Congenital heart surgery outcomes in Turner syndrome: the society of thoracic surgeons database analysis
Open accessAbstract
Background Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45,XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to individuals without genetic syndromes.Methods The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000 to 2017 in pediatric patients (aged 0-18 years) with and without TS. Analyses were stratified by the most common operations, including coarctation repair, aortic arch repair, partial anomalous pulmonary venous return repair, Norwood, superior cavopulmonary anastomosis (Glenn), and Fontan.Results Included were 780 operations in TS and 62,659 operations in controls. The most common TS operations were coarctation repair in 274 (35%), aortic arch repair in 116 (15%), and Norwood in 59 (8%). Compared with controls, TS patients had lower weight-for-age Z-scores across all operations (P < .01 for all); however, operative mortality rates did not differ significantly. The chylothorax rate was higher in TS after coarctation repair (8.8% vs 2.8%, P < .001) and Norwood (22% vs 8.1%, P < .001). The median (interquartile range) postoperative length of stay was longer in TS for coarctation repair (6.5 [5.0-15.5] days vs 5.0 [4.0-9.0] days, P < .001), aortic arch repair (15.0 [8.0-27.5] days vs 11.0 [7.0-21.0] days, P = .004), and Glenn (9.0 [6.0-16.0] days vs 6.0 [5.0-11.0] days, P = .013).Conclusions Turner syndrome patients most commonly underwent operations for left-sided obstructive lesions. Despite increased morbidity for select operations, TS was not associated with increased operative mortality.
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Operative management can be more challenging given the medical complexity of TS.
“Operative management can be more challenging given the medical complexity of TS.410-413”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [413]
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- Authors
- Chew, J. D., Hill, K. D., Jacobs, M. L., Jacobs, J. P., Killen, S. A. S., Godown, J., Wallace, A. S., Thibault, D., Chiswell, K., Bichell, D. P., & Soslow, J. H.
- Affiliations
- Vanderbilt University Medical Center (US)
- Duke Medical Center (US)
- Johns Hopkins University (US)
- Corresponding author
- Chew, Joshua D.
- ORCID
- Venue
- The Annals of Thoracic Surgery
- Publisher
- Elsevier BV
- Published
- 2019-11
- Volume
- 108
- Issue
- 5
- Pages
- 1430-1437
- ISSN
- 0003-4975 (ISSN-L)
- OpenAlex
- W2959043312
- Cited by
- 19
- References
- 20
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 4 · 2023: 3 · 2022: 2 · 2021: 2 · 2020: 3 · 2019: 1
- FWCI
- 1.52
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Annals of Thoracic Surgery (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Surgery › Cardiac and Coronary Surgery Techniques
- Health Sciences › Medicine › Surgery › Pectus Deformity Diagnosis and Treatment
- Keywords
- Turner syndrome, congenital heart disease, congenital heart surgery, surgical outcomes, postoperative mortality, chylothorax, aortic arch surgery, Norwood procedure, superior cavopulmonary anastomosis, Fontan procedure, partial anomalous pulmonary venous return, postoperative hospital stay, pediatric cardiac surgery, weight-for-age z-score
- MeSH terms
- Adolescent, Child, Child, Preschool, Female, Heart Defects, Congenital, Cardiac Surgical Procedures, Humans, Infant, Postoperative Complications, Societies, Medical, Thoracic Surgery, Turner Syndrome, Cohort Studies, Databases, Factual, Treatment Outcome
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