581851

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SCVXXX10.1177/1089253215581851Seminars in Cardiothoracic and Vascular AnesthesiaFiles and Arya

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Seminars in Cardiothoracic and

Preoperative Physiology, Imaging, and

Vascular Anesthesia
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Great Arteries DOI: 10.1177/1089253215581851

scv.sagepub.com

Matthew D. Files, MD1, and Bhawna Arya, MD1

Abstract
Transposition of the great arteries (TGA) refers to hearts with concordant atrioventricular connections but discordant
ventriculoarterial connections. In this lesion, the aorta arises from the right ventricle and the pulmonary artery arises
from the left ventricle. As such, the pulmonary and aortic circulations run in parallel as opposed to in series, and this lesion
is not compatible with survival without adequate mixing of these circulations. The management and outcomes of TGA
parallels the field of pediatric cardiac surgery itself. Uniformly fatal in childhood, palliative procedures from the 1950s to
1970s offered survival, albeit at a high early and late cost. In the 1970s, the arterial switch operation (ASO) provided an
anatomical “cure,” with survival to adulthood in the current era of around 90%. Detailed perioperative imaging, attention
to associated lesions, and comprehension of the physiology are critical to medical and surgical management.

Keywords
cardiac surgery, neonate, cerebral oximetry, children, congenital heart disease, neuroprotection, pediatric intensive care,
postoperative cognitive dysfunction

Introduction month and 90% at 1 year, which challenged surgeons to

Transposition of the great arteries (TGA) refers to hearts
with concordant atrioventricular connections but discor-
dant ventriculoarterial connections. In this lesion, the aorta
arises from the right ventricle (RV) and the pulmonary
artery arises from the left ventricle (LV). As such, the pul-
monary and aortic circulations run in parallel as opposed to
in series, and this lesion is not compatible with survival
without adequate mixing of these circulations. The man-
agement and outcomes of TGA parallels the field of pediat-
ric cardiac surgery itself. Uniformly fatal in childhood,
palliative procedures from the 1950s to 1970s offered sur-
vival, albeit at a high early and late cost. In the 1970s, the
arterial switch operation (ASO) provided an anatomical
“cure,” with survival to adulthood in the current era of
approximately 90%. Detailed perioperative imaging, atten-
tion to associated lesions, and comprehension of the physi-
ology are critical to medical and surgical management.
apply early cardiac surgery procedures to small infants.1
As survival was highest in those with a large atrial septal
defect (ASD), the Blalock-Hanlon atrial septectomy pro-
vided relief of severe cyanosis but with 60% mortality.2
Percutaneous balloon atrial septostomy (BAS), first
refined by William Rashkind in 1966, created an atrial
communication with significantly lower mortality. With
the advent of cardiopulmonary bypass in the 1950s,
Senning and Mustard achieved success in different ver-
sions of the atrial switch procedure, creating a physiologi-
cal but not anatomical correction that was utilized for 3
decades. The atrial switch procedures were successful in
recreating an in-series circulation by creating “double dis-
cordance.” However, this circulation maintains the RV as
the systemic ventricle, which is prone to failure over the
long term. Advances in neonatal and microsurgical tech-
niques led to the anatomical correction via the ASO by
Adib Jatene in 1975, but mortality remained high for a

Background 1
Seattle Children’s Hospital, Seattle, WA, USA
Initially described in the late 1700s, further characteriza-
Corresponding Author:
tion occurred during the mid-1900s as interest in palliative Matthew D. Files, Seattle Children’s Hospital, 4800 Sand Point Way
procedure for congenital heart disease began to increase. NE, PO Box 5371, Seattle, WA 98105, USA.
Natural history studies demonstrated 50% mortality at a Email: matthew.files@seattlechildrens.org

