RESUMEN
BACKGROUND: Patients with inherited arrhythmogenic diseases (IADs) are often prescribed preventative implantable cardioverter-defibrillators (ICDs) to manage their increased sudden cardiac arrest risk. However, it has been suggested that ICDs in IAD patients may come with additional risk. We aimed to leverage the PainFree SmartShock Technology dataset to compare inappropriate therapies, appropriate therapies, mortality, and complications in patients with and without IAD. METHODS: This retrospective analysis included extracted, physician-adjudicated, arrhythmic episodes from ICD devices. The incidence of arrhythmic events was estimated with the Kaplan-Meier method using the log-rank test. Cox proportional hazards regression was used to estimate hazard ratios (HRs) with their 95% confidence intervals (CIs). RESULTS: Of the 1699 ICD patients, 77 patients (4.5%) had IAD. Incidence of inappropriate shock was similar in both patients with (3.2% at 24 months) and without (3.8% at 24 months) IAD (HR: 0.80, CI: 0.19-3.30, p = 0.76). In a multivariable analysis IAD was not significantly associated with reduced mortality (HR: 0.64, CI: 0.08-4.80, p = 0.66). The rates of complications were numerically lower in patients with IAD vs without (8.8% vs 9.6% at 24 months respectively), but not statistically significant (HR: 0.83, CI: 0.20-3.38, p = 0.79). CONCLUSIONS: IAD patients showed a very low annual rate of inappropriate therapy. This suggests that newer algorithms, such as the SST algorithm, are equally good at identifying and treating life-threatening arrhythmias in patients regardless of whether they have IAD.