Journal of Medical Ultrasound (2012) 20, 65e71

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REVIEW ARTICLE

Diagnosis of Transposition of the Great Arteries

in the Fetus
Jin-Chung Shih 1*, Shu-Chien Huang 2, Chia-Hui Lin 1, Tzu-Hung Lin 1,
Ying-Ning Su 1, Shin-Yu Lin 1, En-Ting Wu 3, Ming-Kwang Shyu 1,
Chien-Nan Lee 1

1
Department of Obstetrics and Gynecology, 2 Department of Surgery, and 3 Department of Pediatrics, National Taiwan Univer-
sity Hospital and National Taiwan University College of Medicine, Taipei, Taiwan

Received 12 October, 2011; accepted 4 November, 2011

KEY WORDS Transposition of the great arteries (TGA) is a group of congenital cardiac defects characterized
antenatal diagnosis, by ventriculoarterial discordance. It is one of the most common cyanotic heart diseases, and
fetus, most affected neonates are undiagnosed as fetuses. The sequelae of the complex congenital
transposition of the heart diseases could be severe if undiagnosed before birth. However, the outcome of fetuses
great arteries, with TGA is improved with prenatal detection of this condition. The purpose of this article is to
ultrasound review the gold standard and additional signs for the diagnosis of fetal TGA.
ª 2012, Elsevier Taiwan LLC and the Chinese Taipei Society of Ultrasound in Medicine. All rights
reserved.

Introduction ventricular septum. A few decades ago, Van Praagh et al

Transposition of the great arteries (TGA) was first described
by Mathew Baillie in 1797 [1]. The term transposition was
first used by Farre in 1814, in which “trans” means “across”
and “position” means “placement.” Transposition means
that the aorta and pulmonary artery are placed across the
* Correspondence to: Dr. Jin-Chung Shih, Department of Obstet-
rics and Gynecology, National Taiwan University Hospital, 15F,
Number 8, Chung-Shan South Road, Taipei 10002, Taiwan.
E-mail address: jcshih@ntu.edu.tw (J.-C. Shih).
suggested that the aberrations in conotruncal development
could result in a continuum of malpositions of the great
arteries [2]. TGA is simply one subtype of these malposi-
tions. Other subcategories of these malpositions include
double-outlet right ventricle, double-outlet left ventricle,
and anatomically corrected malposition [3].
The reported prevalence of congenital heart disease
(CHD) varies between four and 10 per 1000 live births
[4e6]. Due to advances in fetal ultrasound resolution and
techniques, obstetricians have the increasing ability to
detect fetal CHD prenatally, thereby prompting timely
referral for adequate perinatal management. Based on

