Spontaneous fistulization of a caseous calcification of the mitral annulus: an exceptional cause of stroke

Abstract

We present the case of a caseous calcification of the mitral annulus, responsible for two strokes, in a 72-year-old female patient. The brain computed tomography (CT) scan confirmed the presence of a calcific embolus. The echocardiography showed a liquidy, pseudotumoral mass combined with numerous calcifications located in the posterior part of the mitral annulus and extending toward the inferior surface of the left ventricle. During surgery, we found a direct communication between the caseous necrosis and the lumen of the left ventricle at the level of its inferior wall. We performed a valve repair procedure and excision of the caseous necrosis, combined with injection of bioglue into the cavity, to avoid recurrence. Six months after the procedure, the patient was in good health, and had no recurrence of stroke with a satisfactory echocardiography. This is the first description of spontaneous fistulization of a caseous necrosis in the lumen of the left ventricle, explaining a new mechanism for cerebral embolism during the course of calcifying diseases of the mitral annulus.

Caseous calcification of the mitral annulus associated with stroke: Report of two cases

“…In the absence of vascular risk factors or widespread atherosclerosis and when a cardiac embolism is strongly suspected based on radiological criteria such as in our second case, CCAM can be considered as the most probable cause of stroke, and surgery is considered as the treatment of choice [5,15,23]. In these cases, stroke could be the consequence of embolization of small calcification, of thrombus from ulceration of the surface, or fistulization of the caseous necrosis in the lumen of left atrium or ventricle [5,23].…”

Risk of Cerebral Embolization with Caseous Calcification of the Mitral Annulus: Review Article

“…Thorough review of all cases of CCMA presenting with a stroke excluded thrombus formation and other possible intracardiac source of embolism by TEE or during operation in all 25 cases. The possible mechanisms for cerebral embolization from CCMA lesions include: (1) spontaneous fistulization and embolization of caseous material that may leak directly into the left atrium or ventricle [18, 20]; (2) embolization of caseous necrotic debris from the CCMA lesion [25]; and (3) embolization of calcium and cholesterol particles [32]. …”

Surgical Management of Caseous Calcification of the Mitral Annulus

“…Published operative experience with CCMA is limited to few case reports that describe wide d ebridement and excision of the mass cavity [3,4]. In our early experience, this technique was associated with a massive perioperative embolic stroke.…”

“…C aseous calcification of the mitral annulus (CCMA) is a known variant of mitral annular calcification in which liquefaction and caseation forms a mass within the mitral leaflet at the level of the annulus [1]. Although the reported incidence is rare, affecting an estimated 0.5% to 1.0% of patients with mitral annular calcification, fistulization or erosion of the contents of the mass into the left ventricle and systemic circulation can result in devastating embolic events [2][3][4].…”