Caseous calcification of the mitral annulus: case report and brief review

Abstract

BackgroundCaseous calcification of the mitral annulus (CCMA) is a very rare variant of mitral annular calcification, which is typically asymptomatic but can manifest as a cardiac tumour, abscess, or in the form of mitral valve dysfunction.Case summaryWe present a patient who developed shortness of breath and was initially thought to have an intracardiac tumour, but ultimately was recognized as massive calcification of the mitral valve by computed tomography angiogram. This finding was unfortunately made only shortly before the patient’s clinical deterioration, and the specific diagnosis of CCMA was made on post-mortem findings, precluding any directed treatment for this entity.DiscussionCaseous calcification of the mitral annulus can lead to significant pathology, including mitral regurgitation, stenosis, or systemic embolization of caseous material. Due to its relative scarcity and tendency to be asymptomatic, a diagnosis of CCMA is often difficult to make or easily overlooked. Early recognition and appropriate treatment is crucial for avoiding these potential complications.

Ruptured caseous calcification of the mitral annulus

“…Clinical presentation, size, shape, location, mobility and other echocardiographic findings can help differentiate the aetiology. 6 In regard to treatment, it is important to distinguish between CCMA and MAC, as anticoagulation and antiplatelet therapy is ineffective in the prevention of cardioembolic stroke in patients with CCMA. 15 CCMA maybe at increased risk of cardioembolic stroke, which is not related to AF.…”

“…Majority of patients are treated conservatively, with most having spontaneous resolution back to typical MAC. 6 Surgery is indicated and has been relatively successful in those patients who have severe mitral valve dysfunction or systemic embolisation of the caseous material. Despite therapy, some patients have had reoccurring CCMA, even after surgical excision.…”

“…4,5 Its appearance is ovoid in shape, focal mostly to the posterior annulus and found with internal, caseous fluid-like calcifications and debris. 6 CCMA contains a mixture of calcium, salts, fatty acids and cholesterol and results in a soft mass in the mitral annulus. 7 Although often picked up incidentally on imaging, MAC and CCMA are often asymptomatic, benign and can be misdiagnosed as thrombus, abscess, cardiac tumour or vegetation often resulting in unnecessary surgery.…”

Caseous mitral valve calcification and concurrent hypertrophic obstructive cardiomyopathy: A rare cause of stroke

“…It can also lead to the same hemodynamic consequences of constrictive pericarditis. While all these complications significantly increase the risk of CVA, we believe that CCMA independently carries a higher risk of cerebrovascular complications especially in presence of HOCM [1] , [3] , [5] , [6] .…”

“…CCMA is a distinctive and special variant of MAC that results from liquefactive necrosis of the posterior annulus of the mitral valve and is usually considered a benign condition [1] , [2] . In rare cases, however, it may compromise the normal function of the mitral valve, subsequently causing regurgitation or mitral stenosis [3] . The later can cause significant left ventricular inflow tract obstruction.…”