Caseous calcification of mitral annulus in the setting of multivessel disease

Abstract

C aseous calcification of the mitral annulus (CCMA) is a chronic degenerative process affecting the mitral valve fibrous ring. It is a rare variant of mitral annular calcification (MAC) that is frequently misdiagnosed as endocarditis, cardiac tumor, or abscess. Patients can present with palpitations and dyspnea; however, CCMA does not have a specific clinical presentation and diagnosis is typically made incidentally by presence of an intracardiac mass on cardiac imaging. Transthoracic echocardiography (TTE) remains the preferred imaging modality for diagnosis of CCMA; however, multimodality imaging with transesophageal echocardiography (TEE), cardiac computed tomography (CT), or cardiac MRI (CMRI) may be necessary when the diagnosis remains unclear. Although CCMA is frequently benign, it has been associated with cerebral embolization and valvular dysfunction. Multimodality imaging can clearly differentiate CCMA from other lesions and should be employed when diagnosis remains unclear. Uncomplicated CCMA can be managed conservatively and surgical intervention with mitral valve replacement is reserved for complicated cases. Calcifications of the mitral valve annulus have a higher prevalence in patients with multiple cardiovascular risk factors, as was seen in our patient. While her symptoms of chronic angina, dizziness, dyspnea, and palpitations were likely due to multivessel coronary artery disease (CAD), evaluation of her symptoms led to incidental discovery of a large mobile cardiac mass with high risk for embolization. This case demonstrates the importance of employing multimodality imaging to accurately diagnose CCMA in a highrisk patient (elderly, female, and multiple CAD risk factors). We present the case of a 68 years old Hispanic female with past medical history hypertension, hyperlipidemia, heart failure with preserved ejection fraction, and diabetes mellitus (DM) who presented to the emergency department with one year history of typical anginal chest pain with radiation to the back, dyspnea on exertion, and occasional nighttime dizziness and palpitations. She had an outpatient stress TTE to evaluate chronic angina which revealed a mobile echoic mass on the posterior mitral valve leaflet concerning for tumor, thrombus, or vegetation. Stress echo was prematurely terminated upon detection of the mass and the patient was instructed to start Coumadin and seek further evaluation in the emergency department. Upon admission, patient’s vital signs were within normal limits; basic labs and cultures obtained to rule out concerns for an infectious process were negative for acute infection. CT chest, however, demonstrated extensive calcification in the area of the mitral of the mitral valve annulus. Cardiology was consulted for a transesophageal echocardiogram to further characterize the mass. TEE revealed a hypermobile 0.7 × 1.0 cm pseudo-pedunculated hyperechogenic structure with areas of central lucency extending from the posterior mitral valve annulus. There was trace mitral regurgitation however no evidence of mitral stenosis or outflow tract obstruction. Surgical removal of the mass was pursued as its large size and degree of mobility conferred a high risk of embolization. Pre-operative left heart catheterization was perJournal of Geriatric Cardiology