Caseous calcification of the mitral valve annulus
Developed attacks of fainting, initial investigation demonstrated abnormal ECG findings with atrial fibrillation pattern. Additional echocardiography demonstrated cardiac mass.
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Axial non-enhanced CT demonstrates large dense calcified mass lesion involving the posterior commisure of the mitral valve
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Cardiac MRI showed intense blooming of the entire mass due to calcifications (axial gradient echo), the mass primarily originated and extended along the mitral valve annulus posterior surface (sagittal gradient echo) and was associated with high grade mitral valve regurgitation manifested by the strong aliasing toward the atrium during ventricular contraction (axial gradient echo arrow). The mass demonstrated intense enhancement at the delayed scans.
Caseous calcification of the mitral annulus (CCMA) is a rare form of mitral annular calcification occurring in 0.6% of the cases of MAC 1. It is a degenerative disorder common in the elderly, in particular women, that typically affects the posterior annulus commissure 1. This disorder carries a benign prognosis. However, physicians who are not familiar with this condition and may interpret it as a (benign or malignant) cardiac tumor, vegetation or calcified thrombus 2,3,4, therefore, a punctual differential diagnosis is required. CCMA rarely may be complicated with mitral valve dysfunction, systemic embolization, or conduction abnormalities 5. CCMA may demonstrate dynamic course, it may resolve spontaneously or transform back to an isolated form of simple mitral annulus calcification. However, it may recur even after surgical excision 6. Currently, CCMA is managed conservatively except when significant associated mitral valve dysfunctions or associated distant embolic events 4.
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- 2. Fujiwara M, Watanabe H, Iino T, Kobukai Y, Ishibashi K, Yamamoto H, Iino K, Yamamoto F, Ito H. Two cases of calcified amorphous tumor mimicking mitral valve vegetation. (2012) Circulation. 125 (10): e432-4. doi:10.1161/CIRCULATIONAHA.111.072793 - Pubmed
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- 5. Davidson MJ, Cohn LH. Surgical treatment of caseous mitral valve annulus calcification. (2006) The Journal of thoracic and cardiovascular surgery. 131 (3): 738-9. doi:10.1016/j.jtcvs.2005.11.025 - Pubmed
- 6. Koito H, Nakamura C, Suzuki J, Takahashi H, Iwasaka T. Reduced size of liquefaction necrosis of mitral annular calcification in chronic renal failure by using low calcium concentration hemodialysis. (1999) Japanese circulation journal. 63 (6): 490-2. Pubmed