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      Caseous calcification of the mitral annulus associated with a complete atrioventricular block: a case report

      case-report

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          Abstract

          Background

          Caseous calcification of the mitral annulus (CCMA), a subtype of mitral annulus calcification, is rarely encountered. Although most cases of CCMA are asymptomatic and have a benign course, there are several reports of mitral valve dysfunction, stroke, and myocardial infarction. However, few reports have been published on conduction disturbances.

          Case summary

          We encountered a case of an atrioventricular block, which is a rarely reported complication, in a 70-year-old woman who presented with heart failure. The patient’s anatomy suggested a conduction disturbance caused by CCMA extending from the posterior apex of the mitral annulus to the ventricular septum. Our heart team discussed whether surgical resection should be performed. We concluded that bradycardia was the most likely cause of the current symptoms and that resection of the extensively infiltrated calcification was risky; therefore, we decided to proceed with pacemaker implantation followed by careful observation of the mass. The patient had undergone permanent pacemaker implantation and has been asymptomatic ever since.

          Discussion

          Few reports on conduction abnormalities caused by CCMA have been published. A mass involving the left ventricular septum and posterior mitral annulus may lead to conduction abnormalities, such as a complete atrioventricular block, in the future. We suggest that careful follow-up is required for CCMA, as it has been determined that surgical intervention is not required.

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          Most cited references12

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          2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

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            Caseous calcification of the mitral annulus: a neglected, unrecognized diagnosis.

            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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              The incidence and clinical course of caseous calcification of the mitral annulus: a prospective echocardiographic study.

              Mitral annular calcification (MAC) is a common echocardiographic finding. Caseous calcification of the mitral annulus (CCMA) is, on the other hand, a less known, rarely described variant, seen as a round mass with a central echolucent area composed of a puttylike admixture of fatty acids, cholesterol, and calcium. The aims of this study were to assess the prevalence of CCMA, assess its morphologic changes over the course of time, and evaluate the patients' characteristics and clinical outcome on follow-up. Between January 2002 and December 2004, 20,468 consecutive patients, referred for transthoracic echocardiography, were included in the study. All patients underwent echocardiographic examinations. Four echocardiographic laboratories participated in the registry. CCMA was defined as a large, round, echodense mass with smooth borders located in annular region, without acoustic shadowing and with central areas of echolucencies resembling liquefaction. A total of 2169 (10.6%) patients were given the diagnosis of MAC by 2-dimensional echocardiography. A total of 14 patients (0.64% of all MACs, 0.068% of all studies) were given the diagnosis of echocardiographic findings compatible with CCMA. Six (43%) patients underwent transesophageal echocardiography (TEE) to better evaluate the nature of the mass. A complete TEE examination was performed using 2-dimensional and color flow Doppler, and the best visualizations of the mass were performed by midesophageal 4-chamber view, midesophageal 2-chamber view, and midesophageal long-axis view. More detailed imaging of the masses, above all a better visualization of the central areas of echolucency, the assessment of the posterior mitral leaflet motion, and the assessment of the correct location of the mass was achieved by TEE views. All calcifications were confined to the mitral annulus. The most common symptom was palpitation, which occurred in 43% of the patients. During a mean follow-up of 3.4 +/- 1.2 years, one patient died. The cause was unrelated to the annular mass; it was the result of neoplasm. During the follow-up period, in 6 (43%) cases, the studies changed, in regard to the features of CCMA, in comparison with baseline studies, thus likely suggesting a changeable condition. This study confirms prior observations that CCMA is a rare and benign condition. It illustrates the potential role of TEE in confirming the precise location of the lesion and in more clearly defining the extent of the involvement of the posterior mitral leaflet. There were no typical clinical characteristics in patients with CCMA although the absolute number of patients with CCMA was too small to be statistically significant. However, CCMA does tend to occur in older patients and all 14 patients with CCMA in this study had hypertension. CCMA may be a dynamic process based on the observation that 3 patients with MAC progressed to CCMA and 3 patients with CCMA reverted back to MAC during the study period. To avoid diagnostic mistakes such as tumor, abscess, or thrombus among echocardiographers, it is important for us to consider a more widespread knowledge of this rare lesion.
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                Author and article information

                Contributors
                Role: Handling Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Journal
                Eur Heart J Case Rep
                Eur Heart J Case Rep
                ehjcr
                European Heart Journal. Case Reports
                Oxford University Press (UK )
                2514-2119
                March 2025
                14 February 2025
                14 February 2025
                : 9
                : 3
                : ytaf079
                Affiliations
                Department of Cardiology, Aso Iizuka Hospital , Yoshio-machi 3-83, Iizuka, Fukuoka-ken 8208505, Japan
                Department of Cardiology, Aso Iizuka Hospital , Yoshio-machi 3-83, Iizuka, Fukuoka-ken 8208505, Japan
                Department of Cardiology, Aso Iizuka Hospital , Yoshio-machi 3-83, Iizuka, Fukuoka-ken 8208505, Japan
                Author notes
                Corresponding author. Tel: +81 0948 22 3800, Fax: +81 0948 29 5744, Email: koji2132@ 123456hotmail.co.jp

                Conflict of interest. None declared.

                Author information
                https://orcid.org/0009-0002-7212-892X
                Article
                ytaf079
                10.1093/ehjcr/ytaf079
                11879389
                40046972
                541499e9-5556-4938-99de-88a24c77f44e
                © The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 04 September 2024
                : 24 November 2024
                : 12 February 2025
                : 04 March 2025
                Page count
                Pages: 6
                Categories
                Case Report
                Cardiovascular Imaging
                AcademicSubjects/MED00200
                Eurheartj/31
                Eurheartj/35
                Eurheartj/32

                caseous calcification of the mitral annulus,complete atrioventricular block,conduction disturbance,cardiac mass,case report

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