Noncardiac surgery in patients with hypoplastic left heart syndrome (HLHS) and other variants of single-ventricle physiology presents unique risks, especially prior to and after the first stage of surgical palliation. Although there are a number of cardiac defects that may be treated with single-ventricle palliation, there are principles that may be generalized for the perioperative care of these patients. Preoperative assessment of their status and knowledge of their pathophysiology may aid the anesthesiologist in mitigating these risks. Laparoscopy remains controversial in this population, as patients with single-ventricle physiology may be ill equipped to tolerate the physiologic derangements that laparoscopy induces. This chapter presents an overview of single-ventricle physiology including bedside clinical assessment, as well as the expected physiologic changes associated with laparoscopy. It then reviews the published outcomes of laparoscopic versus open Nissen fundoplication. Finally, it reviews specific and devastating perioperative complications that may occur in patients with stage I palliation.