2012 年Brugada Syndrome 診斷與治療準則 - tsoc.org.tw

1 102 3 2 Brugada Syndrome (BrS) polymorphic ventricu-lar tachycardia ventricular fibrillation sudden cardiac death 2012 Paola Berne Josep Brugada Brugada Syndrome Review 1 Brugada Syndrome 1~5/10,000 12/10,000 80% 2 Brugada type type I II III 2012 type I & II coved type saddle back type 2012 Brugada Syndrome BrS 3 BrS V1 V2 sensitivity specifi city Brugada Syndrome BrS Type I ECG BrS BrS:1.

2 Family history: SCD in a family member younger than 45 years ECG type 1 in family members2. Arrhythmia-related symptoms: Syncope Seizures Nocturnal agonal respiration3. Documented ventricular arrhythmias: PVT VF. BrS V1 V2 102 3 4 BrS BrS Brugada Syndrome BrS Auto-somal dominant with incomplete penetrance BrS SCN5A mutations 20% 1% 300 SCN5A mutations Na+ channel Brugada Syndrome BrS Brugada-type ECG ECG vagotonic agents Class I AAD Brugada-type ECG baseline ECG BrS type I ECG Brugada Syndrome BrS repolarization depolarization Repolar-ization

3 Inward positive current outward positive current action potential notch ST segment Epicardium transmural repolarization dis-persion phase 2 reentry depolarization V1 V2 V1 V2 5 BrS 102 3 6 BrS BrS A Flecainide ECG B BrS type 1 ECG 7conduction delay BrS ECG long PR interval, com-plete bundle branch block EPS study prolonged H-V intervals body surface, en-docardial, epicardial mapping late potential Nademanee endo/epi mapping RVOT epicardium low voltage late potential prolonged duration fractionation 89% BrS SCD syncope ven-tricular arrhythmia SCD BrS ventricular arrhythmia 17-62% syncope BrS ventricular arrhythmia 6-19% ICD SCD Class I indication ventricular tachycardia SCD BrS ICD Class IIa indication BrS 1~8% EPS BrS Brugada et al EPS

4 Sustained ventricular arrhythmia cardiac event BrS BrS type 1 ECG EPS class IIb indication SCD BrS SCN5A trunca-tion mutations missense mutations Na channels BrS SCN5A missense mutations Na current BrS syncope Brugada Syndrome ICD BrS ICD SCD Isoproterenol ICal BrS electrical storm (Class IIa indica-tion) Quinidine Class Ia AAD Ito 102 3 8I-Kr BrS electrical storm (Class IIb indication) Dysopiramide orciprenaline electrical storm tedisamile Ito phos-phodiesterase III inhibitors cilostazol dimethyl lithospermate B Additional Management Recommendations BrS type 1 ECG PVT/VT ECG BrS Reference1.

5 Berne P, Brugada J. Brugada Syndrome 2012. Circ J 2012; 76: Bayes de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for di-agnosis of Brugada pattern: a consensus report. J Electrocardiol 2012; 45: 433-42.