Neurodevelopmental Outcome in Children With Congenital Heart Disease: A Work in Progress Leonard Rappaport, MD, MS When I began my residency in 1977, treatment of children with congenital heart defects was evolving in the context of an inspiring collaboration between pediatric cardiology, cardiac surgery, and cardiac anesthesia. In the ensuing decades, thousands of infants were saved by novel imaging, new operations, and refined supportive methods. The impact on these children was, in many instances, not only lifesaving but even miraculous. A similar phenomenon was occurring nationally and internationally, and advances in diagnosis, treatment, surgical interventions, and support techniques changed faster than in perhaps any other pediatric field. By the mid-1980s, many previously fatal congenital cardiac lesions had become operable, and survival rates had improved. In addition, new surgical techniques were developed to repair even the most complex lesions during early infancy rather than in 2 stages (ie, first palliative, then later reparative surgery), and morbidity and mortality declined. Dazzling progress in the sophistication of surgical repair is illustrated nowhere better than by the transition of surgery for dextrotransposition of the great arteries from atrial switch surgery (eg, Mustard or Senning procedure) and then the 2-stage arterial switch operation to the primary arterial switch operation. Pushing the field toward earlier age at repair was an article by Newburger et al 1 describing decrements in developmental outcomes in children who remained cyanotic past the age of 2. Newburger’s findings also sounded a warning that management decisions should weigh not only mortality but the kind of life each child would lead. Cardiologists who followed these new survivors of complex congenital heart disease began to realize that with only anecdotal observations of individual patients, complex relationships between interventions and neurodevelopmental outcome could not be discerned. As a junior faculty member in developmental–behavioral pediatrics, I was invited by Jane Newburger, MD, author of the first cardiac development study, to become part of a team to study the developmental outcomes in the first randomized clinical trial of deep hypothermic circulatory arrest versus low-flow bypass in the arterial switch operation for dextrotransposition of the great arteries. 2 Cardiac surgeons had been using different techniques of vital organ support and were committed to their particular approach, but investigators at Boston Children’s Hospital were willing to subject their beliefs to rigorous scrutiny. This trial gave us much new knowledge about the limits of deep hypothermic circulatory arrest and changed the conduct of infant cardiac surgery. Additionally, the trial ushered in a new era of randomized clinical trials and detailed retrospective studies aimed at improving neurodevelopmental outcomes. Future studies would examine the effects of hematocrit levels and pH management, 3–5 among other support techniques, and studies used Division of Developmental Medicine, Department of Medicine, Boston Children’ s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts Opinions expressed in these commentaries are those of the author and not necessarily those of the American Academy of Pediatrics or its Committees. www.pediatrics.org/cgi/doi/10.1542/peds.2015-0719 DOI: 10.1542/peds.2015-0719 Accepted for publication Feb 25, 2015 Address correspondence to Leonard Rappaport, MD, MS, Boston Children’ s Hospital, Fegan 10, 300 Longwood Avenue, Boston, MA 02115. E-mail: leonard.rappaport@childrens.harvard.edu PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2015 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose. COMPANION PAPER: A companion to this article can be found on page 816, and online at www.pediatrics. org/cgi/doi/10.1542/peds.2014-3825. COMMENTARY PEDIATRICS Volume 135, number 5, May 2015