Transcript 2008_07_31-Thompson-Brugada_syndrome

Brugada Syndrome
Carly Thompson
MD CCFP
EM Resident
July 31, 2008
Overview
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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
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Brugada syndrome can be identified by
ECG and successfully treated by ICD
Brugada Syndrome: ECG Changes
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First described in 1992 by J & P Brugada
What is It?
 Pseudo-RBBB
 ST Elevation V1-V3
Normal vs RBBB vs Brugada Syndrome
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Normal
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RBBB
– QRS ≥120ms
– Terminal R wave in V1 (RSR1)
– Slurred S wave in I and V6
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Brugada Syndrome
PseudoRBBB
(but no
slurred S
in V6)
 ST
Elevation
V1-V3
 T wave
inversion
= Brugada
Syndrome
ECG
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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
B
C
3 Different Patterns
Feature
Type 1
Type 2
Type 3
J wave
≥2mm
≥2mm
≥2mm
T wave
Negative
ST-T
Coved
Positive or Positive
biphasic
Saddleback Saddleback
ST Segment
Terminal
Portion
Gradual
descent
Elevated
≥1mm
Elevated
<1mm
Transient Nature
Epidemiology
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Prevalence:
– Japan 1.0%, Type 1 is common up to 0.16%
– Finland 0.6%, Type 1 is rare
– USA 0.4%
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Gender:
– Male (Up to 9x more common!)
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Children
– consider fever, syncope
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Age
– average age of diagnosis is 41
Pathogenesis
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Genetics
– Autosomal dominant inheritance with variable
expression
– Cardiac sodium channel gene
– No structural abnormalities
Arrhythmias in Brugada Syndrome
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Ventricular Arrhythmias
– Localized reentry -> PVCs -> VT or VF
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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
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– 19% arrhythmia in 33 months
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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
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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
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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
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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
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Summary
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Think of Brugada syndrome in a patient
with palpitations or syncope!
– Pseudo-RBBB
– ST Elevation V1-V3
– Family history of sudden cardiac death
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Send patients with suspicious ECGs to
cardiology / electrophysiology for drug
challenge or electrophysiology testing.
References
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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=D12C3F88B51A46E
D8A7848CD24B9A9C6
Wylie et al. Brugada syndrome and sudden cardiac
arrest. Up To Date. June 2008.
Questions?
Thanks for listening!