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

Abstract

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] P <0.001) had higher adjusted risk of mortality. Patients who had >1 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.