ABSTRACT
Acute aortic syndrome (AAS) is a term that describes interrelated aortic emergencies with similar clinical characteristics and challenges. These are aortic dissection (AD), intramural hematoma (IH), and penetrating atherosclerotic ulcer (PAU). The incidence of AAS is three cases per 100.000 persons per year. Diverse genetic disorders and acquired conditions have been related to the pathogenesis of this disease. Clinical features of patients with any of the three conditions comprising AAS are very similar. A high degree of clinical suspicion and imaging studies are necessary for an accurate diagnosis. Prognosis is clearly related to underlying diagnosis and appropriate surgical repair, in the case of proximal involvement of the aorta. Involvement of distal segments of the aorta may require medical or endovascular therapy according to the presence of complications. After hospital discharge, patients require lifelong follow-up.