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2 Seminars in Cardiothoracic and Vascular Anesthesia 
decade. In the 1980s, further refinements to the surgical
technique, coronary translocation, and the Lecompte
maneuver improved survival and outcomes.3 In the past
decade, neonatal ASO has come to be associated with a
30-day surgical mortality of 3%4 and excellent long-term
outcomes.
Epidemiology
TGA represents 5% to 7% of all congenital heart disease,
yet is one of the most frequent causes of newborn cyano-
sis. The prevalence is 0.2 to 0.3 per 1000 live births, with
a greater than 2-fold male predominance.5 TGA carries a
low recurrence risk (0.3%) in siblings and offspring com-
pared with all other forms of congenital heart disease.
Although diabetes was previously considered to be a risk
factor for TGA, this has recently been refuted by a large
population-based study.6 There are currently no known
associated fetal environmental risk factors.
Embryology
TGA is considered within the spectrum of conotruncal
defects. Embryologically, TGA is a result of abnormal
rotation and septation of the truncus arteriosus, whereby
the aorta is connected to the RV via a subsemilunar valve
muscular conus, and the pulmonary artery is connected to
the LV with mitral-pulmonary fibrous continuity. For the
purpose of this discussion, TGA will refer to hearts with
isolated ventriculoarterial discordance, meaning that the
aorta is connected to an anatomical RV and the pulmonary
artery is connected to the anatomical LV. We will exclude
patients with discordant atrioventricular connections and
ventriculoarterial connections (“congenitally corrected”
TGA), univentricular hearts, and forms of double-outlet
RV with transposed great vessels. The majority of patients
have normal atrial and abdominal situs. Compared with all
forms of complex congenital heart disease, there is a rela-
tively low (12%) association with extracardiac malforma-
tions or genetic syndromes.5 The spatial relation of great
vessels to each other can be variable, but the most common
is the aorta anterior and rightward of the pulmonary artery;
other relations include side-by-side positioning or directly
anterior-posterior. Typically, there is complete muscular
conus beneath the aorta, but bilateral conus is also possi-
ble, being more common in cases with ventricular septal
defects (VSDs). Unlike the normal heart, where the out-
flow tracts “cross” each other, the orientation of the great
vessels in TGA is parallel with a resultant straight ven-
tricular septum.7
Around 40% of cases of transposition are associated
with VSDs, which can be found in any portion of the ven-
tricular septum.8 VSDs below the outlets can cause
malalignment between the muscular trabecular and outflow
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septa. Posterior deviation of the outlet septum causes sub-
pulmonary obstruction, whereas anterior deviation may
cause subaortic obstruction and a spectrum of both outflow
tracts’ commitment to the RV (Taussig-Bing anomaly).
Thorough understanding of anatomy allows preoperative
stabilization and informs surgical technique.
Hemodynamics and Physiology
Fetal Circulation
The fundamental circulatory abnormality in TGA is that
the pulmonary and systemic circulations run in parallel as
opposed to series. The presence and extent of associated
lesions (mainly ASDs, VSDs, and pulmonary stenosis) in
TGA determines the specific circulatory dynamics and the
resultant physiology. Whereas the presence of the foramen
ovale and ductus arteriosus are essential for fetal viability,
there are important considerations for the fetal circulation.
In the normal fetal circulation, the most oxygen-rich and
glucose-rich blood is delivered to the ascending aorta and
subsequently to the brain. With TGA, the blood delivered
to the brain has a lower saturation, and cerebral autoregu-
lation must account for this reduced oxygen delivery, as
evidenced by Doppler studies.9 This factor may account
for the lower head circumference, altered brain metabo-
lism, and structural abnormalities found in TGA fetuses.10-13
Additionally, the shunting of oxygen-rich and glucose-rich
blood through the ductus arteriosus to the descending aorta
may explain the higher somatic size and association with
pancreatic hypertrophy, hyperinsulinism, and a tendency
toward postnatal hypoglycemia.14
Transitional and Postnatal Circulation
The postnatal circulation is dependent on some form of
shunting at the atrial, ventricular, or great vessel level to be
compatible with life. As the circulations run in parallel, oxy-
gen supply to the tissues is not possible without shunting.
The typically used terms such as left-to-right shunt must be
used with caution, and more descriptive terms are used to
avoid confusion in this lesion.15 The term anatomical left-
to-right shunt involves all the blood that passes from the left
to the right side of the heart. The physiological left-to-right
shunt involves all the blood that is recirculated through the
pulmonary circulation. Finally, the effective pulmonary
blood flow represents desaturated systemic venous blood
that reaches the pulmonary vascular bed. Likewise the effec-
tive systemic blood flow represents the oxygen-rich pulmo-
nary venous blood that reaches the systemic circulation.
This concept is illustrated in Figure 1 and is critical to the
understanding of this circulatory physiology.
Large-volume physiological shunting (parallel circula-
tions) and small-volume anatomical or effective shunting
Files and Arya 3

Figure 1.  Schematic demonstrating the circulation of transposition of the great arteries with intact ventricular septum, with
intercirculatory mixing at the atrial and great artery levels. The majority of the systemic and pulmonary blood flow is recirculated
within the parallel circulations and is ineffective. An anatomical left-to-right shunt is seen at the atrial level, which ultimately enters
the systemic circulation as effective, oxygenated systemic blood flow. An equal volume passes from the aorta to the pulmonary
artery as effective pulmonary blood flow. As seen in the shunt at the PDA, a small portion of the anatomical shunt is derived from
the pulmonary circulation; thus, the anatomical shunt may be greater than the effective pulmonary blood flow. This concept is
important when understanding bidirectional shunting.
Abbreviations: AO, aorta; ASD, atrial septal defect; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PDA, patent ductus arteriosus; RA, right
atrium; RV, right ventricle.

