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Brugada Syndrome

Carly Thompson
MD CCFP
EM Resident
July 31, 2008

Overview

Importance of Brugada Syndrome


ECG Changes in Brugada Syndrome
Epidemiology
Pathogenesis
Diagnosis
Treatment
ECG Practice

Sudden Cardiac Arrest


Cardiac Arrest in a Structurally Normal
Heart

Long QT Syndrome
Preexcitation Syndrome
Commotio cordis
Brugada Syndrome

Importance of Brugada
Syndrome
Mortality rate up to 10% / year in
untreated patients with typical ECG
changes!

Importance of Brugada
Syndrome
Brugada

syndrome can be identified


by ECG and successfully treated by
ICD

Brugada Syndrome: ECG


Changes
First

described in 1992 by J & P


Brugada

What is It?
Pseudo-RBBB
ST Elevation V1-V3

Normal vs RBBB vs Brugada


Syndrome

Normal

RBBB

QRS 120ms
Terminal R wave in V1 (RSR1)
Slurred S wave in I and V6

Brugada Syndrome

PseudoRBBB
(but no
slurred S
in V6)
ST
Elevation
V1-V3
T wave
inversion
= Brugada
Syndrom
e ECG

ST Patterns in Brugada
Syndrome
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

What Type is It?


A

3 Different Patterns
Feature

Type 1

Type 2

Type 3

J wave

2mm

2mm

2mm

T wave

Negative Positive or
biphasic
Coved
Saddlebac
k
Gradual Elevated
descent 1mm

ST-T
ST
Segment
Terminal
Portion

Positive
Saddlebac
k
Elevated
<1mm

Transient Nature

Epidemiology

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

Pathogenesis
Genetics

Autosomal dominant inheritance with


variable expression
Cardiac sodium channel gene
No structural abnormalities

Arrhythmias in Brugada
Syndrome
Ventricular

Arrhythmias

Localized reentry -> PVCs -> VT or VF


Atrial

Fibrillation

More common!

Provoking Factors
Sodium Imbalances
Drugs: Cocaine
TCAs and Neuroleptics
in overdose
Sodium channel
blockers: procainamide
Electrolyte Imbalances:
Sodium, Calcium
Lithium

Drugs
B-blockers
Local anesthetics
Autonomic Tone
Fever
Night
Valsalva
Pacing

Risk Factors for Sudden


Cardiac Death
Male
Family

history
Abnormal ECG
Inducible VT or VF
Previous syncope
19% arrhythmia in 33 months
Previous

arrest

62% arrhythmia in 33 months

Outcome for Brugada


Patients

Diagnosis
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

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

Case 1

Male 62 years old presents to Foothills

Hx
Presented to family MD, asymptomatic
No syncope
No family history of sudden cardiac death
PMHx
Htn, Hyperlipidemia

RBBB + ST Elevation V1-V3


Type 1 Brugada ECG Pattern

Case 1
Referred

to electrophysiology for
further testing, and possible ICD
implantation.

Case 2
29 year old male
Hx
Cocaine use
No personal or family hx of syncope,
sudden cardiac death

RBBB, ST elevation V1-V3, T wave


inversion
Brugada-Type 1 ECG Changes

Case 2
ECG

when not using cocaine


normalized over several days
IV Procainamide failed to produce
Brugada changes
Diagnosis
Cocaine-induced ECG changes

Case 3
29 year old Female
Hx
Presented to the ER after 3 episodes of palpitations
over 3 days, and a feeling of impending doom
Hx of sudden cardiac death in uncle at age 45
No hx of syncope
PMHx
Healthy
Meds
No medications

Subtle downsloping of ST in V1 and V2

Case 3
Cardiology

consult:
Patient was admitted to hospital
Procainamide challenge -> VT
ICD placed
Patient discharged home in stable
condition

Summary
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.

References

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.

Questions?

Thanks for
listening!