0929-6441/$36 ª 2012, Elsevier Taiwan LLC and the Chinese Taipei Society of Ultrasound in Medicine. All rights reserved.
doi:10.1016/j.jmu.2012.04.005
66
a review of the literature, the antenatal diagnosis and
transfer of neonates with CHD could improve both short-
term and long-term outcomes, especially in those with
cyanotic congenital heart diseases [6,7].
In a population-based review, cardiac defects account
for almost half of the neonatal deaths attributed to
congenital malformations [8]. Tetralogy of Fallot and TGA
are the most common cyanotic heart defects, each
constituting approximately 10% of fetal congenital heart
defects [9e13]. However, in most fetuses, TGA remains
undiagnosed before birth. According to a population-based
review [14], TGA is diagnosed prenatally in only 17% of
affected neonates. In contrast, approximately one-half of
cases of tetralogy of Fallot can be correctly diagnosed in
utero. We conducted a comprehensive review for the
diagnosis of TGA in the fetus because of this low detection
rate.
Terminology
TGA as well as other CHD are best diagnosed using the
segmental approach of the fetal heart [15e17]. To enhance
understanding of the terminology used in the segmental
approach for the diagnosis of TGA, the following terms
should be clarified in advance [2,3,16,18e20].
Atrioventricular (A-V) discordance: The morphologic
right atrium (RA) is erroneously connected to the morpho-
logic left ventricle (LV); and the morphologic left atrium
(LA) is incorrectly drained to the morphologic right
ventricle (RV).
Ventriculoarterial (V-A) discordance: The pulmonary
artery (PA) arises from a morphologic LV, and the aorta (Ao)
arises from a morphologic RV.
Complete transposition of the great arteries (TGA):
Indicates the conditions of ventriculoarterial discordance.
The Ao arises from a morphologic RV, and the PA arises from
a morphologic LV. The A-V connection is correct.
Congenitally corrected transposition of great arteries
(ccTGA): Refers to the condition of A-V discordance plus V-A
discordance. In brief, the RA enters the LV, which gives rise
to the PA. The LA connects the RV, which gives rise to the
Ao. Thus, the circulation becomes physiologically corrected
although double errors occur.
d-loop: Denotes the normal rightward (dextro, d ) loop
of the embryonic cardiac tube. The inflow portion of the RV
is to the right of the morphologic LV.
l-loop: Denotes the cardiac tube bending leftward (levo,
l ) during embryogenesis. The inflow portion of the
morphologic RV is to the left of the morphologic LV.
Detecting transposition of the great arteries in the
fetus
According to the policy of the Bureau of Health Promotion
in the Department of Health of Taiwan, general practi-
tioners should perform routine ultrasound to evaluate
fetal growth at 20 weeks’ gestation. The diagnosis of TGA
can be made by carefully and appropriately evaluating the
anatomic locations of cardiac chambers and the connec-
tions between the atria, ventricles, and great arteries at
this gestational age with high-resolution ultrasound.
J.-C. Shih et al.
However, it must be emphasized that diagnostic accuracy
is only half based on the literature [21e23]. The gold
standard and the additional signs of ultrasound in the
diagnosis of fetal TGA are discussed in the following
paragraphs.
The localization of cardiac chambers and great
arteries
The first step in the diagnosis of fetal TGA is the localization
of the cardiac chambers and their connections with the
great arteries [22]. This is best done by the segmental
approach as described in the literature [15,24e26]. These
cardiac structures can be identified on the basis of their
specific morphologic features.
Anatomically, the differentiation of the atrial chamber
is based on the morphologic aspect of the atrial append-
ages. The atrial appendages are the earlike extensions of
the atria. Typically, the RA appendage is triangular in
shape, whereas the LA appendage is fingerlike [15].
However, differentiation of atrial appendages is difficult
during the antenatal period. In addition, systemic and
pulmonary venous connections may provide important clues
to define situs of the atria in the fetus. The supra-
diaphragmatic portion of the inferior vena cava (IVC)
provides a reliable landmark to identify the anatomic RA
(because of the rule of venoatrial concordance) [15], and
the drainages of four pulmonary veins usually define the
location of anatomic LA (Fig. 1A and B).
Certain features that could help differentiate the right
and left ventricles are the texture and distribution of
internal trabeculae. The trabeculae are coarse in the RV,
but thin and delicate in the LV (Fig. 1B and 1C). More-
over, the papillary muscles of the RV are attached to
both the interventricular septum and the lateral wall, yet
the two papillary muscles of the LV are attached only to
the lateral wall of myocardium [15,27,28] (Fig. 1C).
Nevertheless, these anatomic features may not be
apparent in the fetus. The insertion of the tricuspid valve
to the ventricular septum is lower than that of the mitral
valve, and the location of the moderator band in the
apical area identifies the location of the RV (Fig. 1B).
Both characteristics are valuable in defining the
morphologic RV.
The major characteristic of the PA is that the three
branches (right and left PA, ductus arteriosus) immediately
emerge from the main PA when it arises from the RV
(Fig. 1D). In contrast, the Ao does not branch into the
ascending portion except for the coronary arteries. The
aortic arch can be seen by positioning the transducer to
the left parasagittal plane. In the view, the Ao is seen to
arise from the middle of the fetal thorax with acute
curvature (usually described as having a ‘candy cane’
appearance). Three branches from the transverse arch can
be identified as the innominate, the left common carotid,
and the left subclavian arteries (Fig. 1E). The ductal arch
can be seen by sliding the transducer to the left from the
aortic arch (with a slight tilt of the transducer). In the
normal fetus, the ductal arch arises from the anterior
thorax with a wide curvature (usually described as having
a ‘hockey stick’ appearance). There is no branch in the
Transposition of the Great Arteries 67

Fig. 1 (A) Parasagittal view of the fetal heart. The drainage of IVC identifies the location of the RV. (B) Four-chamber view of the
fetal heart. The MB indicates the location of the morphologic RV, whereas the drainage of normal pulmonary veins denotes
the location of the LA. The trabeculae are coarse in the RV but thin and delicate in the LV. (C) Anatomically, the insertion of the
tricuspid valve to the ventricular septum is lower than the mitral valve (arrows). In addition, the papillary muscles of the RV are
attached to both the interventricular septum and the free wall (asterisks), yet the two papillary muscles of the LV are attached only
to the free wall. These landmarks are useful features for the identification of the morphologic RV. (D) The major characteristic of
the pulmonary artery is that the main pulmonary trunk branches into the right and left pulmonary arteries and the ductus arte-
riosus. The ductus arteriosus is not shown here because it is at the higher position. (E) The ‘candy cane’ appearance of the aortic
arch, which originates from the middle thorax. 1, the innominate artery; 2, the left common carotid artery; 3, the left subclavian
arteries. (F) In the normal fetus, the ductal arch arises from the anterior thorax with a wide curvature (the ’hockey stick’
appearance). Ao Z aorta; IVC Z inferior vena cava; LA Z left atrium; LPA Z left pulmonary artery; LV Z left ventricle;
MB Z moderator band; MPA Z main pulmonary artery; MV Z mitral valve; PA Z pulmonary artery; PV Z pulmonary vein;
RA Z right atrium; RPA Z right pulmonary artery; RV Z right ventricle; TV Z tricuspid valve.