characterizes the postnatal circulation. The actual cardiac
output of each ventricle is typically increased 2 to 4 times
the normal15; however, the majority of this cardiac output
is ineffective. Furthermore, the anatomical left-to-right
shunt must match the anatomical right-to-left shunt over a
short period of time. Because the circulations run in paral-
lel, any sustained anatomical shunt in one direction would
result in a depletion of blood from that circuit.
TGA With Intact Ventricular Septum (TGA-IVS)
In TGA-IVS, the physiology is dependent on the size and
direction of shunting at the atrial or great artery level.
Similar to normal fetal to neonatal circulatory transition,
the expansion of the lung and removal of the placenta
allows for a rapid fall in pulmonary vascular resistance and
increase in systemic vascular resistance, respectively. With
normal transitional circulation, these changes result in an
increase in the LV diastolic pressure that is transmitted to
the atria. As the left atrial pressure exceeds the right atrial
pressure, the flap of the foramen ovale shuts, effectively
eliminating significant atrial level shunting. With TGA,
the right ventricular and right atrial pressures are increased,
which often is sufficient to keep the foramen ovale open
with bidirectional shunting.
The size of shunt through the foramen ovale is variable.
A true secundum ASD is less commonly encountered.
Shunting at the atrial level is complex and depends on
many factors, the majority of which are not modifiable.15
The right atrial a wave typically exceeds the left atrial a
wave, and shunting is anatomical right to left during atrial
systole (effective pulmonary blood flow). During early

