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

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.

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 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: 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 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 in Southeast Asia characteristic ECG pattern – ST elevation in V1 – V3 ICD implantation in symptomatic patients

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