What is tetralogy of Fallot (TOF)?

In 1888, Fallot described a congenital heart defect composed of four characteristics (a) large ventricular septal defect (VSD), (b) right ventricular outflow obstruction, (c) overriding aorta, and (d) right ventricular hypertrophy. The complex of anatomic malformations results from an anterior displacement of the conoseptum toward the right ventricle creating a malalignment VSD and a narrowing of the outflow tract of the right ventricle (RV). The aorta is displaced anteriorly, straddling the muscular septum and arising from both ventricles (overriding aorta). See Fig. 9.1. Figure 9.1. Tetralogy of Fallot (From Moller DH, Neal WA, Hoffman WR, eds. A parent's guide to heart disorders. Minneapolis: University of Minnesota Press, 1988:44, with permission.) View Figure

The obstruction to outflow of the right ventricle usually involves the infundibulum of the right ventricle but can arise from the pulmonic valve, its annulus, the main pulmonary artery (PA), or even in the peripheral pulmonary arteries. Elevated pressures in the right ventricle from right ventricular outflow obstruction and exposure to systemic pressure from the overriding aorta lead to compensatory right ventricular hypertrophy. Back to Quick Links Castaeda AR, ed. Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:215219. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:303304. Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:810. A.2. What other conditions may produce cyanosis in the first year of life? Any condition that results in right-to-left shunting will produce cyanosis. The classic cyanotic congenital heart lesions are the 5 Ts:

TOF Transposition of the great arteries Truncus arteriosus

Tricuspid atresia Total anomalous venous return

Other cardiac lesions that lead to cyanosis are

Single ventricle Hypoplastic left heart syndrome Double outlet right ventricle Single atrium Pulmonic stenosis (PS) or atresia

Back to Quick Links Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18101811. A.3. Describe the pathophysiology of TOF. The main characteristic of TOF is cyanosis. Cyanosis can result from three separate mechanisms: (a) inadequate pulmonary blood flow, (b) right-to-left shunting, or (c) intrinsic pulmonary disease. In TOF, cyanosis results from a right-to-left shunt at the level of the ventricles and inadequate pulmonary blood flow. Because of the right ventricular outflow obstruction, blood ejected from the right ventricle crosses the VSD and enters the overriding aorta. This reduces the amount of pulmonic blood flow available for oxygenation and adds desaturated blood to the systemic circulation leading to cyanosis. Pressures in the right ventricle are near or equal to systemic pressure. The pressure load on the right ventricle causes the compensatory right ventricular hypertrophy. The degree of shunting across the VSD is proportional to the relationship of PVR to systemic vascular resistance (SVR). Pulmonary resistance is mostly fixed but can vary with activity. SVR is variable. Decreases in SVR or increases in PVR will worsen the degree of cyanosis. Back to Quick Links Castaeda AR, Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:219220. A.4. What is a "pink tet"? A pink tet is a patient with TOF and a source for adequate pulmonary blood flow. The additional pulmonary blood flow can come from a patent ductus arteriosus (PDA), aorto-pulmonary collaterals, or other naturally occurring collateral vessels to the PA (bronchial, intercostal, or coronary arteries). In the rare case in which the right ventricular outflow obstruction is not significant, the degree of right-to-left shunt is reduced in favor of an increase in pulmonary blood flow, also causing a pink tet. With time, the degree of outflow obstruction worsens and this type of pink tet evolves into cyanotic TOF.

