Anaesthesia for correction of congenital

heart disease (for the specialist or

senior trainee) Matrix reference 3D00

James M Peyton MB ChB MRCP FRCA

Michelle C White MB ChB DCH FRCA

Key points Congenital heart disease is common occurring Preoperative considerations

in 1% of live births.1 The aim of surgery is
Preoperative assessment includes: Many children attend a preoperative assessment
assessment of recent infection to achieve a physiologically correct, biventricu-
clinic for both psychological and medical prep-
and need for i.v. hydration lar repair. However, this is not always possible,
(cyanotic children) and aration before surgery. Psychological prepar-
and instead, a palliative approach may be
antifibrinolytics (neonates and ation for parents and children is vital. This
cyanotic children are at increased required resulting in a single-ventricle circula-
includes realistic expectations of the day of
risk of bleeding). tion. Anaesthesia for correction of congenital
surgery and duration of procedure, post-
Before initiation of heart disease requires an understanding of (i)
cardiopulmonary bypass (CPB), operative intensive care, and subsequent re-
the principles of neonatal and paediatric anaes-
heparin 3 mg kg21 is habilitation. Cardiac liaison nurses play a key
administered to ensure an thesia, (ii) the anatomy and physiology of con-
role and help provide continuity in this area.
activated clotting time of at least genital heart disease, (iii) the principles of
400 s or three times the baseline. Routine medical investigations include baseline
cardiopulmonary bypass (CPB), and (iv) the
Before separation from CPB, blood tests (full blood count, coagulation
expected postoperative complications of paedi-
ventilation, heart rate and screen, and renal and liver function),
rhythm, temperature, acid–base, atric cardiac surgery.
methicillin-resistant Staphylococcus aureus
and electrolytes must be Success requires a multidisciplinary team
satisfactory and inotropes and screening, ECG, chest X-ray, and a recent
approach. In addition to the anaesthetist, other
blood products are nearly always echocardiogram. Investigations usually occur
needed in infants. key members are the paediatric cardiac
1–2 weeks before surgery to allow time for
surgeon, paediatric cardiologist, perfusionist,
Haemodynamic instability and reporting and collating of results.
haemodilution (anaemia, intensive care staff, and cardiac liaison nurses.
During the anaesthetic preoperative visit, all
thrombocytopaenia, and Congenital heart disease can be classified in
reduction in clotting factors) are investigations including the recent echocardio-
a number of ways. A simple physiological clas-
complications of CPB in children. gram and any angiography must be reviewed to
sification (based on shunt type and outflow
Arrhythmias, bleeding, low ensure a thorough understanding of the child’s
cardiac output syndrome, tract obstruction) is helpful for anaesthesia as
anatomy, physiology, planned procedure, and
pulmonary hypertension, and shown in Table 1.
systemic inflammatory response potential complications. This usually involves
syndrome are the main discussion with the cardiac surgeon and
immediate postoperative cardiologist.
complications. Aims and limitations Routine history and examination should pay
This review aims to discuss the principles of particular attention to the following areas:
James M Peyton MB ChB MRCP FRCA
preoperative preparation, the management of an-
Specialist Registrar in Anaesthesia
Royal United Hospital
aesthesia and CPB, and the common post- (i) Any history of recent upper respiratory
Bath operative complications of children undergoing tract infection, fever, nocturnal cough,
UK cardiac surgery. Owing to the complexity of moist cough, or chest wheeze as these
Michelle C White MB ChB DCH FRCA congenital heart disease and the variety of surgi- predispose to adverse respiratory events.3
Consultant in Paediatric Anaesthesia and cal procedures, a detailed discussion is beyond Also, ‘acute green runny nose’ or a
Intensive Care the scope of this article. Furthermore, since an- raised C-reactive protein, liver transami-
Department of Paediatric Intensive Care
aesthesia for paediatric cardiac surgery is nases, and/or white cell count may be in-
Bristol Royal Hospital for Children
Marlbrough Street regarded as a ‘super-specialist’ consultant post, dicative of the presence of infection
Bristol BS2 8BJ a detailed knowledge is not required as part of which could be poorly tolerated after the
UK
the curriculum for a Certificate of Completion immunomodulation associated with CPB.
Tel: þ44 117 342 8843
Fax: þ44 117 342 8910 of Training in Anaesthetics.2 Therefore, this (ii) Evidence of cardiac failure suggested by
E-mail: [email protected] review is limited to a brief overview of the poor feeding, failure to gain weight,
(for correspondence)
subject and the discussion of key concepts. sweating, tachypnoea, tachycardia, and
doi:10.1093/bjaceaccp/mkr050 Advance Access publication 21 December, 2011
23 Continuing Education in Anaesthesia, Critical Care & Pain | Volume 12 Number 1 2012
& The Author [2011]. Published by Oxford University Press on behalf of the British Journal of Anaesthesia.
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 Anaesthesia for correction of congenital heart disease

