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Aim: We investigated the prognostic relevance of serum carbohydrate antigen 125 (CA125) levels in non-ischemic dilated cardiomyopathy (NICMP) and assessed whether increased levels relate to the degree of functional mitral regurgitation (FMR).Methods and Results: Seventy-seven patients with NICMP were enrolled and followed-up for 10 ± 2 months. FMR was assessed by using the PISA and vena contracta methods. ROC analysis established a cut-off CA125 value of 25 U/mL for predicting mortality. Patients were divided into two groups as: CA125 < 25 U/mL (n=58) and CA125 > 25 U/mL (n=19). Patients with high CA125 values had statistically worse functional status, higher BNP levels, higher LV volumes, lower EF, higher E/Em ratio, higher PAP, and more severe FMR. On the multivariate analysis, serum CA125 (p=0.002) and severe FMR (p=0.04) were identified as the independent predictors of mortality. Serum CA125 levels also correlated with BNP levels and FMR severity statistically.Conclusion: CA125 is a powerful prognostic biomarker that is associated with several echocardiographic parameters including LV volumes, systolic and diastolic functions, pulmonary artery pressure and the degree of FMR. Serum CA125 was also shown as an independent predictor of mortality during 10 ± 2 months of follow-up.