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J Thorac Cardiovasc Surg 2005;129:1438-1440
© 2005 The American Association for Thoracic Surgery

Brief Communication

Caseous calcification of the mitral annulus

Hatem Alkadhi, MDa,*, Sebastian Leschka, MDa, René Prêtre, MDb, Aurel Perren, MDc, Borut Marincek, MDa, Simon Wildermuth, MDa

a Institute of Diagnostic Radiology, University Hospital Zurich, CH-8091 Zurich, Switzerland.
b Clinic for Cardiovascular Surgery, University Hospital Zurich, CH-8091 Zurich, Switzerland.
c Department of Pathology, University Hospital Zurich, CH-8091 Zurich, Switzerland.

Received for publication October 27, 2004; accepted for publication November 4, 2004.

* Address for reprints: Hatem Alkadhi, MD, Department of Medical Radiology, Institute of Diagnostic Radiology, University Hospital Zurich, Rämistrasse 100, CH-8091 Zurich, Switzerland (Email: hatem.alkadhi{at}usz.ch).

Caseous calcification of the mitral annulus is a rare variant of mitral annular calcification that should be included in the differential diagnosis of intracardiac masses.1 We present the computed tomography (CT), intraoperative, and histopathologic findings of a 70-year-old woman with mitral stenosis caused by a large caseous calcification located in the posterior mitral annulus.

Clinical Summary

A 70-year-old woman was admitted with progressive shortness of breath, orthopnea, and several syncopal episodes in the last 6 months. Her history was remarkable for a long-standing hypertonia. A transthoracic echocardiogram revealed an echo-intense mass with central echolucencies attached to the posterior mitral annulus causing mitral stenosis. For further characterization of the lesion, a 64-slice multidetector row CT (Sensation 64, Siemens, Germany) with retrospective electrocardiographic triggering after intravenous administration of iodinated contrast material was performed. CT showed an oval 3 x 3-cm mass located in the posterior mitral annulus with peripheral calcifications and central hyperdensity. Reconstructions during mid-systole showed the broad-based attachment of the lesion to the posterior leaflet; during mid-diastole, the mass hindered a complete opening of the posterior leaflet, thus leading to mitral stenosis (Figure 1).


Figure 1
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  		Figure 1. Computed tomography (CT) reconstructions parallel to the long-axis of the left ventricle during mid-systole (A) and mid-diastole (B) demonstrate the centrally hyperdense, peripherally calcified mass (arrowheads) located in the posterior mitral annulus and attached to the posterior leaflet. Complete opening of the valve leaflets during diastole (arrows) is hindered by the attachment of the caseous calcification to the posterior leaflet.

 
At surgery, the nodular, exophytic mass was excised, and a pasty, white material that filled the center of the mass was aspirated (Figure 2). The mitral valve was subsequently reconstructed with ring annuloplasty. Cultures for bacteria, fungi, and acid-fast bacilli did not yield infectious organisms, and histochemical stains showed negative results. Histopathologic examination of the pasty material showed a dense, amorphous, acellular and basophilic substance with scattered calcifications and sparse histiocytes (Figure 3). Inflammatory cells were absent. Thus, the diagnosis of caseous calcification of the mitral annulus was established.


Figure 2
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  		Figure 2. Intraoperative photograph demonstrating the caseous calcification after incision (A). Pasty white material was subsequently aspirated (B).

 

Figure 3
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  		Figure 3. Histologic section of the aspirate shows an amorphous, basophilic substance with scattered calcifications and sparse histiocytes (magnification x200; hematoxylin-eosin stain).

 
Discussion

Mitral annular calcification is a degenerative abnormality of the cardiac fibrous skeleton that occurs mainly in elderly individuals.2 It usually involves the mid-base of the posterior leaflet but may also involve other segments of the mitral annulus. Caseous calcification is a less-known and rarely described entity representing a variant of mitral annular calcification, which is typically located in the posterior mitral annulus.1 It presents as a soft, periannular calcification and is composed of an admixture of calcium, fatty acids, and cholesterol with a "toothpaste-like" texture. The echocardiographic prevalence of caseous calcification is 0.6% in patients with mitral annular calcification and 0.06% to 0.07% in large series of patients of all ages.3 The prevalence in necropsy series has been reported to be 2.7%,2 however, which indicates that this entity is not yet adequately recognized by most clinicians.1,3

In the largest reported series of 18 patients, the echocardiographic finding of caseous calcification in all except 1 patient was incidental and mostly unrelated to symptoms.1 Similar to the case in this study, the symptomatic patient had mitral stenosis caused by the mass obstructing the mitral inflow. Baseline clinical characteristics of patients with caseous calcification and mitral annular calcification are comparable; therefore, the diagnosis of caseous calcification cannot be made on the basis of clinical symptoms and findings.1 Similarly, autopsy findings such as severe coronary artery disease, aortic atherosclerosis, and a history of hypertension are equally prevalent among patients with both mitral annular calcification and caseous calcification.2

Several misdiagnoses of caseous calcification as abscesses and cardiac tumors have been reported.1 Therefore, the imaging appearance of this entity deserves further elucidation. As was the case in this patient, caseous calcification is usually detected by transthoracic or transesophageal echocardiography and is characterized as a round or semilunar, echo-dense mass with smooth borders, sometimes encircling a central area of echolucency resembling liquefaction.1,3–5 The CT appearance, which has never been demonstrated before, consisted of a well-defined, oval, hyperdense mass with peripheral calcifications in our patient. The central hyperdensity on CT is most likely explained by the pathologic finding of a dense, tenacious substance filling the center of the mass. Distinguishing features between mitral annular abscess and caseous calcification include the lack of larger amounts of calcification in the case of mitral abscesses.4 A cardiac tumor can be ruled out on CT by the lack of soft-tissue density and absence of contrast enhancement.

In summary, caseous calcification represents a rare, underappreciated variant of mitral annular calcification that should be differentiated from an abscess or tumor. It is likely that the growing clinical use of cardiac CT may increasingly uncover this rare entity.

Footnotes

The work was supported by the National Center of Competence and Research, Computer Aided and Image Guided Medical Interventions of the Swiss National Science Foundation.

References

  1. Harpaz D, Auerbach I, Vered Z, Motro M, Tobar A, Rosenblatt S. Caseous calcification of the mitral annulus. a neglected, unrecognized diagnosis. J Am Soc Echocardiogr 2001;14:825-831.[Medline]
  2. Pomerance A. Pathological and clinical study of calcification of the mitral valve ring. J Clin Pathol 1970;23:354-361.[Abstract/Free Full Text]
  3. Novaro GM, Griffin BP, Hammer DF. Caseous calcification of the mitral annulus. an underappreciated variant. Heart 2004;90:388.[Free Full Text]
  4. Kronzon I, Winer HE, Cohen ML. Sterile, caseous mitral anular abscess. J Am Coll Cardiol 1983;2:186-190.[Abstract]
  5. Teja K, Gibson RS, Nolan SP. Atrial extension of mitral annular calcification mimicking intracardiac tumor. Clin Cardiol 1987;10:546-548.[Medline]



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