Time-Dependent Changes in Porcelain Aorta and Aortic Stenosis in a Patient with Systemic Lupus Erythematosus

Porcelain aorta, defined as extensive calcification of the ascending aorta or aortic arch, is a reported risk factor for embolic stroke during cardiac surgery. However, the time course of the progression of aortic calcification leading to porcelain aorta has not been elucidated. We herein describe a 70-year-old woman who was followed up for systemic lupus erythematosus and antiphospholipid syndrome for approximately 20 years. A routine computed tomography scan revealed progression of ascending aortic calcification to porcelain aorta. The calcification was absent during the preceding 12 years, partial 6 years later, and total after another 3 years. Computed tomography also demonstrated aortic and mitral valve calcification in the development of porcelain aorta. During the 3 years prior to the last admission, annual echocardiography examinations showed progression of calcific aortic stenosis with symptoms. The patient was admitted to our institution for aortic valve replacement. Considering the high risk of perioperative stroke associated with porcelain aorta, transcatheter aortic valve implantation was performed. Postoperative transthoracic echocardiography revealed improvement of the aortic stenosis with no symptoms. The present case revealed aortic calcific progression to porcelain aorta during an approximately 10-year period with deterioration of aortic stenosis within a short time. The aortic and valvular calcification could be attributed to the inflammatory process of systemic lupus erythematosus and antiphospholipid syndrome. The presence of aortic and mitral annular calcification in the serial imaging can provide information on aortic and valvular atherosclerotic progression, which may be modifiable by early steroid-lowering therapy.