Brugada’s Syndrome and Sudden Cardiac Death

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

Cardiac Chanellopathies?

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

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

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

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

Brugada Syndrome: ECG Coved ST Saddle-back ST

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

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.

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

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

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