Purposeful use of multimodality imaging in the diagnosis of caseous mitral annular calcification: a case series report

Background Caseous mitral annular calcification (CMAC) is a rare liquefactive variant of mitral annular calcification (MAC) and superficially mimics a cardiac vegetation or abscess. CMAC is viewed as a benign condition of MAC, while MAC has clinical implications for patients’ lives. Correctly diagnosing CMAC is essential in order to avoid unnecessary interventions, cardiac surgery or even psychological suffering for the patient. Case presentation We report on 6 patients with suspected intra-cardiac masses of the mitral annulus that were referred to our institution for further clarification. A definitive diagnosis of CMAC was achieved by combining echocardiography (Echo), cardiac magnetic resonance imaging (MRI) and cardiac computed tomography (CT) for these patients. Echo assessed the mass itself and possible interactions with the mitral valve. MRI was useful in differentiating the tissue from other benign or malign neoplasms. CT revealed the typical structure of CMAC with a “soft” liquefied centre and an outer capsule with calcification. Conclusion CMAC is a rare condition, and most clinicians and even radiologists are not familiar with it. CMAC can be mistaken for an intra-cardiac tumour, thombus, vegetation, or abscess. Non-invasive multimodality imaging (i.e. Echo, MRI, and CT) helps to establish a definitive diagnosis of CMAC and avoid unnecessary interventions especially in uncertain cases.

Recurrent ST-Elevation Myocardial Infarction: Case Report of A Rare Complication of Caseous Mitral Annular Calcification

Background Caseous mitral annular calcification is an under-diagnosed division of calcific mitral valve disease that has recently been reported to have increased propensity for embolic disease. Early recognition of this entity as a cause of embolic disease can lead to prevention of occlusive vascular disease and long-standing complications. Case Summary We present the case of a patient with end-stage renal disease who presented for evaluation of chest pain and was found to have ST-segment myocardial infarction. Despite thrombectomy and stenting, he had multiple recurrent events, and imaging evaluation demonstrated caseous mitral annular calcification with mobile components. He was taken for surgical replacement of the mitral valve, with pathology confirming diagnosis. Discussion Caseous mitral annular calcification may represent an increased risk of embolic disease. Better understanding of this pathology and it’s propensity for embolic disease will be important to best determine treatment plans and timing of operative intervention.

Abstract 16851: Simultaneous Mitral Valve Replacement, Aortic Valve Replacement, Tricuspid Valve Repair, Left Atrial Appendage Ligation, Maze Procedure And Cabg In A 57-year Old Female With History Of Tavr And Severe Mitral Annular Calcification

Background: Open heart surgery is preferred for patients with multiple valvular issues with conflicting physiology. Case: A 57-year-old female with a past medical history of Type I Diabetes Mellitus complicated by kidney & pancreatic transplant in 1999 s/p failed kidney transplant in 2016 subsequently back on dialysis, aortic stenosis leading to Transcatheter Aortic Valve Replacement (TAVR) in 2016 who presented to the hospital for profound cardiogenic shock and was found to have severe tricuspid regurgitation, severe mitral stenosis secondary to mitral annular calcification with severe concentric hypertrophy of left ventricle. Decision Making: Her conflicting right and left heart physiology due to Tricuspid Regurgitation and Mitral Stenosis was very difficult to manage medically. She was not deemed a candidate for a heart transplant due to a history of a failed kidney transplant. She underwent surgery which included Mitral Valve Replacement, explant TAVR and repeat aortic valve replacement, Tricuspid repair, Left atrial appendage ligation and maze procedure, and CABG x1 with saphenous vein graft to PDA. She stayed on V-V extracorporeal membrane oxygenation (ECMO) post-operatively and was discharged on post-operative Day # 14. Conclusion: Severe tricuspid Regurgitation & coexisting mitral stenosis pose a dilemma for medical management and only feasible option is surgery which could be extremely challenging.