RESUMO
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. Constant awareness for aortic aneurysms is essential in the Fallot tetralogy population, nevertheless, several causes may contribute to the acute worsening of the clinical condition until the patient's death.