The document summarizes the history of the arterial switch operation for treating discordant ventriculoarterial connections. It describes how early pioneers in the 1950s and 1960s attempted the operation but faced failures, leading to its abandonment. The first successful arterial switch was performed in 1975 by Jatene. Through the 1980s, surgical technique and timing indications were perfected, establishing the arterial switch as the standard treatment. Currently, arterial switch is performed neonatally, with hospital mortality under 10% depending on anatomy. However, long-term follow up continues to find residual issues, showing the operation is not a complete cure.

1. Cardiology in the Young (2012), 22, 724–731
doi:10.1017/S1047951112001746
r Cambridge University Press, 2012
Original Article
Past, present, and future of the arterial switch operation:
historical review
Ali Dodge-Khatami,1
Constantine Mavroudis,2
Constantine D. Mavroudis,3
Jeffrey P. Jacobs4
1
Division of Pediatric and Congenital Cardiac Surgery, University Heart Center, University of Hamburg, Hamburg,
Germany; 2
Johns Hopkins Children’s Heart Surgery, Florida Hospital for Children, Orlando, Florida; 3
Division of
Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia,
Pennsylvania; 4
Johns Hopkins Children’s Heart Surgery, All Children’s Hospital and Florida Hospital for Children,
Saint Petersburg, Tampa, and Orlando, Florida, United States of America
Abstract The arterial switch operation is the extant surgical correction after a long series of palliations attempted
and/or successfully achieved for the treatment of discordant ventriculoarterial connections. As early as 1954,
pioneers such as Mustard, Bailey, Kay, and Idriss led the way with at first disheartening failures, temporarily
leading to abandoning the procedure. The first successful atrial baffle procedure in 1958 established itself as the
procedure of choice for treating discordant ventriculoarterial connections, but tenacity, courage, and vision to
pursue anatomic correction finally led to the first successful arterial switch in 1975 by Jatene. After a decade to
perfect surgical technique and timing indications for the various anatomic subtypes, the new era of the neonatal
arterial switch since the late 1980s set the very high standards that we all know and expect today. Despite
excellent early and long-term survival, important residual lesions are increasingly being recognised. Expected
anatomic residuals include supravalvar pulmonary stenosis, neoaortic valve insufficiency, and coronary ostial
stenosis. Reinterventions and rare, but challenging surgical reoperations address these residual findings with
satisfactory outcomes. Quality of life into young adulthood is satisfactory, but functional problems include
reduced exercise capacity, diffuse coronary insufficiency, and neurodevelopmental shortcomings, of which the true
incidence and potential clinical implications are still unknown. The arterial switch is a spectacular anatomic
correction for a once lethal condition and currently the best surgical solution for patients with discordant
ventriculoarterial connections. It is, however, far from a true cure; closer and ongoing follow-up for future care will
continue to be required.
I
N INTRODUCTION TO THIS REVIEW OF THE DEVELOPMENT
and results of the arterial switch operation for
discordant ventriculoarterial connections, it seems
necessary to attempt a definition, in surgical historical
terms, of the past, present, and future. Although the
future may simply be defined as everything that will
happen after tomorrow, defining the past is more
subtle, and its border with the present is a vague and
wider time zone shaped by human memory. Few
complex congenital heart lesions have seen such a
spectacular change in outcome occur as for discordant
ventriculoarterial connections, owing to the develop-
ment of bold and ingenious surgical techniques,
rendering what was universally a lethal condition into
one with survivors, after surgical correction with the
arterial switch operation, currently expected to lead
near-normal lives. The anatomy and even physiology
of discordant ventriculoarterial connections were
brilliantly described by Baillie and Clift1
in 1799.
Since that time, the road to the current status required
brave attempts and temporary success at palliation,
then unsuccessful trials of anatomic correction and
abandoning the arterial switch, physiologic correction
with the atrial (venous) switch, culminating in what
today is regarded as the anatomic and physiologic
Correspondence to: Dr A. Dodge-Khatami, MD, PhD, Division of Pediatric and
Congenital Cardiac Surgery, University of Hamburg, Martinistrasse 52, 20246
Hamburg, Germany. Tel: 149 7410 58221; Fax: 149 7410 58275; E-mail:
adodgekhatami@googlemail.com

2. correction of the defect, the arterial switch operation.
