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Background  Brugada syndrome (BrS) is an inherited primary arrhythmia disorder leading to sudden cardiac arrest.   SCN 5A , encoding the α‐subunit of the cardiac sodium channel (Nav1.5), is the most common pathogenic gene of BrS. An implantable cardioverter defibrillator ( ICD ) is the standard treatment for secondary prevention. This study aimed to evaluate association of the   SCN 5A  variant with this cardiac conduction disturbance and appropriate  ICD  shock therapy in Thai symptomatic BrS patients with  ICD  implants.    Methods and Results  Symptomatic BrS patients diagnosed at university hospital were enrolled from 2008 to 2011. The primary outcome of the study was an appropriate  ICD  shock defined as having non‐pacing‐associated  ICD  shock after the occurrence of ventricular tachycardia or ventricular fibrillation. Associations between   SCN 5A  polymorphisms, cardiac conduction disturbance, and potential confounding factors associated with appropriate  ICD  shock therapy were analyzed. All 40 symptomatic BrS patients (median age, 43 years) with  ICD  implantations were followed for 24 months. There were 16 patients (40%) who had the appropriate  ICD  shock therapy after  ICD  treatment. An independent factor associated with appropriate  ICD  shock therapy was   SCN 5A‐ R1193Q with an adjusted hazard ratio of 10.550 (95%  CI , 1.631–68.232).    Conclusions    SCN 5A‐ R1193Q is associated with cardiac conduction disturbances. It may be a genetic marker associated with ventricular arrhythmia leading to appropriate  ICD  shock therapy in symptomatic BrS patients with  ICD  treatment. Because of the small sample size of study population and the appropriate  ICD  shock outcome, further large studies are needed to confirm the results of this study.

SCN 5A Genetic Polymorphisms Associated With Increased Defibrillator Shocks in Brugada Syndrome