Aortic dilatation and outcome in women with Turner syndrome.
BACKGROUND
Women with Turner syndrome (TS) are at increased risk of aortic dissection, which is related to ascending aortic diameter. However, the relation between aortic diameter and outcome is not well determined. This study evaluates the prevalence of aortic dilatation, the growth rate of the aorta and the risk of aortic complications in adults with TS.


METHODS
Single centre, retrospective study of all women with TS followed with a strict protocol in an outpatient TS clinic. Aortic diameters were analysed using advanced imaging. The primary outcome was a combined endpoint of aortic-related mortality, aortic dissection and preventive aortic surgery. The secondary endpoint was aortic growth and prevalence of aortic dilatation, defined as an aortic size index >20 mm/m2 at baseline.


RESULTS
At least one cardiac MR/CT was available in 268 women with TS, having median age of 28.7 (IQR: 21.3-39.7) years. Aortic dilatation was present in 22%. Linear regression identified independent factors associated with larger aortic diameters: age (coefficient=0.23; p<0.001), hypertension (coefficient=2.7; p<0.001), bicuspid aortic valve (coefficient=3.3; p<0.001), 45XO karyotype (coefficient=1.7; p=0.002), weight (coefficient=0.075; p<0.001) and growth hormone treatment (coefficient=1.4; p=0.044). During follow-up (6.8±3.2 years), five women (2%) reached the primary endpoint (two dissections, three aortic surgery). Women withmore than one scan (n=171; 1015 patient-years follow-up), the median aortic growth was 0.20 (IQR: 0.00-0.44) mm/year. In multivariate analysis, aortic growth was not associated with baseline aortic diameter or other variables.


CONCLUSIONS
Aortic dilatation is common and known associations were confirmed in large adult TS cohort However, aortic dissection, related mortality and preventive aortic surgery are rare. Growth hormone treatment in childhood was associated with aortic dimensions.
['Duijnhouwer|Anthonie L|AL|0000-0001-5064-0143', 'Bons|Lidia R|LR|', 'Timmers|Henri J L M|HJLM|', 'van Kimmenade|Roland R L|RRL|', 'Snoeren|Miranda|M|', 'Timmermans|Janneke|J|', 'van den Hoven|Allard T|AT|', 'Kempers|Marlies|M|', 'van Dijk|Arie P J|APJ|', 'Fleischer|Kathrin|K|', 'Roos-Hesselink|Jolien W|JW|']
D000328:Adult D000784:Aneurysm, Dissecting D001013:Aorta, Thoracic D017545:Aortic Aneurysm, Thoracic D004452:Echocardiography D004562:Electrocardiography D005260:Female D005500:Follow-Up Studies D006801:Humans D019028:Magnetic Resonance Imaging, Cine D009426:Netherlands D015995:Prevalence D012189:Retrospective Studies D015996:Survival Rate D014057:Tomography, X-Ray Computed D014424:Turner Syndrome D055815:Young Adult
aortic and arterial disease aortic aneurysm congenital heart disease