Although fascinating subjects in themselves, the
various attempts and successes of palliation such as
the Blalock–Hanlon operation or the Senning and
Mustard atrial baffle procedures will not be examined
in this review. We focus solely on the arterial switch
operation, and for the sake of simplicity, the past
includes the earliest unsuccessful attempts at the
arterial switch dating back to 1954 by William
Mustard2
until 1975. The present could be sum-
marised as the period between the first successful
arterial switch operation, performed in 1975 by Jatene
et al,3
until now. Finally, the future is open-ended, and
we shall cover the important residual lesions facing our
patients and the new challenges facing the medical
community treating them.
The past: Mustard, Bailey, Kay, and
Idriss – the early pioneers
During the early 1950s, mechanical cardiopulmo-
nary bypass was still in its developing stages, cross-
circulation championed by C. Walton Lillehei was
making its debut, and William T. Mustard was using
explanted monkey lungs in his cardiopulmonary
bypass circuit.4,5
Using biological oxygenators in
animal experiments since 1951, Mustard was embol-
dened to attempt new surgical techniques in humans,
stating that ‘‘ysurgery in the human being could
be undertaken in hopeless casesyTransposition of the
great vessels was the condition selected’’.2
In 1954, he
reported on seven patients with discordant ventricu-
loarterial connections operated at Toronto’s Hospital
for Sick Children, of whom three underwent the
arterial switch operation. A 6-week-old baby died of
aortic suture line bleeding, a 19-day-old baby died of
left coronary artery occlusion, and another 19-day-old
baby died from ventricular fibrillation. Mustard
transferred the left coronary artery only and felt that
leaving the right coronary artery arising from the
pulmonary artery would be compatible with long-
evity: all three died in the operating room.2
He noted
two important anatomic features, which would play
a key role in future successes of the operation: the
size discrepancy between the great vessels and the
variation in coronary artery patterns.2
The same year, Charles Bailey used hypothermia to
perform the ‘‘switchover’’ operation at Hahnemann
Hospital in Philadelphia.6
In this procedure, per-
formed in three patients with simple transposition, the
coronary arteries were not transferred, and no patient
survived.6
A year later in 1955, Earle Kay attempted
a similar procedure in two patients – a 4-week-old
baby and a 3-month-old baby – at St. Luke’s Hospital
in Cleveland,7
whereby both great vessels were
transected, and after disconnecting both pulmonary
arteries the pulmonary trunk was sutured to the
underside of the aortic arch. The transected pulmonary
arteries were then reconnected on both sides of the
original ascending aorta, directly on the right, and
with an interposition graft for the left pulmonary
artery. The coronaries were not transferred, and both
patients died.7
Following experimental surgery in 19 dogs under-
going the ‘‘switchover’’ procedure with cardiopul-
monary bypass, Farouk Idriss attempted the arterial
switch in 1961 at Children’s Memorial Hospital in
Chicago in two patients, one 7 years old and another
3 months old.8
On pre-operative cardiac catheterisa-
tions, the left ventricular pressure was only one-third
of the systemic right ventricular pressure in the first
patient and was nearly equal in the second. His
procedure involved transecting an intact cuff of the
aorta with both coronary arteries still attached,
flipping it upside down and transposing it to the
left ventricular outflow tract, incorporating it as a
slice-in between the left ventricle and the aorta.8
Both
patients died in the operating room, from left
ventricular failure in the older patient and from
excessive bleeding in the second patient. In his
discussion, Idriss comments on the importance of
patient selection, noting that the ‘‘first patient died
because the left ventricle was unable to handle the
sudden load imposed on ity[and]yin spite of two
clinical failures, we think this operation is worthy of
further trials’’.8
These early attempts required tremendous courage
and ingenuity, but were met with failure and 100%
mortality owing to many factors, for example imper-
fect bypass technology and gross suture material,
unrelated to the correct and logical concept of the
arterial switch. It led to abandoning the arterial switch
contemporary to the first successful atrial or venous
switch operation performed by A˚ ke Senning in
1958.9,10
Although the venous switch provided only
physiologic repair, leaving the right ventricle in the
systemic circulation, it was safe, reproducible, and
rapidly became the standard operation for discordant
ventriculoarterial connections with excellent short-
to mid-term results. In this setting, pursuing or
justifying a different approach required tenacity, more
courage, vision to predict systemic right ventricular