Back to Quick Links Castaeda AR, Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:219220. A.5. What are hypercyanotic spells? How are they treated? Hypercyanotic spells or "tet spells" are paroxysmal episodes in which the cyanosis acutely worsens. Crying, feeding, or defecating can bring on these episodes. The common pathway for all these activities is an increase in right-to-left shunting. Three mechanisms can explain the increase in shunt:

Increase in PVR o This is associated with a reduction in pulmonary blood flow and increase in rightto-left shunt. Treatment is to lower PVR through hyperventilation with 100% O2 and bicarbonate administration to temper the effects of acidosis on PVR. Dynamic outflow obstruction o Tachycardia, hypovolemia and increased myocardial contractility can cause infundibular spasm. Similar to an increase in PVR, spasm decreases blood flow into the PA and worsens right-to-left shunt. The spasm can be treated with blockers, with volume, and by deepening the level of anesthesia to decrease catecholamine levels. Morphine can be given to diminish the hyperpneic response. Decrease in SVR o This will favor right-to-left shunting through the VSD. Treatment is volume administration to ensure adequate filling of the right ventricle and an adrenergic agonist to increase the SVR. SVR can also be increased by flexing the legs or by compressing the abdominal aorta directly. Children will squat during a hypercyanotic spell to increase their SVR and cause a decrease in the right-to-left shunt.

Back to Quick Links Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:810812. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:305. A.6. Describe natural history of the disease. Without treatment, 25% of infants with TOF and PS die in the first year of life, 40% will die by the age of 4 years, 70% by 10 years, and 95% by 40 years. Cyanosis may not become evident until closure of the ductus arteriosus occurs or hypertrophy of the right ventricle leads to an increase in right ventricular outflow obstruction. The increase in outflow obstruction decreases pulmonary blood flow and increases the right-to-left shunt. The resulting chronic hypoxemia leads to an increase in erythropoietin production and polycythemia. Long-term polycythemia is evident from clubbing of the nailbeds on physical examination. Pulmonary, renal, and cerebral

thrombosis can occur as a result of hyperviscosity when the hematocrit reaches 65%. Late causes of death include cardiomyopathy, hypoxia, and aortic valve insufficiency with cardiac failure. Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:305306. A.7. What are the associated anomalies? Associated anomalies that can influence the surgical repair are

Multiple muscular VSDs (about 3% to 15%) Right-sided aortic arch (25%) Atrial septal defect (ASD) (9%) Persistent left superior vena cava (8%) Abnormalities in the coronary arterial origin and distribution (5%) PDA (4%) Partial anomalous pulmonary venous drainage (1%)

Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:305306. A.8. What are the significant variables in TOF? The variables in TOF are the location and degree of right ventricular outflow obstruction and the size of the VSD. These factors, coupled with the relationship of PVR to SVR, all influence the amount of right-to-left shunting. The right ventricular outflow obstruction can range from mild to complete (TOF with pulmonary atresia). The location of the obstruction can be anywhere along the right ventricular outflowinfundibulum, pulmonic valve, the annulus of the pulmonic valve, the main PA, or in the branch or peripheral pulmonary arteries. VSD size can limit the amount of blood shunted into the aorta. Infants with less shunt and smaller degrees of cyanosis have improved outcomes. Back to Quick Links Castaeda AR, Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:215219. A.9. What techniques or surgical procedures are available for treating this patient? Palliative Procedures

Balloon dilatation of the pulmonic valve can be performed during catheterization. Systemic-pulmonary arterial shunts o Classic Blalock-Taussig shunt: anastomosis of the right or left subclavian artery to the PA o Modified Blalock-Taussig shunt: artificial tube graft between the right or left subclavian artery and pulmonary arteries o Central shunt: artificial tube graft between the aorta and PA

