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A previously healthy 42 year old woman was referred after the discovery of a 3/6 diastolic murmur maximal at the aortic region, with vigorous pulsation of the suprasternal notch. Echocardiography followed by magnetic resonance imaging (left) revealed a fusiform aneurysm of the ascending aorta measuring 7 cm in diameter, without evidence of dissection, extending from the aortic root up to the brachiocephalic artery, associated with severe aortic regurgitation. The patient underwent a Bentall procedure. Histological examination of the operative material showed Erdheim-Gsell cystic medial necrosis of the aorta (below).