Anna Terra França

Introduction: Gain‐of‐function variants in SCN5A may cause multifocal ectopic Purkinje‐related premature contractions (MEPPC), with a high premature ventricular contraction burden that may lead to PVC‐induced cardiomyopathy. Pharmacological therapy is usually required, as catheter ablation is ineffective because of the multifocal origin of the ectopy. Methods and Results: We report two siblings with the pathogenic SCN5A p.Arg222Gln variant. In both, ranolazine markedly suppressed ventricular ectopy, improved or preserved left ventricular function, and enabled amiodarone withdrawal. Conclusion: Ranolazine may represent a useful mechanism‐guided option for SCN5A‐related MEPPC.

Introduction: Despite advancements in implantable cardioverter‐defibrillator (ICD) technology, sudden cardiac death (SCD) remains a persistent public health concern. Chagas disease (ChD), prevalent in Brazil, is associated with increased ventricular tachycardia (VT) and ventricular fibrillation (VF) events and SCD compared to other cardiomyopathies.Methods: This retrospective observational study included patients who received ICDs between October 2007 and December 2018. The study aims to assess whether mortality and VT/VF events decreased in patients who received ICDs during different time periods (2007–2010, 2011–2014, and 2015–2018). Additionally, it seeks to compare the prognosis of ChD patients with non‐ChD patients. Time periods were chosen based on the establishment of the Arrhythmia Service in 2011. The primary outcome was overall mortality, assessed across the entire sample and the three periods. Secondary outcomes included VT/VF events and the combined outcome of death or VT/VF.Results: Of the 885 patients included, 31% had ChD. Among them, 28% died, 14% had VT/VF events, and 37% experienced death and/or VT/VF. Analysis revealed that period 3 (2015–2018) was associated with better death‐free survival (p = .007). ChD was the only variable associated with a higher rate of VT/VF events (p < .001) and the combined outcome (p = .009).Conclusion: Mortality and combined outcome rates decreased gradually for ICD patients during the periods 2011–2014 and 2015–2018 compared to the initial period (2007–2010). ChD was associated with higher VT/VF events in ICD patients, only in the first two periods.