Caseous Calcification of the Mitral Annulus With Aortic Root Fistula and Pseudoaneurysm

BACKGROUND: Caseous calcification of the mitral annulus (CCMA) fistulizing into the aortic root has not been described. CASE SUMMARY: A 66-year-old man with severe regurgitation from mitral valve prolapse presented with a calcified mass extending from the anterolateral trigone of the mitral valve to the anterolateral papillary muscle. Multimodality imaging revealed a pseudoaneurysm in the anterolateral trigone communicating with the aortic root without aortic valve dysfunction. A 3-dimensional (3D) printed model delineated anatomy and supported valve repair, fistula closure, and aortic valve preservation. DISCUSSION: This novel case of CCMA with pseudoaneurysm fistulizing into the aortic root demonstrated how multimodality imaging and 3D modeling facilitate valve-sparing repair. TAKE-HOME MESSAGES: Fistulization of CCMA into the aortic root is exceptionally rare. Multimodality imaging and 3D modeling enabled precise anatomic characterization, mitral valve repair, and closure of the fistulous tract. Advanced imaging can expand surgical options and support preservation of native valves in anatomically complex diseases.