Elective Aortic Surgery for Prevention of Aortic Dissection in Turner Syndrome: The Potential Impact of Updated European Society of Cardiology and International Turner Syndrome Consensus Group Guidelines on Referrals to the Heart Team.

Abstract

Objective: The risk of aortic dissection is increased in Turner Syndrome (TS). Aortic dilation is thought to contribute to this risk and may be managed with elective aortic surgery. New TS guidance has lowered the aortic size thresholds for consideration of aortic surgery. We investigated the impact of new guidance on potential heart team referrals in a UK cohort of TS individuals.

Methods: A cross-sectional study of 156 individuals with TS was performed. Up to date transthoracic echocardiography or cardiac MRI derived aortic dimensions, anthropometric data and the presence of aortic dissection risk factors were analysed.

Results: Twenty-one individuals (13%) met updated guideline criteria for consideration of aortic surgery, 15 more than met 2016 TS guideline criteria. Use of aortic size index (ASI) and aortic height index (AHI) together identified additional individuals meeting criteria for surgical consideration compared with the use of ASI or AHI alone. Z-score identified no additional individuals for surgical consideration, nor did it reclassify any individuals into moderate or severe aortic dilation groups. Twelve of 13 individuals with moderate aortic dilation met criteria for surgical consideration due to the presence of additional risk factors for aortic dissection. There was no positive correlation between height or body surface area and ascending aorta diameter in this cohort.

Conclusions: New TS guidelines are likely to significantly increase the number of individuals with TS who might be considered for elective aortic surgery. Centres caring for individuals with TS should re-evaluate their TS cohorts for aortic dissection risk considering these new guidelines.