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Inherited Coagulation Disorders Hemophilia The hemophilias are a group of X-linked recessive bleeding disorders characterized by insufficient production of coagulation factor VIII (hemophilia A) or factor IX (hemophilia B). Formation of the platelet plug (primary hemostasis) is normal; however, stabilization of the plug by fibrin (secondary hemostasis) is defective because an insufficient amount of factor VIII or IX results in ineffective thrombin generation. Hemophilia A results…

Gastroesophageal Reflux Gastroesophageal reflux (GER) is a term used to describe a leaky lower esophageal sphincter that causes a child to regurgitate recently ingested meals. It is common in the first year of life, especially in children born premature, and usually resolves during early childhood. Children with GER related to neuromuscular disorders, such as cerebral palsy, will continue to reflux throughout childhood. Because of weakened airway…

Cerebral palsy and seizure disorders are very common in the pediatric population, thus anesthesia providers should be familiar with their clinical characteristics and the pharmacologic agents used for their treatment. Although less common, myopathies are associated with significant morbidity in children, and are noteworthy because of their potential association with malignant hyperthermia and the potentially catastrophic hyperkalemic response to administration of succinylcholine (and in some cases,…

Upper Respiratory Infection Upper respiratory infection (URI) is common in children presenting for anesthesia and the most common infection overall in children. The average child will have 6 to 8 URIs per year. Thus, it is important to understand the pathogenesis, clinical features, risk stratification, and anesthetic management of children with a URI. URIs are almost always viral. Rhinoviruses are the most common, but influenza, parainfluenza,…

The overall incidence of congenital heart disease (CHD) is approximately 8 per 1000 live births and is usually divided into two categories: cyanotic (the defect contains a right-to-left shunt) and acyanotic (the defect may contain a left-to-right shunt). The most common cyanotic lesions, in order of decreasing frequency, are pulmonary stenosis (PS) , transposition of the great arteries (TGA) , tetralogy of Fallot (ToF), tricuspid atresia…

Developmental physiology describes the bodily changes that take place during early development. Anesthesiologists must be familiar with these changes as they pertain to the different organ systems. Developmental pharmacology describes the changes in pharmacokinetics and pharmacodynamics during early life. In pediatric anesthesia, this is especially important because it influences the administration of intravenous and inhaled anesthetic agents in young children. Respiratory Physiology A term newborn will…

