RESUMO
Unicuspid aortic valve is a rare congenital anomaly. Two forms of unicuspid aortic valve exist: acommisural and unicommisural forms, both with a predisposition to aortic dilatation. Most patients with this congenital abnormality will eventually require therapeutic intervention for resulting severe aortic stenosis during infancy or childhood. In adults, the most common treatment applied is aortic valvular replacement associated with replacement of the aortic root and ascending aorta. We describe a 43-year-old man admitted to our hospital complaining of chest pain with the diagnosis of acute coronary syndrome. Transthoracic and transesophageal echocardiography showed a unicommisural unicuspid aortic valve associated with an aneurysmally dilated ascending aorta. The patient underwent a Bentall operation and was discharged uneventfully.