1. Brugada’s Syndrome and Sudden Cardiac Death
Petra Lovrec
Mentor: A. Žmegač Horvat
Petra Lovrec

2. Sudden Cardiac Death Incidence (cases/year) Survival Worldwide
‹1%
U.S. 5%
W. Europe
‹5%

3. Causes of Sudden Cardiac Death
80% - coronary artery disease
15% - cardiomyopathy
5% - other

4. Cardiomyopathy dilated cardiomyopathy hypertrophic cardiomyopathy
arrythmogenic right ventricular cardiomyopathy
left ventricular non-compaction
restrictive cardiomyopathy

5. Cardiac Chanellopathies
Brugada syndrome
long QT syndrome
short QT syndrome
idiopathic VF
short coupled Torsades

6. Brugada Syndrome
first described in 1992 in 8 patients with aborted sudden cardiac death
history since 1986 – Brugada brothers

7. 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

8. Brugada Syndrome: ECG

9. 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.

10. Brugada Syndrome: ECG

11. 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.

12. Brugada Syndrome: Clinical Features
unexpectad sudden death
syncope, seizures
agonal nocturnal respirations
affects male patients predominantly (8:1)
cases reported worldwide

13. 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

14. Brugada Syndrome: ECG

15. 12-lead ECG during administration of ajmaline (50 mg in 5 min).
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.

16. 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

17. 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

19. Brugada Syndrome: Diagnosis
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

20. 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:
1. family history of sudden cardiac death
2. syncope of unknown origin
3. documented ventricular tachycardia/fibrillation
4. genetic mutation of ion channels

21. Brugada Syndrome: Treatment
IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR (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

25. Potential Antiarrhythmic Drugs in Brugada Syndrome
Quinidine (non specific I blocker)
Isoproterenol (open L type calcium channel)
Cilostazol (augment I Ca)

26. Conclusion sudden cardiac death in Southeast Asia
characteristic ECG pattern – ST elevation in V1 – V3
ICD implantation in symptomatic patients

27. Thank you!