Abstract Prosthetic valve endocarditis is a rare complication following surgical aortic valve replacement, accounting for a small fraction of all endocarditis cases. This complication is typically caused by bacterial pathogens and carries a high mortality rate. In rare cases, prosthetic valve endocarditis may be attributed to fungal organisms. We present the case of a 47-year-old woman who developed prosthetic valve endocarditis from a fungus known as Exophiala dermatitidis. Prosthetic valve endocarditis caused by this pathogen is extremely rare, with only 3 previously reported cases of prosthetic valve endocarditis attributed to this fungus since 1969. Review of the previous literature revealed an additional 3 cases originating in native aortic valves. These cases tended to occur in relatively young patients and those with risk factors for infective endocarditis such as immunosuppression and intravenous drug use. Septic emboli to various organs, particularly the brain and spleen, appear to be a complication of fungal endocarditis caused by this organism. Management can be difficult due to the lack of guidelines to treat such rare infective complications, but replacement of the infected valve and a prolonged course of antifungal therapy appear to be effective in some cases.
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