Congenital heart diseaseCohort study
Long-Term Survival and Patient-Reported Outcomes After Staged Reconstructive Surgery for Hypoplastic Left Heart Syndrome
- Retrospective cohort study
Authors' conclusion
In this cohort of newborns undergoing staged reconstructive surgery for HLHS, fewer than one-third are alive without a transplant at 35 years of age. Survival has not improved in recent years. However, there is a group of survivors who report good to excellent outcomes and QOL, consistent with a "high-performing" Fontan phenotype.
Study context
- Population
- Neonates with hypoplastic left heart syndrome (HLHS) and variants undergoing Norwood or hybrid procedure, January 1984–December 2023
- Participants
- 2,012 neonates
- Exposure
- Norwood vs hybrid procedure
- Norwoodn = 1,921
- Hybridn = 91
- Outcome
- Death or cardiac transplantation
- Follow-up
- Last
Main finding
As reported in the abstract
Transplant-free survival · 35 years
Transplant-free survival at 35 years
31.0%
Observational data: associations, not effects.
Further findings
Patient-reported general health in adults (≥18 years)
- Most responders reported good to excellent general health
Original publication
Long-Term Survival and Patient-Reported Outcomes After Staged Reconstructive Surgery for Hypoplastic Left Heart Syndrome. J Am Coll Cardiol. Published 24 June 2025. doi:10.1016/j.jacc.2025.04.028
Summarised from the published abstract; every number is checked against it