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The available literature suggests high morbidity and mortality. To better characterize the shared experience of these cases and their outcomes, we performed a systematic review. We investigated all published reports of systemic fungal infections\u2014fungemia and fungal vegetative disease\u2014in the context of CIED, drawing from PubMed, EMBASE, and the Cochrane database of systematic reviews, inclusive of patients who received treatment between January 2000 and May 2020. Exclusion criteria included presence of ventricular assist device and concurrent bacteremia, bacterial endocarditis, bacterial vegetative infection, or viremia. Among 6261 screened articles, 48 cases from 41 individual studies were identified. <jats:italic>Candida<\/jats:italic> and <jats:italic>Aspergillus<\/jats:italic> species were the most commonly isolated fungi. There was significant heterogeneity in antifungal medication selection and duration. CIED extraction\u2014either transvenous or surgical\u2014was associated with increased survival to hospital discharge (92%) and clinical recovery at latest follow\u2010up (81%), compared to cases where CIED extraction was deferred (56% and 40%, respectively). Importantly, there were no prospective data, and the data were limited to individual case reports and one small case series. In summary, CIED extraction is associated with improved fungal clearance and patient survival. Reported antifungal regimens are heterogeneous and nonuniform. Prospective studies are needed to verify these results and define optimal antifungal regimens.<\/jats:p>","DOI":"10.1111\/pace.14090","type":"journal-article","created":{"date-parts":[[2020,10,14]],"date-time":"2020-10-14T13:24:34Z","timestamp":1602681874000},"page":"159-166","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Management of systemic fungal infections in the presence of a cardiac implantable electronic device: A systematic review"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6214-5542","authenticated-orcid":false,"given":"Jayson R.","family":"Baman","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine Northwestern University Feinberg School of Medicine  Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ankit N.","family":"Medhekar","sequence":"additional","affiliation":[{"name":"Department of 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