Caseous calcification of the mitral annulus with mitral regurgitation and impairment of functional capacity: a case report

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Abstract

Introduction
Mitral annular calcification (MAC) is a chronic degenerative process, which occurs mainly in older patients, particularly in women and in patients with end-stage renal failure on chronic dialysis [1]. Caseous calcification of the mitral annulus (CCMA) is a relatively rare variant with an echocardiographic prevalence of 0.6% in patients with MAC and 0.06% to 0.07% in large series of patients of all ages [2,3].

We describe a patient who was referred to our echocardiographic laboratory because of progressive impairment of functional capacity (up to New York Heart Association (NYHA) class III), and in whom moderate to severe mitral regurgitation (MR) and CCMA were found.

Case presentation
A symptomatic 69-year-old woman (NYHA functional class III) underwent a transthoracic echocardiographic (TTE) examination to assess her left ventricular function. Her past history included hypercholesterolaemia, hypothyroidism and paroxystic atrial fibrillation. A DDD type pacemaker had been implanted due to sick sinus syndrome one year previously. She had marked limitation of physical activity. She was comfortable at rest but breathless on mild exertion. Physical examination revealed a pansystolic murmur of grade 3/6 audible in the mitral area. An electrocardiogram was completely normal. Laboratory examinations were as follows: haemoglobin 12.3 g/dl, glycaemia 72 mg/dl, urea 37 mg/dl, creatinine 0.9 mg/dl, calcium 8.7 mmol/l, phosphate 3.4 mmol/l, serum cholesterol 217 mg/dl and tryglicerides 148 mg/dl.

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TTE revealed an echodense spherical, tumour-like mass (3.0 ─éÔÇö 3.5 cm) located in the peri-annular posterior region close to the atrial side of the posterior mitral leaflet with an internal echolucent area, without acoustic shadowing (Figures 1a and 2a). On Doppler colour flow mapping, moderate to severe mitral regurgitation was seen in the left atrium, but no obstruction to the diastolic transmitral flow was found (Figure 3a). The left ventricle was hypertrophic (interventricular septum at end of diastole was 16 mm, left ventricular posterior wall at end of diastole was 14 mm) without wall motion abnormalities. The left atrium was dilated (anteroposterior diameter 48 mm). The right ventricle and right atrium were normal and the pacemaker lead was confirmed as inserted normally. The aortic valve was tricuspid and showed some calcification with mild stenosis and regurgitation.

  Figure 1
Two-dimensional echocardiogram, apical four-chamber view. (a) Pre-operative: a round echodense large mass attached to the calcified mitral annulus is seen. (b) Postoperative: a smaller round echodense mass attached to the calcified annulus is seen.
  Figure 2
Two-dimensional echocardiogram, apical four-chamber view (detail). (a) Pre-operative: dyshomogeneous echodensity of the mass is evident. (b) Postoperative: the mass has central echolucency surrounded by a hyperechogenic region.
  Figure 3
Two-dimensional echocardiogram, apical four-chamber view, colour Doppler. (a) Pre-operative: moderate to severe mitral regurgitation is present. (b) Postoperative: trivial mitral regurgitation is seen in the left atrium.

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References
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