Case Studies - Tetralogy of Fallot

5/17/2020 Case Studies: Tetralogy of Fallot
<BACK TO: CD-ROM Main Contents | Contents of Resource Shown:
Anesthesiology: Problem-Oriented Patient Management < Previous Chapter | Next Chapter >
CASE 9: Tetralogy of Fallot

Serle K. Levin
Vittoria Arslan-Carlon
Quick Links to Sections in this Chapter
–A. Medical Disease and Differential Diagnosis
–B. Preoperative Evaluation and Preparation
–C. Intraoperative Management
–D. Postoperative Management
A 10-month-old girl was admitted to the hospital for cardiac catheterization. Her history included cyanosis
noted at about 6 weeks of age, increasing over the last 7 months and becoming more severe with crying or
physical activity. A presumptive diagnosis of tetralogy of Fallot (TOF) was made on admission.
Related Links in other Resources
Congenital heart disease, Chapter 21 from Cardiac Anesthesia: Principles and Practice
Postoperative management of tetralogy of fallot in Chapter 18 from Cardiac Anesthesia:

Principles and Practice
Tetralogy of Fallot in Chapter 14 from A Practical Approach to Cardiac Anesthesia
A. Medical Disease and Differential Diagnosis

1. What is tetralogy of Fallot (TOF)?
2. What other conditions may produce cyanosis in the first year of life?
3. Describe the pathophysiology of TOF.
4. What is a "pink tet"?
5. What are hypercyanotic spells? How are they treated?
6. What is the natural history of the disease?
7. What are the associated anomalies?
8. What are the significant variables in TOF?
9. What techniques or surgical procedures are available for treating this patient?
B. Preoperative Evaluation and Preparation

1. What preoperative information do you want?
2. What nothing by mouth (NPO) guidelines do you follow and what premedication do
you give?
tele.med.ru/book/cardiac_anesthesia/text/ya/ya009.htm 1/16
3. Discuss preoperative antibiotics.
C. Intraoperative Management
1. What emergency drugs will you prepare?
2. What monitoring will be necessary for a palliative shunt?
3. What monitoring would be necessary in a definitive surgical repair?
4. How would you perform the anesthesia for a shunt procedure?
5. What is the effect of each of these drugs on pulmonary vascular resistance (PVR):
barbiturates, ketamine, narcotics, halothane, sevoflurane, isoflurane, desflurane, and
nitrous oxide?
6. How would you perform the anesthesia for a definitive surgical correction?
7. How are hypercyanotic spells managed during anesthesia?
8. Discuss blood replacement therapy.
9. What is monitored during cardiopulmonary bypass (CPB)?
10. Which measurements are made after separation from CPB?
11. What inotropic agents can be used to help separation from CPB and in the
postoperative period?
12. How is heparin reversed?
13. What is a protamine reaction?
14. Can regional anesthesia be used as an adjuvant to general anesthesia in the shunting
or definitive correction procedures?
D. Postoperative Management
1. Discuss postoperative ventilation.
2. What are the requirements for extubation?
3. Discuss postoperative complications in a shunt procedure and in a definitive

correction.
4. How would you manage an uncorrected patient for noncardiac surgery?
A. Medical Disease and Differential Diagnosis

