Background: To evaluate the risk for ventricular arrhythmia (VA) and sudden cardiac death (SCD) in patients with cardiac sarcoidosis (CS) and determine the prognostic factors.
Methods and results: PUBMED, EMBASE and SCOPUS were searched up to 14th April 2020. Studies reporting the incidence of SCD, appropriate ICD therapy in CS patients, or relevant prognostic information in patients having undergone MRI, PET, or programmed electrical stimulation (PES) were included. Nineteen studies consisting of 1247 patients, reported the risk of ICD therapies or SCD over a follow-up period of 1.7-7 years. 22.7% (n = 9; 22.7, 95%CI [16.10-29.36]) of patients in primary and 58.4% (n = 9; 58.42, 95% CI [38.61-78.22]) in secondary prevention cohorts experienced appropriate device therapy or SCD events. 18% (n = 2; 18, 95%CI [14-23]) of patients received ≥5 appropriate therapies. 9 out of 664 patients with confirmed cardiac sarcoidosis but without implanted ICDs died suddenly. 17.9% of patients (n = 4; 17.9, 95%CI [10.80-25.03]) experienced inappropriate device therapy. Positive LGE-MRI and PES were associated with an 8.6-fold (n = 6; RR = 8.60, 95%CI [3.80-19.48]) and 9-fold (n = 5; RR = 9.07, 95%CI [4.65-17.68]) increased risk of VA respectively. Positive LGE-MRI and PET with associated with a 6.8-fold (n = 12; RR = 6.82, 95%CI [4.57-10.18]) and 3.4-fold (n = 7; RR = 3.41, 95%CI [2.03-5.74]) respectively for increased risk of major adverse cardiac events.
Conclusions: The risk of appropriate ICD therapy or sudden cardiac death is high in patients with CS. The presence of LGE-MRI and positive electrophysiology study identify patients at increased risk of ventricular arrhythmias. [CRD42019124220].
Keywords: Cardiac sarcoidosis; EPS; LGE-MRI; PES; Sudden death; Ventricular arrhythmias.
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