Single-ventricle physiology can arise from various anatomic and physiologic features beyond the most well-known hypoplastic left heart syndrome. In conditions that affect the pulmonary outflow tract, such as pulmonary atresia, initial palliative surgery often involves the placement of a modified Blalock–Taussig shunt, followed by a bidirectional Glenn and Fontan procedures. Older patients with Fontan physiology can have significant sequelae that are important to assess for preoperatively. Perioperative management of these patients requires attention to maintenance of adequate preload, ventilator strategies to promote pulmonary blood flow, and anesthetic techniques that account for potential long-term single-ventricle sequelae.

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.

The Fontan operation is the final stage of single-ventricle palliation that effectively separates the pulmonary and systemic circulations. The procedure connects the inferior vena cava directly to the pulmonary arteries or to the previously created superior cavopulmonary anastomosis, which allows all of the deoxygenated systemic venous return to flow directly to the lungs. The single ventricle then pumps oxygenated pulmonary venous blood to the systemic circulation. Baseline higher central venous pressure drives the Fontan circulation. There is an increased incidence of severe spinal deformities in children with congenital heart disease that will require corrective surgery, including posterior spinal fusion. Anesthesia for posterior spinal fusion in Fontan patients presents significant challenges, especially as the patient is in the prone position, which further exacerbates hemodynamic instability. This chapter discusses the perioperative management of a Fontan patient for posterior spinal fusion.