transverse portion of the ductal arch, which can be used to
differentiate the aortic arch (Fig. 1F).
‘Gold standard’ of the diagnosis of fetal TGA
The diagnosis standard of fetal TGA is the demonstration of
V-A discordance. The Ao arises from the morphologic RV
while the PA arises from the morphologic LV [18] (Fig. 2). The
diagnosis of V-A discordance can be achieved by rotating the
transducer from the four-chamber view to the left
ventricular outflow tract view and the right ventricular
outflow tract view subsequently. Sometimes these two
views can be obtained simultaneously in the same plane.
These two views essentially establish the diagnosis of fetal
TGA.
After demonstrating V-A discordance, the A-V connec-
tion should be inspected to differentiate complete trans-
position (d-TGA) from congenitally corrected transposition
(ccTGA). If V-A discordance combined with a correct A-V
connection is noted, the diagnosis is complete transposition
of the great arteries (d-loop TGA, or d-TGA). In contrast,
68
Fig. 2 The ventriculoarterial (V-A) discordance and the
parallel alignment of the great arteries essentially establish
the diagnosis of transposition of the great arteries in the fetus.
The asterisk indicates the location of moderator band.
Ao Z aorta; LV Z left ventricle; RV Z right ventricle;
PA Z pulmonary artery.
when V-A discordance plus A-V discordance (Fig. 3) is
demonstrated, the diagnosis is changed to congenitally
corrected transposition of the great arteries (ccTGA; l-loop
TGA). These two entities should be differentiated because
the clinical manifestation, outcome, and surgical options
are totally different.
The additional signs of the diagnosis of fetal TGA
Several additional scanning planes and signs of the diag-
nosis of fetal TGA have been described in the literature
[21,22,29e33]. These additional views, although not
essential to the prenatal diagnosis, may help to identify
Fig. 3 The atrioventricular (A-V) discordance. The LA, which
receives the drainages from the PV, connects to the morpho-
logic RV (identified by the presence of the moderator band).
The diagnosis of ccTGA can be made in coexistence with the V-
A discordance. AO Z aorta; ccTGA Z congenitally corrected
transposition of great arteries; LA Z left atrium;
PV Z pulmonary vein; RV Z right ventricle.
J.-C. Shih et al.
TGA or assist diagnosis in certain conditions such as unfa-
vorable fetal position or diagnostic dilemma between
similar pathologies. The identification of fetal TGA may
improve perinatal outcome with well-timed transport of
the mother to a tertiary center for neonatal cardiovascular
assistance [34,35].
1. Three-vessel and trachea view: The three-vessel or
three-vessel-trachea view is a transverse view of the
fetal upper mediastinum [32,36,37]. In this plane, the
main PA, Ao, and superior vena cava (plus the cross
section of trachea in front of the spine) are conse-
quently demonstrated from the left side (Fig. 4).
However, in the fetus with TGA, this anatomic
sequence is altered and usually demonstrates a solitary
transverse Ao in the same plane. The PA and superior
vena cava sometimes may be seen on the same side of
the transverse Ao (Fig. 5).
2. The arch view: In the fetus with TGA, the aortic arch
may be shifted to the anterior fetal thorax, with
a ductal arch in its posterior aspect. Both arches may
demonstrate a parallel configuration without a normal
spiraling relationship (Fig. 6).
3. The outflow tract view: In the normal fetus, the great
arteries emerging from the bilateral ventricles show
a ‘crossover’ relationship. However, in the fetus with
TGA, the great arteries are usually aligned in the
parallel course, which allows visualization of both great
arteries from ventricles in a single scanning plane
(Fig. 4). However, not all fetuses with TGA have this
parallel relationship of the great arteries.
4. The ‘big-eye frog’ view [21]: Using four-dimensional
spatiotemporal image correlation technique, the en-
face view of the fetal A-V valves could be seen. In
this view, the PA is at the 1-2 o’clock direction (left
anterior side) of the Ao. However, the anatomic rela-
tionship is altered in the fetuses with d-TGA. In those
fetuses with TGA, the PA deviates posteriorly to the
directions of 3 or 4-5 o’clock direction of Ao (i.e., PA
parallel or left posterior to the Ao) (Fig. 7).
Fig. 4 The three-vessel tracheal view through the upper