Back to Quick Links
A.1. 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
Castañeda AR, ed. Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:215–219.
Lake CL, Pediatric cardiac anesthesia3rd ed. Stamford, CT: Appleton & Lange, 1998:303–304.
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, 2000:1810–1811.
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
Castañeda AR, Cardiac surgery of the neonate and infant Philadelphia: WB Saunders, 1994:219–220.
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
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
This is associated with a reduction in pulmonary blood flow and increase in right-to-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
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
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, 1999:810–812.
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
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
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 outflow-infundibulum, 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
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
Classic Blalock-Taussig shunt: anastomosis of the right or left subclavian artery to the PA
Modified Blalock-Taussig shunt: artificial tube graft between the right or left subclavian artery
and pulmonary arteries
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 1999:68:1344–1349.
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]123–129.
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 boot-shaped 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
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, 1999:795–796.
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 1999:90:896–905. 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.
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 g/kg
Atropine 20 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, 2002:470–473.
Hensley FA Jr, Martin DE, A practical approach to cardiac anesthesia2nd ed. Philadelphia: Little, Brown and Company,
1995:340–341.
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 selected—
one 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
1995:340–342.
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
1995:341–342.
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
1995:344–345.
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
1995:341–344.
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 25°C to 28°C. Should the repair require deep hypothermic circulatory arrest,
the temperature is decreased to 18°C to 20°C. 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 36°C 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
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
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
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 25°C
to 30°C 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
1995:345–346.
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
1995:347.
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
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, 1999:807–809.
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, 1999:807–808.
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 2000:95:1020–1024.
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
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
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
Low output state
Residual right ventricular outflow tract (RVOT) obstruction
Residual VSD
Coagulopathies
Heart block
Renal failure
Phrenic nerve injury
Stroke
Infection
Late complications
RVOT obstruction
RVOT aneurysm
Residual VSD
Dysrhythmia and sudden death
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 2000:356:975–981.
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 surgery—maintain 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.
Back to Quick Links
Gregory GA, Pediatric anesthesia4th ed. New York: Churchill Livingstone, 2002:489–491, 501.

Case Studies - Tetralogy of Fallot

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Case Studies - Tetralogy of Fallot

Uploaded by

Copyright:

Available Formats

5/17/2020 Case Studies: Tetralogy of Fallot

<BACK TO: CD-ROM Main Contents | Contents of Resource Shown:

CASE 9: Tetralogy of Fallot

Quick Links to Sections in this Chapter

–A. Medical Disease and Differential Diagnosis

–B. Preoperative Evaluation and Preparation

–C. Intraoperative Management

–D. Postoperative Management

Related Links in other Resources

Postoperative management of tetralogy of fallot in Chapter 18 from Cardiac Anesthesia:

Tetralogy of Fallot in Chapter 14 from A Practical Approach to Cardiac Anesthesia

A. Medical Disease and Differential Diagnosis

3. Describe the pathophysiology of TOF.

4. What is a "pink tet"?

5. What are hypercyanotic spells? How are they treated?

6. What is the natural history of the disease?

7. What are the associated anomalies?

8. What are the significant variables in TOF?

B. Preoperative Evaluation and Preparation

3. Discuss preoperative antibiotics.

2. What monitoring will be necessary for a palliative shunt?

3. What monitoring would be necessary in a definitive surgical repair?

4. How would you perform the anesthesia for a shunt procedure?

7. How are hypercyanotic spells managed during anesthesia?

8. Discuss blood replacement therapy.

9. What is monitored during cardiopulmonary bypass (CPB)?

10. Which measurements are made after separation from CPB?

12. How is heparin reversed?

13. What is a protamine reaction?

2. What are the requirements for extubation?

3. Discuss postoperative complications in a shunt procedure and in a definitive

4. How would you manage an uncorrected patient for noncardiac surgery?

A. Medical Disease and Differential Diagnosis

A.1. What is tetralogy of Fallot (TOF)?

Back to Quick Links

Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:810.

Any condition that results in right-to-left shunting will produce cyanosis.

The classic cyanotic congenital heart lesions are the 5 Ts:

Transposition of the great arteries

Total anomalous venous return

Other cardiac lesions that lead to cyanosis are

Hypoplastic left heart syndrome

Double outlet right ventricle

Pulmonic stenosis (PS) or atresia

Back to Quick Links

Miller RD, Anesthesia5th ed. Philadelphia: Churchill Livingstone, 2000:1810–1811.

A.3. Describe the pathophysiology of TOF.

Back to Quick Links

A.4. What is a "pink tet"?

Back to Quick Links

A.5. What are hypercyanotic spells? How are they treated?

Dynamic outflow obstruction

Back to Quick Links

Kaplan JA, Cardiac anesthesia4th ed. Philadelphia: WB Saunders, 1999:810–812.

A.6. Describe natural history of the disease.

Back to Quick Links

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%)