Frequency and outcomes of cardiac operations and catheter interventions in Turner syndrome
Abstract
Cardiac malformations occur commonly in Turner syndrome (TS), but the outcomes of cardiac operations and catheter-based procedures are unknown. The Pediatric Cardiac Care Consortium database was queried for individuals with TS and other female subjects without genetic abnormalities or syndromes (non-TS [NTS]). Procedures for left-sided heart lesions represented most TS procedures (95.2%). Three hundred ninety-eight patients with TS who underwent 637 of these procedures of interest were compared with 25,913 female NTS subjects who underwent 56,625 procedures. The numbers of procedures per admission (1.47 vs 1.61, p = 0.01) and per patient (1.85 vs 2.16, p <0.0001) were significantly lower in patients with TS. Procedures for cyanotic heart disease other than hypoplastic left heart (HLH) were performed 4.5-fold less frequently in patients with TS. Patients with TS and NTS subjects had equivalent hospital lengths of stay, except for patients with TS who underwent hypoplastic aortic arch operations, patent ductus arteriosus ligation, pulmonary artery balloon dilation, balloon atrial septostomy, and catheter closure of atrial septal defects. There were 34 deaths among patients with TS and 1,795 among NTS subjects (8.6% vs 7.2%, p = 0.30). When HLH was excluded, mortality was lower in the TS group (3.9% vs 6.5%, p = 0.05). Operations for partial anomalous pulmonary venous connection (14.3% vs 1.9%, p = 0.03) and HLH (90.4% vs 70.5%, p = 0.08) were more likely to result in death in patients with TS. In conclusion, given generally comparable lengths of stay and numbers of procedures as well as uniformly excellent results, these data suggest that the diagnosis of TS does not increase the utilization of limited health care resources. Operations for HLH and partial anomalous pulmonary vein connection carry additional risk for those with TS. These results will permit risk stratification, prognostication, and counseling of individuals with TS and their families.
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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 [410]
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- Authors
- Madriago, E., Nguyen, T., McFerson, M., Larson, E. V., Airhart, N., Moller, J. H., & Silberbach, M.
- Affiliations
- Oregon Health & Science University (US)
- University of Minnesota (US)
- Corresponding author
- Silberbach, Michael
- Venue
- The American Journal of Cardiology
- Publisher
- Elsevier BV
- Published
- 2012-08
- Volume
- 110
- Issue
- 4
- Pages
- 580-585
- ISSN
- 0002-9149 (ISSN-L)
- OpenAlex
- W2111172386
- Cited by
- 34
- References
- 14
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 2 · 2023: 3 · 2022: 1 · 2021: 5 · 2020: 2 · 2019: 4 · 2018: 3 · 2017: 2 · 2016: 4 · 2015: 2 · 2013: 2 · 2012: 1
- FWCI
- 1.19
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The American Journal of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Keywords
- Turner syndrome, congenital heart disease, cardiac surgery, catheter-based interventions, hypoplastic left heart syndrome, partial anomalous pulmonary venous return, cyanotic congenital heart disease, procedural mortality, health care utilization, ductus ligation, atrial septal defect closure, risk stratification
- MeSH terms
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Female, Cardiac Catheterization, Cardiac Surgical Procedures, Hospitalization, Humans, Infant, Infant, Newborn, Length of Stay, Middle Aged, Retrospective Studies, Turner Syndrome, Case-Control Studies, Treatment Outcome, Young Adult
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