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ANESTHESIA IN A CHILD WITH CONGENITAL HEART DISEASE FOR NON CARDIAC SURGERY

Dr. Aruna Parameswari

INTRODUCTION

Congenital heart disease has an incidence of 8 in 1000 live births and this incidence has not changed
much in the last 30 – 40 years. 25% of these children have extra cardiac anomalies like trachea-
esophageal fistula, anorectal anomalies, cleft lip and palate and skeletal anomalies for which they may
require non-cardiac surgery. It has also been shown that the incidence of cardiac arrest and 30 day
mortality is higher in these children for both major and minor procedures. Therefore, it is imperative to
have a thorough knowledge of the specific cardiac anatomy, cardiorespiratory physiology and the
potential risk of complications while anesthetizing them. Children most at risk of perioperative
morbidity are those with major cardiac anomalies, cyanosis, pulmonary hypertension, congestive cardiac
failure, are < 2 years of age and those undergoing emergency surgery.

CLASSIFICATION OF CONGENITAL HEART DISEASE

1. Simple Shunts
Simple left to right shunt lesions Simple right to left shunts
a) Atrial septal defect (ASD) a) Tetrology of Fallot (TOF)
b) Ventricular septal defect (VSD) b) Pulmonary atresia
c) Atrioventricular septal defect c) Tricuspid atresia
d) Patent ductus arteriosus (PDA) d) Ebstein’s anomaly
e) Aortopulmonary window
2. Complex shunts
a) Transposition of the great arteries
b) Truncus arteriosus
c) Total anomalous pulmonary venous drainage
d) Double-outlet right ventricle
e) Hypoplastic left heart syndrome
3. Obstructive lesions
a) Coarctation of the aorta
b) Interrupted aortic arch
c) Aortic stenosis
d) Pulmonary stenosis

PATHOPHYSIOLOGY

Shunt lesions

Shunts are intracardiac connections between the chambers of the heart or extracardiac connections
between a systemic and pulmonary artery. The direction of blood flow through a shunt is dependent on
the size of the shunt orifice, the pressure difference between the chambers and the relative resistances
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on either side of the shunt. With a large, non restrictive VSD or PDA that does not impede blood flowing
freely in each direction, the main determinant of blood flow is the resistance of the pulmonary and
systemic vascular beds. The direction and magnitude of shunt at the atrial level are governed by the
relative differences in ventricular compliance and respective atrioventricular valve function.

The effect that a shunt lesion has on the cardiovascular system, the lungs and the rest of the body
depends on its size and direction. Left to right shunts occur when the pulmonary vascular resistance
(PVR) is lower than the systemic vascular resistance (SVR) and blood flow is directed preferentially
toward the lungs, resulting in increased pulmonary blood flow. The effects of this are threefold: 1)
Pulmonary congestion and predisposition to pulmonary edema with pulmonary vascular occlusive
disease (PVOD) and right heart failure developing with chronic elevation of pulmonary blood flow 2)
volume overload of cardiac chambers leading to congestive heart failure and 3) decreased cardiac
output. Development of PVOD can lead to right ventricular hypertrophy and reversal of shunt (L- R
becomes R – L shunt) that is referred to as Eisenmenger’s syndrome. Once established, this condition is
irreversible and the correction of any underlying cardiac defect is not possible. Increased pulmonary
blood flow also leads to decreased compliance of the lungs, increased work of breathing and the need
for higher ventilatory pressures.

Right to left shunts occur when pulmonary vascular or right ventricular outflow tract resistance exceeds
SVR, thereby reducing PBF. The systemic circulation receives an admixture of deoxygenated blood via
the shunt and this manifests clinically as cyanosis and hypoxemia. Chronic cyanosis is associated with
several changes in the body. It results in a compensatory erythrocytosis, that is an adaptive response to
hypoxia to allow normal systemic oxygen delivery without a sustained increase in cardiac output. With
polycythemia, the blood viscosity increases, which in turn can affect microcirculatory blood flow and
predispose to renal, pulmonary and cerebral thrombosis. Children under 5 years of age are at the
highest risk, especially in the presence of fever, dehydration and iron deficiency. 20% of children with
cyanotic congenital heart disease have abnormal laboratory tests of hemostasis. Prolongation of
prothrombin time, partial thromboplastin time are the commonest abnormalities, though they can have
thrombocytopenia, platelet dysfunction, hypofibrinogenemia and accelerated fibrinolysis. Even when all
coagulation tests are normal, these children have an excessive risk of bleeding. Cyanosis also results in
growth retardation as hypoxemic children expend more metabolic activity on non-growth functions.
Hypoxemia induced myocardial dysfunction can also occur leading to reduced maximal exercise
capacity.

