Attrition between the superior cavopulmonary connection and the Fontan procedure in hypoplastic left heart syndrome

Commentary: Attrition after superior cavopulmonary connection; we don't have to be perfect but better than yesterday

“…In the current issue of the Journal, Lawrence and colleagues 1 from Philadelphia review their large cohort of 856 children born with hypoplastic left heart syndrome (HLHS) who survived to hospital discharge following superior cavopulmonary connection (SCPC). They report the progression of these children subsequent to SCPC and focus on those who failed to move toward undergoing the Fontan operation.…”

“…They inferred that strategies to improve survival in this interstage period are needed. 1 Multistage palliation management of children with HLHS continues to evolve, and advances in all aspects in the care of these challenging patients have resulted in a remarkable improvement of their outlook. 2 Much of the emphasis has been on the greatest-risk period that includes perioperative care and interstage period between Norwood and SCPC.…”

“…2 The findings from the authors' experienced, large center are significant and reflect that despite our success in decreasing early death, we are unable to sustain survival toward the final palliation stage in a number of children, and that we have failed to make a marked improvement in the outlook of these children during that stage over the past 3 decades. 1 In our continuous task to advance the management of children with complex congenital heart defects, we should constantly examine opportunities to improve survival and life quality. In the case of interstage attrition between SCPC and Fontan, the question is: are there any potentially correctable factors that would allow us to decrease this attrition and increase the total number of children who successfully complete their multistage palliation strategy and receive the Fontan operation?…”

“…They further suggest that failure to improve this attrition in the recent era might be related to the relative increase use of RVPA shunt. 1 However, is that enough evidence against the use of RVPA shunt? Other series, including the Single Ventricle Reconstruction trial, similarly showed that despite the greater early survival with the use of RVPA shunt as opposed to the modified Blalock-Taussig shunt, the eventual survival balances within few years from Norwood with more attrition in the RVPA shunt group.…”

Commentary: Late attrition in Norwood populations: Kicking the can down the road?

“…We continue to learn from the unique experience of the Children's Hospital of Philadelphia's (CHOP) analysis of their Norwood program for hypoplastic left heart syndrome, with the notable finding that the attrition between stage 2 and stage 3 has remained unchanged over the past 30 years. 1 It is an unexpected finding given the remarkable progress that has been made over this same time period. The dramatic improvements seen in stage 1 and interstage 1 or 2 outcomes have still meant better overall survival for this population.…”