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Acute aortic dissection (AAD) is the most common aortic catastrophe. The mortality rate of type A dissection approaches 40% to 50% in 48 hours. Causes of death include rupture, aortic insufficiency, or malperfusion involving the coronary arteries, head vessels, visceral arteries, and lower extremities. Other acute aortic conditions can be confused with AAD. Emergent surgery is usually recommended, although there are some situations in which initial management of malperfusion or conservative therapy can be considered prior to proximal aortic repair. Various surgical techniques are employed to manage AAD. This article reviews the etiology, clinical presentations, and management of patients with type A AAD.