Table 1 A physiological classification of congenital heart disease cryoprecipitate, platelets, and/or fresh frozen plasma should
1. ‘Simple’ left-to-right shunt lesions—these cause an increased pulmonary blood be anticipated.
flow (PBF) (v) Potential sites for peripheral and central venous access.
a. Atrial septal defect Previous surgery or recent cardiac catheterization may make
b. Ventricular septal defect (VSD)
c. Atrioventricular septal defect (AVSD) this difficult.
d. Patent ductus arteriosus (vi) Building a rapport with the child and family, and a frank
e. Aortopulmonary window discussion of perioperative risk.
NB. The effect of the shunt on right ventricular (RV) and respiratory physiology
differs depending on the level at which shunting occurs (vii) Presence of congenital syndromes associated with heart
defects which have implications for perioperative care. The
2. ‘Simple’ right-to-left shunt lesions—these cause a reduction in PBF with cyanosis
a. Tetralogy of Fallot. Consists of RV outflow tract obstruction, RV hypertrophy, range of syndromes is large but common and important ones
VSD, and an overriding of the aorta include: Di George syndrome which requires irradiated
b. Pulmonary atresia
blood products, CHARGE syndrome where choanal atresia
c. Tricuspid atresia
d. Ebstein’s anomaly. Consists of downward displacement of an abnormal may preclude nasal intubation and midface hypoplasia and
tricuspid valve into the RV cavity, part of the RV is thus incorporated into the micrognathia can make airway management increasingly dif-
right atrium (atrialized RV), and the remaining RV cavity is malformed
ficult with age.
3. Complex shunts—these cause mixing of PBF and SBF. Cyanosis occurs as a result
of complex interactions between systemic SVR and PVR Premedication is often unnecessary in infants under a few months
a. Transposition of the great arteries (TGA) of age but is not contraindicated. For other children, sedative pre-
b. Truncus arteriosus
medication is commonly used to avoid distress, minimize oxygen
c. Total anomalous pulmonary venous drainage (TAPVD)
d. Double outlet RV consumption, and may reduce the amount of induction agent so
e. Hypoplastic left heart syndrome minimizing reductions in systemic vascular resistance (SVR).
Most of these lesions (except TAPVD) are examples of a parallel circulation
Benzodiazepines are commonly used, but the use of other agents
4. Obstructive lesions such as triclofos or clonidine is also reported.
a. Coarctation of the aorta
b. Interrupted aortic arch
c. Aortic stenosis Management of anaesthesia
d. Pulmonary stenosis
The usual anaesthetic technique involves a gaseous or i.v. induc-
tion, muscle relaxation, opioid analgesia, and maintenance with a
volatile agent, although other methods are described.
hepatomegaly. These children have poor cardiac reserve and In small infants, venous access may be difficult so a cautious
may be very sensitive to the vasodilatory effects of induction gas induction with sevoflurane is frequently used. Ketamine has no
agents. Many will have large left-to-right shunts and so high effect on SVR, increases mean arterial pressure (MAP), and is well
concentrations of oxygen will increase shunt flow at the tolerated in children with PHT, making it the i.v. agent of
expense of systemic perfusion. choice.4,5 Etomidate also provides haemodynamic stability but
(iii) Pulmonary hypertension (PHT) which is a risk in children may be associated with adrenal suppression. Propofol profoundly
with increased pulmonary blood flow (left-to-right shunt), decreases SVR and MAP, which alters shunt dynamics.4,5 In
obstructed pulmonary venous drainage, or increased left children with a right-to-left shunt, propofol worsens cyanosis by
atrial pressure (LAP). PHT develops earlier in some lesions increasing shunt flow. Therefore, propofol is unsuitable for many
(e.g. atrioventricular septal defects, tricuspid atresia) and in children with heart disease.6 Induction times are prolonged in
certain patient groups (e.g. Trisomy 21). Separation from children with cardiac failure so patience is required to prevent
CPB will require attention to minimizing pulmonary vascu- excessive drug administration.
lar resistance (PVR) and nitric oxide may be required. Any long- or medium-acting neuromuscular blocking agent
(iv) Cyanosis which increases the risk of hyperviscosity before may be used. Pancuronium is commonly used in neonates because
operation and increased bleeding after operation. it produces a tachycardia. This is helpful because the cardiac
Hyperviscosity can cause cerebral vein and sinus thrombosis. output in neonates is rate-dependent, and it also offsets the brady-
Risk factors include: age ,5 yr, dehydration, fever, and iron cardic effect of large doses of opioid. Pancuronium in combination
deficiency anaemia. Preoperative i.v. fluid therapy may be with fentanyl produces very stable cardiovascular conditions.
used to minimize the risk especially in children with a High-dose opioid techniques (fentanyl 25–50 mg kg21) and spinal
haemoglobin concentration of 18 g dl21 or greater. Abnormal anaesthesia have been shown to reduce the stress response in
laboratory tests of haemostasis are documented in 20% of infants undergoing cardiac surgery.7,8 However, recent years have
children with cyanosis, but all children with cyanosis are at seen a reduction in the amount of opioid used, in order to facilitate
increased risk of postoperative bleeding. Therefore, consider- early extubation either immediately in theatre or within a few
ation should be given to the use of antifibrinolytics such as hours of admission to the intensive care unit (ICU). Spinal anaes-
tranexamic acid, and the need for blood products such as thesia is rarely used in UK practice. Either sevoflurane or isoflurane

