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

Open AccessPublished:November 24, 2022DOI:https://doi.org/10.1016/j.jtcvs.2022.11.018
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      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 centre-volume and long-term survival in f-SV CHD within the centralised health care service of England and Wales.

      Methods

      A retrospective cohort study of children born with f-SV CHD between 2000-2018, using the national CHD procedure registry, with survival ascertained in 2020.

      Results

      Of 56,039 patients, 3,293 (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 (HLHS) 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%CI 75.3%,78.2%) and 72.1% (95%CI 70.6%,73.7%), respectively. The unadjusted hazard ratio (HR) for each 5 additional f-SV patients starting treatment per centre per year, was 1.04 (95%CI 1.02,1.06) p<0.001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), HR for f-SV centre-volume was 1.01 (95%CI 0.99,1.04) p=0.28. There was strong evidence that more complex f-SV patients (HLHS, Norwood pathway) were treated at centres with higher f-SV case-volume (p<0.001).

      Conclusion

      After adjustment for case mix, there was no evidence that f-SV centre-volume was linked to longer-term survival, in the centralised health service provided by the ten children’s cardiac centres in England and Wales..

      Key words

      Abbreviationslist:

      AVSD (atrioventricular septal defect), CHD (congenital heart disease), DILV (double inlet left ventricle), f-SV (functionally single ventricle), FUH (functionally univentricular heart), HLHS (hypoplastic left heart syndrome), IQR (interquartile range (second and third quantiles)), NCHDA (National Congenital Heart Diseases Audit), ONS (Office of National Statistics)