KEY POINTS

1. Organ system maturation, from birth through adolescence (e.g., cardiovascular, central nervous system, pulmonary, renal, hematologic), affects physiologic function and, therefore, anesthetic and surgical management and outcome.
2. Physiologic understanding of congenital heart disease and subsequent anesthetic management is based on the pathophysiologic determinants of four categories of defects: shunts, mixing lesions, stenotic lesions, and regurgitant lesions.
3. The chronic sequelae of congenital heart disease—repaired, palliated, or unrepaired—affect anesthetic management: ventricular failure, residual hemodynamic effects (e.g., valve stenosis), arrhythmias, and pulmonary blood flow changes (e.g., pulmonary artery hypertension).
4. Preoperative assessment of cardiac status (e.g., review of history and physical, echocardiography, and catheterization data, speaking with patient's cardiologist) and planning are the keys to a successful anesthetic outcome.
5. Intraoperative transesophageal echocardiography and central nervous system monitoring enhance surgical outcome and reduce morbidity.
6. Selecting an induction technique is dependent on the degree of cardiac dysfunction, the cardiac defect, the degree of sedation provided by the premedication, and the presence or absence of an indwelling venous catheter. The maintenance of anesthesia depends on the age and condition of the patient, the nature of the surgical procedure, the duration of cardiopulmonary bypass, and the need for postoperative ventilation.
7. The physiologic effects of CPB on neonates, infants, and children are significantly different than the effects on adults. During CPB, pediatric patients are exposed to biologic extremes not seen in adults, including deep hypothermia (18°C), hemodilution (three- to fivefold greater dilution of circulating blood volume), low perfusion pressures (20 to 30 mm Hg), and wide variation in pump flow rates (ranging from 200 mL/kg/minute to total circulatory arrest).
8. After the repair of complex congenital heart defects, the anesthesiologist and surgeon may have difficulty separating patients from CPB. Under these circumstances, a diagnosis must be made and includes (1) an inadequate surgical result with a residual defect requiring repair, (2) pulmonary artery hypertension, and (3) right or left ventricular dysfunction.
9. The use of MUF reverses the deleterious effects of hemodilution and the inflammatory response associated with CPB in children. Perioperative blood loss and blood use are significantly reduced when MUF is used. MUF also improves left ventricular function and systolic blood pressure and increases oxygen delivery. Pulmonary compliance and brain function after CPB are also improved.
10. Neonates, infants, and children undergoing cardiac surgery with CPB have a higher rate of postoperative bleeding than that seen in older patients. This is due to several factors: (1) There is disproportionate exposure to the nonendothelialized extracorporeal circuit, which produces an inflammatory-like response. This inflammatory response to CPB is inversely related to patient age; the younger the patient, the more pronounced the response. (2) The type of operations performed in neonates and infants usually involves more extensive reconstruction and suture lines, creating more opportunities for surgical bleeding than in adult cardiac patients. (3) Operations are frequently performed using deep hypothermia or circulatory arrest, which may further impair hemostasis. (4) The immature coagulation system in neonates may also contribute to impaired hemostasis. (5) Patients with cyanotic heart disease demonstrate an increased bleeding tendency before and after CPB.
11. The guiding principle in the management of the postoperative patient is an understanding of both normal and abnormal convalescence after anesthesia and cardiac surgery. The immediate postoperative period, even that of normal convalescence, is one of continuous physiologic change because of the pharmacologic effects of residual anesthetic agents and the ongoing physiologic changes secondary to abrupt alteration in hemodynamic loading conditions, surgical trauma, and extracorporeal circulation.
12. There are additional anesthetic considerations in patients with congenital heart disease who undergo transplantation, closed heart operations without CPB, cardiac interventional procedures, and non-cardiac surgery.