Transcript Brugada’s Syndrome and Sudden Cardiac Death

Brugada’s Syndrome
and Sudden Cardiac
Death
Brugada’s Syndrome and
Sudden Cardiac Death
Petra Lovrec Petra Lovrec
Sudden Cardiac Death
Incidence
(cases/year)
Survival
Worldwide
3 000 000
‹1%
U.S.
450 000
5%
W. Europe
400 000
‹5%
Causes of Sudden Cardiac Death
• 80% - coronary artery disease
• 15% - cardiomyopathy
• 5% - other
Cardiomyopathy
• dilated cardiomyopathy
• hypertrophic cardiomyopathy
• arrythmogenic right ventricular
cardiomyopathy
• left ventricular non-compaction
• restrictive cardiomyopathy
Cardiac Chanellopathies
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Brugada syndrome
long QT syndrome
short QT syndrome
idiopathic VF
short coupled Torsades
Brugada Syndrome
• first described in 1992 in 8 patients with
aborted sudden cardiac death
• history since 1986 – Brugada brothers
Brugada Syndrome
• characterized by:
– ECG findings of RBBB and persistent ST
elevation in V1 – V3
– structurally normal hearts
– propensity for life-threatening ventricular
arrhythmias
– J point elevation
Brugada Syndrome: ECG
Brugada Syndrome: ECG
Typical electrocardiogram of Brugada syndrome.
Note the pattern resembling a right bundle branch block,
the P-R prolongation and the ST elevation in leads V1-V3.
Brugada Syndrome: ECG
Brugada Syndrome: ECG
Spontaneous polymorphic ventricular tachycardia recorded
during monitoring in a patient with Brugada syndrome.
The arrhythmias are fast and need DC shock to terminate.
Brugada Syndrome: Clinical
Features
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unexpectad sudden death
syncope, seizures
agonal nocturnal respirations
affects male patients predominantly (8:1)
cases reported worldwide
Brugada Syndrome: Incidence and
Distribution
• responsible for up to 50% of sudden death
victims with structurally normal hearts in
Thailand
• recognized in Asia for decades:
– Philippines: Bangungut = scream followed by
sudden death during sleep
– Thailand: Lai tai = death during sleep
– Japan: Pokuri = unexpected death during
sleep
Brugada Syndrome: ECG
Brugada Syndrome: ECG
12-lead ECG during administration of ajmaline (50 mg in 5 min).
Note the progressive appearance of the ST elevation in V1-V3.
Brugada Syndrome: Factors /
Drugs that Enhance ECG Pattern
• Na+ channel blockers
• alpha agonists, vagotonic agents, beta
blockers
• fever
• alcohol, cocaine
• severe ischemia
• tricyclic antidepressants, antihistaminics
Brugada Syndrome: Genetics
• autosomal dominant
• 60 different mutations in the SCN5A
(chromosome 3) gene have been linked to
the syndrome
• failure of alpha subunit of sodium channel
to express
• shift in voltage and time dependence on
I(Na+) activation, inactivation or
reactivation
Brugada Syndrome: Diagnosis
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symptoms: syncope, SCD (usually during sleep)
physical exam: normal
family history: strong history of SCD
ECG: best test to identify Brugada patients
– may require Flecainide or Procainamide to bring out
typical findings
– ST elevation, RBBB
• imaging tests: usually no underlying structural
disease
• stress tests: symptoms and ECG findings not
usually reproducible with exercise
Brugada Syndrome: Diagnostic
Criteria
• major criteria:
– 1. presence of ECG marker in structurally normal
hearts
– 2. appearance of ECG marker after administration of
Na+ channel blockers
• minor criteria:
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1. family history of sudden cardiac death
2. syncope of unknown origin
3. documented ventricular tachycardia/fibrillation
4. genetic mutation of ion channels
Brugada Syndrome: Treatment
• IMPLANTABLE CARDIOVERTERDEFIBRILLATOR (ICD)
• symptomatic with the disease: ICD
• asymptomatic with family history of SCD: with normal
ECG – OBSERVE
• asymptomatic with prolonged H-V interval and inducible
VT/VF: controversial ICD
Potential Antiarrhythmic Drugs in
Brugada Syndrome
• Quinidine (non specific I blocker)
• Isoproterenol (open L type calcium
channel)
• Cilostazol (augment I Ca)
Conclusion
• sudden cardiac death in Southeast Asia
• characteristic ECG pattern – ST elevation
in V1 – V3
• ICD implantation in symptomatic patients
Thank you!