Other shunts such as the Waterston and Potts shunts are of historic interest now. Definitive Surgical Correction About 70% of patients with TOF with PS require surgery within the first year of life. In the past, children underwent placement of a systemic-pulmonary shunt followed by a definitive repair at an older age. As surgical and anesthetic techniques improved, corrective surgery was undertaken at a younger age. Currently, most patients with TOF have a full correction between the ages of 2 to 10 months. The diagnosis of TOF is made at or soon after birth. The infants are followed until evidence of hypercyanotic episodes occur and then referred for surgery. A push had been made for neonatal correction of this disease, but the increased surgical morbidity and mortality did not support surgery at that early age. The definitive correction of TOF requires CPB and includes closure of the VSD with a patch, a right ventriculotomy with division of muscle bundles, and enlargement of the RV outflow tract. The outflow augmentation is done with a pericardial patch that can include the outflow tract, valve annulus, and even extend onto the main and branch pulmonary arteries. The closure of the VSD is completed with a patch that closes the defect and leaves the aorta arising from only the left ventricle. Nothing is done to the overriding aorta. Following correction, the pressures in the right ventricle should be at least half of the systemic pressure. The only contraindication to a repair after the first 2 months of life is an anomalous origin of the right coronary artery. In this case a shunt procedure is done initially, followed by reconstruction with RV to PA conduit at a later date. Patients with TOF with pulmonary atresia require a more complex correction involving reconstruction of their pulmonary arteries with multiple surgeries as well as closure of the VSD. Initially the outflow tract obstruction is relieved, the VSD is left open, and the pulmonary circulation is reconstructed in a unifocalization procedure. The concept of unifocalization is to use the largest of the aortopulmonary collaterals to "build" right and left pulmonary arteries. The newly constructed pulmonary arteries can be connected to the right ventricle with homograft, allowing flow from the right ventricle into the pulmonary arteries and to help them grow. Eventually, the VSD is closed and any residual outflow tract stenosis is relieved. When necessary a valved homograft is interposed between the RV and the PA. Back to Quick Links

Caspi J. Zalstein E. Zucker N, et al:. Surgical management of tetralogy of Fallot in the first year of life. Ann Thorac Surg 1999:68:13441349. Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:812. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:223229. Van Arsdell GS. Maharaj GS. Tom J, et al.What is the optimal age for repair of tetralogy of Fallot?. Circulation 2000:102:[Suppl III]123129. B. Preoperative Evaluation and Preparation Back to Quick Links B.1. What preoperative information do you want? A preanesthesia history and physical examination, accompanied by appropriate laboratory data, are vital. Special attention is directed to information pertaining to hypercyanotic spells frequency, severity, and methods to treat them. Weight gain, growth, development, and level of activity can indicate the severity of the cardiac disease. An understanding of the patient's medications and their effects is also necessary. In addition, common preoperative problems for pediatric patients need to be addressed. Recent respiratory tract infection and the presence of loose teeth should be noted. Hematocrit is frequently elevated in cyanotic patients and intraoperative phlebotomy can be used to prevent the problems associated with polycythemia. Chest radiographs demonstrate a bootshaped heart secondary to right ventricular hypertrophy and concave PA segment. An extensive cardiac evaluation to identify other congenital anomalies is prudent. Cardiac catheterization can provide information on PVR, the ratio of pulmonary blood flow to systemic blood flow (Qp : Qs), and degree of valvar PS. Angiography will display the coronary and PA anatomy, pulmonary collateral circulation, and other cardiac anomalies. Much of this information can be gathered from transthoracic echocardiography. Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:306307. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18141815. B.2. What NPO guidelines do you follow and what premedication do you give? General NPO guidelines can be followed. The child is allowed any solid food and particulate fluid (like milk, formula, or breast milk) up to 6 hours before surgery and clear liquids up to 2 hour before surgery.

