BSPED2024 Poster Presentations Miscellaneous/Other 2 (9 abstracts)
Optimising risk stratification for aortopathy in turner syndrome using aortic size index
Alexander James. Fletcher 1,2 , Aoife Carr 1 , Lindsey McVey 1 , Martin Denvir 2,3 , Ailsa Gebbie 4 , Guilherme Guaragna-Filho 5 , Lindsey Hunter 6 , Stuart Hutchinson 3 , Shruti S. Joshi 3 , Sofia Helena. Valent. de Lemos-Marini 5 , Renata Isa. Santoro 7 , Nicola Walker 8 , Avril Mason 1 & Sze Choong. Wong 1
1Developmental Endocrinology Research Group, Royal Children Hospital Glasgow, Glasgow, United Kingdom; 2Centre for Cardiovascular Science, Edinburgh, United Kingdom; 3Department of Cardiology, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom; 4Chalmers Centre for Sexual and Reproductive Health, Edinburgh, United Kingdom; 5Paediatric Endocrinology Unit, Faculty of Medical Sciences, State University of Campinas, Campinas, Brazil; 6Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, United Kingdom; 7Paediatric Cardiology Unit, Faculty of Medical Sciences, State University of Campinas, Campinas, Brazil; 8Scottish Adult Congenital Cardiac Service, Golden Jubilee National Hospital, Clydebank, Glasgow, United Kingdom
Background: Turner Syndrome (TS) is a complete or partial loss of the second X chromosome affecting approximately 1:2000 females. Cardiovascular complications in TS include increased risk of congenital cardiac malformations, hypertension, and aortic dissection. The Clinical Practical Guidelines for the Care of Girls and Women with Turner Syndrome (published 2024) guidance suggests using aortic size index (ASI, aortic diameter divided by body surface area) thresholds. The ASI is feasibly influenced by BSA equation selection, and falsely reassuring in those with very high weight. We analyse the influence of BSA estimation equation selection and after adjustment for ideal body weight on those meeting threshold for surgery.
Methods: A retrospective observational cohort study was carried out in three tertiary centres of patients with confirmed TS attending for routine monitoring of their aortic diameter through echocardiography or magnetic resonance imaging. ASI error from DuBois was calculated and Bland-Altman plots for each of five alternative body surface area estimation equations were analysed. Numbers reaching the threshold for surgical intervention were determined for each of ASI and ideal-weight-aortic size index (iwASI, weight adjusted to the same z-score for height).
Results: 132 patients were included in the study. Mean age at imaging was 27.9 years (range 16 – 58 years.) 112 subjects had karyotypes available, 50 (45%) were 45X, 50 (45%) were 45X/46XX or other variants, 12 (11%) had Y material. Compared to DuBois, all alternative BSA estimation equations underestimated ASI, with the greatest degree of underestimation when Furqan or Boyd equations were used. In a sub-analysis of 53 subjects with clinical risk factors, up to seven out of the 53 subjects (13%) had aortic risk category lowered simply by using an alternative BSA estimation equation for adjustment of ASI. Using iwASI, four subjects were identified as meeting the criteria for surgical intervention, that were not identified using standard ASI; one of which experienced a fatal aortic dissection. All four subjects had BMI > 35 kg/m2.
Conclusion: Our results demonstrated that compared with Dubois, alternative BSA estimation equations lead to underestimation of ASI. Our findings suggest using iwASI values should be considered when interpreting ASI in TS, particularly at extremes of weight.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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