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A thoracic aortic aneurysm (TAA) is characterized by dilation of the aortic root or ascending aorta; it is a heritable disease in 10% to 20% and of unknown origin in the remainder; it is an increasingly recognized condition that is often diagnosed incidentally. It could be potentially life-threatening if left untreated due to its catastrophic rupture. The pooled incidence and prevalence of TAAs is about 5.3 per 100 000 individuals/year and 0.16%, respectively. The pooled incidence of ruptured aneurysms was estimated at 1.6 per 100 000 individuals/year. Genetic studies indicate that the somatic <i>JAK2</i><sup>V617F</sup> sequence variation is strongly and independently linked with TAAs. Investigators have suggested that irrespective of the absolute aortic diameter values, patients with an aortic height index (AHI) exceeding 2.9 cm/m should be referred to an aortic center for multidisciplinary risk assessment. All the issues concerning ascending aorta aneurysm are herein detailed; the results of meta-analyses of relevant studies are discussed and tabulated and the types of aneurysms and diseases of the ascending aorta and the surgical repair techniques of these pathologies are described and pictorially illustrated.