-RBBB
› ST Elevation V1-V3
› Family history of sudden cardiac death
 Send patients with suspicious ECGs to
cardiology / electrophysiology for drug
challenge or electrophysiology testing.

26.  Brugada. Brugada Syndrome: The Official Website of
the Ramon Brugada Senior Foundation.
http://www.brugada.org/
 Laszlo et al. Brugada-type electrocardiographic pattern
induced by cocaine. Mayo Clin Proc. 2000;75:845-849.
http://www.mayoclinicproceedings.com/inside.asp?
AID=1503&UID
 Watrich et al. Brugada syndrome in a young patient
with palpitations. CJEM 2005; 7(5): 347.
http://www.caep.ca/template.asp?
id=D12C3F88B51A46ED8A7848CD24B9A9C6
 Wylie et al. Brugada syndrome and sudden cardiac
arrest. Up To Date. June 2008.

27. Thanks for listening!