Oral premedication is recommended in children who suffer from hypercyanotic spells. Midazolam 0.5 to 1.0 mg/kg can be given by mouth 10 to 20 minutes before induction in children older than 9 months or pentobarbital 2 to 4 mg/kg 45 minutes before induction in children older than 6 months. In children older than 1 year, adding oral meperidine 3 mg/kg to the pentobarbital further eases the separation from parents. An anticholinergic agent, atropine 0.1 to 0.15 mg/kg by mouth, can prevent bradycardia associated with some anesthetic inductions. Propranolol should be continued up to and including the day of surgery. Back to Quick Links Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:795796. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:307308. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:1815. Practice guidelines for preoperative fasting and the use of pharmacologic agents to reduce the risk of pulmonary aspiration: application to healthy patients undergoing elective procedures. Anesthesiology 1999:90:896905. 32 B.3. Discuss preoperative antibiotics. Antibiotics should be administered intravenously before induction, if possible. In the event that intravenous access is obtained after induction, antibiotics should be given as soon as possible. In patients with no known allergies, cefazolin 25 mg/kg is appropriate. If there is a penicillin or cephalosporin allergy, vancomycin 20 mg/kg over 1 hour can be substituted. Antibiotics should be repeated after separation from CPB. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:183. Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:798. C. Intraoperative Management Back to Quick Links C.1. Which emergency drugs will you prepare? Standard emergency drugs for pediatric cardiac case should be available. Epinephrine 10 Atropine 20 g/kg

g/kg

Calcium chloride 10 mg/kg In addition, phenylephrine 2 to 10 g/kg and propranolol 10 to 50 g/kg should be available to assist in the treatment of hypercyanotic spells. Infusions should be readily available if inotropic support is necessary in the postbypass period. Dopamine, dobutamine, isoproterenol, epinephrine, or phosphodiesterase inhibitors are all useful in these cases. Infusions can be prepared in the operating room, by the pharmacy, or in the pediatric intensive care unit based on a standardized concentration that can be used for the postoperative period. The dosages of the drips and emergency drugs for pediatric patients at our institution are available through a computerized form available on our hospital website. See Table 9.1. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:470473. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:340341. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:308309. The New York Presbyterian Hospital Protocol http://infonet.nyp.org/pedicalc/default.htm C.2. What monitoring will be necessary for a palliative shunt? Standard monitoring includes

Electrocardiogram (ECG) Pulse oximetry. Because pulse oximeters require pulsatile flow to work properly, the probe should be placed on the hand opposite to the side of the proposed shunt. Appropriate care in placing the pulse oximeter will prevent a loss of signal during shunt placement. Occasionally, two sites are selectedone probe on an upper extremity and a second probe on a lower extremity. Blood pressure. An automated blood pressure cuff can be used during induction. After endotracheal intubation, an arterial line can be placed if deemed necessary. The need for this line is surgeon and patient dependent. The site of arterial cannulation should avoid arteries affected by the current procedure or previous shunts. An arterial line allows for continuous blood pressure measurement and access for arterial blood gas sampling. End-tidal carbon dioxide. This helps determine correct placement of the endotracheal tube and assists in managing ventilation. Rectal and esophageal temperature Urine output

At least one, but preferably two, large bore intravenous line(s) are placed after induction.

Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:488489. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:340342. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18151816. C.3. What monitoring would be necessary in a definitive surgical repair? In addition to standard monitoring, an arterial line is an absolute necessity. The location of the arterial line again depends on the location of previous shunts and catheterizations. Depending on the size of the child, a central venous catheter can be inserted into the right internal jugular vein or the femoral vein. A double-lumen catheter is useful to allow infusion of medications and measurement of central venous pressure (CVP) in separate lines. In about 8% of patients there is a persistent left superior vena cava demonstrated during preoperative catheterization that can influence line placement. Transthoracic monitoring lines can be placed by the surgeon before separation from CPB, in lieu of preoperative central lines. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:488489. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:341342. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18151816. C.4. How would you perform the anesthesia for a shunt procedure? The main goal of induction in a cyanotic patient is to establish a necessary level of anesthesia without increasing the right-to-left shunt. The stress of the induction can lead to a hypercyanotic spell. Our objectives are to

Maintain SVR. Avoid drugs that decrease SVR and treat decreases in blood pressure promptly with vasoconstrictors. Decrease PVR to maintain or improve pulmonary blood flow. Hypercarbia, hypoxia, light anesthesia, atelectasis, polycythemia, acidosis, and elevated airway pressures increase PVR. Hypocarbia, anemia, alkalosis, high oxygen concentrations, and deep anesthesia decrease PVR. PVR can also be decreased using drugs such as nitroglycerin, sodium nitroprusside, phentolamine, tolazoline, prostaglandin E1, or inhaled nitric oxide. Favor mild myocardial depression and euvolemia because they can help prevent or limit a hypercyanotic spell. Most volatile anesthetics provide some myocardial depression with halothane having the most pronounced effect. Slow heart rate to reduce the likelihood of infundibular spasm.