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 Anaesthesia for correction of congenital heart disease

can be used for maintenance and both have been shown to have no temperature, the circulation can then be either completely arrested
effect on shunt fraction in children undergoing cardiac catheteriza- (known as deep hypothermic circulatory arrest) or selective brain
tion.9 Desflurane and propofol infusions have also been used, but perfusion maintained by a carotid artery cannula while the perfu-
their effects on shunt fraction and cardiac output are unknown.6 sion to the rest of the body is arrested (known as regional low
A nasal rather than oral tracheal tube is common practice in flow perfusion). Perfusion pressure, venous saturations, cerebral
infants and small children. Nasal tracheal tubes make ICU nursing oxygenation, haematocrit, and electrolyte and acid –base status are
care easier and may be more stable. Arterial and central venous continually monitored. Target perfusion pressure varies with age
access is secured and a urinary catheter inserted. Cerebral monitor- and ranges from an MAP of 30 to 50 mm Hg (neonates –young
ing, using near-infrared spectroscopy to give a cerebral tissue oxy- adults). At low temperatures, acid– base management becomes
genation index, is now considered mandatory by many experts.10 more complex and either an a- or pH-stat strategy may be used.
The same technology can also be used to monitor splanchnic/renal Both produce similar results at 378C but differ significantly below
and skin perfusion. 27 –308C. During hypothermic CPB in children, pH-stat manage-
Before skin incision, surgical prophylactic antibiotics are admi- ment reduces seizures, ICU length of stay, and mortality. As tem-
nistered according to local guidelines. Antifibrinolytic therapy (e.g. perature decreases, Hþ and OH2 dissociation constants increase
tranexamic acid) should be considered in patients at high risk of so the Hþ concentration decreases and pH rises. Therefore, since
bleeding (neonates, cyanotic patients, and those undergoing redo electrochemical neutrality must be maintained, at 378C, the cellu-
or complex surgery requiring prolonged CPB). Steroids (dexa- lar pH is 7.4, whereas at 208C, the cellular pH is 7.8. As tem-
methasone or methylprednisolone) may also be used to reduce the perature decreases, cellular pH is mediated by carbon dioxide
inflammatory response to CPB. (CO2). Solubility of CO2 increases as temperature decreases,
causing a decrease in partial pressure (at 378C: PCO2 ¼40 mm Hg,
at 208C: PCO2 ¼16 mm Hg). pH-stat management involves add-
Management of CPB
ition of CO2 to the CPB circuit so that the total content of blood
Good communication between the surgeon, anaesthetist, and perfu- CO2 is increased. Thus, at 208C, the PCO2 is maintained at 40
sionist is required during CPB. In infants, the priming volume of mm Hg rather than being allowed to decrease to expected values
the CPB pump can be more than twice the child’s total blood of 16 mm Hg (i.e. if the total CO2 content was kept the same)
volume. This causes significant haemodilution with anaemia, and the pH is maintained at 7.4 instead of rising to 7.8.
thrombocytopaenia, and a reduction in clotting factors, thereby Therefore, if blood gases are temperature-corrected, the pH would
contributing to the coagulopathy associated with CPB in children. be more acidotic and the CO2 higher than expected at
In infants, blood is usually added to the prime aiming for a haem- normothermia.
atocrit of 21–24%. Once surgery is completed, the child can be separated from
Before the institution of CPB, baseline arterial blood gases and CPB. However, before this can happen, several criteria must be
activated clotting time (ACT) should be checked. Vigilance is met. These include:
required during surgical dissection and aortic and venous cannula-
† Ventilation re-established and both lungs seen to fully inflate.
tion because there may be significant haemodynamic instability
† Heart rate and rhythm acceptable (if not will need pacing).
due to anatomical distortion or arrhythmias. At the request of the
† Vasoactive infusions commenced (most infants require ino-
surgeon, heparin 3 mg kg21 is administered and the ACT mea-
tropic support such as dopamine or low-dose epinephrine
sured. An ACT of 400 s (or at least three times the baseline) is
after CPB and a lusitrope such as milrinone is also frequently
required before institution of CPB and is monitored throughout the
used especially for complex surgery).
duration of CPB.
† Core temperature .368C, and peripheral temperature at least
During CPB, ventilation is stopped but anaesthesia, analgesia,
348C.
and muscle relaxation must be maintained. Isoflurane (or sevoflur-
† Normal acid– base and electrolyte status.
ane) is added to the CPB circuit in a concentration of 0.5–1.0%
† Blood available and blood products if required.
or a propofol infusion administered. Additional opioid and muscle
relaxation is administered either to the child before the com-
mencement of CPB or added to the CPB prime. Further doses of
benzodiazepines may also be used. Depending on the type of
Management after separation from CPB
surgery, surgical technique, and/or surgical preference, the perfu-
sionist will allow the child’s core temperature drift to 34 –358C After separation from CPB, transoesophageal or epicardial echo-
(so-called ‘warm bypass’) or the child is actively cooled to 328C cardiography is performed to evaluate the adequacy of surgical
or lower (minimum of 158C). For certain types of surgery involv- repair. Modified ultrafiltration (MUF) can then begin if required.
ing the ascending aorta and aortic arch, it can be impossible to MUF removes excess body water thereby increasing haematocrit
perfuse the body via the aortic cannula; therefore, the child is and also removes some inflammatory mediators. MUF has been
cooled to 15 –178C and ice packs applied to the head. At this shown to improve cardiac output and decrease PVR. When MUF

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 Anaesthesia for correction of congenital heart disease

