A population-based analysis of mortality in patients with Turner syndrome and hypoplastic left heart syndrome using the Texas Birth Defects Registry: Lara et al.
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BACKGROUND: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population. METHODS: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999-2007. We used Kaplan-Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS-). RESULTS: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects or genetic disorders, and 463 had neither. The median follow-up time was 4.2 y (interquartile range [IQR] 2.1-6.5). Comparing those with HLHS/TS+ to HLHS/TS-, 100% versus 35% were female (P < .001), and median birth weight was 2140 g (IQR 1809-2650) versus 3196 g (IQR 2807-3540, P < .001). Neonatal mortality was 36% in HLHS/TS+ versus 27% in HLHS/TS- (log rank = 0.431). Ten of the 11 TS+ patients died during the study period for cumulative mortality of 91% versus 50% (hazard ratio (HR) for TS+: 2.90, 95% CI 1.53-5.48). Six patients died prior to surgery, 5 underwent Stage 1 palliation (S1P), 3 died after S1P, 2 survived past S2P, and one of these died at age 19 mo. The underlying cause of death was listed as congenital heart disease on all the death certificates of HLHS/TS+ patients. In multivariable analysis controlling for low birth weight (<2500 g), TS remained associated with significantly increased cumulative mortality, although females without TS had higher mortality than males (HR for TS+ versus males: 2.42, 95% CI 1.24-4.73; HR for TS- females versus males: 1.41, 95% CI 1.08-1.83). CONCLUSION: TS with HLHS is associated with significant mortality. The increased mortality in females without documented TS calls to question if TS is undetected in a portion of females with HLHS.
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Several studies have reported 2-3-fold higher mortality in neonates with TS with hypoplastic left heart (HLH) compared to all cases of HLH, suggesting an independent risk of TS to mortality.
“Similarly, several studies have reported 2-3-fold higher mortality in neonates with TS with hypoplastic left heart (HLH) compared to all cases of HLH, suggesting an independent risk of TS to mortality.633-635”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [633]
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Hypoplastic Left Heart Syndrome
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Patient With Poor Prognosis, Family Wishes to Pursue All Options, Care Team Cannot Reach Consensus
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Self-citationThe Heart and Vasculature in Turner Syndrome: Development, Surveillance, and Management
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Preoperative Diagnostic Evaluation
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Prenatal Genetic Consultation and Testing
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Hypoplastic left heart syndrome: anatomical spectrum, clinical management, and brain defects
Bell-Cheddar, Y. R., Devine, W. H., Diaz-Castrillon, C. E., Tang, R., Castro-Medina, M., Solt, S., Christopher, A. B., Morales, R., Xu, X., Lo, C. W. Y., et al. (2026) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2026.1877717
- Authors
- Lara, D. A., Ethen, M. K., Canfield, M. A., Nembhard, W. N., & Morris, S. A.
- Affiliations
- Baylor College of Medicine (US)
- Texas Department of State Health Services (US)
- Arkansas Children's Research Institute (US)
- Corresponding author
- Lara, Diego A.
- Venue
- Congenital Heart Disease
- Published
- 2016-09-29
- Volume
- 12
- Issue
- 1
- Pages
- 105-112
- ISSN
- 1747-079X (ISSN-L)
- PMC
- PMC7321826
- OpenAlex
- W2525919158
- Cited by
- 47
- References
- 50
- Citations by year
- 2026: 3 · 2025: 6 · 2024: 7 · 2023: 3 · 2022: 8 · 2021: 3 · 2020: 4 · 2019: 5 · 2018: 4 · 2017: 4
- FWCI
- 4.06
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Congenital Heart Disease (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Centers for Disease Control and Prevention (U50DD613232)
- National Heart, Lung, and Blood Institute (L40 HL124303, K23HL127266)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
- Keywords
- Turner syndrome, hypoplastic left heart syndrome, mortality, long-term survival, low birth weight, sex differences, neonatal mortality, stage 1 palliation, stage 2 palliation
- MeSH terms
- Adolescent, Cause of Death, Child, Child, Preschool, Female, Cardiac Surgical Procedures, Humans, Infant, Infant, Newborn, Male, Registries, Retrospective Studies, Risk Factors, Sex Factors, Texas, Time Factors, Turner Syndrome, Proportional Hazards Models, Treatment Outcome, Hypoplastic Left Heart Syndrome, Kaplan-Meier Estimate
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