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

Brown, K. L., Huang, Q., Espuny-Pujol, F. ORCID: https://orcid.org/0000-0001-9085-7400, Van Doorn, C., Stoica, S., Tsang, V., Pagel, C., Franklin, R. C. G. and Crowe, S. (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). pp. 316-324. ISSN 1097-685X doi: 10.1016/j.jtcvs.2025.02.029

Abstract/Summary

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.

Item Type	Article
URI	https://centaur.reading.ac.uk/id/eprint/122001
Identification Number/DOI	10.1016/j.jtcvs.2025.02.029
Refereed	Yes
Divisions	Science > School of Mathematical, Physical and Computational Sciences > Department of Computer Science
Publisher	Elsevier
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