Case Presentation: A 39-year-old woman presented to the emergency room with dyspnea and substernal chest pain following a dialysis session. Her history included end stage renal disease, hypertension and diabetes. Physical exam revealed a 2/6 systolic murmur at the apex. Electrocardiogram (ECG) returned with changes concerning for inferolateral ischemia prompting ST-elevation myocardial infarction (STEMI) activation.Left heart catheterization revealed non-occlusive coronary artery disease with a 20-30% lesion in the proximal segment of the left circumflex artery. Transthoracic echocardiogram (TTE) demonstrated a poorly characterized mass with concern for tumor or infection (Figure 1). Subsequent transesophageal echocardiogram (TEE) was completed and the mass was described as a severe caseous calcification of the mitral annulus (CCMA) with an associated mobile plaque. Repeat ECG was largely unremarkable and did not have the prior inferolateral ischemic changes.The patient was ultimately placed on a high intensity statin as well as dual anti-platelet therapy. She was referred to cardiothoracic surgery, though left the hospital against medical advice and chose to pursue outpatient follow up. While no aspiration of the plaque was performed during catheterization, the size and mobile nature of the plaque very likely played a significant role in the patient’s transient coronary embolization event.
Discussion: Mitral annulus calcification (MAC) remains a common finding on cardiovascular imaging with an incidence that widely depends on underlying patient risk factors. It is typically found in older patients with significant co-morbidities. A variant of MAC, caseous calcification of the mitral annulus (CCMA) can often affect younger patients with end stage renal disease. Given the rarity of CCMA, it poses a diagnostic challenge. CCMA also has a multitude of clinical implications and complications, including embolic stroke and mitral regurgitation, making the disease important to recognize and appropriately treat.
Conclusions: While this case was appropriately escalated to cardiology, general internal medicine services still played a pivotal role. Most MAC will be found incidentally on TTE and this finding often requires further diagnostic imaging to fully characterize the pathology and is associated with an increased atherosclerotic burden. This case illustrates the importance of recognizing CCMA, leading to the subsequent medical management in mitigating risk as well as possibility of surgical referral to fully treat the patient.
