Odd association of left atrial appendage aneurysm and caseous mitral annular calcification in an asymptomatic adult

A 58-year-old woman underwent cardiac evaluation in our hospital. She had a family history as two of her sisters passed away from unexplained sudden deaths before the age of 50. She was asymptomatic. Her electrocardiogram showed atrial fibrillation. Transthoracic echocardiogram revealed a large anechogenic mass communicating with the left atrium (Panel A). Cardiac magnetic resonance (CMR) revealed the nature of the mass; it was a very enlarged left atrial appendage (LAA) aneurysm, measuring 72 × 38 mm, connected to the left atrium through a neck 37 mm in diameter (Panels B and C). Also present was caseous mitral annular calcification (CCMA) (Panel D), as well as disjunction and bi-leaflet ballooning. Left atrial appendage aneurysm is a very rare entity, and only a few dozen cases have been reported so far.1 Cardiac magnetic resonance plays an essential diagnostic role, but cardiac computed tomography can also provide valuable data.2 Most cases are congenital, but some cases were secondary to mitral valve disease.3 However, this case is, to our knowledge, the first reported association of CCMA and LAA aneurysm. Surgical aneurysmectomy is often proposed even in asymptomatic patients to prevent major complications such as life-threatening arrhythmia and thromboembolism. Mitral annular disjunction also increases arrhythmic risk.4 Some patients were spared surgery, with good results.5 As our patient declined the surgery, we opted for optimal medical therapy including beta-blockers and oral anticoagulants, with good outcomes after a 2-year follow-up. LA, left atrium; LV, left ventricle; RA, right atrium; RVOT, right ventricular outflow tract; panel B star, left atrial appendage aneurysm; panel D star, caseous mitral annular calcification. Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with the Committee on Publication Ethics (COPE) guidance. Funding: None declared. No new data were generated or analysed in support of this research.