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4 Seminars in Cardiothoracic and Vascular Anesthesia 
ventricular filling, the left atrial v wave predominates
causing an anatomical left-to-right shunt (effective sys-
temic blood flow). Respiratory variation also influences
atrial shunting. During inspiration, an increase in systemic
venous return and a decrease in pulmonary venous return
create a predominant right-to-left anatomical shunt,
whereas expiration has the opposite effect. The effects of
positive-pressure ventilation on atrial shunting in TGA
have not clearly been documented.
Immediately after birth, shunting through the patent
ductus arteriosus (PDA) is typically pulmonary to aorta in
systole and reversed in diastole. This may account for
reverse differential cyanosis, which refers to higher satura-
tions in the lower extremities than in the arms. This unique
clinical scenario is found nearly exclusively16 in TGA
(either with persistent pulmonary hypertension and/or a
preductal coarctation) and represents an anatomical left-
to-right shunt and effective systemic blood flow. However,
as the postnatal pulmonary vascular resistance falls, the
majority of the PDA shunting will transition from the aorta
to the pulmonary circulation, which represents an effective
source of pulmonary blood flow. Postnatal circulation with
a PDA results in a large volume of pulmonary blood flow,
the majority of which is ineffective because it remains
within the pulmonary circuit. As the venous return to the
left atrium and ventricle increases, the left atrial pressure
rises, forcing the flap of the foramen ovale shut, which
may eliminate this important source of atrial mixing. With
closure of the foramen ovale, profound hypoxemia and
hypoxia can develop within hours or days after birth, with
saturations <70%, PaO2 <35 mm Hg, and ensuing acidosis.
In this circumstance, the medical team must decide
whether a temporizing BAS is indicated to improve mix-
ing and hypoxemia or whether urgent surgical correction is
indicated (see management below).
TGA With VSD
VSD is found in ~40% of patients with TGA; however,
one-third of these VSDs are small and not functionally sig-
nificant.8 For patients with a large, nonrestrictive VSD,
this form of mixing between the circulations may avoid
acidosis but sets up a very-large-volume pulmonary over-
circulation and ensuing heart failure. Shortly after birth, as
the pulmonary vascular resistance falls, ventricular level
shunting is anatomically right to left in systole, which is
transmitted to the pulmonary circulation. As the left atrial
pressure and left ventricular end-diastolic pressure
increase, diastolic anatomical left-to-right shunting
increases, which may avoid hypoxemia and cyanosis, typi-
cally resulting in saturations >80% and PaO2 >60 mm Hg.
Although a moderate to large VSD typically creates ade-
quate circulatory mixing, occasionally, these patients may
still have significant hypoxemia. The combination of
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high-pressure and high-volume pulmonary blood increases
the likelihood for pulmonary vascular disease, which will
be discussed below.
TGA With Left Ventricular Outflow Tract
Obstruction (LVOTO)
LVOTO is an important consideration in the postnatal
physiology and may influence the surgical management
considerably. LVOTO can occur in the presence or
absence of a VSD, which has important implications for
the postnatal physiology. Additionally, LVOTO can be
acquired in infants with TGA-IVS who do not undergo an
early ASO. In these hearts, as the left ventricular pressure
falls (subsequent to the postnatal decline in pulmonary
vascular resistance), the interventricular septum bulges
from the pressure loaded RV into the left ventricular out-
flow tract, which causes dynamic obstruction. Over time,
this obstruction can become fixed and require alternative
surgical strategies.3
Most series report around 10% incidence of “true”
LVOTO, with the majority having a VSD.8 TGA with
LVOTO and VSD creates a large-volume anatomical left-
to-right shunt at the ventricular level. The limitation in this
circulation is the amount of effective pulmonary blood
flow that is most reliably carried through the PDA. In the
presence of a VSD, careful attention to the detailed anat-
omy of the subpulmonic outflow tract, hypoplasia and ste-
nosis of the pulmonary valve, and branch pulmonary
arteries is especially important. Adequacy of the LVOTO
and pulmonary valve will be important factors to consider
when contemplating the ASO or alternative surgical strate-
gies (see acompaning surgical article).
TGA With Right Ventricular Outflow Tract
Obstruction (RVOTO)
Obstruction to the systemic circulation is less common
than LVOTO and occurs in less than 10% of cases. A
detailed evaluation of the aortic arch anatomy and of any
coarctation and/or aortic arch hypoplasia are important
surgical considerations. The systemic saturations can be
variable depending on the degree of RVOTO. As discussed
above, reverse differential cyanosis indicates significant
anatomical left-to-right shunting at the PDA level and
should prompt concern for coarctation of the aorta.
Imaging
Fetal Diagnosis
TGA remains one of the most difficult congenital heart
defects to diagnose prenatally because of the relatively
normal appearance of the standard 4-chamber view.
Files and Arya 5
Figure 2.  Two-dimensional subcostal short-axis view oriented
posteriorly and leftward demonstrating the great vessels
in parallel, with the aorta arising rightward from the right
ventricle (RV) and the pulmonary artery arising leftward from
the left ventricle (LV).
Detection rates are low, between 3% and 41%, with the
highest detection rates occurring in more recent years in
regions near tertiary care centers.17-22 It is important to
note that the American Congress of Obstetricians and
Gynecologists added assessment of the outflow tracts to
the 2013 guidelines for universal ultrasound screening,
which has the potential to significantly improve prenatal
detection rates. Without a prenatal diagnosis, most infants
with TGA are born outside of tertiary care centers, which
delays management of hypoxemia and hemodynamic
compromise that may result from delayed diagnosis. This
may result in mortality, multiorgan failure, or neurocogni-
tive damage. Prenatal diagnosis of TGA has been shown to
improve preoperative and postoperative survival.18,23-25
Children with TGA diagnosed prenatally have better early
complex cognitive skills compared with those diagnosed
postnatally, possibly because of the decreased incidence of
preoperative acidosis and hypoxemia in those prenatally
diagnosed.24,26
Diagnosis of TGA can be made prenatally as early as in
the late first trimester; however, a complete fetal echocar-
diogram should be performed between 18 and 24 weeks’
gestational age to confirm the diagnosis.27 A standard
4-chamber view may appear normal in a fetus with TGA.
Thus, cephalad sweeps to the outflow tracts and a three-
