Cerebral oxygen consumption accounts for around 20% of the whole-body oxygen consumption. In the perioperative period,monitoring the adequacy of cerebral perfusion and oxygen delivery is desirable, since they can be compromised by several factors related to surgery and anaesthesia Cerebral oximeters are monitoring devices based on near infrared spectroscopy (NIRS) providing a continuous and non-invasive assessment of cerebral oxygenation. Cerebral oximetry is commonly adopted in cardiac surgery, with the widest application in aortic arch procedures and pediatric congenital heart surgery.There is growing interest in its application in non-cardiac surgery settings. Whilst the evidence for a role in impacting patient outcomes is still not completely clear even in cardiac surgery, cerebral oximetry can be a useful adjunct in perioperative monitoring and in hemodynamic management, provided that it is interpreted in the context of other physiological variables.

Pulmonary hypertension in preterm infants is defined by the same guidelines used for adults and children: mean pulmonary arterial pressure =20 mm Hg, pulmonary capillary wedge pressure <15 mm Hg, and indexed pulmonary vascular resistance >3 Wood units/m2. Bronchopulmonary dysplasia, the major cause of chronic lung disease in preterm infants, is defined as the need for oxygen at 36 weeks postconceptual age in infants born at or before 32 weeks’ gestation. The disruption of growth and function of the pulmonary vasculature observed with bronchopulmonary dysplasia contributes to the development of pulmonary hypertension. Abnormal pulmonary vasculature develops increased pulmonary vascular resistance and mean pulmonary artery pressures. As many of 20%–40% of patients with bronchopulmonary dysplasia have persistent pulmonary hypertension and the combination is accompanied by significant morbidity and mortality. This chapter details the assessment and perioperative considerations involved in caring for a preterm infant with these conditions undergoing multiple surgical procedures.

An atrioventricular septal defect is a defect in the center of the heart consisting of a common atrioventricular valve, a primum atrial septal defect, and an inlet ventricular septal defect. When the common valve is not positioned equally over both ventricles it is called an unbalanced atrioventricular septal defect. This unbalance leads to a decreased inflow of blood to one of the ventricles, and that ventricle and outflow tract are often hypoplastic. The degree of unbalance can vary widely, ranging from mild, which in effect is similar to a large ventricular septal defect, to severe, which can often involve a hypoplastic ventricle and dependence on a patent ductus arteriosus to provide adequate flow to the affected circulation. This chapter discusses the anesthetic management of a 1-day-old patient with trisomy 21 and an unrepaired, unbalanced atrioventricular septal defect undergoing a laparoscopic repair of duodenal atresia. Anesthetic concerns regarding patients with trisomy 21, physiologic changes with laparoscopic surgery and implications of an unbalanced atrioventricular septal defect are all examined in detail.

Truncus arteriosus is a rare form of cyanotic congenital heart disease in which the embryologic separation of the arterial trunk remains incomplete. The primary anatomic lesion is a common arterial trunk that supplies the pulmonary, coronary, and systemic circulations. Patients become symptomatic early in infancy because pulmonary vascular resistance falls over the first days to weeks of life, resulting in pulmonary overcirculation. Ideally, truncus arteriosus repair occurs within the first weeks of life. The most common extracardiac congenital malformations associated with truncus arteriosus are those within the heterogeneous spectrum of 22q11 deletion syndrome. Features of 22q11DS can pose multiple challenges to anesthesiologists. Dysmorphic facies, including micrognathia, retrognathia, palatal abnormalities, and laryngotracheomalacia, can impede airway management. This chapter describes the care of a child with repaired truncus arteriosus and 22q11 deletion syndrome for palatal surgery and the perioperative management considerations.

Shone’s complex is a group of left-sided, typically obstructive, lesions of the heart, also referred to as Shone’s syndrome, disorder, or anomaly. Shone’s complex was first described in 1963 as four left-sided heart lesions consisting of a supravalvular ring of the left atrium, a “parachute” mitral valve, muscular or membranous subaortic stenosis, and a coarctation of the aorta. These anomalies cause a progressive limitation of inflow into the left ventricle and outflow obstruction from the left ventricle and aorta. There is also an association with a smaller left ventricular size and decreased left ventricular function. Lesions may progressively worsen over time, causing significant heart failure symptoms, pulmonary hypertension, and arrhythmias. Surgical intervention may range from a single intervention such as aortic arch repair to a combination of multiple procedures such as repair of aortic coarctation along with mitral valve repair and aortic valvotomy. This chapter details the perioperative management of a child with multilevel left-sided obstructive lesions presenting for noncardiac surgery.

Tetralogy of Fallot (TOF) with pulmonary atresia and multiple aortopulmonary collaterals represents an extreme variation of tetralogy physiology. While TOF with pulmonary atresia includes an aorta that "overrides" an unrestrictive ventricular septal defect and right ventricular hypertrophy, instead of pulmonary stenosis there is complete atresia of the pulmonary valve and right ventricular outflow tract.Multiple aortopulmonary collaterals are vessels that arise from the descending thoracic aorta or any of its branches (subclavian, bronchial, celiac, or intercostal), often anastomosing proximal to branch pulmonary arteries. Pulmonary blood flow via these vessels is variable and nonuniform. This chapter discusses the anesthetic considerations and complexities involved in caring for a child with palliated TOF with pulmonary atresia and aortopulmonary collaterals for noncardiac surgery.

Ebstein’s anomaly is a rare heart defect affecting the tricuspid valve and right ventricle. The tricuspid valve is dysplastic, with downward displacement of the septal and posterior leaflets inferiorly into the right ventricle. While the mildest forms of Ebstein’s anomaly include minimal tricuspid regurgitation and tricuspid valve displacement, more severely affected patients will have greater tricuspid valve displacement with resultant loss of right ventricular volume and right ventricular dysfunction. An atrial level communication, either an atrial septal defect or patent foramen ovale, also exists in nearly all patients. In more severely affected patients right-to-left shunting at the atrial level results in varying degrees of cyanosis. This chapter discusses the considerations involved in the perioperative care and management of a patient with mild right ventricular dysfunction and tricuspid regurgitation undergoing urgent noncardiac surgery.

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.

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.

Subvalvular aortic stenosis is a progressive left-sided obstructive lesion, usually consisting of a discrete membrane, fibromuscular ridge, or tunnel. Surgical intervention is preferred over catheter intervention when the gradient across the lesion is greater than 40 mm Hg or aortic insufficiency has developed across the aortic valve. Perioperative anesthetic goals include maintaining adequate preload, afterload, and a low to normal heart rate.

The risk of perioperative morbidity and mortality is increased in patients with pulmonary hypertension regardless of the procedure. This is particularly true for patients with multifactorial pulmonary hypertension. We present such a patient here with a history of Down syndrome and repaired congenital heart disease who is scheduled to undergo an elective tympanoplasty. Anesthetic issues relevant to the care of a patient with Down syndrome, atrioventricular septal defect, and pulmonary hypertension are discussed, with a focus on assessment and intraoperative planning.