If intravenous access is present, induction can be accomplished with ketamine (2 mg/kg) or fentanyl (25 g/kg). An intravenous induction is faster in patients with a right-to-left shunt because peak receptor site concentrations are attained faster. If intravenous access is absent, induction can be accomplished with an intramuscular injection of ketamine (4 mg/kg) especially in patients in whom a mask induction would be frightening. The option of a mask induction with oxygen and halothane or sevoflurane is always a possibility, keeping in mind that a significant right-to-left shunt may delay induction. Once the induction is completed and intravenous access is established, vecuronium or pancuronium (0.2 mg/kg) can be given to facilitate endotracheal intubation. Maintenance of anesthesia is accomplished with oxygen, volatile anesthetic agents, intravenous opiates, and benzodiazepines. An appropriately sized endotracheal tube should be placed according to the size and age of the patient. Whether a cuffed tube is used is institution dependent, but a leak above 22 to 25 cm H2O is preferred. Intravenous tubing must be meticulously checked to ensure that no air bubbles enter the patient's circulation. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:506508. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:344345. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:307308. C.5. What is the effect of each of the following anesthetics on pulmonary vascular resistance (PVR): barbiturates, ketamine, narcotics, halothane, sevoflurane, isoflurane, desflurane, and nitrous oxide? Barbiturates have a vasodilating effect, lowering both SVR and PVR. Ketamine has been shown to decrease PVR in adults but not in children. Its sympathomimetic effect helps in maintaining SVR, and it is considered one of the safest induction techniques for a cyanotic patient. Narcotics provide good hemodynamic stability without altering PVR. Fentanyl and sufentanil are used for maintenance of anesthesia because they blunt the sympathetic stress response. Halothane and sevoflurane are both considered safe for inhalation induction. They both decrease SVR but have a small effect on PVR. Sevoflurane is less of a myocardial depressant than halothane.

Desflurane and isoflurane decrease SVR in a dose-dependent manner and are mild myocardial depressants. Desflurane at higher concentrations (above 6%) is associated with increased sympathetic stimulation, which can be detrimental in TOF. Both volatile agents have a very pungent odor and have been associated with laryngospasm and bronchospasm, during inhalational induction, can be used for maintenance of anesthesia. Nitrous oxide does not increase PVR in infants, but 100% oxygen is preferred to avoid hypoxemia. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:491495. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:341344. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:307309. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18181821. C.6. How would you perform the anesthesia for a definitive surgical correction? The management of preoperative period and induction are essentially the same as that for placement of a shunt. The main difference is the need for CPB to perform the definitive correction. After induction of anesthesia, invasive monitors are placed and the surgeon prepares the patient for CPB. An activated clotting time (ACT) is measured as a baseline and again after administration of 300 to 400 units/kg of heparin to achieve an ACT greater than 480 seconds. At our institution, the surgeon administers heparin directly into the right atrium, but it also can be given through a central line. After CPB is instituted, maintenance of anesthesia is accomplished with narcotics, benzodiazepines, and muscle relaxant. Initiation of CPB causes hemodilution. The hematocrit is allowed to go as low as 20% and the temperature is lowered to 25C to 28C. Should the repair require deep hypothermic circulatory arrest, the temperature is decreased to 18C to 20C. Blood cardioplegia solution with high-dose potassium (30 mEq/L) is used to arrest the heart and protect the myocardium. Any preexisting pulmonary-to-systemic shunt is clamped or ligated on institution of CPB to avoid pulmonary hyperperfusion. After surgical repair is completed and the heart is closed, the patient is placed in Trendelenburg position until air bubbles are eliminated from the chambers of the heart. When the temperature reaches 36C and the heart has a stable rate and rhythm, weaning from CPB is attempted. Inotropic support, if needed, is achieved with epinephrine, dobutamine, dopamine, or milrinone infusions.

Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:307309. C.7. How are hypercyanotic spells managed during anesthesia? Hypercyanotic spells manifest with a sudden decrease in the oxygen saturation. Without quick intervention, they can progress to bradycardia and systemic hypotension. Treatment is to increase the depth of anesthesia, hyperventilate with 100% oxygen, administer volume, increase SVR with phenylephrine, and decrease infundibular spasm with -blockers. Sodium bicarbonate should be considered to treat any ensuing metabolic acidosis. Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:305. C.8. Discuss blood replacement therapy. Blood is very rarely needed in shunt procedures. It should be present in the operating room in the event of any untoward events. Because of the hemodilution associated with CPB, blood is often used to "prime" the bypass circuit. The requirement for blood depends on the size and starting hematocrit of the patient. After bypass, the volume in the bypass circuit is concentrated and returned to the patient to limit the homologous blood exposure. In smaller patients, plasma and platelets are required to stop postbypass bleeding secondary to dilutional thrombocytopenia and coagulopathy. Some institutions prefer to use fresh whole blood to restore the level of coagulation factors that are severely decreased by CPB. Back to Quick Links Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18181821. C.9. What is monitored during cardiopulmonary bypass (CPB)?

Perfusion pressure is traditionally lower in children than adults while on CPB. Pressure is usually maintained at 30 to 50 mm Hg despite higher flow rates. Pump flow rates vary with the size of the patient ranging from 200 mL/kg/minute in the neonate to 100 mL/kg/minute in older infants and children. Blood gases are monitored every 20 to 30 minutes while on CPB to ensure adequate gas exchange and perfusion. Hematocrit can be assessed at this time and corrected if it falls below acceptable levels. Respiratory acidosis is corrected by increasing the gas sweep through the oxygenator, and metabolic acidosis is corrected with sodium bicarbonate.

Mixed venous oxygen saturation can be used to determine adequate tissue perfusion. A decrease in mixed venous oxygen saturation can reveal poor perfusion, light anesthesia, or increased oxygen consumption. Urine output should be 1 to 2 mL/kg/hour. Temperature is monitored with a bladder, rectal, or nasopharyngeal probe. Patients are cooled to 25C to 30C while on bypass. Moderate hypothermia helps decrease metabolic demand while on CPB. The ACT indicates the level of anticoagulation. An ACT is repeated every 20 to 30 minutes after heparin administration to ensure an adequate degree of anticoagulation (ACT greater than 480 seconds) for CPB. If necessary, additional heparin is administered.

Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:498501. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:345346. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:308309. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18211825. C.10. Which measurements are made after separation from CPB bypass? CVP is routinely measured after separation from CPB bypass. The CVP is used to estimate the filling and function of the RV following correction. In some institutions, PA and left atrial lines are also used. The need for these additional monitors is surgeon and institution dependent. Postbypass transesophageal echocardiography can assess left and right ventricular function, the presence of residual VSDs, and the pressure gradient across the pulmonic valve. It can also be useful in detecting intracardiac air before separation from pump. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:500501. Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company, 1995:347. Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:803. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:309. C.11. What inotropic agents can be used to help separation from CPB and in the postoperative period?

