Outcome After Implantable Cardioverter-Defibrillator In Patients With Inherited Cardiac Arrhythmias

[Objective] To investigate the efficacy of implantable cardioverter-defibrillator(ICD) in patients with inherited cardiac arrhythmias.[Methods] A retrospective analysis is conducted in patients with Long-QT syndromes (LQTS), Brugada syndrome, Hypertrophic cardiomyopathy and Arrhythmogenic right ventricular cardiomyopathy (ARVC) who underwent ICD implantation between2004and2011in Fuwai Hospital.[Results]43patients (mean age41.5±15.8years;58%male) were enrolled. During the follow-up period of37.0±20.6months, nobody died and21patients(48.8%) had ventricular tachycardia/ventricular fibrillation(VT/VF),while5of whom were due to inappropriate detection.1patient had an undetected sustained ventricular tachycardia episode. A total of382episodes of VT/VF were documented and ICDs intervened498times by delivering381ATP,83CV and34SHOCK.51.6%of VT episodes were successfully terminated by ATP. The average time from implantation to the first documented VT/VF episode was6.4±8.9months. The cumulative event rate was84.6%at1-year follow-up. Inappropriate therapies accounted for47.6%of all therapy episodes and were mainly caused by T wave oversensing.3patients experienced "firing storm" during the follow-up,2/3were caused by inappropriate detections and1/3finally resulted in battery depletion.[Conclusion] Patients with inherited cardiac arrhythmias have a high recurrence rate of ventricular malignant arrhythmias, and ICD is effective in preventing these patients from sudden cardiac death. However, inappropriate detection/therapy and firing storm should be payed more attention in this population.