Complex shunts or mixing lesions:

In these defects, the mixing between the pulmonary and the systemic circulation is so large that the
systemic and pulmonary artery oxygen saturations approach each other. The pulmonary to systemic
flow ratio (Qp/Qs ratio) is independent of shunt size and totally dependent on vascular resistance or
outflow obstruction. With no obstruction, flow to the systemic or pulmonary circulation is dependent on
the relative vascular resistances of both circuits, such as with univentricular hearts or double-outlet right
ventricle.
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Obstructive lesions

These include left and right ventricular outflow tract obstructions which produce a pressure overload on
the corresponding ventricle. The pathophysiologic problems in critical neonatal left-sided heart
obstructive lesions include a) profound left ventricular failure b) impaired coronary perfusion with an
increased incidence of ventricular ectopy c) systemic hypotension d) PDA-dependent systemic
circulation and e) systemic hypoxemia. The pathophysiologic problems in critical neonatal right-sided
heart obstructive lesions include a) right ventricular dysfunction b) decreased PBF c) systemic
hypoxemia and d) PDA – dependent PBF.

Children with congenital heart disease can be scheduled for non cardiac surgery with their cardiac lesion
corrected, uncorrected or palliated. Since the physiology varies depending on the stage at which they
are scheduled, it is important to formulate a plan individualized to the child and tailored to the type of
surgery.

The four major complications of congenital heart disease in children include arrhythmias, cardiac failure,
cyanosis and pulmonary hypertension. Arrhythmias are more common in children who have had
previous cardiac surgery. Children at high risk are those with extensive atrial sutures or those who have
undergone ventriculotomy. Ventricular ectopics are an ominous sign as about 30% of these children die
suddenly. Cardiac failure can be due to a volume overloaded, pressure overloaded heart or both.
Children with cardiac failure are at very high risk, have a higher incidence of perioperative cardiac arrest
and a higher requirement for perioperative inotropic support. Cyanosis is commonly seen in unrepaired
or palliated congenital heart disease. Pulmonary hypertension is defined as a mean PA pressure >
25mmHg at rest or 30 mmHg during exercise. These children are eight times more likely to develop a
major complication.

The indices of critical impairment in congenital heart disease include

 Chronic hypoxemia (arterial saturation < 75%)

ANESTHETIC MANAGEMENT

Preoperative evaluation and preparation

The anesthesiologist should be thoroughly familiar with the child’s cardiac anatomy and
pathophysiology. Valuable information can be obtained by discussion with the child’s cardiologist.
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A history of either exercise intolerance in the older child or feeding intolerance in the infant are reliable
symptoms of cardiopulmonary decompensation. The presence of even mild upper respiratory infection
is a contraindication to elective surgery, as it can cause changes in airway reactivity and PVR which
might be poorly tolerated by these children. History of cyanotic spells and triggering events, episodes of
unconsciousness or convulsions should be specifically asked for. Any additional systemic disease and
non-cardiac anomalies should be thoroughly evaluated. Airway compression by enlarged cardiovascular
structures or vascular rings, tracheal stenosis due to previous prolonged ventilation after cardiac surgery
and difficult airway in syndromic children are some of the airway issues we could encounter.

A laboratory evaluation should include Hemoglobin (raised in cyanotics), coagulation profile (cyanotics),
electrolytes and blood gases. A room air oxygen saturation should be recorded. Chest X ray, ECG, ECHO
and cardiac catheterization would provide complete details about the anatomy and physiology of the
lesion.

Preoperative fasting guidelines

The avoidance of preoperative dehydration is important in children with cyanotic congenital heart
disease (especially when Hb > 18g/dl). Fasting guidelines will be dictated by the age of the child and the
nature of the surgery.

Premedication

The potential advantages of preoperative sedation in children with congenital heart disease include easy
separation from parents, less crying, decreased oxygen consumption and decreased levels of
intraoperative anesthetic requirements. The disadvantage of sedative premedication is respiratory
depression with desaturation in children with cyanotic congenital heart disease, whose resting
oxyhemoglobin saturation may lie on the steep portion of the curve. Premedication should hence be
administered in a controlled environment with monitoring of vitals. Most centers use midazolam orally
or nasally. Oral and intranasal ketamine can also be used in specific situations. Cardiac premedication
like antiarrhythmics, beta blockers and diuretics should be continued preoperatively.

Infective endocarditis prophylaxis

As per the recent guidelines published by the American Heart association (2007), the following are the
cardiac lesions where prophylaxis is highly recommended: Unrepaired cyanotic CHD, including palliative
shunts and conduits, completely repaired congenital heart defect with prosthetic material or device,
whether placed by surgery or by catheter intervention, during the first 6 months after the procedure
and repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or
prosthetic device (which inhibit endothelialization).

Induction of anesthesia

Inhalational induction is generally well tolerated by children with minor cardiac defects. Sevoflurane
provides better cardiovascular stability compared to halothane and has less myocardial depressant and
arrhythmogenic properties. Intravenous induction is usually the preferred method in patients with
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severe cardiovascular limitation or when an intravenous cannula has already been inserted. Children
with well compensated congenital heart disease tolerate intravenous induction with thiopental or
propofol in conjunction with an opioid well, provided the children are euvolemic. Etomidate may have
less hemodynamic side effects and can be used for children with limited reserve. Ketamine as sole
induction agent may be advantageous when preservation of heart rate, blood pressure and ejection
fraction seems to be important, that is, in cyanosed children or those with congestive cardiac failure.

