Dilation of the ascending aorta entails a high risk of dissection or aortic rupture in the absence of surgical treatment. Overall, it represents 50% of all thoracic aneurysms, but can be separated into two distinct entities, according to aetiology and surgical management: (1) the aortic root aneurysm, concerning the initial portion, the so called “aortic root”, that includes the sinuses of Valsalva; and (2) the “supravalvular aortic aneurysms” above the sinuses of Valsalva up the brachiocephalic trunk (fig 1). In contrast to the supravalvular aneurysm that can be treated by a simple supracoronary tube graft, the aortic root aneurysm involves the aortic valve which needs to be spared or replaced. Recent surgical advances have been developed for aortic root aneurysms, which are detailed in this report.

INCIDENCE AND RISK FACTORS

Aortic aneurysms remain the 13th leading cause of mortality in western countries.^1,2 The incidence of thoracic aortic aneurysms is estimated to be 4.5 cases per 100 000.^1–3 Supravalvular aortic aneurysms are less common, and predominantly affect male patients (ratio 3:1); the mean age at the time of diagnosis ranges from 59–69 years.³ In the case of aortic root aneurysms, patients are younger (30–50 years), with a 1:1 sex ratio.

Supravalvular aortic aneurysms are caused by atherosclerosis in relation to hypertension, whereas aneurysm of the aortic root is related to dystrophic degeneration of the aortic wall—so called cystic medial necrosis.^1,3 Aneurysm of the aortic root (annulo-aortic ectasia) can be either idiopathic or associated with well-defined connective tissue disorders such as Marfan syndrome, Ehler Danlos syndrome, or bicuspid valve^1,3 (figs 2 and 3).

Twenty per cent of patients with Marfan syndrome (autosomal dominant connective tissue disorder, incidence 2/5000) will be operated …