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

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

Quantifying Additional Procedures in Functionally Single-Ventricle Disease: A National Cohort Study

Background: Given their importance as a metric for health care evaluation, this study’s aim was to evaluate the rates of surgical and catheter reinterventions for children with functionally single-ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation. // Methods: We undertook a retrospective cohort study of children born with f-SV CHD between 2000 and 2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, during follow-up, accounting for competing events of death or transplantation. // Results: Of 56,039 patients who received an intervention for CHD, 3307 (5.9%) had f-SV. The largest diagnostic subcategories were hypoplastic left heart syndrome (1266 [38.3%]), tricuspid atresia (448 [13.5%]), and double-inlet left ventricle (328 [9.9%]). During a median follow-up of 5.4 (interquartile range, 0.8-10.8) years, 921 (27.9%) patients had at least 1 additional interstage surgery and 1293 (39.1%) had at least 1 additional interstage catheter intervention. The cumulative incidence of additional surgery at 6 months after stage 1 was 17.6% (95% CI, 16.2%-19.0%); at 2 years after stage 2, 8.3% (7.2%-9.5%); and at 5 years after stage 3, 8.4% (7.0%-9.9%). The cumulative incidence of additional catheter at 6 months after stage 1 was 18.0% (16.6%-19.4%); at 2 years after stage 2, 14.7% (13.3%-16.2%); and at 5 years after stage 3, 23.7% (21.5%-26.0%). // Conclusions: It is important to quantify additional procedures for children with f-SV disease to inform parents and health professionals, potentially facilitating the development of interventions that aim to reduce these important adverse outcomes

Retrospective Cohort Study of Additional Procedures and Transplant‐Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales

Background: Reinterventions may influence the outcomes of children with functionally single‐ventricle (f‐SV) congenital heart disease. / Methods and Results: We undertook a retrospective cohort study of children starting treatment for f‐SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant‐free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f‐SV, 909 (27.5%), had no follow‐up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f‐SV had transplant‐free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5‐year transplant‐free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%–94.4%) and 79.3% (95% CI, 77.4%–81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87–8.27] P1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52–0.96] P=0.03) had a lower likelihood of achieving Fontan. / Conclusions: Among children with f‐SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood

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

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

A retrospective cohort study of additional procedures and transplant-free survival for patients with functionally single ventricle (f-SV) disease undergoing staged palliation in England and Wales

Background: Reinterventions may influence the outcomes of children with functionally single ventricle (f-SV) congenital heart disease (CHD).Methods: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000-2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond one-year of age. Amongst patients who had transplant-free survival beyond one-year of age, we explored the relationship between reinterventions in infancy, and the outcomes of survival and Fontan completion, adjusting for complexity.Results: Of 3,307 with f-SV, 909 (27.5%), had no follow up beyond one-year of age, amongst whom 323 (35.3%) had one or more reinterventions in infancy; and 2,398 (72.5%) of f-SV patients had transplant-free survival beyond one-year of age, amongst whom 756 (31.5%) had one or more reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, amongst those who survived infancy, were 93.4% (95% CI: 92.4%-94.4%) and 79.3% (95%CI 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had more than one additional surgery (adjusted hazard ratio (aHR) 3.93 (1.87-8.27) P&lt;0.001) had higher adjusted risk of mortality. Patients who had more than one additional interventional catheter (adjusted subdistribution hazard ratio (aSHR) 0.71 (0.52-0.96) P=0.03) had lower likelihood of achieving Fontan.Conclusions: Amongst children with f-SV, the occurrence of more than one reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.</div

A retrospective cohort study of additional procedures and transplant-free survival for patients with functionally single ventricle (f-SV) disease undergoing staged palliation in England and Wales

Background: Reinterventions may influence the outcomes of children with functionally single ventricle (f-SV) congenital heart disease (CHD).Methods: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000-2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond one-year of age. Amongst patients who had transplant-free survival beyond one-year of age, we explored the relationship between reinterventions in infancy, and the outcomes of survival and Fontan completion, adjusting for complexity.Results: Of 3,307 with f-SV, 909 (27.5%), had no follow up beyond one-year of age, amongst whom 323 (35.3%) had one or more reinterventions in infancy; and 2,398 (72.5%) of f-SV patients had transplant-free survival beyond one-year of age, amongst whom 756 (31.5%) had one or more reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, amongst those who survived infancy, were 93.4% (95% CI: 92.4%-94.4%) and 79.3% (95%CI 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had more than one additional surgery (adjusted hazard ratio (aHR) 3.93 (1.87-8.27) P&lt;0.001) had higher adjusted risk of mortality. Patients who had more than one additional interventional catheter (adjusted subdistribution hazard ratio (aSHR) 0.71 (0.52-0.96) P=0.03) had lower likelihood of achieving Fontan.Conclusions: Amongst children with f-SV, the occurrence of more than one reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.</div