vessel view are necessary to identify the parallel relation-
ship of the great arteries.28,29 VSDs can also be demonstrated
by fetal echocardiogram; however, small defects may be
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missed. Prenatal evaluation of the atrial septum for poten-
tial restriction of the atrial communication or presence of
an intact atrial septum is important for delivery planning.
The following features of the atrial septum primum have
been shown to predict atrial septal restriction: hypermobil-
ity, bowing into the left atrium, lack of normal swinging
motion, and acute angle of septum primum to the atrial
septum (<30°). Small ASD size, diastolic flow reversal in
the PDA, and abnormal ASD flow pattern are also useful
predictors.30-32 Because of the difficulty in predicting
which fetuses with TGA will ultimately develop a restric-
tive atrial septal communication, current guidelines rec-
ommend that all TGA fetuses are delivered at a hospital
that can manage the hypoxia and hemodynamic compro-
mise associated with restrictive ASD.33
Preoperative Imaging
Transthoracic echocardiography is the imaging modality
of choice for the diagnosis of TGA. Surgical decisions are
made based solely on echocardiographic findings. Thus, a
comprehensive study evaluating the anatomical risk fac-
tors for surgical repair is essential. In addition, echocar-
diography guides BAS prior to surgical repair.
Segmental Anatomy and Spatial Relationship of the Great Ves-
sels.  The subcostal view provides a view of the heart and
blood vessels and their relationships to each other, allow-
ing for rapid diagnosis of TGA. A subcostal long-axis view
sweep from posteroinferior to anterosuperior demonstrates
the abdominal situs, systemic venous connections, the
atrial septum, atrioventricular and ventriculoarterial con-
nections, and the great vessels. The LV is visualized, giv-
ing rise to the pulmonary artery that bifurcates into the
right and left pulmonary arteries. The aorta is visualized
arising anteriorly and rightward from the superior aspect
of the RV. A subcostal short-axis view sweep from right to
left demonstrates the parallel course of the great arteries
(Figure 2). A parasternal short-axis view demonstrates the
spatial relationship between the semilunar valves and great
vessels, with simultaneous appearance of the aortic and
pulmonary valve in cross-section as 2 circles (Figure 3).
The aortic valve is most commonly seen anterior and right-
ward of the pulmonary valve, but other relationships are
possible (anterior-posterior, side by side). Visualization of
the commissure alignments of each semilunar valve is
important for planning coronary transfer during surgical
repair.
Atrial Septum.  The atrial septal communication is critical
to the preoperative circulation in TGA, and echocardio-
graphic assessment of the communication is paramount
once the diagnosis of TGA is made. The type of interatrial
communication (secundum defect vs patent foramen
6 Seminars in Cardiothoracic and Vascular Anesthesia 
Figure 3.  Two-dimensional high parasternal short-axis view
demonstrating the great vessels en face with the aortic valve
anterior and rightward and the pulmonary valve posterior and
leftward. The arrows point to the distal portions of the right
and left coronary arteries, which can also be visualized in this
view.
ovale) and location can be assessed by 2-dimensional
imaging and is best visualized in the subcostal views
(Figure 4). Color Doppler interrogation of the communica-
tion demonstrates flow direction and velocity, and spectral
Doppler is utilized to calculate a mean pressure gradient
across the defect. Features concerning for a restrictive
ASD include small size, flow turbulence with color Dop-
pler, and increased flow velocity and mean gradient. Sec-
ondary signs of atrial communication restriction include
left atrial dilation and flow reversal into the pulmonary
veins during atrial systole. Similar imaging techniques are
utilized for guidance during BAS procedures (Figure 5).
Interventricular Septum.  TGA can be associated with VSDs.
Evaluation involves full sweeps of the interventricular
septum in multiple views by 2-dimensional and color Dop-
pler set to a low Nyquist limit scale because the right and
left ventricular pressures are near equal.
Perimembranous VSDs, the most common type in
TGA, are visualized in subcostal short- and long-axis
views, apical 5-chamber view, and anteriorly tilted para-
sternal long-axis view. Muscular VSDs are best imaged
with a posterior-anterior sweep through an apical view of
the interventricular septum, along with a cross-sectional
short-axis view of the ventricles, with a focus on the
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Figure 4.  Two-dimensional and color Doppler subcostal long-
axis (A) and short-axis (B) view of the right atrium (RA) and
left atrium (LA) with left-to-right shunt flow across the patent
foramen ovale (PFO).
interventricular septum either in a parasternal or subcostal
short-axis view.
Malalignment VSDs (conoventricular septal defects)
are often associated with outflow tract obstruction. Those
with anterior deviation of the conal septum can be seen in
all views but are optimally demonstrated in the subcostal
short-axis and parasternal long-axis views. Malalignment
VSDs with posterior deviation of the conal septum are best
viewed from the subcostal long- and short-axis and para-
sternal long-axis views.
Inlet VSDs are often associated with chordal attach-
ments of the atrioventricular valves to the crest of the sep-
tum or straddling the atrioventricular valve. The tricuspid
valve is more commonly involved. Because straddling
valves and chordal attachments crossing the interventricu-
lar septum can complicate surgical closure of the VSD,
this area must be imaged meticulously. This region is best
visualized in a posterior angled apical view, demonstrating
the VSD at the base of the septum with the atrioventricular
valves forming the superior border of the defect.
Outflow Tracts and Semilunar Valve Function.  Because TGA
can be associated with RVOTO or LVOTO, the subaortic
and subpulmonary regions should be evaluated closely,
recognizing that the pulmonary outflow tract will become
the neoaortic outflow tract and the aortic outflow tract will
Files and Arya 7
Figure 5.  Two-dimensional subcostal long-axis view
demonstrating a septostomy balloon across the atrial septum
in the LA during balloon atrial septostomy. The atrial septum is
delineated with arrows.
Abbreviations: LA, left atrium; RA, right atrium.
become the neopulmonic outflow postoperatively. The
semilunar valves are assessed for structural abnormalities
as well as the presence of stenosis and/or regurgitation.
2-Dimensional, color, and spectral Doppler should be uti-
lized to assess the degree of obstruction, velocity, and peak
and mean gradients. Spectral Doppler may overestimate
the degree of outflow tract obstruction because of the high
