ArticleHeart (British Cardiac Society)2022
Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018).
Article in Heart (British Cardiac Society), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 23 citations in OpenAlex.
- Factors Influencing Weight Gain at Different Stages of the Fontan Pathway for Children with Functionally Single Ventricle Heart Disease.Pediatric cardiology · 2026Article
- Congenital heart disease in England: a national cohort study from fetal diagnosis to end of infancy.Heart (British Cardiac Society) · 2026Article
- Impact of Collateral Vessels on Fontan Circulation: 0- to 1-Dimensional Fontan Circulation Model and Concept of Oxygen Supply and Consumption.Annals of thoracic surgery short reports · 2025Article
- Retrospective Analysis of a Multicenter Observational Study of the Relationship Between Social Determinants of Health and Complications After Children's Heart Surgery.Critical care explorations · 2025Observational
- Peripandemic outcomes of infants treated for sentinel congenital heart diseases in England and Wales.Open heart · 2025Article
- Evaluating Long-Term Outcomes of Children Undergoing Surgical Treatment for Congenital Heart Disease for National Audit in England and Wales.Journal of the American Heart Association · 2024Article
- The Long-term Outcomes and Risk Factors of Complications After Fontan Surgery: From the Korean Fontan Registry (KFR).Korean circulation journal · 2024Article
- Retrospective Cohort Study of Additional Procedures and Transplant-Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales.Journal of the American Heart Association · 2024Article
- Quantifying Additional Procedures in Functionally Single-Ventricle Disease: A National Cohort Study.Annals of thoracic surgery short reports · 2024Article
- Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.Journal of the American Heart Association · 2024Article
- Article
- Hospital resource utilization in a national cohort of functionally single ventricle patients undergoing surgical treatment.JTCVS open · 2023Article
Corrections and comments
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Authors and funding
11 authors at 5 institutions in 1 country.
Funding
Abstract
objectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.
methodsWe performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020.
resultsOf 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5-43.5) days, cavopulmonary shunt 9.2 (IQR 6.0-17.1) months and Fontan 56.2 (IQR 45.5-70.3) months. The median follow-up time was 10.8 (IQR 7.0-14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation.
conclusionAlthough treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.
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