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. 2023 Nov 27;12(6):569-576.
doi: 10.21037/acs-2023-adw-0083. Epub 2023 Aug 24.

Outcomes of cardiothoracic surgery in women with Turner syndrome

Affiliations

Outcomes of cardiothoracic surgery in women with Turner syndrome

Lucas Ribé et al. Ann Cardiothorac Surg. .

Abstract

Background: To describe short- and mid-term surgical outcomes of patients with Turner syndrome (TS) after cardiovascular interventions.

Methods: All individuals >12 years of age at the time of surgical repair for cardiovascular disease (valve or coarctation repairs, aortic disease, aortic dissection) from 2002 to 2022 were eligible. The primary endpoint was complications or death within 30 days of intervention. Secondary outcomes included late complications and reinterventions within six months. Combined data from the University of Texas Health Science Center at Houston and the Turner Syndrome Society of the United States were included in the analysis.

Results: We identified 22 patients who met the inclusion criterion. The median age was 46 years (range, 21-75 years), with 86% having estrogen replacement therapy. The most common medical condition was hypertension (77%), followed by hypothyroidism (59%). The most frequent indication for surgery was aortic root or ascending aortic aneurysms (68%), followed by symptomatic aortic stenosis in patients with bicuspid aortic valve (64%), coarctation of aorta (45%), and acute aortic dissection (18%). Respiratory complications were the most common (68%). Pleural effusions were the most frequent found sign on imaging studies (68%). Thoracentesis, or chest tube placement, was required in 33% (5/15). Respiratory failure requiring specific support with high flow oxygen and/or thoracentesis occurred in 36% (8/22).

Conclusions: Patients with TS may be at an increased risk for postoperative complications after aortic surgery. Bicuspid aortic valve (59%) and coarctation of the aorta (45%) were the most common congenital malformations among our study group. Our study showed that respiratory complications were the most common, with pleural effusions being the most common post-surgery complication.

Keywords: Aneurysm; Turner syndrome (TS); aortic repair; women.

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Conflict of interest statement

Conflicts of Interest: ROA is a consultant for Medtronic and EndoRon Ltd. ALE is a consultant for WL Gore, CryoLife, Edwards Lifesciences, and Terumo Aortic. SKP is supported by a gift from the Turner Syndrome Society of the United States. The other authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Kaplan-Meier estimates for survival in our TS group. TS, Turner syndrome.

References

    1. Schoemaker MJ, Swerdlow AJ, Higgins CD, et al. Mortality in women with turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 2008;93:4735-42. 10.1210/jc.2008-1049 - DOI - PubMed
    1. Prakash SK, Bondy CA, Maslen CL, et al. Autosomal and X chromosome structural variants are associated with congenital heart defects in Turner syndrome: The NHLBI GenTAC registry. Am J Med Genet A 2016;170:3157-64. 10.1002/ajmg.a.37953 - DOI - PMC - PubMed
    1. Stochholm K, Juul S, Juel K, et al. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006;91:3897-902. 10.1210/jc.2006-0558 - DOI - PubMed
    1. Fuchs MM, Attenhofer Jost CH, Said SM, et al. Cardiovascular surgery in Turner syndrome. Early outcome and long-term follow-up. World J Cardiol 2020;12:97-106. 10.4330/wjc.v12.i3.97 - DOI - PMC - PubMed
    1. Chew JD, Hill KD, Jacobs ML, et al. Congenital Heart Surgery Outcomes in Turner Syndrome: The Society of Thoracic Surgeons Database Analysis. Ann Thorac Surg 2019;108:1430-7. 10.1016/j.athoracsur.2019.05.047 - DOI - PubMed