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Background and Purpose: Candida endocarditis is an infrequent disease with a high mortality rate, which commonly occurs in immunosuppressed patients with cardiac valve replacement. We reported a 70-year-old woman diagnosed with Candida prosthetic valve endocarditis (PVE). This study also involved a review of all published cases of Candida PVE from 1970. Case report: Herein, we reported a 70-year-old woman with the history of severe mitral stenosis and myelodysplasia syndrome. She underwent mitral valve replacement for two times. The blood cultures were positive, and phenotypic identification of the isolates at the species level was performed based on microscopic and macroscopic characteristics. In the second prosthetic valve replacement, huge fungal white and creamy vegetation was observed which was identified as Candida albicans based on the conventional and molecular methods. Despite the administration of antifungal treatments, the patient passed away probably due to the multidrug-resistant Candida PVE. Conclusion: As PVE is a late consequence of prosthetic valve replacement, extended follow-up visits, early diagnosis, repeating valve replacement surgeries, and timely selective antifungal treatments are warranted. Keywords: Amphotericin B, Antifungal resistant, Azoles, Candida endocarditis, Multi-drug resistant, Myelodysplasia syndrome, Prosthetic valve replacement

Introduction: Fungal prosthetic valve endocarditis (PVE) is rare but serious complication of valve replacement surgery. Candida species, particularly Candida albicans is the most common isolated pathogen in fungal PVE (1–6%of cases). Case Presentation: We describe a 35-year-old woman who underwent mechanical mitral valve replacement about 3 years ago. She was admitted with neurological symptoms and later with dyspnea and hypotension. Transesophageal echocardiography showed large and mobile prosthetic valve vegetation. She underwent mitral valve surgery. The explanted valve and vegetation revealed lots of budding yeasts and the isolated yeast was identified as C. parapsilosis. Amphotericin B and broad spectrum antibiotic were started immediately. Unfortunately, the patient died two days after surgery, due to sepsis probably related to the candidemia. Conclusions: Fungal endocarditis is uncommon infection, but it is a serious problem in patients with prosthetic valve. Fungal PVE can occur years after the surgery, thus long-term follow-up is essential.