Abstract
26-A-8616-ACC GENETIC INFLUENCES ON HEART FAILURE, MORTALITY, AND COMORBIDITIES AMONG CHILDREN WITH SINGLE VENTRICLE DISEASE
Journal of the American College of Cardiology, Vol.87(13 Supplement), p.A345
04/2026
DOI: 10.1016/j.jacc.2026.02.854
Abstract
Background
Single ventricle disease (SVD) is associated with high morbidity, mortality, and resource utilization. Survival to Fontan completion is ∼50-60% with clinical outcomes influenced by associated comorbidities, notably heart failure (HF). We evaluated the association of genetic syndromes with outcomes including HF, mortality, and comorbidities among children with SVD.
Methods
This retrospective study used CDC-funded surveillance data across 7 sites (AZ, GA, IA, NC, NY, SC, UT), the largest SV dataset to date. Children with SVD age <6 years (during 2010-2019) were identified using ICD-9/10 diagnostic codes. We applied Poisson log-linear regression to evaluate associations between presence of genetic syndrome(s) and incidence of each outcome, adjusting for covariates and reporting as incidence rate ratio (IRR). Person-years at risk were included as the offset.
Results
Among 6,225 SV patients, 72% were <1 year old at study entry, 57% male, 50% had hypoplastic left heart syndrome, 36% tricuspid atresia, and 14% other SV. In the overall cohort, 44.7% developed HF, 5.3% required cardiac transplantation, and 13.0% died during the surveillance period. A total of 1163 patients (18.7%) had a genetic syndrome, with Down syndrome (23.0%) and 22q11 deletion (9.7%) as the most common. Patients with genetic syndromes had significantly (p<0.001) higher prevalence of HF (51.3% vs 43.2%), pulmonary hypertension (17.5% vs 10.9%), valve disease (47.4% vs 41.6%), arrhythmias (30.0% vs 20.7%), chronic kidney disease (4.7% vs 1.9%) and mortality (17.5% vs 11.9%) than those with no genetic syndrome. Presence of genetic syndrome was independently associated with increased HF and mortality risk (IRR 1.27, p<0.001) and (IRR 1.37, p<0.001) respectively. These associations were similar when limiting analysis to patients <1 year of age, suggesting results were not substantially impacted by possible survival bias.
Conclusion
In this cohort of young SVD patients, nearly half developed HF over a 10-year surveillance period. Children with genetic syndromes had higher comorbidities and mortality, underscoring the need for targeted surveillance and early management strategies in this vulnerable group.
Details
- Title: Subtitle
- 26-A-8616-ACC GENETIC INFLUENCES ON HEART FAILURE, MORTALITY, AND COMORBIDITIES AMONG CHILDREN WITH SINGLE VENTRICLE DISEASE
- Creators
- Adeola Awujoola - Centers for Disease Control and PreventionKatie Freedy - Centers for Disease Control and PreventionSteven Kemp - Centers for Disease Control and PreventionKaren E. Chiswell - Centers for Disease Control and PreventionAlfred D'Ottavio - Centers for Disease Control and PreventionAllison Levin - Centers for Disease Control and PreventionAnaclare Sullivan - Centers for Disease Control and PreventionAndrew Landstrom - Centers for Disease Control and PreventionCharlie J. Sang - Centers for Disease Control and PreventionCheryl Lynne Raskind-Hood - Centers for Disease Control and Preventionemily Olsen - Centers for Disease Control and PreventionJennifer AndrewsJill Glidewell - Centers for Disease Control and PreventionKarl Welke - Centers for Disease Control and PreventionKristin Conway - Centers for Disease Control and PreventionKristin Sommerhalter - Centers for Disease Control and PreventionLorenzo Botto - Centers for Disease Control and PreventionMatthew Reeder - Centers for Disease Control and PreventionMichael Walsh - Centers for Disease Control and PreventionPaul Romitti - Centers for Disease Control and PreventionScott E. Klewer - Centers for Disease Control and PreventionTimothy M. Hoffman - Centers for Disease Control and PreventionWendy M. Book - Centers for Disease Control and PreventionJennifer Li - Centers for Disease Control and Prevention
- Resource Type
- Abstract
- Publication Details
- Journal of the American College of Cardiology, Vol.87(13 Supplement), p.A345
- DOI
- 10.1016/j.jacc.2026.02.854
- ISSN
- 0735-1097
- Publisher
- Elsevier
- Language
- English
- Date published
- 04/2026
- Academic Unit
- Epidemiology; Biostatistics
- Record Identifier
- 9985149412102771
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