Long-term rheumatic heart disease complicated with 'porcelain atrium'.

A 71-year-old female patient was admitted to the hospital due to ‘progressive chest tightness and shortness of breath for 2 years, aggravated for 8 months’. She had a history of rheumatic heart disease and underwent mitral valve bioprosthesis replacement plus tricuspid valvuloplasty 18 years earlier. The patient was also complicated with atrial fibrillation for more than 40 years and received regular long-term medication. Physical examination: cyanosis of the lips, irregular heart rhythm, and bilateral lower extremity oedema. Cardiac auscultation revealed systolic murmurs in the mitral and tricuspid valve areas. Laboratory examinations indicated haemoglobin 36 g/L and coagulation dysfunction. Echocardiography suggested bilateral atrial enlargement, severe tricuspid regurgitation, and severe deterioration of the mitral bioprosthesis. Cardiac computed tomography angiography (CTA) demonstrated extensive calcification of the left atrial wall and interatrial septum (Panels A–F, asterisks), consistent with the manifestation of ‘porcelain atrium’ (see Movies S1 and S2). The patient underwent transcatheter mitral valve-in-valve replacement. Despite aggressive supportive treatment, the patient progressed to refractory cardiogenic shock and multiple organ dysfunction syndrome and was clinically deceased 11 days after surgery. ‘Porcelain atrium’, also called ‘coconut atrium’, was first described in 1898. It is characterized by massive left atrial wall calcification, predominantly affecting middle-aged and elderly females with rheumatic mitral valve disease and atrial fibrillation. Interatrial septum involvement significantly increases the difficulty and risk of mitral valve surgery, accelerates bioprosthetic deterioration, and elevates risk further when complicated with pulmonary hypertension or tricuspid regurgitation.1,2 Computed tomography can clearly visualize these calcifications, and three-dimensional (3D) post-processing accurately delineates their location and extent, offering more precise imaging support for preoperative evaluation and diagnostic–therapeutic decisions in such high-risk patients.