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Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification (MAC), in which the core of the calcification undergoes a caseous transformation. CCMA can cause dysfunction of the mitral valve or embolization of caseous material, requiring surgery. There is currently no clear consensus on the optimal treatment strategy for CCMA.

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Unlabelled: Mitral annular calcification (MAC) is a chronic degenerative process involving the fibrous support structure of the mitral valve. The prevalence of this condition significantly increases with age, and is higher in patients with cardiovascular risk factors or end-stage renal disease. However, patients with systemic lupus erythematosus (SLE) may develop atherosclerosis and MAC at a relatively young age.

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We present 2 cases of caseous mitral annulus calcification (MAC) in which one patient was asymptomatic whereas the second experienced left hemianopsia. Both patients underwent transthoracic and transesophageal echocardiography exams which revealed severe MAC with a mass consistent with caseous MAC. A mobile component of the caseous MAC was observed in the patient with left hemianopsia.

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