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      Spontaneous Resolution of a Caseous Calcification of the Mitral Annulus

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          Abstract

          INTRODUCTION An asymptomatic 76-year-old woman with a prior history of mitral annular calcification underwent a transthoracic echocardiographic examination for evaluation. Her risk factor for atherothrombotic disease was hypertension, and she had no prior medical history of hyperlipidemia or diabetes. She was given the following pharmacologic treatments: an angiotensin-converting enzyme (ACE) inhibitor (quinapril 5 mg), a calcium antagonist (lercanidipine 10 mg), a loop diuretic (furosemide 25 mg), and an antiplatelet agent (acetylsalicylic acid 150 mg). A round, echo-dense structure was visualized in the left atrioventricular groove region (Fig. 1, upper panels). A transesophageal echocardiographic examination (TEE) was performed to better evaluate this intracardiac mass (Fig. 1, lower panels). A large (2.14 × 1.82 cm), round, echo-dense mass containing central areas of echolucencies (central liquefaction) was revealed by TEE. No acoustic shadowing could be detected behind the mass, suggesting an absence of dense calcium deposition. The mass had sharp and distinct borders, with an echogenicity distinct from that of the adjacent myocardium (Fig. 2, left panel). The echocardiographic findings were consistent with the diagnosis of Caseous Calcification of the Mitral Annulus (CCMA). The patient was asymptomatic and was treated conservatively. A TEE conducted nine months later revealed the disappearance of the mass (Fig. 2, right panel). DISCUSSION Mitral Annular Calcification (MAC) is a chronic degeneration of the mitral valve fibrous ring involving the posterior annulus. This disorder is common in the elderly, particularly in women.1 It may also occur in younger patients with advanced renal disease or other metabolic disorders that result in abnormal calcium metabolism. MAC is a consequence of atherosclerosis, with risk factors identical to those of cardiovascular disease. CCMA is a less-known and rarely described entity. This condition is an extensive “soft” periannular calcification, resembling a tumor, that is composed of an admixture of calcium, fatty acids, and cholesterol with a “toothpaste-like texture.” CCMA is often misdiagnosed as a myocardial abscess,2,3 a tumor,4,5,6 or a thrombus,7 in some cases2 leading to unnecessary explorative cardiotomy. Differential diagnosis of round echogenic structures adjacent to the left atrioventricular groove should also include infected mitral calcification, lipomatosis of the atrioventricular groove, and enlarged lymph nodes.8 In this case, a correct diagnosis was made according to clinical presentation and echocardiographic findings. Generally, valvular endocarditis is unlikely in the absence of mitral valve regurgitation, fever, or positive blood cultures or laboratory examinations for infectious disease. Clinical signs of malignancy were absent. The round, echodense lobular aspect and mobility of the mass favored the diagnosis of myxoma; however, myxomas seldom originate from valves and are often found attached to the inter-atrial septum in the fossa ovalis region. Instead, specific echocardiographic features of CCMA favor the diagnosis: a large, round echodense mass in the posterior periannular mitral region that is heterogeneous and contains central echolucent areas of necrosis. The density of the mass suggested calcification and ruled out a thrombus diagnosis. Harpaz and colleagues conducted a prospective series of echocardiographic examinations to define the echocardiographic appearance, prevalence, and prognosis of CCMA.9 They described the typical echocardiographic appearance of CCMA as a large, round, echo-dense mass with smooth borders situated in the periannular region, lacking acoustic shadowing artifacts, and containing central areas of echolucencies resembling liquefaction. They emphasized that it is possible to make a correct diagnosis by transthoracic examination alone, since no additional significant information was obtained by TEE in the 19 cases they examined. In this patient, however, TEE gave a more precise and detailed image of the mass and a better definition of the motion of the posterior mitral leaflet. Recently, we10 have confirmed that CCMA is a rare and benign condition (14 patients with CCMA, 0.64% of all mitral annular calcifications, 0.068% of all studies). This study revealed no clinical characteristics typical of patients with CCMA. However, CCMA did tend to occur in older patients, and all 14 patients with CCMA had hypertension. We suggested that CCMA may be a dynamic process based on the observation that three patients with MAC progressed to CCMA and three patients with CCMA reverted back to MAC during the study period. It is important to differentiate between CCMA and MAC; the former is a larger, well-delineated structure surrounded by clear borders with a distinct echolucency and a “softer” appearance that is less reflective and does not have acoustic shadowing. When a CCMA is perforated, a large amount of toothpaste-like, milky, caseous material is exuded from the mass. This material it is not truly calcific, but rather consists of caseous, putty-like material. After the material is removed, only a calcified envelope remains. In the case of large dense deposits of calcifications in the left atrioventricular groove, significant acoustic shadowing artifacts may rule out the diagnosis of CCMA. However, if the calcified envelope of the CCMA is very large, acoustic shadowing could result in an erroneous non-diagnosis of CCMA.10 The mechanisms involved in the liquefaction necrosis of CCMA are not well understood. Hypercholesterolemia and dissolution of lipid-laden macrophages might be implicated; high serum cholesterol is associated with MAC and may be responsible for liquefaction necrosis. The peculiarity of this case is in its spontaneous resolution ten months later. A previous similar case11 in the literature showed a spontaneous resolution of a CCMA. In this previous case, the patient had a prior history of hemodialysis treatment, but no change was made in the hemodialysis regimen. In another similar case12 with a prior history of hemodialysis treatment, the reduction of the mass was observed after treatment with low calcium hemodialysis, so the authors suggested that the acute change in the serum calcium level might have contributed to the dissolution of the mass. In our case, we hypothesize that central liquefaction and dissolution of the material through a rupture of external wall occurred without evident clinical consequences. In order to avoid misdiagnoses of CCMA as tumors, abscesses, or thrombi by echocardiographers, it is important that knowledge of this rare lesion becomes more widespread.