failure after the venous switch, and the need to
surmount the ethical considerations pertinent to
abandoning an existing successful surgical strategy.11
After further experimental work in calves and dogs by
Anagnostopoulos in Chicago,12
it would take another
decade and a half for Adib Jatene to perform the first
successful arterial switch operation in 1975 in Sao
Paolo, Brazil.2
However, after his initial spectacular
breakthrough, he reported five deaths in the adden-
dum to his 1976 paper,13
making many surgeons
Vol. 22, No. 6 Dodge-Khatami et al: Past, present, and future of the arterial switch operation 725

3. hesitant to undertake such a daunting procedure
instead of the safer Senning operation. Some continued
to perform the switch without coronary transfer, with
surprising initial success in case reports.14
It would
eventually take more than another decade for the
arterial switch to become the gold standard operation
for the treatment of discordant ventriculoarterial
connections. Where to draw the line between the
past and the present is probably debatable: does the
present era of the arterial switch begin in 1975 after
a first success or in the late 1980s when it was more
widely performed? In any event, the surgical history of
the arterial switch operation is a fascinating illustra-
tion of how a surgical operation established itself
based on logic, trial and error, and a visionary shift in
long-term expectations.
The present
Currently, for discordant ventriculoarterial connec-
tions with intact ventricular septum, the arterial
switch is electively undertaken between the first
day and the first 2 weeks of life. For discordant
ventriculoarterial connections with a ventricular
septal defect, the repair is usually recommended by
2 to 3 months. The majority of initial successful
arterial switch procedures were undertaken in older
patients with a ventricular septal defect or a large
patent arterial duct, whereby the left ventricle was
pre-operatively still submitted to systemic pressures
and was therefore able to handle its systemic role
after an arterial switch. One of the initial successful
series was reported by Pacifico et al.15
However, the
majority of babies with discordant ventriculoarterial
connections do not have a ventricular septal defect,
and very early on in the experience it was recognised
that an unprepared left ventricle in patients with an
intact ventricular septum would fail. The concept
of two-stage anatomic correction was proposed by
Yacoub et al16
in 1977 and included pulmonary
arterial banding followed by debanding and an
arterial switch. This initial approach involved a
period of banding of a year or more, further
prolonging the duration of cyanosis, and imposing
surgical morbidity such as time-related left ventri-
cular injury, branch pulmonary arterial distortion,
and insufficiency of the neoaortic valve.17,18
The
concept was later refined in an accelerated rapid, two-
stage strategy by Jonas in 1989, by banding with or
without an aortopulmonary arterial shunt, proving the
feasibility of retraining the left ventricle by doubling
its mass through hypertrophy after a week only.17,18
The current guidelines indicating the adequacy of
left ventricular training after pulmonary arterial
banding include an inferior limit of left ventricular
mass at 35g/m2
,19
a left-to-right ventricular pressure
ratio .0.85, left ventricular end-diastolic volume
.90% of normal, or posterior wall thickness .4mm,
allowing for a safe arterial switch after debanding.20
In
developing countries and/or in situations with late
diagnosis and referral, the time limits to perform an
arterial switch with or without prior left ventricular
training by banding are being redefined and vary
depending on the presence or absence of a ventricular
septal defect,21–24
details of which are beyond the
scope of this review. It is worth noting, however, that
the age boundaries to directly perform an arterial
switch without prior banding in babies with an intact
ventricular septum have been pushed to 6 months.21
This strategy needs to take into consideration the
potential need for post-operative mechanical circula-
tory support during the recovery period.21
In patients
with a ventricular septal defect with or without
Taussig–Bing morphology, patients of up to 19 years
of age have effectively been treated by a direct arterial
switch operation and closure of all defects, including a
post-operative protocol of inhaled nitric oxide and oral
sildenafil or bosentan.23
Concerns regarding ongoing attrition of untreated
patients with discordant ventriculoarterial connec-
tions and intact ventricular septum and/or the added
surgical morbidity of the two-stage approach moti-
vated Paul Ebert and the San Francisco group to
embark on a brave approach to performing the
neonatal arterial switch operation, amongst other
neonatal repairs.25
Early on, two of the first six babies
died, one required reoperation for pulmonary artery
