Left atrial calcification is a rare radiographic finding. Here, we describe a case of left atrial calcification associated with congenital pulmonic valve disease requiring multiple interventions.
A 50-year-old female presented with exertional dyspnoea, hypoxia, and orthopnoea. History was significant for congenital pulmonic valve stenosis surgically repaired at age 4 that degenerated to severe insufficiency requiring surgical pulmonary valve replacement at age 31. The bioprosthetic valve subsequently developed severe stenosis that required balloon valvuloplasty 8 months prior to presentation. Surface echocardiogram on admission revealed severe pulmonic insufficiency and significant left atrial calcifications. The mitral valve had posterior annular calcification with normal leaflet mobility and function (Video 1). Computed tomography chest revealed a heavily calcified left atrium (Figure 1). The patient underwent percutaneous endovascular pulmonic valve replacement during which invasive haemodynamic pressure measurements revealed an elevated pulmonary capillary wedge pressure and normal left ventricle end-diastolic pressure consistent with a stiff left atrium. She was diuresed and had significant improvement in her symptoms.
Figure 1.
A sagittal image from a non-contrasted chest computed tomography. Note the diffuse calcifications involving the left atrium (black arrow).
A ‘porcelain’ left atrium, better termed massive left atrial calcification (MLAC), was first described by Claude and Levaditi1 in 1891, and since that time has continued to be sporadically published in the medical literature. Massive left atrial calcification is most commonly associated with rheumatic heart disease; other associations include end-stage renal disease, tuberculosis, radiation, and cardiac surgery.2,3 Its cause is ultimately unknown, but the associated conditions suggest that chronic inflammation is a major driver of MLAC.3
The treatment for MLAC is surgical endoatrioectomy, however, this is only possible in a subset of MLAC patients who do not have involvement of the interatrial septum, the endocardium, or the mitral annulus.3 Medical management consists of diuresis for venous congestion and anticoagulation if indicated for atrial fibrillation or thrombus.
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 COPE guidance.
Conflict of interest: None declared.
Funding: None declared.
References
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