cardiac output by both ventricles. Both outflow tracts are
visualized in parasternal long-axis or subcostal short-axis
views. The left ventricular outflow tract (pulmonary) can
also be evaluated in an apical view angled anteriorly to a
5-chamber view.
Patent Ductus Arteriosus. The orientation of the PDA in
TGA is parallel to both the long axis of the aortic arch and
main pulmonary artery, and is, thus, best visualized in a
suprasternal long-axis view. The 3 vessels are demon-
strated in 1 plane, and the aortic and pulmonary connec-
tions of the PDA are seen. Color and spectral Doppler
interrogation display PDA flow directionality.
Coronary Artery Anatomy. Evaluation of coronary artery
anatomy is particularly important in TGA because it
involves critical preoperative factors that influence surgi-
cal course as well as early- and late-term outcomes. Early
mortality after ASO is almost always a result of difficulty
with coronary artery translocation.34-36 Specifically, intra-
mural coronary course or single coronary artery pattern are
associated with increased risk of death.36
To understand coronary artery patterns, one must first
review the naming system for the aortic sinuses of Valsalva
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in TGA (Figure 6). The sinuses are named based on their
relationship to the pulmonary root: 2 pulmonary “facing”
sinuses and 1 “nonfacing” sinus. Depending on the spatial
relationship of the great arteries, the facing sinuses are
named left anterior facing (left facing or anterior facing in
anterior-posterior or side-by-side relationship, respec-
tively) and right posterior facing (right facing or posterior
facing in anterior-posterior or side-by-side relationship,
respectively).37 Alternatively, the Leiden system describes
the origin of the coronary arteries from the facing aortic
sinuses of Valsalva as viewed from the noncoronary sinus
looking at the pulmonary root. From this viewpoint, sinus
1 is defined as the sinus to the right of the observer and
sinus 2 is defined as the sinus to the left of the observer.38
The origin and proximal course of the coronary arteries
is variable; however, in the majority of patients, the coro-
nary arteries arise from the aortic sinuses facing the pul-
monary root. The 8 major anatomical patterns of the
coronary arteries and their incidence in TGA are illustrated
in Figure 7. The most common or “usual” coronary artery
pattern in TGA involves the left anterior descending and
left circumflex arteries arising from the left-facing sinus
(sinus 1) and the right coronary artery (RCA) arising from
the right-facing sinus (sinus 2). The second most common
pattern involves the left anterior descending artery arising
from the left-facing sinus and the RCA and left circumflex
artery arising from the right-facing sinus.
A high parasternal short-axis view is ideal for evaluation
of the origin and proximal course of the coronary arteries
using 2-dimensional and color Doppler set to a low Nyquist
limit scale (Figure 3). In the usual TGA coronary artery pat-
tern, the left coronary artery is best seen with a slight clock-
wise rotation of the transducer (index marker at 3 o’clock),
and the RCA is visualized with a slight counterclockwise
rotation of the transducer (index marker at 1 o’clock).
Parasternal long-axis, apical, and subcostal views provide
additional information regarding the relationship of the
coronary arteries to the great arteries, especially in cases
where the coronary artery pattern is not usual.
Coronary evaluation should include assessment for
intramural course of a coronary artery because its presence
increases operative risk. Generally, an intramural coronary
artery originates from the aortic sinus of Valsalva that
faces the pulmonary valve with its proximal course run-
ning within the wall of the aorta. In a parasternal long-axis
view, the coronary artery will course between the aorta and
pulmonary artery. If there is an additional coronary artery
arising from the same sinus of Valsalva, it is important to
determine whether it shares a common origin with the
intramural coronary artery or whether there are 2 separate
orifices.
Ventricular Size and Function. Assessment of biventricular
size and function can be performed in multiple views and
8 Seminars in Cardiothoracic and Vascular Anesthesia 
Figure 6.  Designations of the aortic sinuses of Valsalva.a
a
Copyrighted images from Lai et al, Echocardiography in Pediatric and Congenital Heart Disease.
includes ventricular dimensions, wall thickness, and sys-
tolic and diastolic functional measurements. Usually, func-
tion is preserved in neonates with TGA. Calculation of left
ventricular mass, wall thickness, and systolic function is
particularly important when considering a “late” ASO as
described below.
Preoperative Management
For patients without prenatal diagnosis, congenital heart
disease should be suspected in the immediate newborn
period if there is cyanosis that does not respond to oxygen.
Although certain fetal echocardiographic features are pre-
dictive of the need for BAS in those with a prenatal diag-
nosis, these predictions are not accurate enough to risk
stratify, and all infants prenatally diagnosed with TGA
should be delivered in a center equipped to perform a
BAS. There is growing evidence to support better out-
comes after cardiac surgery in higher-volume surgical
centers.4
Newborns with TGA are classically not in significant
respiratory distress and frequently will be comfortable
with fairly severe cyanosis and only mild tachypnea.
Additionally, the finding of reverse differential cyanosis
(described above) is unique to TGA and can be sufficient
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to make the provisional diagnosis. The physical exam is
often innocuous in the first days, with perhaps only the
murmur of a closing PDA. As in all cyanotic heart disease
presenting in the newborn period, initiation of prostaglan-
din E1 (PGE) and rapid transfer to a center with expertise
in congenital surgery is indicated. After the diagnosis of
TGA is made, a multidisciplinary team must weigh the
risks and benefits of BAS, continuation of PGE, and tim-
ing of surgery.
Balloon Atrial Septostomy
The team must decide the timing of ASO and if a preopera-
tive BAS is indicated to improve mixing and oxygen deliv-
ery to the tissues. There are no evidence-based criteria for
the performance of BAS for infants with TGA. Reported
incidence ranges from 20% to 75%.39 Some use criteria of
a PaO2 <30 mm Hg and/or a size of the patent foramen
ovale <3 mm as indications for performance of a septos-
tomy. The benefits of acute and dramatic improvement in
saturations must be weighed against the risk of vascular
trauma, arrhythmias, and rare complications such as atrial
perforation and avulsion of the inferior vena cava.
Although McQuillen et al40 reported an increase in preop-
erative stroke in 2006, this has been refuted by several
Files and Arya 9