stenosis, and another had neoaortic valve insufficiency,
leading him to discontinue the programme tempo-
rarily. The LeCompte manoeuvre was introduced in
1982, making a case for reconstructing the right
outflow tract without a prosthetic conduit.26
In 1984,
using the LeCompte manoeuvre, Castaneda et al
reported the first encouraging results in 14 neonates
with transposition and intact septum with only one
death, making a strong case for an early primary
arterial switch while the left ventricle is still capable
of effectively sustaining the systemic circulation.27
From this point in the mid-1980s, the stage of the
present era was finally set: the indications and timing
relating to anatomic subtypes were better defined, the
surgical technique was refined, the coronary artery
anatomic variations were better understood, cardio-
pulmonary bypass was up to par, and neonatal
intensive care improved. Various international groups
could define the standard of care and current
expectations for a lesion, which only three decades
prior had eluded all hope of treatment. In the present
era, which has continued for 25 years, operative
mortality for each anatomic subtype – simple
transposition, complex transposition including a
ventricular septal defect, arch obstruction, coarctation
726 Cardiology in the Young December 2012

4. of the aorta, Taussig–Bing anomaly, and intramural
coronaries – is clearly defined,28–40
and surgical
performance can be measured. Hospital mortality for
simple transposition is up to 5% and for complex
transposition is 5–10%.28–40
With these figures as a
benchmark, it is easy to see that the vast majority of
babies with discordant ventriculoarterial connections
are expected to survive the arterial switch operation
with a heart that is cured. Or is it really? In 1990,
Kirklin et al41
and others27–29
were able to foresee the
possibility of morbidity or residual lesions to be
found after the arterial switch operation, making it
perhaps the best surgical solution currently available
for patients with discordant ventriculoarterial con-
nections but certainly not a cure.
An unusual but increasingly encountered indica-
tion for the arterial switch operation is for patients
with discordant ventriculoarterial connections and
a failing systemic right ventricle after a Senning
or Mustard-type atrial switch.42–45
Indeed, the two
most common and feared mid- to long-term
complications after the atrial baffle procedure are
intractable atrial arrhythmia and systemic right
ventricular failure,42–45
estimated at ,10% after
the first decade and expected to increase with
time.43,45
In this setting, as with unoperated patients
with discordant ventriculoarterial connections referred
late for an arterial switch, a chronically underloaded
left ventricle cannot be expected to suddenly handle a
systemic workload if (re)-switched back into the
systemic circulation. The concept of left ventricular
retraining by pulmonary arterial banding before an
atrial baffle takedown, debanding, and arterial switch
operation was introduced by Roger B Mee in 1986.44
To reach this goal, one or multiple pulmonary arterial
banding operations and a period of 1–5 years is
thought to be required to achieve adequate left
ventricular retraining.42,44,45
Echocardiographic and
cardiac catheterisation criteria to suggest adequate left
ventricular function before an arterial switch include a
left ventricular posterior wall thicker than 8 mm
during diastole and a left-to-right ventricular systolic
pressure ratio .80%.42
At the time of debanding and
arterial switch, concomitant antiarrhythmia surgery
and dealing with neoaortic valve insufficiency should
be applied as appropriate, despite an already extensive
surgical procedure.42
The staged strategy has met
with success in a very select group of younger
children with good long-term outcomes, and its
indication may be broadening. However, complex
coronary anatomy and older age beyond 12–16 years
are reported risk factors for mortality or failure to
complete the full protocol.42,45
In this case, the only
alternatives are to leave the banding as destination
therapy or proceed to cardiac transplantation.42,45
It is
therefore crucial but very difficult to distinguish
between adolescents with discordant ventriculoarterial
connections who will continue to do well long after a
Senning or Mustard palliation, and others to commit
early enough to a left ventricular retraining protocol,
in view of debanding and a successful arterial switch
operation.42,45
Predictable residuals and potential
problems for the future
There exist predictable but treatable surgical problems
pertaining to the pulmonary arteries, the neoaortic
valve, and the coronary arteries, as well as more obscure
residua involving the myocardium, coronary reserve,
exercise capacity, and neurodevelopmental issues faced
by patients long after the arterial switch operation,
with future true implications still unknown.
