Ascending aortic dilatation in Turner syndrome: the natural history
Kristian Mortensen1, Britta Hjerrild1, Kirstine Stochholm1, Niels Andersen2, Keld Soerensen2, Erik Lundorf2, Jens Christiansen1 & Claus Gravholt1
Background: Aortic dissection causes excess mortality in Turner syndrome, and this increased risk is associated with aortic dilation, congenital cardiovascular abnormalities, karyotype, and blood pressure. But risk factor identification is inadequate, and the natural course of aortopathy in Turner syndrome is poorly investigated.
Design and methods: Prospective follow-up study in women with Turner syndrome (n=102, examined twice) and healthy age-matched controls (n=65, examined once), using magnetic resonance imaging (MRI) of thoracic aorta combined with 24-h ambulatory blood pressure monitoring and echocardiography (for aortic valve morphology).
Results: The mean follow-up time was 2.4±0.4 years (range: 1.4 to 3.5 years). Of the 102 TS women enrolled at baseline, one had a chronic dissection, three died unexpectedly, and eight were lost to follow-up. Eleven TS women were excluded due to technically unsuccessful MRI scans, leaving 80 to participate. The sinotubular aortic diameter enlarged with 0.16 mm/year (P=0.02) and the mid-ascending aorta with 0.23 mm/year (P=0.001). The other thoracic aortic diameters were unchanged. Twenty-two patients (30%) increased their aortic diameter increment above the interobserver limits of agreement (rates ranging from 0.7 to 2.7 mm/year). The aortic growth rates were not predicted by congenital abnormalities, karyotype, age, or blood pressure. Pulse rates were higher with faster aortic dilatation rates (P<0.03).
Conclusion: A general ascending aortopathy was evident with increment of sinutubular and mid-ascending aortic size, and a third of the cohort has a seemingly more pronounced aortic growth. The previously identified indices of risk for aortic dilatation and dissection did not predict the increase in aortic size.