Background:Caseous calcification of the mitral annulus (CCMA) is believed to have a benign prognosis. Several authors have recommended conservative management in asymptomatic patients. However, the prevalence of cerebrovascular events (CVE) in patients with CCMA has never been evaluated before. The aims of this study are to investigate whether patients with CCMA are at increased risk of cerebral embolization, and to determine whether elective surgical resection of CCMA should be considered to prevent a cardioembolic stroke.Methods:A comprehensive literature search was obtained from MEDLINE via PubMed.gov, ScienceDirect.com, and Google Scholar using the following search queries: caseous calcification of the mitral annulus, intracardiac pseudotumor, mitral annular calcification, and cardioembolic stroke.Results:From our initial search that yielded 1,502 articles, we identified a total of 130 patients with CCMA reported in 86 publications. Literature review revealed that the prevalence of CVE associated with CCMA is 19.2% (25 of 130) which is significantly higher than the prevalence of CVE reported with mitral annular calcification (MAC), 11.8% (214 of 1818) (range 4.8% to 24.1%) (P = 0.01796) (odds ratio = 1.78; 0.95 confidence interval = 1.1278 – 2.8239). Only four of 25 (16.0%) patients with CCMA who suffered a CVE had history of atrial fibrillation (AF).Conclusion: Based on our review, it would be reasonable to consider elective surgical resection of CCMA in asymptomatic patients who are good surgical candidates, because patients with CCMA may be at increased risk of embolic strokes, which are unrelated to AF.

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.