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Left‐to‐right shunt lesions are common among congenital heart defects and present in a variety of forms. This chapter summarizes the general concepts and pathophysiology that these lesions share, and details the incidence, anatomy, natural history, pathophysiology, surgical and transcatheter approaches and outcomes, and anesthetic considerations for each of eight left‐to‐right shunt lesions. These...
Left‐sided obstructive lesions of the heart may occur at various anatomic levels and with varying degrees of severity, ranging from a bicuspid aortic valve with minimal hemodynamic compromise to aortic atresia and hypoplastic left heart syndrome with profound hemodynamic derangements. These abnormalities in systemic blood flow may occur due to obstruction at multiple levels, such as seen in patients...
Right‐sided obstructive heart lesions contain various heart defects which present with a wide spectrum of physiological impact. Patients may present as neonates with severe congestive heart failure to adults with vague complaints of fatigue with exercise. Right‐to‐left shunting of blood can be evident due to the almost universally present septal defect. Obstruction to blood flow can occur in a single...
This chapter focuses on the congenital cardiac lesion of transposition of the great arteries and intends to give an overview of the condition. It is divided into two sections addressing the more common lesion of d‐TGA in part I and the rarer defect of congenitally corrected transposition of the great arteries (ccTGA) in the part II. For both of these lesions the anat‐omy, pathophysiology, natural...
Patients with a single functional systemic ventricle comprise approximately 5% of patients with congenital heart disease at birth. In the modern era, virtually all of these patients now undergo a staged palliation strategy, most often consisting of a neonatal surgery, followed by a superior cavopulmonary anastomosis (SCPA) at age 3‐6 months, and Fontan completion at 18 months‐3 years. This chapter...
The congenital cardiac anesthesiologist is often faced with caring for patients with lesions and diseases that do not fit neatly into the anatomic and surgical classifications presented elsewhere in this book. This chapter presents vascular rings, coronary artery anomalies, pericardial effusion and tamponade, mitral regurgitation, cardiac tumors, aortic aneurysm and aortopathy, and mediastinal masses...
This chapter begins with a review of the indications for heart transplantation, then presents recipient evaluation and pretransplant management. Then, donor management, surgical technique, and anesthetic management are discussed. Immunosuppression and rejection therapy are then summarized. Next, heterotopic heart transplantation, combined heart and liver transplantation, and retransplantation are...
Pulmonary hypertension (PH) is a multifactorial disease requiring thorough evaluation and classification followed by focused therapy. Several factors associated with the perioperative period are potential triggers for a pulmonary hypertensive crisis, and patients with PH have significant risks of cardiac arrest or death. Safe and successful anesthetic management of these patients requires understanding...
There has been a continuing trend of decline in the number of diagnostic cardiac catheterizations and a rise in interventional and hybrid procedures. With advances and an increasingly broader spectrum of interventions being offered, higher risk patients are presenting. It is vital therefore for close cooperation between the anesthesiologist and cardiologist to allow meaningful data collection for...
With a growing population of children, adolescents and adults with congenital heart disease of all types and severities, the number of these patients presenting for noncardiac surgery and imaging procedures will also increase. Pediatric patients on ventricular assist device support or with pacemakers and/or defibrilllators requiring anesthetic care are no longer rare. A combination of proper planning...
This chapter begins with a discussion of pathophysiology and implications of broad classes of congenital cardiac defects, including mixing lesions, shunts, and outflow obstruction lesions. Next, airway and ventilation management is presented, focusing on cardiorespiratory interactions, and the influence of lung volumes, intrathoracic pressure, and positive end‐expiratory pressure on cardiac physiology...
Mechanical devices used to support the failing heart or respiratory systems continue to improve and their use is continuing to increase. As outcomes have improved the number of indications for use has increased and the threshold for use continues to decline, recognizing that a persistent low cardiac output state has greater risk of organ injury or death than institution of mechanical support of the...
Over the last 70 years, pediatric cardiac anesthesia has developed as a subspecialty of pediatric anesthesia. Advancement in surgical treatment of CHD started with simple surgical ligation of a patent ductus arteriosus (PDA) and evolved to sophisticated, staged repair of complex intracardiac lesions in low‐birth‐weight neonates requiring cardiopulmonary bypass (CPB) and circulatory arrest to the most...
Advances in diagnosis in pediatric cardiology, medical management, cardiac surgery, and cardiac anesthesia throughout the world have drastically increased the survival of children with congenital heart disease (CHD) to over 90 % and as a result, there are more adults than children living with congenital heart disease today. Very few resources existed in terms of providing training and experience with...
This chapter begins with a discussion of errors and outcomes in surgery and anesthesia, emphasizing communication and teamwork. Next, systems for prospective risk assessment in pediatric cardiac surgery are discussed. Then, an analysis of closed malpractice claims in anesthesia is presented, and subsequently a discussion focusing on pediatric cardiac anesthesia morbidity and mortality. Finally, database...
As a stage in cardiology has been reached at which most advances will relate to an understanding of developmental biology, knowledge regarding cardiovascular development is relevant to practitioners who care for patients with congenital heart disease (CHD). It is equally important to be thoroughly conversant in the nomenclature used to describe the resulting morphologic and anatomic abnormalities...
This chapter presents a developmental perspective on cardiovascular physiology and cellular biology, which is essential to understand the pathophysiology of congenital heart disease. After presenting the change in circulatory pathways from the fetus, through the transitional circulation, to postnatal circulation, the chapter then reviews the developmental physiology of myocardial contractility. An...
This chapter reviews the cardiovascular effects of commonly used anesthetic and sedative agents, focusing wherever possible on human studies in infants and children with congenital heart disease. Volatile agents are discussed first, followed by nitrous oxide, and then opioids and benzodiazepines. Next, intravenous agents propofol, ketamine, etomidate, and dexmedetomidine are presented. Special conditions...
This chapter reviews the principles and practice of pediatric cardiopulmonary bypass (CPB) necessary for the anesthesiologist to be a contributing member of the congenital heart surgery team. The basic CPB systems, including circuits, oxygenator‐heat exchangers, tubing, cannulae and cannulation techniques, pumps, and prime composition are presented. Adult and pediatric cardiopulmonary bypass are then...
Surgical management of congenital cardiac disease in children produces a profound insult to immature organ systems. Because of the size of the cardiopulmonary bypass circuit is relatively larger in children as compared to adults, there is greater blood exposure to the artificial surface and more significant hemodilution producing a profound inflammatory response and activation of the coagulation cascade...
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