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Introduction
Aortic dissection is six times more common in Turner syndrome (TS) than in the general population, with an estimated incidence of 40 cases per 100 000 TS years. 1 It most commonly affects women in their mid-30s but can occur during childhood and adolescence. 2-4 The annual incidence of dissection in TS is 15 cases per 100 000 for children and adolescents, 78 cases per 100 000 for women 20-40 years, and 50 cases per 100 000 for older women with TS. 1 Typically, this complication is recognised late and is often fatal, accounting for 8% of all TS deaths. 5 A recent report from the International Turner Syndrome Aortic Dissection Registry has helped characterise the profile of women at risk of aortic dissection. 6 This voluntary registry retrospectively recruited 20 patients with TS with dissection, through the Turner Syndrome Society of the USA, following completion of a questionnaire by the survivors themselves or individuals aware of a dissection in a patient with TS. The most frequent associations were a bicuspid aortic valve, antecedent aortic dilatation and hypertension. In addition, a trend to delay seeking medical advice was seen in younger patients with TS, substantially reducing their likelihood of survival.
Earlier papers have also reported on aortic dissection in TS and these included children and adolescents. 2 3 One paper recorded 46 cases of dissection in patients with TS, 9 of whom were children or adolescents, and the youngest was 4 years of age. 2 In these younger patients, dissection was also strongly associated with the presence of congenital heart defects. 2 The documentation of blood pressure (BP) in these younger patients was generally poor, but studies show that hypertension is common in children and adolescents with TS. 7 8 In addition, one case report describes progressive aortic dilatation during childhood, eventually ending in dissection and rupture in adolescence. 4 However, routine screening for aortic dilatation during childhood and adolescence is not often performed. Since guidance is lacking, we have reviewed the literature on these key risk factors for aortic dissection, and propose recommendations on the optimal imaging for congenital heart defects and aortic dilatation, and on the assessment and management of BP in this patient group.
Risk factors for aortic dissection
Congenital heart...