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      A Large Cardiac Mass: Diagnosis of Caseous Mitral Annular Calcification and Determining Optimum Management Strategy

      case-report

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          Abstract

          A 64-year-old woman with dizziness and blurry vision underwent an evaluation for a possible stroke with a head-neck CT scan and a transthoracic echocardiogram. The head-neck CT scan was unremarkable, but the echocardiogram was notable for a 2.0 × 2.3 cm heterogeneous echodensity attached to the mitral valve. After a transesophageal echocardiogram and chest CT scan, the mass was determined to be a caseous mitral annular calcification, CMAC. This entity is a rare variant of MAC with an estimated prevalence of 0.068%. Echocardiographic techniques can distinguish CMAC from other intracardiac masses such as tumor, cyst, or abscess. CMAC is associated with cerebrovascular accidents; however, optimal treatment is controversial given the rarity of this clinical finding. Management strategies should be tailored based on the patient's presentation, risk factors, and overall clinical circumstances.

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          Mitral annular calcification predicts cardiovascular morbidity and mortality: the Framingham Heart Study.

          R Vasan, Emelia Benjamin, … (2003)
          Mitral annular calcification (MAC) has been associated with stroke in longitudinal, community-based cohorts and cardiovascular disease (CVD) outcomes in many small retrospective studies. Prospective data are limited on the relation of MAC with CVD morbidity and mortality. We examined the association between MAC assessed by M-mode echocardiography and the incidence of CVD, CVD death, and all-cause death over 16 years of follow-up in the Framingham Heart Study subjects who attended a routine examination between 1979 and 1981. Cox proportional hazards models were used to estimate hazard ratios (HRs) associated with the presence of MAC for each outcome. Of 1197(445 male, 752 female) subjects who had adequate echocardiographic assessment, 14% had MAC. There were 307 incident CVD events and 621 deaths. In multivariable adjusted analyses, MAC was associated with an increased risk of incident CVD (HR, 1.5; 95% CI, 1.1, 2.0), CVD death (HR, 1.6; 95% CI, 1.1, 2.3), and all-cause death (HR, 1.3; 95% CI, 1.04, 1.6). For each 1-mm increase in MAC, the risk of incident CVD, CVD death, and all-cause death increased by approximately 10%. The independent association of MAC with incident CVD and CVD death underscores that cardiac calcification is a marker of increased CVD risk.
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            Mitral annular calcification and the risk of stroke in an elderly cohort.

            Steven P Wolf, Emelia Benjamin, K Comai … (1992)
            Previous clinical studies have suggested that there is an association between mitral annular calcification and the risk of stroke, but it is unclear whether this association is independent of the traditional risk factors for stroke. We examined the relation between mitral annular calcification and the incidence of stroke in a population-based study. Subjects in the Framingham Study receiving a routine examination underwent M-mode echocardiography to determine the presence and severity (thickness in millimeters) of mitral annular calcification. The incidence of stroke during eight years of follow-up was analyzed with a proportional-hazards model adjusting for the calcification, age, sex, systolic blood pressure, diabetes mellitus, cigarette smoking, atrial fibrillation, and coronary heart disease or congestive heart failure. Among 1159 subjects whose echocardiograms could be assessed for mitral annular calcification and who had no history or current evidence of stroke at the index examination (51 percent of all subjects), the prevalence of mitral annular calcification was 10.3 percent in the men and 15.8 percent in the women. Multivariate analysis demonstrated that the presence of mitral annular calcification was associated with a relative risk of stroke of 2.10 (95 percent confidence interval, 1.24 to 3.57; P = 0.006). There was a continuous relation between the incidence of stroke and the severity of mitral annular calcification; each millimeter of thickening as shown on the echocardiogram represented a relative risk of stroke of 1.24 (95 percent confidence interval, 1.12 to 1.37; P less than 0.001). Furthermore, even when subjects with coronary heart disease or congestive heart failure were excluded from the analysis, subjects with mitral annular calcification still had twice the risk of stroke. In an elderly, longitudinally followed population-based cohort, mitral annular calcification was associated with a doubled risk of stroke, independently of traditional risk factors for stroke. Whether such calcification contributes causally to the risk of stroke or is merely a marker of increased risk because of its association with other precursors of stroke remains unknown.
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              Caseous calcification of the mitral annulus: a neglected, unrecognized diagnosis.

              Jaime Tobar, M Motro, Z Vered … (2001)
              Mitral annular calcification is a common echocardiographic finding. Caseous calcification is a rare variant seen as a large mass with echolucencies that resembles a tumor, occasionally resulting in exploratory cardiotomy. The aim of this study was to assess the prevalence of caseous calcification of the mitral annulus, to evaluate patient characteristics and the echocardiographic variables for diagnosing this entity, and to describe the clinical outcome on follow-up of such patients. Caseous calcification was defined as a large, round, echo-dense mass with smooth borders situated in the periannular region, with no acoustic shadowing artifacts and containing central areas of echolucencies resembling liquefaction. Eighteen patients were diagnosed by 2-dimensional echocardiography as having caseous calcification of the mitral annulus. One had calcification of the tricuspid annulus. Nine patients underwent transesophageal echocardiographic studies. A typical finding of a round, sometimes semilunar, large, echo-dense, soft mass with central echolucencies seen on both transthoracic and in particular transesophageal echocardiography, resembling a periannular mass, was demonstrated. The mass was posteriorly located in all mitral patients. Transesophageal echocardiography added limited information. Three patients underwent mitral valve replacement. The operative findings were a solid mass adherent to the posterior portion of the mitral valve. Sectioning revealed a toothpaste-like, white, caseous material. Sixteen (84%) patients were treated conservatively. On follow-up of 3.8 +/- 2.4 years, 4 patients died of unrelated causes. The characteristic appearance of a large, soft, echo-dense mass containing central areas of echolucencies resembling liquefaction at the posterior periannular region of the mitral valve on 2D echocardiography is compatible with the diagnosis of caseous abscess. Such a finding should not be confused with a tumor. Transesophageal echocardiography does not appear to contribute to the diagnosis. This rather impressive lesion appears to carry a benign prognosis.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Med
                Journal ID (iso-abbrev): Case Rep Med
                Journal ID (publisher-id): CRIM
                Title: Case Reports in Medicine
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1687-9627
                ISSN (Electronic): 1687-9635
                Publication date (Print): 2014
                Publication date (Electronic): 17 June 2014
                Volume: 2014
                Electronic Location Identifier: 637374
                Affiliations
                1UCSD School of Medicine, La Jolla, CA, USA
                2Department of Radiology, UCSD School of Medicine, La Jolla, CA, USA
                3Department of Medicine and Division of Cardiovascular Medicine, UCSD Sulpizio Family Cardiovascular Center, La Jolla, CA 92093, USA
                Author notes
                *Luis R. Castellanos: lrcastellanos@ 123456ucsd.edu

                Academic Editor: W. Zidek

                Article
                DOI: 10.1155/2014/637374
                PMC ID: 4084408
                SO-VID: f08919dd-ca59-430c-8572-a7179dd4df78
                Copyright © Copyright © 2014 Emanuel A. Shapera et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 8 April 2014
                Date accepted : 21 May 2014
                Categories
                Subject: Case Report

                Data availability:

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