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

Open AccessPublished:November 24, 2022DOI:
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      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.


      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.


      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).


      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


      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)