Pulmonary artery/right ventricular
outflow tract stenosis
Right ventricular outflow tract obstruction, more
specifically supravalvar pulmonary stenosis, is the most
common mid- to long-term complication after the
arterial switch operation, occurring in ,2–17%32–35
of cases. Despite recent advances in reconstruction
technique and the more liberal use of fresh autologous
pericardium to reconstruct the pulmonary arteries,
it still represents about half of all indications for
reintervention/reoperation after the arterial switch,
independently of the material used for reconstruc-
tion.26
Discordant ventriculoarterial connections with a
ventricular septal defect,32,34
prior neopulmonary
artery reconstruction with a Gore-Tex patch34
or an
equine pericardial patch35
have been charged with
increasing the risk for a reintervention on the right
ventricular outflow tract. Fortunately, supravalvar
pulmonary stenosis is easy to treat and can in the vast
majority of cases be performed in the interventional
catheterisation laboratory by percutaneous balloon
dilatation.32–35
As it is almost always amenable to
curative dilatation or surgical correction in the rare
cases requiring a reoperation, it is not expected to
represent a major concern for ongoing or progressive
morbidity in the long-term for these patients.
Neoaortic valve
After the arterial switch, the structurally formed
pulmonary valve becomes the neoaortic valve. Very
early on in the experience, concerns were raised as
to the longevity of the neoaortic valve in its new
role.27,28
Although severe obstruction of the left
ventricular outflow tract or organic narrowing of the
pulmonary valve are still considered contraindications
to perform the arterial switch operation,46
there are no
obvious criteria to proceed with or discard the arterial
switch with borderline anatomy of the future neoaortic
Vol. 22, No. 6 Dodge-Khatami et al: Past, present, and future of the arterial switch operation 727

5. valve, including in patients with a bicuspid pulmo-
nary valve, present in 1–7% of patients with
transposition.47–50
However, there seems to be
increasing evidence that a bicuspid pulmonary
valve is not a contraindication to perform an
arterial switch, as demonstrated by mid- to long-
term follow-up data revealing a low prevalence of
neoaortic valve insufficiency or stenosis, comparable
with that after switching a normal trileaflet pulmo-
nary valve.47–50
So how good is the durability of the
neoaortic valve in general? Answering this question
may be relative in terms of documenting any degree of
valve stenosis/regurgitation or significant neoaortic
valve dysfunction requiring a reoperation.32–35,40,49–62
In mid-term follow-up studies, the incidence of
mild aortic regurgitation occurs in 13–38% of
patients, moderate aortic regurgitation is reported in
0.7–15%, and severe aortic regurgitation in only
0.4–1.5%32,33,52,61
of patients. Freedom from at least
moderate aortic insufficiency is 100%, 96–100%,
94–99%, 91–99%, and 85–98% at 1, 5, 10, 15, and
20 years, respectively.32,54,61
Accordingly, the reopera-
tion rate for aortic valve repair or replacement is very
low, reported at 0.3–5%.32,33–35,40,51,54,55,60,61
Risk
factors predicting neoaortic regurgitation include prior
pulmonary artery banding,34
the trap-door technique
for coronary reimplantation,53
complex coronary
anatomy,34
an augmented sinotubular junction/aortic
root dilatation,56,57,62
size discrepancy of the great
arteries,57
complex form of discordant ventriculoarter-
ial connections – associated with ventricular septal
defect, Taussig–Bing, or arch obstruction,34,35,62
age
greater than 6 months at the time of arterial switch
surgery,62
and left ventricular outflow tract obstruc-
tion.61
It is reassuring that despite a relative high
incidence of neoaortic valve insufficiency, it is often
mild, progresses but not severely, and only rarely leads
to aortic valve reoperation.
