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Key facts rnDefinition:Multiorgan heritable connective tissue disorder characterized by cystic medical nercrosis of large,medium-sized arteries.rnClassic imaging appearance:①Dilatation of aortic root and sinotubular junction causing “tulip bulb aorta” or “pear shaped” aorta.②With or without fusiform arteriopathy,saccular aneurysms.rnAcute dissection:very high mortality,necessitates emergency surgery.Use transesophageal echocardiography (TEE) for rapid and safe assessment.