Disorders of the central nervous system (CNS) in pregnancy continue to be a common reason for morbidity and mortality in mothers. Common CNS diseases during pregnancy and their anesthetic management are described in our chapter. Some disorders of central nervous system preexist pregnancy, like epilepsy, Parkinson disease, multiple sclerosis, intracranial lesions, benign intracranial hypertension, or migraine. Others may have an increased incidence in pregnancy, for example cerebrovascular disorders, including hemorrhagic or ischemic strokes. Experience in treating these CNS diseases is limited, thus management is largely based on isolated case reports, case series, and some review articles. A high suspicion for uncommon CNS conditions is vital since timely and intense treatment significantly affects the outcome. Important aspects of consideration for the care of the parturient with a CNS disorder are the pathophysiology of the lesion, effects of pregnancy on the lesion, and effects of lesion on pregnancy. Other important considerations in management are the effect of medical management, monitoring, and surgery on fetus, maternal versus fetal priority regarding surgical plan, timing, and route of delivery. Most importantly communication and coordination among the patient, her family, and the medical team (neurologist, neurosurgeon, obstetrician, anesthesiologist, medical consultants, and nursing staff) is paramount in the optimal management of mother and fetus.

The Fontan procedure has been adapted as a palliation for a variety of congenital heart diseases that result in single-ventricle physiology. Complications after Fontan palliation are common and include exercise intolerance, hepatomegaly, lymphatic dysfunction with protein losing enteropathy, ventricular dysfunction, and rhythm and conduction disturbances. Patients with Fontan circulation are experiencing improved long-term outcomes and longer life expectancy and hence present more often for noncardiac surgery. A comprehensive understanding of the Fontan circulation is required in order to provide safe care to both well-functioning and failing Fontan patients in the perioperative period.

Idiopathic pulmonary arterial hypertension is a subset of pulmonary hypertension in which progressive narrowing of pulmonary vasculature leads to an increase in pulmonary vascular resistance and eventual right ventricular failure. Survival and quality of life have significantly improved with the advent of targeted therapies that promote pulmonary vasodilation and improve right ventricular function. Children with pulmonary hypertension have a 20-fold higher incidence of perioperative cardiac arrest compared to the general pediatric population. A well-balanced, hemodynamically stable anesthetic that aims to avoid increases in pulmonary vascular resistance and decreases in ventricular function or coronary perfusion is crucial in preventing a pulmonary hypertensive crisis. The anesthetist must anticipate, rapidly recognize, and treat impending signs of pulmonary hypertensive crisis in order to safely anesthetize a child with pulmonary hypertension.

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.