Resuscitation and perioperative management of the high-risk single ventricle patient: first-stage palliation.

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high-risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first-stage clinical management strategies are reviewed.