Epinephrine 0.05 to 0.5 g/kg/minute Dopamine 1 to 20 g/kg/minute Milrinone 0.325 to 0.750 g/kg/minute

Epinephrine is an - and -adrenergic agonist. Its -adrenergic effects increase contractility and heart rate. Its -adrenergic effects will cause vasoconstriction and increased vascular resistance. Dopamine has dose-dependent effects. At low doses (1 to 5 g/kg/minute), the predominant effects are on dopamine receptors that increase renal, splanchnic, coronary, and cerebrovascular blood flow. At intermediate doses (5 to 15 g/kg/minute), the major effects are -adrenergic increased heart rate, contractility, cardiac output, and blood pressure. At higher doses (greater than 15 g/kg/minute), the -adrenergic effects of vasoconstriction and increased blood pressure are most dominant. Milrinone is a phosphodiesterase inhibitor. It inhibits the breakdown of cyclic AMP leading to a decrease in PVR and SVR and an increase in cardiac contractility. Back to Quick Links Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:309. Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:18311834. C.12. How is heparin reversed? After separation from CPB is successfully achieved, heparin is reversed using protamine sulfate. Protamine binds to heparin and forms a stable salt neutralizing heparin's activity on antithrombin III. The dose of protamine is calculated based on the heparin given during the case. It is usually 1 mg of protamine per 100 units of heparin. Back to Quick Links Donnelly AJ, Cunningham FE, Baughman VL, Anesthesiology and critical care drug handbook2nd ed. Hudson OH: Lexi-Comp, Inc, 1999:807809. C.13. What is a protamine reaction? Protamine sulfate causes several hemodynamic reactions when administered. Most commonly, it has vasodilating and negative inotropic effects that result in lower blood pressure. Anaphylactic and anaphylactoid reactions are also possible. The heparin-protamine complex can lead to the release of thromboxane A2. Thromboxane release causes pulmonary vasoconstriction and a rise in PA pressures. The thromboxane-mediated reaction can be severe enough to require significant inotropic and vasopressor support. In rare cases, it is not able to be reversed pharmacologically and requires a return to CPB until the thromboxane is metabolized and the pulmonary effects subside.

To avoid or minimize these adverse reactions, protamine is usually given after a small test dose and over 5 to 10 minutes. Back to Quick Links Donnelly AJ, Cunningham FE, Baughman VL, Anesthesiology and critical care drug handbook2nd ed. Hudson OH: Lexi-Comp, Inc, 1999:807808. C.14. Can regional anesthesia be used as an adjuvant to general anesthesia in the shunting or definitive correction procedures? Use of regional anesthesia techniques in children seems to be more effective in inhibiting the stress response associated with surgery than intravenous narcotics. Spinal injection of narcotics and/or local anesthetics achieves an excellent pain relief allowing extubation in the operating room after procedures that do not require CPB. The doses for spinal anesthetic are shown in Table 9.2. A combination of local anesthetic and narcotic given in the caudal or epidural space at the beginning of the case can dramatically decrease the amount of total narcotic required for the case. Continuous epidural infusion during surgery attenuates the stress response associated with CPB and helps optimize analgesia in the postoperative period. In children, epidural catheters can be easily inserted through the sacrococcygeal membrane and advanced 16 to 18 cm to reach the thoracic epidural space. A combination of bupivacaine and hydromorphone, or bupivacaine and morphine, can be used as an infusion and with the dose adjusted as needed. An example can be an initial bolus with 0.25% bupivacaine 0.5 mL/kg plus hydromorphone 7 to 8 g/kg followed by supplemental doses of 0.25% bupivacaine 0.3 mL/kg without narcotics. For the postoperative period, an infusion of 0.10% bupivacaine plus hydromorphone 3 g/mL is given at a rate of 0.3 mL/kg/hour. Placement of a spinal or epidural anesthetic should occur soon after intubation to maximize the time before anticoagulation. The most common side effects of neuraxial narcotics are nausea, vomiting, and pruritus. Hypotension associated with use of epidural local anesthetics in adults is very uncommon in children. The risk of epidural hematoma is small. The epidural catheter should be removed only after return of normal coagulation parameters postoperatively. Back to Quick Links Hammer GB. Ng OK. Macario A. A retrospective examination of regional plus general anesthesia in children undergoing open heart surgery. Anesth Analg 2000:95:10201024. D. Postoperative Management Back to Quick Links D.1. Discuss postoperative ventilation.

