An extensive caseous calcification of the mitral annulus complicated with severe mitral regurgitation

Caseous calcification of the mitral annulus (CCMA) is considered a rare variant of mitral annular calcification (MAC) due to caseous transformation of the inner material and prevalence of CCMA, about 0.63% of all MAC cases and 0.06%-0.07% of the population. The precise pathophysiology of CCMA is still unknown. It is a chronic degenerative disorder common in the elderly, particularly in women. Caseous calcification of the mitral annulus (CCMA) is a soft peri-annular extensive calcification, resembling cardiac tumors, abscesses, vegetation, or calcified thrombus, which is composed of an admixture of calcium, fatty acids, and cholesterol with a toothpaste-like texture. In the characteristic appearance on echocardiography, the calcification is a round, large, soft mass with a central echo dense area, typically located at the basal area of the posterior mitral valve. It usually carries a benign prognosis but rarely may be complicated with mitral valve dysfunction (valve stenosis/mitral regurgitation) or systemic embolization. The current data suggest conservative medical therapy and clinical follow-up for management of CCMA unless there is a need of cardiac surgery for severe mitral stenosis and/or regurgitation. We present a case, complicated with severe mitral regurgitation, and need surgical resection of the CCMA because of the CCMA-induced symptomatic severe mitral regurgitation despite optimal medical therapy. Macroscopic and microscopic examination of caseous material also demonstrated the characteristic appearance of CCMA.