11 Brugada Syndrome: ECGSpontaneous 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 deathsyncope, seizuresagonal nocturnal respirationsaffects 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 Thailandrecognized in Asia for decades:Philippines: Bangungut = scream followed by sudden death during sleepThailand: Lai tai = death during sleepJapan: Pokuri = unexpected death during sleep
15 12-lead ECG during administration of ajmaline (50 mg in 5 min). Brugada Syndrome: ECG12-lead ECG during administration of ajmaline (50 mg in 5 min).Note the progressive appearance of the ST elevation in V1-V3.
17 Brugada Syndrome: Genetics autosomal dominant60 different mutations in the SCN5A (chromosome 3) gene have been linked to the syndromefailure of alpha subunit of sodium channel to expressshift in voltage and time dependence on I(Na+) activation, inactivation or reactivation
19 Brugada Syndrome: Diagnosis symptoms: syncope, SCD (usually during sleep)physical exam: normalfamily history: strong history of SCDECG: best test to identify Brugada patientsmay require Flecainide or Procainamide to bring out typical findingsST elevation, RBBBimaging tests: usually no underlying structural diseasestress 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 hearts2. appearance of ECG marker after administration of Na+ channel blockersminor criteria:1. family history of sudden cardiac death2. syncope of unknown origin3. documented ventricular tachycardia/fibrillation4. genetic mutation of ion channels
21 Brugada Syndrome: Treatment IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR (ICD)symptomatic with the disease: ICDasymptomatic with family history of SCD: with normal ECG – OBSERVEasymptomatic with prolonged H-V interval and inducible VT/VF: controversial ICD