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make clear their point that a strategy of surgical palliation for treatment of aortic atresia during an earlier generation carried a greater early risk of death than primary transplantation. This observation led them to conclude that it may be reasonable to encourage full utilization of primary pediatric transplantation, including extension to more aggressive use of primary transplantation in patients with aortic atresia today. Although they acknowledge that improvements in outcomes have shifted this analysis considerably, they hold to the conclusion that transplantation remains a comparable therapeutic strategy, and that it might be of value to advocate for primary transplantation both for patient outcomes and to encourage full donor organ utilization.
The authors provide a more detailed analysis of the long-term comparisons between primary transplantation and surgical palliation with analysis of quality of life measures using the PedsQL and PedsQL Cardiac Module, demonstrating comparable results in functional, psychological, and quality of life indicators between children undergoing primary transplantation and those undergoing surgical palliation. Clearly, the Fontan is far from a cure. However, flipping this interpretation, it is equally clear that transplantation itself is simply an alternative but largely equivalent means of palliation.
This study identifies a prescient debate for our profession today, successfully highlighting key decision making at critical times in the life of a neonate, infant, and child, and the potential conflict between what is best for an individual patient versus what is best for our patients as a whole. Without doubt, children at high risk for death with complex palliative operations—for example, patients with hypoplastic left heart syndrome, aortic atresia, severe tricuspid insufficiency, and ventricular dysfunction—would likely benefit from primary transplantation even in the current era. However, it must be recognized that the story is very different for standard-risk aortic atresia patients with survivorship through standard risk palliation exceeding 85% at some centers and all comers in the SVR trial exceeding 60% at 6 years, almost exactly overlapping the Kaplan-Meier curves for death following transplantation, the Figure 1 in the publication (see overlapping figure compilation in Figure 1).
It may be true that all experienced congenital heart surgeons can ruefully recall patients who were at higher risk and for whom the only successful primary treatment strategy would have been primary transplantation. However, essential to this strategy is the ready availability of organs to perform the transplantation within the critical time limit.
There are not enough available hearts to meet the need, particularly in the youngest population.
I applaud the authors on their work and agree with their consideration of transplantation in the higher-risk cases. However, a heart used for transplantation in a standard-risk patient is one that cannot be used for a patient with a much higher predicted palliative mortality rate. There remains a societal obligation to be thoughtful in allocation. Only a balanced approach will translate into the best possible outcomes for both standard-risk and high-risk patients. Only this balanced approach will provide for true strategic optimization.
Surgical palliation or primary transplantation for aortic valve atresia.
The study objective was to describe the surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life.