is complete, protamine 3 mg kg21 is given to reverse the heparin- components of cardiac output, that is, heart rate and stroke volume.
ization and the ACT checked to ensure that it has returned to base- Slow heart rates are treated with pacing and tachyarrhythmias
line. Blood and clotting factors are administered as required and managed aggressively often by cooling, amiodarone, and overdrive
may be guided by thromboelastography. Once haemostasis is pacing. Stroke volume consists of preload, afterload, and contractil-
achieved and the surgery has finished, the child is transferred to ity; preload is maintained by the judicious use of fluids to maintain
the ICU. During transfer, full invasive monitoring is continued, central venous pressure (CVP) and LAP at predetermined values,
and emergency drugs and fluid should be available. A comprehen- milrinone is commonly used to reduce afterload, and dopamine or
sive handover including the surgical details and echocardiography epinephrine is used to improve contractility. Occasionally, sternal
findings must be given to ICU medical and nursing staff. reopening is required to improve a low cardiac output state, or in
complex neonatal surgery, a delayed sternal closure technique may
Surgery without CPB be used from the outset.
Complications after cardiac surgery can be divided into five
Some types of congenital heart surgery are performed without the general areas:
use of CPB, for example, correction of coarctation of the aorta,
pulmonary artery (PA) banding, and shunt procedures (modified (i) arrhythmias
Blalock–Taussig shunt or central shunt). In general, PA banding is (ii) bleeding
done to limit excess pulmonary blood flow until the child is able (iii) systemic inflammatory response syndrome (SIRS)
to have a definitive procedure. This strategy ‘protects’ the lungs by (iv) PHT
reducing the risk of developing PHT caused by excessive pulmon- (v) low cardiac output syndrome (LCOS)
ary blood flow under high pressure. Shunt procedures are per-
formed to augment pulmonary blood flow in situations where Arrhythmias may be a second- or third-degree heart block which
pulmonary blood flow is otherwise inadequate. requires pacing, or a tachyarrhythmia such as supraventricular
tachycardia or junctional ectopic tachycardia. Management of
tachyarrhythmias involves excluding other causes such as pain and
Postoperative care
seizure activity, correcting hypoxia, acidosis, and electrolyte ab-
Although most postoperative care is undertaken by a specialist normalities, ensuring adequate sedation and paralysis, cooling to
paediatric intensivist, the paediatric cardiac anaesthetist must be fa- 358C, and considering amiodarone and overdrive atrial pacing.
miliar with common postoperative complications requiring imme- Bleeding after cardiac surgery is common and reduced by a me-
diate attention. This allows the anaesthetist to anticipate and treat ticulous surgical technique and the use of antifibrinolytics such as
