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

• Published in: Journal of the American Heart Association 2024-07, Vol.13 (14), p.e033068
• Main Authors: Huang, Qi, Ridout, Deborah, Tsang, Victor, Drury, Nigel E, Jones, Timothy J, Bellsham-Revell, Hannah, Hadjicosta, Elena, Seale, Anna N, Mehta, Chetan, Pagel, Christina, Crowe, Sonya, Espuny-Pujol, Ferran, Franklin, Rodney C G, Brown, Katherine L
• Format: Article
• Language: English
• Subjects: Child, Preschool; congenital heart disease; England - epidemiology; Female; Fontan Procedure - mortality; Fontan surgery; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Heart Transplantation - statistics & numerical data; Heart Ventricles - abnormalities; Heart Ventricles - physiopathology; Heart Ventricles - surgery; Humans; Infant; Infant, Newborn; Male; mortality; Original Research; Palliative Care; Registries; reintervention; Reoperation - statistics & numerical data; Retrospective Studies; Time Factors; Treatment Outcome; univentricular heart; Univentricular Heart - mortality; Univentricular Heart - physiopathology; Univentricular Heart - surgery; Wales - epidemiology
• ISSN: 2047-9980; 2047-9980
• DOI: 10.1161/JAHA.123.033068

Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. 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] 1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] =0.03) had a lower likelihood of achieving Fontan. 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.