Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.
Open accessAbstract
Objectives We sought to analyze risk factors for long-term survival (up to 36 years) after surgical repair of tetralogy of Fallot (TOF).Background Survival after repair is excellent, but data >20 years are rare.Methods From 1958 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 +/- 8.6 years [mean +/- SD], range 2 to 67), 39.7% had a previous palliation. Operative (n = 139) and 1-year (n = 29) deaths were excluded for long-term calculations, resulting in a study group of 490 patients.Results Actuarial 10-, 20-, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively. Mortality increased 25 years postoperatively from 0.24%/year to 0.94%/year (p = 0.003). The most common cause of death was sudden death (n = 13), followed by congestive heart failure (n = 6). Multivariate correlates of impaired long-term survival were date of operation (before 1970, p = 0.0104), preoperative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079). Postoperative systolic RV/left ventricular pressure ratio and age showed no influence. Patients without preoperative polycythemia and an RV outflow patch (n = 164) had a 36-year actuarial survival rate of 96% and normal life expectancy.Conclusions Cyanosis, operative experience of the surgeon and an RV outflow tract patch influence long-term outcome after repair of TOF in older children. Early repair by experienced surgeons to avoid polycythemia and excessive RV hypertrophy is supported by this study. However, mortality risk increases 25 years postoperatively, and thus heart monitoring should be intensified.
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Earlier surgical eras had an inverse association with the chances of survival in the early postoperative phase, indicating higher residual postoperative morbidity, which is consistent with previous reports.
“Earlier surgical eras had an inverse association with the chances of survival in the early postoperative phase, indicating higher residual postoperative morbidity, which is consistent with previous reports.8,9,17,19”
From Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium (2019) · cites this paper as [9]
In “Survival according to date of operation” (page unavailable), the paper says operative and 1-year mortality were exactly the same before and after 1970. It reports improved long-term survival in the later era, not an early postoperative survival disadvantage in the earlier era; the passage does not establish higher residual postoperative morbidity. (LLM, conf=0.90)
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Experimental — accuracy not yet measuredThe rates of sudden death and heart failure in our cohort are similar to those reported in other studies.
“The rates of sudden death and heart failure in our cohort are similar to those reported in other studies.9,18”
From Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium (2019) · cites this paper as [9]
The Discussion (page unavailable) reports that sudden cardiac death remained the major cause of late death after tetralogy of Fallot repair. The follow-up passage (page unavailable) reports symptoms of congestive heart failure, but it does not establish a comparable heart-failure rate; the source also cannot assess similarity to the citing authors’ cohort. (LLM, conf=0.78)
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- Authors
- Nollert, G., Fischlein, T., Bouterwek, S., Böhmer, C., Klinner, W., & Reichart, B.
- Affiliations
- Ludwig-Maximilians-Universität München (DE)
- Corresponding author
- Nollert, Georg
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 1997-11
- Volume
- 30
- Issue
- 5
- Pages
- 1374-1383
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W2022043412
- Cited by
- 761
- References
- 32
- Citations by year
- 2026: 11 · 2025: 19 · 2024: 23 · 2023: 25 · 2022: 18 · 2021: 25 · 2020: 39 · 2019: 37 · 2018: 35 · 2017: 36 · 2016: 44 · 2015: 28 · 2014: 37 · 2013: 45 · 2012: 49
- FWCI
- 6.24
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Deutsche Forschungsgemeinschaft
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Keywords
- tetralogy of Fallot, surgical repair, long-term survival, mortality risk factors, sudden death, congestive heart failure, cyanosis, right ventricular hypertrophy, timing of repair, congenital heart disease
- MeSH terms
- Actuarial Analysis, Cause of Death, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Male, Pulmonary Valve Stenosis, Reoperation, Risk Factors, Tetralogy of Fallot, Time Factors, Multivariate Analysis, Survival Analysis, Ventricular Function, Left, Ventricular Function, Right
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