The repair of complex congenital heart disease in the very small neonates has seen a dramatic evolution over the last several decades. The preoperative understanding of specific intracardiac anatomy, the ability to master the surgical techniques required for repair of complex lesions, use of cardiopulmonary bypass to support vital organs, including the brain, and evolution of state-of-the-art postoperative care have all culminated in superior surgical outcomes with mortality for most lesions being less than 2%. (1) These improvements in surgical outcomes have been documented in numerous single-center reviews, many multi-institution studies, and several multi-international longitudinal projects. We should applaud all those who currently care for this complex group of patents while not forgetting the numerous past individuals that set the fecundation the successful standards that we now aspire.

The practice of neonatal congenital heart surgery has undoubtedly experienced a persistent transition from the routine application of palliative procedures in children with complex heart defects to the ubiquitous use of complete and corrective surgery, regardless of the size or physiologic condition of the child. This philosophy has been supported by both an increasing body of literature and the growing experience of many congenital heart centers across the globe. The justification for this position is multifaceted, including an earlier return of the child to a "normal physiologic state", amelioration of detrimental physiology states as chronic hypoxia and low blood flow to vital organs, and avoidance of the operative complications and residual lesions associated with palliative procedures. Although these data are impressive, there does exist a philosophy, support by few, that the insults that are obligatory to a complex operative intervention at a very young age are as detrimental to long-term development the application of early complete repair.

Transposition of the Great Arteries (TGA)is a common congenital heart defect, representing sum 5 to 7 % of all congenital heart disease and the most common form of cyanotic congenital heart disease during the neonatal period. (2) The defect is characterized by atrioventricular concordance and ventriculoarterial discordance. Approximately 5% of patients with TGA have associated hypoplasia of the aortic arch plus or minus coarctation of the proximal descending aorta (3). The operative outcomes following repair of TGA associated with aortic arch hypoplasia are inferior to those of simple TGA, with a mortality range in the current literature of 1.9 to 24 % (4)

Double Outlet Right Ventricle with subpulmonary ventricular septal defect (the Tausig-Bing Heart) accounts for roughly a quarter of the double outlet right ventricle malformations. The anatomic configuration generally consists of the side-by-side position of the great arteries in which both the aorta and the pulmonary artery arise entirely from the right ventricle and supported by bilateral coni. The VSD is located beneath both coni, close to the subpulmonary infundibulum, and often remote from both great arteries. The existence of a subpulmonary infundibulum depth exists along a spectrum, with some hearts completely lacking this structure. Hypoplasia of the aortic arch with or without coarctation of the aorta is associated with 50% of the case of Tausig-Bing heart. (4)

In a recent publication of the Journal of Thoracic and Cardiovascular Surgery, Fricke at al; presented their single-center experience with the arterial switch operation in neonates with associated aortic arch hypoplasia or obstruction. (5) The authors retrospectively reviewed 83 neonates (9.8% of the total individual that underwent an arterial switch operation in their center) with either Transposition of the Great Arteries or Tausig- Bing anomaly with a concomitant aortic arch obstruction that underwent biventricular repair utilizing the arterial switch operation with the reconstruction of the aortic arch. Seventy-one percent of this patient underwent a single staged operation while the remained had repaired in a two-stage fashion. Early mortality was 6% with a 95% follow-up to a median of 13 years. There were no late deaths. Freedom from reintervention was 68% at 20 years, with correction of right side lesions making up the majority of these interventions. The authors conclude that although operative mortality in this cohort was higher than those patients undergoing an arterial switch operation without requiring repair of an obstructed aortic arch, representing a high-risk group. These patients are also a significant risk of the development of right-sided anatomic lesions over an extended period. The authors also state that although the application of the two-staged procedure can be performed with excellent results, it has "largely been abandoned in favor of single staged repair at most pediatric cardiac centers worldwide."

We should applaud the authors for an excellent review with important contributions to the current literature. As one of those practitioners that currently care and operate on this group of babies with complex congenital heart disease, I take exception with the idea of eliminating any advantage to ensure the superior surgical outcomes, outcomes that are expected by our patients, their families, and the public. I support and practice the application of complete reparative repair of even the most complex congenital heart disease. I believe that the early and full restoration of "normal" physiology as possible in this population will set the stage for the most suitable environment to achieve successful developmental and neurodevelopment outcomes. But, utilization of a two-staged procedure for this complex group of babies should always be considered and applied to those that have higher operative risk associated with other cardiac and non-cardiac anomalies. This has even become more apparent with the widespread use of the "hybrid type palliations" in a high-risk population with successful surgical outcomes following an eventual complete repair. Again, although the majority of patients with this lesion are excellent candidates for these complex single-stage procedure, we should never forget the successful techniques that have laid the foundation for the current results our patients benefit from.