Postoperative ventilation is directed at increasing pulmonary blood flow. As mentioned earlier, increased FIO2, mild hyperventilation (PaCO2 around the low 30s), euthermia, and mild alkalosis promote a decrease in PVR and increased pulmonary blood flow. The greatest benefit of this ventilatory management is seen following placement of a shunt, although it helps after complete correction. The pressures generated during mechanical ventilation are reflected to the pulmonary vascular bed and mediastinal structures. Positive pressure ventilation decreases venous return, increases PVR, and affects the relationship of pulmonary to systemic blood flow. Problems associated with endotracheal intubation and mechanical ventilation include damage to airway mucosa, mechanical obstruction of the tube, endobronchial intubation, and accidental extubation. Pulmonary complications are pneumonia, ventilation-perfusion mismatch, increased dead space, and parenchymal damage. Prolonged intubation is not advised in these patients. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:845851. Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:310. D.2. What are the requirements for extubation? Extubation following an elective shunt procedure can usually be accomplished soon after the completion of surgery. Spontaneous ventilation with normal blood gases, normothermia, adequate pain control, and stable hemodynamics are required before extubation. Extubation is usually performed in the operating room or soon after arrival in the intensive care unit. Emergency shunt placement implies that the patient is unstable before surgery. The probability of hemodynamic, metabolic, and pulmonary problems is high. As such, extubation in these patients should wait until resolution of these issues. Extubation following complete repair follows the same parameters as that for an elective shunt procedure. Other important issues to be considered are postoperative bleeding, the need for inotropic support, and effects of CPB on cardiac and pulmonary function. Back to Quick Links Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:850851. D.3. Discuss postoperative complications in a shunt procedure and in a definitive correction. Shunt Procedure

Bleeding

Pneumothorax Too large of a shunt and excessive pulmonary blood flow. The result can be pulmonary edema and inadequate systemic blood flow Too small of a shunt with little improvement in oxygenation Thrombosis of the shunt PA hypoplasia

D.4. Definitive Correction

Immediate postoperative complications o Low output state o Residual right ventricular outflow tract (RVOT) obstruction o Residual VSD o Coagulopathies o Heart block o Renal failure o Phrenic nerve injury o Stroke o Infection Late complications o RVOT obstruction o RVOT aneurysm o Residual VSD o Dysrhythmia and sudden death o Valvular insufficiency

Back to Quick Links Gatzoulis MA. Balaji S. Webber SA, et al.Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000:356:975981. Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:519520. D.4. How would you manage an uncorrected patient for noncardiac surgery? The management of a patient with TOF undergoing noncardiac surgery is directed at prevention of hypercyanotic spells. A complete history of the frequency, severity, and causes of hypercyanotic spells as well as the methods used to treat them are important. Hematocrit, shunt fraction, and overall health of the patient are essential pieces of information. The severity of resting cyanosis and the hematocrit can indicate severity of the right-to-left shunt. Risk is increased in patients with more severe cyanosis or with hypercyanotic episodes. Patients who have a systemic-to-pulmonary artery shunt have a lower risk. Anesthetic goals are the same as if the procedure was cardiac surgerymaintain SVR and improve pulmonary blood flow. Be prepared to treat a tet spell aggressively with fluids, negative inotropes, oxygen, hyperventilation, and vasoconstrictors.

Monitoring should be standard. A noninvasive blood pressure cuff is sufficient for most routine cases, but longer or more complex cases warrant arterial access. Arterial blood gas analysis can assess the degree of hypoxemia, the presence of acidosis, and serial hematocrits. Keep in mind the location of shunts when placing the blood pressure cuff or arterial line.