As I had hoped and anticipated, the previous contributions have shown that Len and I are involved in a debate, but not an argument. As I am asked in this rebuttal to respond to some of the very important points that Len has made, I shall do so. I think that he was very accurate in distilling, from the study of outcomes over 15 years in Philadelphia, the message that late attrition was quite low. Of the first cohort in that study, undergoing surgery way back in 1984, less than one-third survived. As those of you who are familiar with the study now, it has improved steadily, so that now at least four-fifths of those undergoing first stage palliations survive. But what is really important is that those children who got out to 5 years usually got out to 10. And nearly all of those that got out to 10 years were alive at 15 years. I have already pointed to evidence that rates of survival with respect to initial surgical palliation are improving steadily at centers throughout the United States of America, and also around the world. So that, were it not for the shortage of donor organs, we could get together again in a decade or two from now and debate the merits of these two forms of therapy based on a comparison of the late morbidities associated with the creation of the Fontan circulation, such as sinus nodal dysfunction, protein losing enteropathy, thromboembolic phenomenons, and so on, to those events occurring at later stages associated with organ replacement, such as graft atherosclerosis, lymphoproliferative disorders, late rejection, and so on.

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

As recently as 35 years ago, children born with a functionally single ventricle were destined to a lifetime of multiple palliative operations, and disrupted growth and development. Physical signs of their cardiac disease were evidenced by chronic hypoxemia, polycythemia, clubbing, small stature, and severely reduced exercise ability. A normal lifestyle, with typical landmarks such as uninterrupted adolescence and potential childbearing, was unlikely. The successful application of right heart bypass by Fontan and Kreutzer in the early 1970s heralded an era of multiple medical and surgical breakthroughs that have systematically improved the quality of life and long-term outlook for these children.

In this Supplement, and at its associated Symposium “Controversies of Hypoplastic Left Heart Syndrome”, various contributors presented a variety of topics regarding “state of the art” treatments for hypoplastic left heart syndrome. The extent of the differences within these various presentations attests to the very significant challenges that remain in optimizing care of these patients, and related ones with other types of functionally univentricular hearts. Of these challenges, the initial surgical approach, be it staged palliation or cardiac transplantation, has been a hotly debated issue, with diametrically opposed schools of thought.1 Today, with further experience, technical advances, and dramatically improved outcomes, there seems to be an emerging complimentary role for both options, favoring a staged reconstructive approach in the majority of circumstances. We review here the salient issues that have lead to this position, and discuss strategies for maintaining a program offering both staged palliation and transplantation for patients with hypoplasia of the left heart.