Figure 7.  The most common patterns of coronary anatomy in patients with D-loop transposition of the great arteries.a
a
Copyrighted images from Lai et al, Echocardiography in Pediatric and Congenital Heart Disease.

studies.39,41,42 A successful BAS is typically associated
with a large increase in the oxygen saturations within min-
utes. Occasionally, BAS is associated with hypotension,
presumably because of relief of left atrial hypertension and
increased PDA shunting. This hypotension is typically
transient and responds to fluid. Peripheral saturations are
typically in the 80s following BAS. As indicated in the
physiology section, the presence of a VSD is not necessar-
ily associated with adequate mixing, and BAS is some-
times indicated in these patients as well.
Prostaglandin
PGE usually improves the saturations in TGA and should
be initiated for any infant with a prenatal diagnosis of TGA
and when cyanotic congenital heart disease is suspected. A
theoretical benefit of a PDA is promoting a large-volume
shunt to cause left atrial hypertension that may stretch the
left atrium and enlarge the foramen ovale, resulting in
improved mixing. Additionally, as the net anatomical
shunts in either direction must equal each other, a shunt
from the aorta to the pulmonary artery (anatomical right to
left) will be matched by an atrial or ventricular shunt in the
opposite direction, thus increasing intercirculatory mixing.
However, a large-volume aorta to pulmonary shunt through
the PDA can also have deleterious effects. Left atrial
hypertension creates pulmonary edema that may compli-
cate the preoperative and postoperative course.
Additionally, runoff from the PDA can decrease the over-
all systemic output that may be marginal to begin with.