In children with CHD with decreased pulmonary blood flow, the transfer of the inhalational agent from
the lungs to the arterial blood is slow. Inhalational induction in a child with a right to left shunt can be
prolonged. In children with left to right shunt, the speed of inhalational induction is unchanged,
provided systemic perfusion is preserved.

In children with left to right shunting, an intravenous bolus of a given drug reaches the brain at the same
time as if no shunt existed. Its initial peak concentration is lower and its effect is prolonged. In lesions
with right to left shunting, in which systemic venous blood bypasses the pulmonary circulation, the
bolus reaches the brain sooner than predicted. Both anesthetic effect and cardiovascular toxicity may
therefore appear more rapidly after IV administration of intravenous agents in these patients.

Circulatory time may be prolonged in patients with cardiac failure, and adequate time must be allowed
for intravenous drugs to achieve full effect. Care should be taken to avoid injecting air bubbles.

Maintenance

This can be done with inhalational agents, additional opioids, muscle relaxants and regional blocks. The
anesthetic goals and concerns have to be framed individually for the child, taking into account the type
of lesion and the surgery. In children with left to right shunt, we have to avoid any fall in PVR or increase
in SVR that would increase the shunt flow and precipitate congestive heart failure and pulmonary
edema. In children with TOF, any fall in SVR or anything which precipitates infundibular spasm should be
avoided. In right to left shunts, any rise in PVR or fall in SVR increases the R – L shunt and worsens
hypoxemia and should be avoided. Nitrous oxide is avoided due to the risk of air embolism.

Factors that decrease PVR include high FIO2, hypocapnia, respiratory alkalosis, factors that increase SVR
include light planes of anesthesia, vasoconstrictors, factors that increase PVR include hypoxia,
hypercapnia, acidosis, high airway pressures, PEEP, inadequate anesthesia, hypothermia and factors
that decrease SVR include hypotension and hypovolemia

Monitoring

Standard monitoring includes ECG, noninvasive blood pressure, pulse oximetry and capnography.
Measured end-tidal carbon dioxide tension underestimates mean arterial carbon di oxide tension in
cyanotic patients with CHD. Pulse oximeters reliably predict saturation between 70% and 90%. However,
below 70%, they have limited accuracy. It is intuitive that acute desaturations <70%, regardless of the
accuracy of the pulse oximeter should be taken to indicate deleterious hypoxemia and corrective
measures should be promptly instituted. Invasive monitoring would be required for children with more
complex lesions and also for major surgery. Regarding central venous catheters, it is important to
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remember that children with Ebstein’s anomaly have some prolapsed right ventricular tissue in the right
atrium, which can be stimulated by the CVP catheter causing ventricular arrhythmias. It is better to
avoid central lines in children who have had a Fontan procedure. Temperature monitoring is indicated in
prolonged surgery.

POSTOPERATIVE MANAGEMENT

Pain should be adequately treated with analgesics as it can increase SVR and PVR and worsen the
infundibular spasm of TOF. As these children do not tolerate even mild decrease in O2 saturation, it is
important to avoid respiratory depression and the resultant hypoxia and hypercarbia.

CONCLUSION

As congenital heart diseases represent a wide spectrum of different lesions, it is important for the
anesthesiologist to be familiar with the anatomy and pathophysiology, based on which an individualized
anesthetic plan can be formulated for the patient. The specific type of defect, the presence of
complications (cardiac failure, arrhythmias, pulmonary hypertension, cyanosis) and the type of surgery
dictate the anesthetic approach.

RECOMMENDED READING

1. White MC. Anaesthetic implications of congenital heart disease for children undergoing non-
cardiac surgery. Anaesth Intens Care Med 2009; 10: 504-509.
2. Sumplemann R, Osthaus WA. The pediatric cardiac patient presenting for non cardiac surgery.
Curr Opin Anesthesiol 2007; 20: 216-220.
3. Bailey PD, Jobes DR. The Fontan patient. Anesthesiology Clinics 2009; 27: 285-300.
4. Litman RS. Anesthetic considerations for children with congenital heart disease undergoing
noncardiac surgery: Answers to common questions. Anesthesiology Clinics 1997; 15:94-104.
5. Lake C and Booker PD. Pediatric Cardiac Anesthesia. 4th edition. Philadelphia: Lippincott,
Williams and Wilkins; 2005.
6. Nayak S, Booker PD. The Fontan circulation. CEACCP 2008;8:26-30.
7. White MC and Peyton JM. Anaesthetic management of children with congenital heart disease
for non-cardiac surgery. CEACCP 2012;12:17-22.