We begin this journey into the magical world of pediatric anesthesia by describing fetal cardiopulmonary physiology and the physiologic changes that occur during birth. Knowledge of these changes is important for understanding the pathophysiologic conditions that occur in neonates when these changes do not occur normally. First, for clarity and consistency across the pediatric anesthesia literature, and to make sure we are all on the same…
Key Points 1. Major adverse cardiac events (MACEs) are relatively common in patients undergoing noncardiac surgical procedures. The incidence of perioperative myocardial infarction (PMI) is about 0.9%. However, a larger percentage of patients experiences a perioperative increase in cardiac troponins without other criteria for myocardial infarction (myocardial injury after noncardiac surgery [MINS]). 2. Preventive and therapeutic strategies for acute coronary syndromes are well established in the…
Key Points 1. Cerebrovascular accidents are some of the most significant perioperative complications because they increase long-term disability, hospital length of stay, and mortality. 2. Recent stroke is associated with a 1.8-fold increased risk of death in noncardiac surgery. 3. There are a broad spectrum of critical care issues involving the pulmonary system, ranging from chronic diseases to surgical and anesthesia complications. 4. Postoperative respiratory failure…
Key Points 1. The postanesthesia care unit (PACU) is a specialized unit designed to monitor for early postanesthesia and surgical adverse events. 2. Early cardiac decline after noncardiac surgery requires prompt evaluation, early intervention, and cardiologist consultation for advanced care. 3. Neurohormonal changes and sympathetic nervous system activation can impair cardiac function in patients with cardiac disease. 4. Anesthetic management can be used to suppress adverse…
Key Points 1. The costs of healthcare are escalating. Goal-directed fluid therapy (GDT) and multimodal pain relief are ways to control cost while improving quality. 2. GDT is an integral part of enhanced recovery programs (ERPs), as is multimodal pain management. 3. The traditional, liberal approach to perioperative fluid management has no sound evidence base and causes perioperative fluid and salt overload. “Zero fluid balance” is…
Key Points 1. The rate of maternal heart disease is increasing and complicates up to 4% of pregnancies. It is a leading cause of maternal, fetal, and neonatal morbidity and mortality. 2. The diagnosis of new cardiovascular disease during pregnancy may be challenging because both symptoms and physical signs often overlap with normal healthy pregnancy. 3. The preferred test during pregnancy to screen for structural cardiac…
Key Points 1. Cardiomyopathy and ischemic coronary artery disease are the most common cardiac conditions in liver transplant candidates. 2. There are no clear recommendations for the assessment of cardiac status before liver transplantation, but each patient must be individually evaluated. 3. Indications for liver transplant have shifted, which has resulted in more patients with underlying cardiac disease presenting for liver transplantation. 4. Pulmonary hypertension and…
Key Points 1. Cardiac patients presenting for emergency noncardiac surgery have higher risk for perioperative morbidity and mortality. Emergency surgeries are associated with two to five times the risk of major adverse cardiac events compared with elective procedures. Preoperative evaluation and patient optimization are limited by the nature of emergency surgery. 2. The main anesthetic goals in patients with cardiac disease undergoing emergency noncardiac surgery are…
Key Points 1. Tachyarrhythmias result from one of three mechanisms (reentry, automaticity, and triggered activity), with reentry being the mechanism most commonly treated in the electrophysiology (EP) laboratory. 2. Anesthetic agents influence cardiac conduction and arrhythmogenesis and can adversely affect EP procedures. 3. Opioids may have an antiarrhythmic effect in the setting of myocardial ischemia. 4. While the arrhythmic properties of volatile anesthetics are controversial, overall…
Key Points 1. Cardiac patients and those who have had previous cardiac surgery often present for intrathoracic diagnostic or therapeutic noncardiac procedures. 2. Patients with coronary artery disease, valvular heart disease, cardiomyopathies, or pulmonary hypertension may require surgery involving lung isolation and one-lung ventilation (OLV). OLV can carry a significant risk for hypoxia. A stepwise approach to management of hypoxia during OLV is important. 3. Patients…
Key Points 1. Patients who present for cerebrovascular, aortic, or peripheral arterial interventions are at elevated risk for concomitant coronary artery disease. 2. A thorough preoperative assessment for cardiovascular disease and medical optimization of any comorbid conditions are essential before elective vascular surgery. This preoperative process is typically not possible for emergency vascular procedures. For urgent but not truly emergent procedures, an expedited workup and targeted…
Key Points 1. With monitored anesthesia care, the stress response to surgery may not be adequately blocked, and the resulting tachycardia may aggravate the patient's underlying cardiac disease. 2. A decrease in minute ventilation and increase in carbon dioxide associated with sedation can have deleterious effects on patients with right heart dysfunction. 3. The recommended dose of epinephrine is reduced to less than 1 µg/kg in the…
Key Points 1. Intraoperative hemodynamic instability may be associated with increased cardiovascular complications and represents one of the most common findings associated with mortality. 2. Phenylephrine can be used for the treatment of intraoperative hypotension through an increase in stroke volume and cardiac output in patients with preload-recruitable stroke work. 3. Binding of vasopressin to its cognate receptor (V 1 ) leads to potent vasoconstriction and…
Key Points 1. The portability, ease of use, and rapid diagnostic capability of transesophageal echocardiography (TEE) make it the diagnostic modality of choice during acute hemodynamic instability. 2. Qualitative analysis of a condensed TEE examination aids in efficiency during the rapid diagnostic demands required in the emergency setting. 3. Rescue echocardiography is a process, not an event, and thus requires continuous reevaluation when treating hemodynamic instability.…