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A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales

Brown, Katherine L; Huang, Qi; Espuny-Pujol, Ferran; Van Doorn, Carin; Stoica, Serban; Tsang, Victor; Pagel, Christina; ... Crowe, Sonya; + view all (2025) A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales. The Journal of Thoracic and Cardiovascular Surgery , 170 (2) 316-324.e13. 10.1016/j.jtcvs.2025.02.029. Green open access

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Abstract

Objectives: To ascertain rates of completion of essential cardiac procedures and their overall contribution to longer-term mortality in children with congenital heart disease (CHD). // Methods: In this cohort study using the United Kingdom National CHD Audit, we described the pathway operations required for treatment for 9 sentinel CHDs—hypoplastic left heart syndrome (HLHS), non-HLHS functionally univentricular heart, ventricular septal defect, tetralogy of Fallot, coarctation, aortic stenosis, atrioventricular septal defect, transposition of the great arteries, and pulmonary atresia—that were undertaken at the population level and report the mortality associated with these pathway operations by age 5 years. // Results: Among 28,806 patients, over a median follow-up of 9.8 years (interquartile range, 4.2-15.4 years), 839 (2.9%) had undergone pre-pathway procedures only, 1135 (3.9%) had undergone initial palliation only (inclusive of functionally univentricular and biventricular reparative pathways), 2001 (6.9%) had undergone stage 2 palliation and/or total cavopulmonary connection (TCPC), 2254 (7.8%) had undergone staged biventricular repair after palliation, and 22,572 (78.3%) had undergone primary biventricular repair. Of the 23,239 children with 5 years of follow-up, 1794 (7.7%) had died by age 5 years, consisting of 409 (1.8%) who died following an initial palliative procedure, 43 (0.2%) who died following a stage 2 palliation or TCPC, 379 (1.6%) who died following a biventricular reparative procedure, and 963 (4.1%) who died in other circumstances, such as interstage or following reinterventions. // Conclusions: The outcome metrics of surgical pathway completion (biventricular repair or TCPC) and overall mortality at age 5 years can be evaluated using registry data and could contribute to future assessment of overall CHD service quality.

Type: Article
Title: A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jtcvs.2025.02.029
Publisher version: https://doi.org/10.1016/j.jtcvs.2025.02.029
Language: English
Additional information: Copyright © 2025 The Authors. Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
Keywords: Congenital heart disease; outcomes; cardiac surgery
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > UCL BEAMS
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Mathematics
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Mathematics > Clinical Operational Research Unit
URI: https://discovery.ucl.ac.uk/id/eprint/10214051
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