Ruptured caseous calcification of the mitral annulus

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. Multimodality 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.

Three Cases of Caseous Calcification of the Mitral Annulus Resulting in Spontaneous Fistulation

“…The diameters of the masses in these cases were 36 × 19, 20 × 15, 6 × 5, and 40 × 30 mm, respectively. 5 , 6 , 7 , 8 Considering our 3 cases, the mean and minimum diameters of CCMA, resulting in suspected spontaneous fistula formation, were 23 and 6 mm at the major axis, respectively.…”

Caseous calcification of the mitral annulus: mimicking a cardiac mass.

“…Sometimes a misdiagnosis as a tumour might lead to unnecessary exposure to explorative cardiotomy [ 3 ] . Caseous calcification is usually a benign condition of a dynamic process [ 8 , 9 , 10 ] . In a follow-up of 14 cases of caseous calcification in the prospective echocardiographic study by Deluca et al, it was found that three patients with mitral annulus calcification progressed to caseous calcification, and three patients with caseous calcification reverted to mitral annulus calcification during the study period [ 8 ] .…”

“…There was a reported case of caseous calcification with spontaneous resolution in a 60-year-old woman [ 9 ] . Another case report found a partial capsule rupture, including mobile remnants at the point of rupture with spontaneous resolution in asymptomatic patients [ 10 ] . Based on these reports and the absence of consensus in managing caseous calcification, we opted for only a follow-up of our patient.…”