Caseous mitral annular calcification: Is it a benign condition?

Caseous mitral annular calcification (CMAC) is a variant of mitral annular calcification (MAC) with a central liquefaction necrosis. The echocardiographic prevalence of CMAC is approximately 0.6% in patients with MAC and 0.06–0.07% in large series of patients of all ages. However, the prevalence in necropsy series has been reported to be 2.7%, which indicates that this condition is yet under-recognized. Furthermore CMAC is probably underappreciated as a source of potentially serious conditions. We report a case of recent stroke associated with CMAC diagnosed by trans-thoracic and confirmed by trans-esophageal echocardiogram (TEE). The importance of correct diagnosis of CMAC extends beyond the possible complications; since several misdiagnoses of CMAC as abscesses and cardiac tumors have been reported leading to inappropriate interventions.