A 70-year-old woman presented to her primary physician with progressive shortness of breath. She was found to have atrial fi brillation and 2/6 pansystolic murmur at the left lower parasternal border on physical examination. Transthoracic echocardiography (TTE) showed mild mitral regurgitation and a cardiac mass at the atrioventricular (AV) groove area. She was referred to our institute for cardiac mass removal (Figs. 3.1 , 3.2 , 3.3 , 3.4 , 3.5 , 3.6 , and 3.7 ).
Learning PointsCaseous calcifi cation of the mitral annulus (CCMA) is a rare variant of mitral annular calcifi cation with an estimated prevalence of 0.068 % [ 1 -3 ]. It is characterized by an echodense outer shell and echolucent core [ 4 ]. The posterior mitral annulus is more often affected than the anterior. The inner core of the lesion represents the byproduct of liquefaction necrosis. Pathological examination reveals sterile, amorphous, acellular eosinophilic material with macrophage and lymphocyte infi ltration [ 5 ].The clinical course of CCMA is generally benign and often dynamic, with reports of spontaneous resolution of progression [ 3 ]. The complications of CCMA include hemodynamically signifi cant mitral stenosis and regurgitation secondary to mass effect [ 5 , 6 ], as well as erosion of the inner core into the left atrial chamber [ 7 ] and the left circumfl ex coronary artery [ 8 ].