Trends in long-term mortality after congenital heart surgery.
Open accessAbstract
Background Congenital heart surgery has improved the survival of patients with even the most complex defects, but the long-term survival after these procedures has not been fully described.Objectives The purpose of this study was to evaluate the long-term survival of patients (age <21 years) who were operated on for congenital heart defects (CHDs).Methods This study used the Pediatric Cardiac Care Consortium data, a U.S.-based, multicenter registry of pediatric cardiac surgery. Survival analysis included 35,998 patients who survived their first congenital heart surgery at <21 years of age and had adequate identifiers for linkage with the National Death Index through 2014. Survival was compared to that in the general population using standardized mortality ratios (SMRs).Results After a median follow-up of 18 years (645,806 person-years), 3,191 deaths occurred with an overall SMR of 8.3 (95% confidence interval [CI]: 8.0 to 8.7). The 15-year SMR decreased from 12.7 (95% CI: 11.9 to 13.6) in the early era (1982 to 1992) to 10.0 (95% CI: 9.3 to 10.8) in the late era (1998 to 2003). The SMR remained elevated even for mild forms of CHD such as patent ductus arteriosus (SMR 4.5) and atrial septal defects (SMR 4.9). The largest decreases in SMR occurred for patients with transposition of great arteries (early: 11.0 vs. late: 3.8; p < 0.05), complete atrioventricular canal (31.3 vs. 15.3; p < 0.05), and single ventricle (53.7 vs. 31.3; p < 0.05).Conclusions In this large U.S. cohort, long-term mortality after congenital heart surgery was elevated across all forms of CHD. Survival has improved over time, particularly for severe defects with significant changes in their management strategy, but still lags behind the general population.
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Linkage of the PCCC to the registries of the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) extends the follow-up time of PCCC-enrolled persons for major outcomes with nearly 90% sensitivity and almost 100% specificity for death and transplant events.
“Linkage of the PCCC to the registries of the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) extends the follow-up time of PCCC-enrolled persons for major outcomes with nearly 90% sensitivity and almost 100% specificity for death and transplant events.14,15”
From Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium (2019) · cites this paper as [15]
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Self-citation
- Authors
- Spector, L. G., Menk, J. S., Knight, J. H., McCracken, C., Thomas, A. S., Vinocur, J. M., Oster, M. E., St Louis, J. D., Moller, J. H., & Kochilas, L.
- Affiliations
- University of Minnesota (US)
- Emory University (US)
- University of Rochester Medicine (US)
- Corresponding author
- Kochilas, Lazaros
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2018-05
- Volume
- 71
- Issue
- 21
- Pages
- 2434-2446
- ISSN
- 0735-1097 (ISSN-L)
- PMC
- PMC5978758
- OpenAlex
- W2803215142
- Cited by
- 230
- References
- 53
- Citations by year
- 2026: 20 · 2025: 34 · 2024: 26 · 2023: 31 · 2022: 30 · 2021: 39 · 2020: 24 · 2019: 18 · 2018: 7
- FWCI
- 17.15
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Georgia Clinical and Translational Science Alliance
- National Institutes of Health (UL1TR000114)
- National Heart, Lung, and Blood Institute (R01HL122392, HL122392)
- National Center for Advancing Translational Sciences
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
- Health Sciences › Medicine › Surgery › Cardiac and Coronary Surgery Techniques
- Keywords
- congenital heart disease, long-term mortality, pediatric cardiac surgery, standardized mortality ratio, transposition of the great arteries, complete atrioventricular canal, single ventricle, patent ductus arteriosus, atrial septal defect, survival trends
- MeSH terms
- Adolescent, Child, Child, Preschool, Female, Follow-Up Studies, Heart Defects, Congenital, Cardiac Surgical Procedures, Humans, Infant, Infant, Newborn, Male, Mortality, Postoperative Complications, Registries, Retrospective Studies, Cohort Studies, Young Adult
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