Coronary arteries
Coronary stenosis after the arterial switch may be a
life-threatening lesion, and its true incidence is
unknown.63,64
Most patients are asymptomatic, as
they feel no chest pain owing to the denervation of the
heart during the arterial switch operation.64
Standard
electrocardiograms or echocardiography usually show
no sign of myocardial ischaemia.51,64–69
Furthermore,
progressive stenosis of one ostium may allow extensive
collateralisation from the other coronary artery, thereby
hindering ischaemia or delaying it in an unpredict-
able manner.68
Almost all authors agree that diagnostic
imaging is immediately necessary in a symptomatic
patient, when there is any doubt as to whether an
electrocardiogram exists, or after difficult coronary
transfer. However, in a completely asymptomatic
patient with prior standard coronary anatomy and an
uneventful post-operative course, no consensus
exists as to when and how often screening should be
performed with elective coronary catheterisation,
multi-slice computed tomography, or magnetic reso-
nance imaging angiography. Independent of the
coronary anatomy, some centres recommend system-
atic coronary angiography for all patients in the
first 2–3 years after repair,65
before the age of 4–5
years,54,70,71
or at 5, 10, and 15 years of age.64,66
Myocardial perfusion scanning has been recommended
in all asymptomatic patients at 3 years.71
The reported incidence of late coronary stenosis
or occlusion ranges from 2% to 9%.51,54,57,63–71
Mechanisms leading to stenosis include ostial fibrosis
at the suture line, mechanical kinking or stretching,
or reactive injury to surgical manipulation.68
Risk
factors for coronary events or the need for reoperation
include single coronary artery57
and complex coronary
anatomy.34,65
Operative techniques include coronary
ostial patchplasty with autologous or biological
patch material, and/or coronary artery bypass graft-
ing.34,65,70
Interventional balloon angioplasty and
stenting have also been performed with success.64,72
As the oldest survivors of the arterial switch are
reaching adulthood, an age where natural athero-
sclerotic disease may affect the coronaries, it remains
unclear whether or not the superimposed surgical
trauma in the newborn period accrues risk in the long
term.68
It would seem wise to recommend lifetime
follow-up at regular intervals with precise coronary
imaging, in order not to miss silent coronary lesions
with life-threatening consequences. However, clear
guidelines have not been clarified.
