1. Brugada’s Syndrome and Sudden Cardiac Death

2. Causes of Sudden Cardiac Death
80% - coronary artery disease
15% - cardiomyopathy (dilated, etc)
5% - other.
Cardiac Chanellopathies

3. Cardiac Chanellopathies?

4. Cardiac Chanellopathies
Brugada syndrome
long QT syndrome
short QT syndrome
idiopathic VF

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

6. Brugada Syndrome: Incidence and Distribution
Inherited condition. A. D. Inheritance.
responsible for up to 50% of sudden death victims with structurally normal hearts worldwide.
First 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

7. 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
imaging tests: usually no underlying structural disease
stress tests: symptoms and ECG findings not usually reproducible with exercise

8. Brugada Syndrome: ECG
Coved ST
Saddle-back ST

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
Spontaneous polymorphic ventricular tachycardia recorded during monitoring in a patient with Brugada syndrome. The arrhythmias are fast and need DC shock to terminate.

11. 12-lead ECG during administration of procainamide.
Brugada Syndrome: ECG
12-lead ECG during administration of procainamide.
Note the progressive appearance of the ST elevation in V1-V3.

12. 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

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

17. Conclusion sudden cardiac death.
characteristic ECG pattern – ST elevation in V1 – V3
ICD implantation in symptomatic patients.
Genetics therapy.
More screening program. (Qatar)

19. Thank you!