problems early while still in theatre. It also helps in addressing tranexamic acid. Blood loss into surgical drains is measured regu-
parental anxiety about what to expect perioperatively. larly. Losses .5 ml kg h21 in the first 2 h or over 1 ml kg h21
Maintaining adequate cardiac output is an important aspect of thereafter warrants attention. Treatment involves correcting hypo-
postoperative care. Cardiac output is difficult to measure in children thermia and clotting factor abnormalities (including checking the
so surrogate markers such as MAP, venous saturations, and serum ACT for adequate heparinization reversal with protamine). Any
lactate are often used. Close attention is also paid to the key blood loss .10 ml kg h21 demands immediate surgical review. A

Table 2 Specific complications associated with specific lesions

Lesion Complication Management

AVSD Atrioventricular valve regurgitation Minimize overload or stretch on the repaired valve using afterload reduction
(milrinone, SNP)
Arrhythmias Maintain normothermia, correct electrolyte abnormalities
Pulmonary hypertension Avoid hypoxia, hypercapnia, acidosis

TGA Coronary ischaemia Avoid overdistension of heart, use small (5 ml kg21) fluid boluse’s
LV dysfunction Afterload reduction
Arrhythmias Maintain normothermia, correct electrolyte abnormalities
RV outflow tract obstruction

Tetralogy of Fallot RV dysfunction, associated with RV RV afterload reduction: maintain high CVP, reduce PVR, reduce LAP (improve
failure and LCOS contractility). Milrinone reduces PVR and improves diastolic function
Arrhythmias Maintain normothermia, correct electrolyte abnormalities

Single-ventricle repair, e.g. Fontan Cardiac output is dependent on PBF, Keep CVP high (head-up and elevate legs); LAP low (maximize contractility,
where PBF¼(CVP2LAP)/PVR maintain sinus rhythm, consider milrinone); PVR low (good oxygenation and
analgesia; early spontaneous ventilation; avoid atelectasis)
Arrhythmias Maintain normothermia, correct electrolyte abnormalities
Pleural effusions and liver dysfunction Monitor and treat accordingly

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 Anaesthesia for correction of congenital heart disease

sudden decrease in drain losses may imply impending cardiac tam- form the foundation for adding a more detailed knowledge and
ponade and requires urgent attention. understanding if a senior trainee or consultant wishes to develop
SIRS is common after cardiac surgery, peaks 8–12 h after oper- this area of their practice.
ation, and may be reduced by the use of steroids and MUF. The
fever component of SIRS predisposes to arrhythmias; capillary
leak worsens the tissue and lung oedema contributing to poor gas Declaration of interest
exchange; and reduced cardiac function worsens the already
None declared.
impaired function caused by ischaemia associated with cross-
clamping and administering cardioplegia to the heart on CPB.
PHT causes an increase in right ventricle (RV) afterload,
decreases RV output which decreases left ventricle (LV) preload,
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