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A 45 year old man who had previously undergone curative surgery and radiotherapy for a thoracic neuroblastoma in childhood was referred for assessment of breathlessness and exertional dizziness. A high resolution thoracic computed tomographic scan demonstrated patchy pulmonary fibrosis and incidentally revealed the presence of localised extensive calcification throughout the left atrium (panel A). He subsequently underwent cardiac catheterisation and fluoroscopy confirmed the presence of gross calcification affecting most of the left atrium (panel B, right anterior oblique view). Angiography revealed normal coronary arteries and left ventricular function with no evidence of mitral regurgitation. The pulmonary artery pressure was 53/12 mm Hg, with a mean of 34 mm Hg, and the mean pulmonary capillary wedge pressure was 20 mm Hg giving a transpulmonary gradient of 14 mm Hg. Strikingly, the phasic pressure tracing demonstrated giant v waves despite the absence of significant mitral regurgitation or a raised left ventricular end diastolic pressure (post a wave 12 mm Hg) (panel C). These findings indicated left atrial non-compliance caused by calcification, suggesting that his symptoms were at least in part caused by a rapid increase in left atrial pressure with exercise. This condition, known as “porcelain” or “coconut” atrium, is rare and can occur in patients with rheumatic mitral valve disease, but has not previously been described in relation to thoracic radiotherapy. Surgical resection by endoatriectomy may occasionally be an option when calcification is non-transmural and spares the septum. Patients may be sensitive to altered loading conditions relating to diuretic or vasodilator treatment, and may be at increased risk of thromboembolism.
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