Myocardial function/exercise capacity
Even in the presence of documented patent coronary
arteries after the arterial switch, it is important to
remember that the myocardium may have suffered
during the initial surgical procedure or in the
immediate post-operative period after a difficult
recovery. It is possible that various baseline coronary
patterns may increase risk in the long term, despite
adequate surgical transfer, leading to progressive
coronary insufficiency. Abnormal myocardial perfusion
scans at rest and at peak exercise have been demon-
strated in otherwise healthy and asymptomatic
patients long after the arterial switch,73
associated
with diminished exercise capacity59,74
and mildly
diminished peak heart rates, oxygen pulse and aerobic
capacity.74
Sterrett et al74
found this to be related to
variant coronary artery patterns, in contrast to normal
exercise perfusion scans documented in patients with
normal coronary patterns. In the absence of a
surgically correctable coronary ostial stenosis, this
728 Cardiology in the Young December 2012

6. type of coronary insufficiency during stress is still
important to diagnose in the early teenage years,74
as
patients may benefit from counselling to limit
strenuous competitive sports. Confirming these find-
ings is a study by Pasquali et al, using exercise stress
testing in 53 patients at a median of 14.1 years (range,
7.7–20.6 years) after an arterial switch operation.69
They found variant coronary patterns – circumflex
from right coronary artery, inverted right coronary
artery and circumflex, single and/or intramural
coronary – and a ventricular septal defect to be asso-
ciated with chronotropic impairment of the heart and
a trend towards lower percent of predicted peak
oxygen consumption.69
In another study excluding
the potential negative bias of overprotection by
parents and child deconditioning from reduced
daily-life activity, van Beek et al75
also found signi-
ficantly reduced peak exercise capacity, diminished
maximal workload, and slower maximal heart rates in
a group of young teenagers after the arterial switch,
compared with healthy controls. Using echocardio-
graphy, Hui et al76
studied 31 asymptomatic children
,10 years of age after the arterial switch with patent
coronary artery ostia and found lower fractional
shortening, ejection fraction, and rate-corrected velo-
city of circumferential fibre shortening. Alarmingly,
dobutamine stress testing further unmasked wall
motion abnormalities in 75% of patients, correspond-
ing to reversible myocardial perfusion defects as
detected by exercise perfusion scanning.76
In the
absence of well-defined coronary artery territorial
involvement, the authors suggest global impairment
of left ventricular function from diffuse coronary
insufficiency, which was associated with older age at
operation, longer cardiopulmonary bypass and circu-
latory arrest times, and unusual coronary patterns.76
The clinical relevance and eventual therapeutic con-
sequences of these findings remain unclear, although
all authors mentioned previously recommend further
follow-up of left ventricular function with regular
myocardial perfusion scanning.59,69–76
Quality of life/neurologic/developmental outcomes
Reports on the perceived quality of life have
shown different results depending on the age group
studied.77–80
Although self-reported quality of life and
the degree of social independence seems excellent in
the majority of teenagers and young adults after the
arterial switch operation,77–79
the same has not been
found in younger patients.80
In their study of 49
11-year-old arterial switch survivors, de Koning et al80
reported poorer health-related quality-of-life percep-
tion with regard to motor functioning and positive
emotional functioning, compared with age-matched
healthy peers. More specifically, neurodevelopmental
assessment in teenagers 16 years after the arterial
switch shows satisfactory average scores compared
with healthy controls, although consistently below
expected levels, independent of the bypass strategy
chosen at the time of surgery.81
In all, one-third
required tutoring; a quarter had received special
education, occupational therapy, or psychotherapy; and
one in six had repeated a grade level at least once.81
Normal coping mechanisms towards young adulthood
include a change in self-perception, explaining the
perceived normalisation of the quality of life from the
teenage years into adulthood.77
However, teenagers
after the arterial switch operation have been shown to
lack insight into their weaknesses,81
and given their
relative unawareness with regard to social cognition
these adolescents should probably remain under
surveillance, so as to detect and hopefully influence/
coach their future chances of occupational success.81
Conclusion
The arterial switch operation remains the procedure of
choice for patients with discordant ventriculoarterial
connections when performed in the first days to weeks
of life. Excellent early and long-term survival is now
expected after successful surgery for a lesion that
50 years ago was considered fatal. Although the
correction is deemed curative, certain residual anatomic
lesions remain, leading to predictable reinterventions
and reoperations, including relief of supravalvar
pulmonary stenosis, left ventricular outflow tract
reconstruction with aortic valve repair/replacement,
and myocardial revascularisation. The overall reinter-
vention rate is low, with freedom from a major event at
10 years nearing 90%.78
These are often difficult
reoperations, requiring tremendous experience and
surgical expertise, but results are excellent in capable
hands. Other functional residua, such as global coronary
insufficiency, diminished exercise capacity, and neuro-
developmental limitations, of which incidence and full
clinical implications are still uncertain, need to be
closely followed. After a more difficult initial adapta-
tion period in children and adolescents,80
the quality of
life is very satisfying in young adults.77–79
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