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Published on: August 28, 2018
Ruptured caseous calcification of the mitral annulus
Richard P Allwood1, Emma L Bonacci1, Erin McKinnon1
1Cardiology Department St Vincent's Hospital Melbourne 41 Victoria Parade Fitzroy 3065 Australia.
Abstract:
Mitral annular calcification (MAC) is considered a chronic and degenerative process involving the fibrous annulus of the mitral valve. The prevalence of MAC has been reported between 8% and 15%. It significantly increases with age, often seen in females, individuals with hypertension, chronic kidney disease and those with multiple cardiovascular risk factors. Caseous calcification of the mitral annulus (CCMA) (also known as caseoma) is a rare variant of MAC and should be considered in the differential diagnosis with other cardiac masses of the mitral valve. An 85-year-old female presented for a transthoracic echocardiogram with a history of hypertension and a systolic murmur. The echocardiogram demonstrated a possible rare variant of MAC, with independent mobile echodensities identified. Further testing using transoesophageal echocardiography was used to confirm diagnosis and ensure appropriate clinical management was arranged. This case demonstrated CCMA, a benign condition that can be mistaken for cardiac tumours, thrombus, vegetations or abscesses. A low prevalence has been reported between 0.06% and 0.07% and 0.6% of patients with MAC using echocardiography. Echocardiography can provide the initial diagnosis for identification and characterisation of MAC. CCMA appears as a calcified mass with an echolucent, liquid-like inner part, located on the posterior mitral valve annulus. Acoustic shadowing is usually absent. Multi-modality imaging can lead to an accurate diagnosis of CCMA without unnecessary interventions. Transoesophageal echocardiography provides further information on the site and composition of the internal material. Computed tomography (CT) and cardiac magnetic resonance imaging (MRI) can also be used, to confirm the diagnosis and to exclude other differential diagnoses.
Insights
Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification (MAC). This benign condition can mimic cardiac tumors but is accurately diagnosed with multi-modality imaging.
Area of Science:
- Cardiology
- Radiology
- Pathology
Background:
- Mitral annular calcification (MAC) is a common degenerative process affecting the mitral valve annulus, with prevalence increasing with age and associated with cardiovascular risk factors.
- Caseous calcification of the mitral annulus (CCMA) is a rare, benign variant of MAC, often presenting as an echodense mass on the mitral valve annulus.
- CCMA requires differentiation from other cardiac masses such as tumors, thrombi, or vegetations.
Observation:
- An 85-year-old female with hypertension and a systolic murmur presented with findings suggestive of a rare MAC variant on echocardiography.
- Transthoracic echocardiography revealed independent mobile echodensities, prompting further investigation with transesophageal echocardiography.
- The case confirmed CCMA, characterized by a calcified mass with an echolucent, liquid-like center on the posterior mitral annulus, typically without acoustic shadowing.
Findings:
- CCMA is a rare condition with a reported prevalence of 0.06%-0.6% among patients with MAC.
- Echocardiography is effective for initial diagnosis and characterization of MAC and CCMA.
- Multi-modality imaging, including transesophageal echocardiography, CT, and MRI, aids in accurate diagnosis and exclusion of differential diagnoses.
Implications:
- Accurate diagnosis of CCMA using imaging techniques prevents unnecessary invasive interventions.
- Understanding CCMA's distinct imaging features is crucial for differentiating it from malignant cardiac tumors or other pathologies.
- This case highlights the importance of considering rare variants of common conditions in clinical practice.
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