Sometimes the best answer is: Reboot.While previously considered unattainable, statistics at experienced centers for cardiac transplantation in the failing Fontan have almost reached survival rates no longer considered “high risk”.See Article page 182.A therapeutic nihilist would posit that the fourth stage of single ventricle palliation remains heart transplantation. Indeed, whereas heart transplantation after Fontan completion once carried a 25% operative mortality, Konstantinov and colleagues present impressive results representing years of iterative improvement on the ultimate ctrl-alt-del operation.Although improvement in surgical technique surely accounts for a large part of this, parallel technical and perioperative management advances (eg, single ventricle mechanical assist options as a bridge to transplant) have also helped to help stabilize and render these challenging patients more transplant-worthy. Transplantation after Fontan completion, by definition, requires extensive extracardiac work—great vessel reconstruction and baffles to adjust for situs abnormalities—compounded by the unappealing inevitability of multiple sternotomies, extensive collaterals, and often poor preoperative nutrition. Where I trained, the complex heterotaxy Fontan transplant was considered the final exam of congenital, now even more so with the prevalence of additional aortic arch pathology (from Norwood type reconstructions, ductal stents, or other perturbations). I, for one, endorse the assertion by Konstantinov and colleagues that one reconstruct as much as possible before the arrival of the donor team, and in so doing reduce cold ischemic time. While this approach may lack the finesse of a reconstructed circulatory system based entirely on donor tissue for augmentation, an implant with this strategy becomes as intuitive as Ikea furniture instructions.Regionalization of care is a third rail in today's environment (at least in the United States), yet the stance of Konstantinov and colleagues that the most complex Fontan candidates be transplanted at a center with extensive experience seems axiomatic. Indeed, it is not only the surgery that must be up to the task; the expert management of patients from all allied specialties contributes to the secret sauce. Above all, chance favors the prepared mind—detailed evaluation by cross-sectional imaging, a huddle before incision that articulates surgical contingencies in the event of calamity, and a shared and realistic mental model of donor and recipient timing—all help to choreograph this complex ballet across 2 operating theaters.Still controversial is the notion that Fontan transplants at present are not risk-stratified in public reporting systems and, because of this, the perception of prohibitively high-risk candidacy could deny many the benefit of this therapy. Complex congenital transplants now account for nearly 50% of patients listed at major US pediatric centers and so Fontan transplants are here to stay—as shown here with results that heretofore would have been considered fantasy. In a different era, equally farfetched might have been envisioning a newborn or infant with hypoplastic left heart syndrome having the luxury to experience Fontan failure. As we continue to leverage advances in other domains (eg, adult aortic reconstruction), certainly multivisceral transplants to address concomitant disease are soon to enter the main stage. The guardrail then, as ever, may be the limited number of donor organs, but perhaps if recent newsworthy stories are correct, while pigs may not fly, they may one day serve as a limitless source for total organ replacement.
Authors: Joseph W Rossano; Wida S Cherikh; Daniel C Chambers; Samuel Goldfarb; Kiran Khush; Anna Y Kucheryavaya; Bronwyn J Levvey; Lars H Lund; Bruno Meiser; Roger D Yusen; Josef Stehlik Journal: J Heart Lung Transplant Date: 2017-07-20 Impact factor: 10.247