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. 2023 Aug;166(2):306-316.e3.
doi: 10.1016/j.jtcvs.2022.11.018. Epub 2022 Nov 25.

Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales

Kate L Brown  1 , Qi Huang  2 , Elena Hadjicosta  3 , Anna N Seale  4 , Victor Tsang  1 , David Anderson  5 , David Barron  4 , Hannah Bellsham-Revell  5 , Christina Pagel  3 , Sonya Crowe  3 , Ferran Espuny-Pujol  3 , Rodney Franklin  6 , Deborah Ridout  7
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Free article

Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales

Kate L Brown et al. J Thorac Cardiovasc Surg. 2023 Aug.
Free article

Abstract

Objectives: Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales.

Methods: This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020.

Results: Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P < .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001).

Conclusions: After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.

Keywords: Fontan; congenital heart disease; hypoplastic left heart syndrome; outcomes.

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