Abstract

Introduction: Cardiac sarcoidosis (CS), often overlooked due to its myriad and non-specific presentation, is mainly understood through small studies. Hypothesis: What comprehensive evidence exists regarding the cumulative clinical outcomes in CS patients? Methods: A literature search in PubMed and EMBASE was conducted until September 8, 2022. Data from Kaplan-Meier curves were reconstructed using WebPlotDigitizer, with subsequent analysis was conducted via Cox proportional hazards models and non-parametric methods. Results: We reviewed 183 studies with 29,830 CS-diagnosed patients, and CS predominantly affected middle-aged, White, and Black individuals, with data primarily derived from Europe, North America, and Japan. The prevalent arrhythmias were ventricular arrhythmias (25%) and conduction block (low-degree, 21%; high-degree, 15%). Steroids were the primary treatment, with implantable cardioverter-defibrillators (ICD) used in 20% of cases (Image 1) . Survival meta-analysis showed that immunosuppressants significantly lower mortality risk in CS patients (HR: 0.52, 95% CI: 0.38-0.7, p < 0.0001) compared with the general CS population. ICD implanted for primary prevention in CS patients was associated with lower risk of inappropriate ICD therapy compared with ICD for secondary prevention (HR: 0.29, 95% CI: 0.19-0.44, p < 0.0001). Post-transplant survival was superior in CS patients than non-CS patients (HR: 0.58, 95% CI: 0.46-0.72, p < 0.0001) (Image 2) . Conclusions: This study is the first to provide comprehensive outcomes in CS patients. We encourage global research efforts to include underrepresented population in order to better understand CS.

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