Porcelain Aorta: Time to Recognize

A 54-year-old female was evaluated in 2012 for management of hypertension and dyslipidemia. Clinical examination was unremarkable except for blood pressure (BP) 160/90 mm Hg and a grade 2/6 ejection systolic murmur along the left sternal border. A skiagram of the chest (posterior-anterior view) revealed a cardiothoracic ratio (CTR) of 0.5, clear lung fields, and extensive calcification outlining the dilated ascending and thoracic aorta (arrows, Fig. 1A), prompting the diagnosis of porcelain aorta (PA). Two-dimensional transthoracic echocardiography (TTE) demonstrated concentric left ventricular hypertrophy (LVH), thickening of aortic valve, dilation of the ascending aorta without any aortic regurgitation (AR) or gradient across the valve, its outflow, and in the supravalvular region. Computed tomography (CT) aortogram showed asymmetric extensive calcification of the wall of dilated thoracic and descending aorta and mild calcification of the ascending aorta (Fig. 1B). Biochemical tests were normal except for deranged lipid profile. During the last 10-year follow-up, she has remained asymptomatic with well-controlled BP and lipids on amlodipine, ramipril, statins, and aspirin. Follow-up X-ray shows a CTR of 0.6, further dilation of the ascending aorta, and extensive calcification in ascending, aortic arch, thoracic, and descending aorta (arrow, Fig. 2A). CT aortogram revealed extension of calcification in the ascending aorta with its aneurysmal dilation as well as ectasia in descending aorta and extension of calcification up to the origins of the renal arteries and celiac trunk (Figs 2B to D). Serial TTE did not demonstrate any AR or hemodynamic alteration.