Extensive myocardial and mitral annular calcification leading to mitral regurgitation and restrictive cardiomyopathy: An unusual case of caseous calcification of the mitral annulus

Abstract

A 62-year-old man with polymyalgia rheumatica and chronic atrial fibrillation with prior atrioventricular node ablation and pacemaker implantation presented with progressive exertional dyspnea. Transthoracic echocardiography showed restrictive filling as well as extensive calcification of the mitral annulus and severe mitral regurgitation from a restricted posterior leaflet. A round, hyperechoic lesion with central lucency was noted within the interventricular septum along with calcification of the basal anterior, lateral, and posterior segments of the left ventricle (Fig. 1; Supplementary Videos 1–2) unchanged since previous echocardiogram 2 years before. An electrocardiographic-gated dual-source cardiac CT (Somatom Definition Siemens Medical Solutions, Forchheim, Germany) confirmed extensive mitral annular calcification and diffuse, near-transmural involvement of a heterogenous peripherally calcified lesion (at largest 5.3×3.3 cm) within the myocardium (Fig. 2). This was more extensive than noted by echocardiography. These findings are consistent with an atypical caseous mitral annular calcification extending along the fibrous skeleton of the heart. Surgical repair was offered, but the patient elected for conservative management. Figure 2Cardiac CT images in short axis further delineating areas of diffuse involvement within the left ventricular myocardium. View Large Image Figure Viewer Download Hi-res image