We report a case of a 44-year-old woman surgically treated for tetralogy of Fallot who experienced an acute and extensive Stanford A type aortic dissection despite the meticulous follow-up. While aortic dilatation is prevalent in individuals with repaired tetralogy of Fallot, aortic dissection represents a rare consequence, that when it appears, is progressive and usually detected during the check-up visits. In the case reported, the dissection was unexpected and severe, and the patient's clinical state worsened suddenly, leading to death after a few days.
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