Caseous mitral annular calcification: Is it a benign condition?

Abstract Background Caseous mitral annular calcification (CMAC) is a rare liquefactive variant of mitral annular calcification (MAC) and superficially mimics a cardiac vegetation or abscess. CMAC is viewed as a benign condition of MAC, while MAC has clinical implications for patients’ lives. Correctly diagnosing CMAC is essential in order to avoid unnecessary interventions, cardiac surgery or even psychological suffering for the patient. Case presentation We report on 6 patients with suspected intra-cardiac masses of the mitral annulus that were referred to our institution for further clarification. A definitive diagnosis of CMAC was achieved by combining echocardiography (Echo), cardiac magnetic resonance imaging (MRI) and cardiac computed tomography (CT) for these patients. Echo assessed the mass itself and possible interactions with the mitral valve. MRI was useful in differentiating the tissue from other benign or malign neoplasms. CT revealed the typical structure of CMAC with a “soft” liquefied centre and an outer capsule with calcification. Conclusion CMAC is a rare condition, and most clinicians and even radiologists are not familiar with it. CMAC can be mistaken for an intra-cardiac tumour, thombus, vegetation, or abscess. Non-invasive multimodality imaging (i.e. Echo, MRI, and CT) helps to establish a definitive diagnosis of CMAC and avoid unnecessary interventions especially in uncertain cases.

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Abstract 11452: Are Patients With Caseous Calcification of the Mitral Annulus But Without Atrial Fibrillation at Increased Risk of Cerebral Embolization?

Circulation ◽

10.1161/circ.130.suppl_2.11452 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Charles A Dietl ◽

Christopher M Hawthorn ◽

Veena Raizada

Keyword(s):

Atrial Fibrillation ◽

Mitral Annulus ◽

Mitral Annular Calcification ◽

Benign Condition ◽

Increased Risk ◽

Cerebral Embolization ◽

Mitral Annulus Calcification ◽

Unusual Variant ◽

History Of ◽

Annular Calcification

Background: Caseous calcification of the mitral annulus (CCMA) is very uncommon, and is frequently misdiagnosed as an intracardiac tumor, thrombus, abscess, or simply as mitral annular calcification (MAC). These masses are generally believed to have a benign prognosis. The aims of this study are to increase the awareness of this unusual variant of MAC, and to determine whether patients with CCMA are at increased risk of cerebral embolization in patients with or without atrial fibrillation (AF). Methods: A comprehensive literature search was done to determine whether patients with CCMA are at increased risk of cerebral embolization, using the following search modules: caseous calcification of the mitral annulus, mitral annular calcification, cerebral embolization with mitral annulus calcification, risk of stroke with mitral annular calcification. Results: Among the 496 articles listed in PubMed.gov, ScienceDirect.com, and Google Scholar, a total of 129 patients with CCMA were identified in 85 publications, and 31 articles were reviewed to evaluate the incidence of stroke in 1800 patients with MAC. Literature review revealed that the incidence of cerebrovascular events (CVE) associated with CCMA is 18.6% (24 of 129) which is even higher than the risk of embolic CVE reported in patients with MAC, 11.6% (209 of 1800) (range 4.8% to 24.1%). Only 2 of 24 patients (8.3%) with CCMA who suffered a CVE had history of AF, whereas the majority (22 of 24, or 91.7%) of CCMA patients with a CVE did not have AF. Conclusions: Despite the fact that several reports suggest that CCMA is a benign condition, CCMA may be potentially serious, because of the increased risk of cerebral embolization, even in patients without atrial fibrillation.

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