Abstract
INTRODUCTION: Sarcoidosis is a systemic inflammatory disorder associated with ventricular arrhythmias (VAs) and sudden death in the context of cardiac involvement. Guidelines advocate implantable cardioverter-defibrillator (ICD) implantation in specific subcohorts, but there is a paucity of data on outcomes. METHODS AND RESULTS: A systematic review was performed to assess outcomes in patients with definite or probable cardiac sarcoidosis (CS) treated with ICD. Observational studies were identified from multiple databases from inception to 21st May 2021. Outcomes of interest included appropriate and inappropriate ICD therapies in addition to all-cause mortality. Study quality was assessed individually using the Newcastle Ottawa Scale (NOS).Eight studies were identified comprising 530 patients, with follow-up period of 24-66 months (weighted average 40 months). Mean age was 53.9 years with ejection fraction of 41.3%. Overall incidence of appropriate therapy was 38.1% during follow-up. Left ventricular systolic dysfunction (LVSD) with ejection fraction <40% was a predictor of appropriate therapy in the majority of studies, as were sustained VAs during electrophysiological testing (EP) in one study. There was no interaction with device indication (i.e. primary or secondary). Where documented, inappropriate therapy was primarily driven by atrial arrhythmias. All-cause mortality was 6.0% over a median follow-up period of 42 months. Only three studies achieved good quality in the comparability domain of NOS. CONCLUSIONS: Appropriate ICD therapy in patients with CS is commonly associated with LVSD, which can act as a surrogate for scar burden. The utility of EP testing in this setting remains unclear.