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10 Seminars in Cardiothoracic and Vascular Anesthesia 
Figure 8.  Color Doppler suprasternal notch view of the
branch pulmonary arteries after the LeCompte maneuver
during arterial switch operation. The right and left pulmonary
arteries (RPA, LPA) straddle the ascending aorta.
Experimental data from near-infrared spectroscopy dem-
onstrated very high cerebral oxygen extraction in the set-
ting of children with PDA, hypoplastic left heart
syndrome,(HLHS) and aortic insufficiency, which sug-
gests decreased cerebral perfusion in the setting of these
runoff lesions.43 PGE also has associated apnea, fever, and
hypotension as well-recognized side effects. For infants
with a sizeable shunt at the atrial or ventricular level, a trial
off PGE before surgery is often utilized while paying close
attention to oxygen saturations and near-infrared spectros-
copy. The majority of infants will continue to need PGE
until the time of surgery.44 Infants with adequate interciru-
latory mixing are often able to be enterally fed with mini-
mal tachypnea and cyanosis.
Timing of Surgery
Advances in early neonatal surgery, bypass, and ICU care
have allowed success rates for the ASO to increase over
the past 3 decades. However, considerable uncertainty
exists as to the optimal timing of surgical repair. A recent
report from Karamlou et al4 lists day of life 6 to 7 as the
average time for ASO in the United States based on the
Society for Thoracic Surgeons Congenital database.
However, knowledge that structural and metabolic brain
abnormalities exist prior to surgery and are associated with
the degree of hypoxia41 reinforces the concept that TGA is
a hemodynamically unstable circulation. A recent retro-
spective review supports day of life 3 as the ideal time for
the ASO, with increasing morbidity if ASO is performed
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earlier or later.45 Other reports show success with ASO
performed in the first hours of life46 for neonates with a
prenatal diagnosis. Although definitive data regarding tim-
ing are lacking, available evidence suggests that surgery
within a few days of life is safe and theoretically may
avoid some adverse long-term effects of this unstable cir-
culation. Details of the surgical strategy, techniques, and
controversies are included in a separate article in this
journal.
Surgical Management of “Late” TGA
Occasionally, ASO is not performed in the early neonatal
period. Although it is still common in countries with less-
developed medical care, this is now only occasionally a
concern in the United States. It is most commonly seen
with TGA and a large VSD or ASD that allows an appro-
priate amount of mixing, so that severe hypoxia and acido-
sis do not develop. Additionally, some infants who are
premature or who sustain severe neurological injury may
have ASO deferred for some time. After a period of time in
TGA-IVS, left ventricular wall thickness decreases
because of the lower afterload of the pulmonary circula-
tion, and the LV may not be able to tolerate the acute after-
load of the systemic circulation following ASO. The
clinician must decide if the LV needs to be “retrained” to
promote ventricular hypertrophy to support the systemic
circulation. This is typically done with a pulmonary artery
band over a short amount (<2 weeks) of time.47 It is worth
noting that training the LV is associated with significant
morbidity and mortality that seems to increase for older
children.48 The upper age for retraining the LV has not
clearly been defined, but many centers have reported one
stage ASO (without retraining) in children <2 months,
with results similar to that in children <3 weeks old.49 A
strategy endorsed at Great Ormond Street is simply pro-
ceeding with primary ASO in late TGA, accepting that a
small percentage may require extracorporeal membrane
oxygenation support postoperatively.49,50
A final consideration for the patient undergoing late ASO
is a high incidence of early pulmonary vascular changes.8,51,52
For reasons incompletely understood, patients with TGA
develop pulmonary vascular disease at an accelerated rate
compared with other patients with high pulmonary blood
flow, particularly when associated with a large VSD. The
incidence of grade 3 to 4 Heath-Edwards histological pulmo-
nary changes is 25% between 3 and 12 months and >75%
after 1 year.8 In addition to the high volume and pressure of
the pulmonary circulation, the bronchial circulation may play
a role in this accelerated change.53 Older children with TGA
have a well-developed bronchial circulation that also perfuses
the pulmonary parenchyma with desaturated blood, which
may contribute to profound vasoconstriction. Although
uncommon, progressive pulmonary arterial hypertension has
Files and Arya 11
been noted in children following ASO, even as early as 2
months.54,55 Although fixed pulmonary vascular disease will
not be a concern for the majority of infants having a well-
timed ASO, vigorous observation for pulmonary hyperten-
sion is required for the late ASO patient. Neonates with
restrictive atrial communication and left atrial hypertension
may have a very reactive pulmonary vascular bed following
ASO, which is typically transient.
Long-Term Follow-up
Whereas the main focus for this discussion has been on
preoperative and perioperative care, it is important to note
long-term outcomes for these children. Boston Children’s
Hospital has recently developed guidelines for long-term
follow-up, which provides recommendations for screening
and imaging. Patients with complex coronary patterns
(intramural, single coronary) may be at risk for future cor-
onary obstruction, occurring in 5% to 7% of survivors.56-58
Although coronary obstruction most often occurs in the
first 3 months following arterial switch, silent long-term
obstruction also occurs, and initial coronary anatomy
should always be considered during long-term follow-up
as screening and detection of myocardial ischemia contin-
ues to evolve. Neo–pulmonary artery stenosis, branch pul-
monary artery stenosis from the LeCompte maneuver
(Figure 8), neoaortic root enlargement, and neoaortic
regurgitation are all common issues that may require rein-
tervention and require ongoing follow-up. Although physi-
cal health is typically very good, there is a striking
prevalence of mild but widespread neurodevelopmental
abnormalities in TGA survivors,59 which are functionally
more significant than their residual cardiac disease. The
etiology of the neurodevelopmental limitations are likely a
multifactorial sequela of neonatal hypoxemia, acidosis,
cardiopulmonary bypass, and low cardiac output as well as
intrinsic and fetal factors. Rigorous work is ongoing to
determine which of these modifiable factors influence
developmental outcomes.
Conclusion
The management of TGA has evolved rapidly and impres-
sively since the introduction of the ASO. The next impor-
tant goals will be public health efforts to increase the
prenatal detection rate to optimize the presurgical course
and problems associated with severe hypoxemia and aci-
dosis. Thorough understanding of the postnatal physiology
and anatomy will help stabilize, risk stratify, and inform
long-term follow-up. Finally, as surgical mortality declines
and outcomes continue to improve, we should focus on
early strategies to optimize the long-term neurodevelop-
mental outcomes that continue to be a significant source of
morbidity for these children.
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Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
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