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          Most cited references33

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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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              Pathological and clinical study of calcification of the mitral valve ring.

              The pathology and clinical features of 258 cases of mitral ring calcification were reviewed. The overall incidence in patients over 50 years of age was 8.5%; it was more than twice as high in women (11.5%) as in men (4.5%) and rose sharply with age. Cardiac failure and systolic murmurs were each noted in over half the patients. Hypertension was slightly commoner than in age- and sex-matched groups without ring calcification, although the difference was not statistically significant. Small nodules of calcification were more frequent in men and heavy deposits in women. Distortion and atrial displacement of the posterior mitral cusp was present in 26% of the hearts with early ring calcification, in 56% of the hearts with moderate, and in almost all hearts with marked changes. Systolic murmurs had been heard in 73% of these cases. ;Caseation' of the calcified ring was seen in seven hearts and haemorrhagic valvulitis in three. Calcium had ulcerated through the cusp in 12 cases, with thrombotic and/or bacterial endocarditis in five. Aortic valve calcification was present in 36% of men and was quantitatively related to the severity of mitral ring calcification. In women the incidence was 30% and there was no corresponding quantitative relationship. Microscopy showed nonspecific chronic inflammatory changes adjacent to calcium in about half the cases in both sexes, with foreign body type giant cells in 6%. Similar inflammatory changes in the valve cusp were almost twice as common in women as in men. There was no evidence that previous endocarditis was responsible for mitral ring calcification, neither did parity influence its incidence. Severe coronary atherosclerosis was unrelated but severe aortic atherosclerosis was commoner in patients with calcified mitral rings. The difference, in women, was statistically significant. The higher incidence of severe degrees of ring calcification, complications, and valvular inflammation in women suggests a sex-determined difference in tissue response in the mitral area. Possible provoking factors apply to both sexes and both left side valves, and such a difference would account for the relative frequency and sex incidence of mitral ring calcification.
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                Author and article information

                Journal
                Clinics (Sao Paulo)
                Clinics (Sao Paulo, Brazil)
                Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo
                1807-5932
                1980-5322
                November 2009
                : 64
                : 11
                : 1130-1132
                Affiliations
                [I ] University of Foggia, Department of Cardiology - Foggia, Italy
                [II ] Andria Hospital, Department of Cardiology - Andria, Italy. Tel: 39 088 173 3652 Email: opsfco@ 123456tin.it
                Article
                cln64_11p1130
                10.1590/S1807-59322009001100015
                2780532
                19936189
                59c85379-d511-4df5-a111-73121356055b
                Copyright © 2009 Hospital das Clínicas da FMUSP
                Categories
                Letters to the Editor

                Medicine
                Medicine

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