Rupture of Caseous Calcification of the Mitral Annulus: Pathophysiology, Diagnosis and Treatment

Caseous calcification of the mitral annulus (CCMA) is a liquefactive necrosis of mitral annular calcification (MAC). CCMA is rare and usually asymptomatic, has a benign course, and, when incidentally found, can be misdiagnosed as a thrombus, abscess, cardiac tumor or vegetation. Although rarely, CCMA may complicate with rupture, which can lead to ventricular-atrial fistulization, pseudoaneurysm, severe mitral regurgitation (with possible heart failure and atrial fibrillation) and systemic embolism of caseous material (with cerebral ischemic events). A significant increase in CCMA dimensions and an infectious involvement of liquefactive necrosis make CCMA prone to rupture. To date, only case reports and some case series have been published on CCMA, without focusing on the pathophysiological mechanisms responsible for rupture, nor recommendations for prevention and management. However, despite general concerns about surgical treatment of CCMA because of high perioperative risks, most published cases actually underwent successful cardiac surgery. In the present review, we conducted a systematic review of the studies published in the medical literature up to March 2025, reporting cases of CCMA and its complications, as identified through the PubMed database. We analyzed clinical and biological risk factors for CCMA rupture and its diagnostic criteria, focusing on imaging features differentiating mitral annular calcification from uncomplicated CCMA and ruptured CCMA. To this regard, we focused on the key role of multimodality imaging in the achievement of the correct diagnosis. Finally, we propose a management strategy for CCMA, with the aim to fill a gap in this field in the current literature.