Massive left atrial calcification, tracheobronchopathia osteoplastica and mitral paravalvular leak associated with cardiac rheumatic disease and previous mitral valve replacement

Massive Left Atrial Calcification in Late Rheumatic Heart Disease

“…While the name “porcelain atrium” refers to the LA calcification that involves the LA appendage, free wall, and mitral valve apparatus, but spares the IAS, 3 the term “coconut atrium” is reserved for the involvement of the entire LA circumference. 1 Whether there are different pathophysiological mechanisms implied in the involvement or sparing of the IAS is still uncertain. Importantly, when this structure is affected, as in the presented case, it represents a major obstacle for surgical and interventional procedures.…”

“…Women are more frequently affected (approximately 74% of cases), 8 and although most patients remain asymptomatic for long periods of time, the progressive LA noncompliance and increased pressure status might lead to pulmonary hypertension, severe tricuspid insufficiency, and development of symptoms like progressive dyspnea and chronic dry cough. 1 …”

“…Within these tissues, the presence of massive left atrial (LA) calcification is extremely rare and has been proposed to represent the result of long-standing RHD, although the mechanisms implied are not fully elucidated. 1 We present a case of complete LA mural calcification of the left atrium (LA) 20 years after surgical mitral valve replacement (MVR) for RHD.…”