This is the first multicenter study exploring outcomes of CCS during pediatric LTx. Importantly, we identified that CCS at the time of LTx in children has acceptable outcomes compared to the larger pediatric LTx cohort in the US. Closure of an ASD was the most common surgery performed concomitantly with LTx in children, especially in patients with PHT.
Recently, a single center case series reported CCS in 9 out of 117 pediatric LTxs with CCS not influencing graft survival [5]. The latter analysis did not report in-hospital or long-term outcomes specific to this cohort.
The current study found that children with PHT, especially PAH and secondary PHT due to lung disease/hypoxemia, are most likely to undergo CCS. PHT is an increasing indication for LTx in children, so we believe that the decision to perform CCS will continue and will likely increase as this population expands [6]. Of the CCS being performed, ASD closure is the most common by far. Due to the majority of these ASD closures occurring in LTx recipients with PHT, there needs to be careful consideration of this, especially in those children with PAH. Children with PHT regardless of PAH or PHT due to lung disease/hypoxemia have a more complicated post-operative course in the intensive care unit with more primary graft dysfunction in the setting of right ventricular recovery after the transplant. Also, even if the PHT has been resolved by LTx the often hypertrophied RV still has significant diastolic dysfunction. In theory, the shunt across the ASD provides volume-unloading to the right ventricle and may assist in post-LTx recovery, which is why the authors do not close ASDs (congenital or created) in their patients. Notably, an analysis of adult patients with Eisenmenger syndrome found superior outcomes in those patients after LTx who had a concomitant ASD repair but not a concomitant VSD repair, as compared to HLTx [7]. Due to the small sample sizes, there are no definitive studies with conclusive results providing recommendations on this issue. Presently, there is no consensus regarding whether ASD closure should be performed during pediatric LTx.
Important limitations of the current study are its retrospective design and analysis of administrative data with risk of coding errors. Granular details such as information from right heart catheterization or the exact indication for CCS were not available. Due to the limited sample size, a comparison of PHT patients with and without concomitant ASD closure was not feasible. Despite these constraints, this study adds value as a multicenter study, which reduces single-center study biases.
In conclusion, 15% of pediatric LTx recipients over the last 20 years also underwent CCS at the time of transplantation. Most of these procedures were ASD closures in the PHT population. The in-hospital and long-term outcomes of these patients were comparable to reported literature. However, we believe that further work is needed to explore what subgroups of the pediatric PHT population undergoing LTx may benefit from not closing the ASD. Moving forward, it will be important to continue seeking out and sharing the best surgical approaches for this high-risk patient population as we work towards a consensus for best practice to improve patient outcomes.