Atrioventricular septal defects are common congenital heart defects affecting the interatrial and interventricular septa as well as the atrioventricular valves. Atrioventricular septal defects occur due to the failure of endocardial cushions to properly develop and migrate to septate the heart during embryonic development; therefore they are also referred to as endocardial cushion defects. There are three common types or descriptions for atrioventricular septal defects: partial, transitional, and complete. An ostium primum type atrial septal defect exists in all types of atrioventricular septal defects. Partial and transitional atrioventricular septal defects have similar physiologic implications and are often diagnosed later in life as left-to-right shunting is not as significant; patients with complete atrioventricular septal defects have more significant left-to-right shunting with more severe pulmonary overcirculation, usually necessitating earlier intervention. This chapter discusses the pertinent findings and perioperative management of a patient with an unrepaired transitional atrioventricular septal defect.

Ventricular septal defects are the most common congenital heart defect, occurring in 50% of patients with congenital heart disease. They may occur in isolation or be part of a constellation of complex cardiac defects. An isolated ventricular septal defect results in the ability to shunt blood between the left and right ventricles. The size of the defect and pulmonary vascular resistance determine blood flow across the defect. Timing for the surgical repair of a ventricular septal defect varies based on patient age and symptomatology as well as the size and location of the lesion, with most nonrestrictive defects closed within the first few years of life to avoid long-term pulmonary sequelae that can lead to eventual shunt reversal and Eisenmenger syndrome. This chapter discusses the implications of caring for a preterm infant with an unrepaired ventricular septal defect for non-cardiac surgery. An understanding of factors impacting pulmonary vascular resistance and systemic vascular resistance is vital to the anesthetic management of the patient with a large ventricular septal defect.

The overall incidence of pediatric cardiomyopathy is estimated to be approximately 1–1.5 cases per 100,000 patients. The majority are due to dilated cardiomyopathy, which accounts for more than 50% of all cases. Hypertrophic cardiomyopathy is the second leading cause, with restrictive cardiomyopathy being the least common. While distinct classifications exist, in practice the variants occur in combination. However, there is generally a predominant phenotype diagnosed by echocardiography that allows for the formation of multicenter registries to track the epidemiologic, management, and outcomes related to pediatric cardiomyopathy. This chapter focuses on mixed hypertrophic and restrictive forms of cardiomyopathy and discusses the perioperative management of a patient with mixed cardiomyopathy.

This chapter, reviews the basics for children undergoing epilepsy surgery. The authors discuss the incidence and types of seizures as well as various modalities for seizure suppression (e.g. ketogenic diet, vagal nerve stimulation). The chapter presents the surgical approaches to epilepsy surgery,MRI mapping followed by laser ablation and electrocorticography with mapping followed by surgical excision. The anesthetic implications related to these complex patients are presented.

This chapter provides a thorough review of scoliosis correction surgery. The authors discuss the differentiation between idiopathic and neuromuscular scoliosis. The pre-operative evaluation of the patients with scoliosis is presented as well as a host of anesthetic considerations related to positioning, neurological monitoring, intravenous anesthesia and blood loss management.

This chapter provides anand in-depth review of the Moya-Moya Syndrome and surgical correction. The authors describe disorders commonly associated with Moya-Moya Disease. The angiographic staging as well as medical and surgical options are presented. A thorough discussion on the perioperative evaluation and anesthetic considerations for this complex disease is presented to the reader.

In this chapter, the authors discuss the issues related to post-operative neonatal apnea with an example of an infant hernia repair. Neonatal apnea, its etiology and associated risk factors is reviewed. The use of infant spinal anesthesia versus general anesthesia and its relationship to neonatal post-operative apnea is discussed.

In this chapter discusses the relevant perioperative anesthetic concerns related to thyroidectomy surgery. Reviewed is Graves’ disease, electrolyte and anatomic considerations of thyroid surgery as well as the timeframe and pathophysiology in relation to surgery.

In this chapter, the ductus arteriosus from development to maintenance in various disease states is discussed. The diagnostic process and hemodynamic consequences of a patent ductus arteriosus are reviewed. The medical and surgical indications for closure are discussed in relation to the anesthetic implications.

This chapter provides a review of anesthetic considerations for children with pyloric stenosis. The disease presentation, genetics and risk factors for pyloric stenosis are presented. The chapter provides detailed analysis of the preoperative work-up and considerations, reviews neonatal dehydration and provides the relevant anesthetic considerations for this surgical group.

In this chapter, pectus excavatum is discussed with regard to diagnosis, surgical indications, evaluation of severity and surgical approaches.The author reviews the anesthesia implications for the minimally invasive thoracoscopic approach (currently the standard technique) as well as a multimodal approach to pain management including regional and medical management options.

This chapter discusses the signs, symptoms, diagnostic work up and anesthetic consideration related to children with anterior mediastinal masses.

In this chapter, the critical pediatric topics of esophageal atresia and tracheoesophageal fistula are reviewed. The diagnosis and pathophysiology and variants of tracheoesophageal fistula are discussed.The author covers the associated anomalies and syndromes often present in patients with tracheoesophageal fistula diagnoses. The preoperative evaluation, anesthetic implications and regional anesthetic considerations are presented.

This chapter presents an overview of a more common pediatric cardiac lesion; tetralogy of Fallot.The authors provide a description of the anatomic variants that comprise the tetralogy spectrum in addition to a detailed pathophysiology discussion. The surgical/catheter-based interventions are presented in reference to the Tetralogy variant.

This chapter, provides an overview of epidurals in pediatric anesthesia practice. The author discusses the consideration for placement in the anesthetized patient, use of test doses, and contraindications to placement. Presented are the differences for consideration between pediatric and adult epidurals.

This chapter reviews the incidence, presentation and types of atrial septal defects. The pathophysiology and hemodynamic effects of atrial septal defects are discussed. The surgical and catheter-based interventions are reviewed in relation to the anesthetic considerations.