mediastinum of the fetus. The pulmonary artery (PA), the aorta
(Ao), and the superior vena cava (SVC) are identified. The
thymus can be seen anteriorly and the trachea posteriorly.
Transposition of the Great Arteries
Fig. 5 The same scanning plane as shown in Fig. 4. The
anatomic relationship is altered in the case of fetal trans-
position of the great arteries. Ao Z aorta; RPA Z right
pulmonary artery.
Cardiac anomalies associated with TGA
Complete transposition of the great arteries (d-TGA)
Approximately one-half of the fetuses with TGA have
associated ventricular septal defect (VSD). The location of
VSD could be anywhere, but is most commonly found at the
part of the outlet portion with an anterior or posterior
malalignment [38]. Anterior malalignment of the outlet
septum may result in aortic root stenosis and widening of
the pulmonary tract. With a great degree of pulmonary
valve overriding the ventricular septum (Fig. 8), TGA may
merge into the continuum of the double outlet of the right
ventricle. In contrast, when there is posterior malalignment
of the outlet septum, pulmonary stenosis with overriding Ao
Fig. 6 In this scan showing transposition of the great
arteries, the spatial arrangement of the aortic arch and the
ductal arch is in reverse. In this fetus with congenitally cor-
rected transposition of the great arteries, the aortic arch
(identified by the presence of three branches in the transverse
arch) appears anterior to the ductal arch. Both arches are
aligned in the parallel course, a finding not seen in the normal
fetus.
69
Fig. 7 Three orthogonal planes in a volume data set of fetal
transposition of the great arteries (TGA). The C plane identifies
the relative locations of the PA to the Ao in this fetus. In the
normal fetus, the PA is at the 1-2 o’clock direction to the Ao.
But in the fetus with TGA, the PA deviated posteriorly, either
side-by-side (the ‘big-eye frog’ sign [21]) or to the 4-5 o’clock
direction of the Ao (as illustrated here). The axis bar shown on
the lower right area illustrates the relative position of the C
plane to the fetal body. Arrows indicate the two coronary
arteries from the ascending aorta. Ao Z aorta; PA = pulmonary
artery. RAA Z right atrial appendage; LAA Z left atrial
appendage.
may be seen. In this condition, it may be difficult to
distinguish tetralogy of Fallot from TGA [1,7,16,18,39].
Left ventricular outflow tract obstruction (LVOTO) is
common in d-TGA due to the posterior malalignment of the
outlet septum, resulting in a small PA in comparison to the
Ao (Fig. 9 A and B). In contrast, right ventricular outflow
tract obstruction (RVOTO) with aortic stenosis is less
common in d-TGA. Premature closure of the foramen ovale
is relatively common in the fetuses with TGA, probably due
Fig. 8 The PA overrides the interventricular septum due to
the anterior malalignment (indicated by the arrow) of the
outlet septum. The asterisk indicates the location of the
ventricular septal defect. Ant Z anterior; Ao Z aorta;
PA Z pulmonary artery; Sp Z spine.
70
Fig. 9 (A) The posterior malalignment of the outlet septum (indicated by the arrow) results in pulmonary stenosis and left
ventricular outflow tract obstruction (LVOTO) in a fetus with transposition of the great arteries. The asterisk indicates the location
of the ventricular septal defect. (B) The same fetus as shown in (A). The posterior malalignment of the outlet septum simulta-
neously results in dilated aortic root, sometimes becoming overriding aorta. Ao Z aorta; LV Z left ventricle; PA Z pulmonary
artery; RV Z right ventricle.
to highly oxygenated blood streaming through the foramen
ovale [40] (Fig. 10). When both VSD and the foramen ovale
are closed or nearly closed, the affected neonates may
require intensive care (including extracorporeal membrane
oxygenation) immediately after birth, and early surgery.
Complete transposition of the great arteries may often
combine with atrial isomerism, common A-V canal, and
double outlet of RV.
Congenitally corrected transposition of the great arteries
(ccTGA)
Complete transposition of the great arteries is often an
isolated lesion. In contrast, 90% of fetuses with ccTGA will
have other cardiac malformations. Approximately 70% of
affected fetuses have VSD, mostly in the perimembranous
portion. LVOTO and tricuspid stenosis occur in 40% and 30%
of fetuses with TGA, respectively. Bradycardia and
complete A-V block were occasionally noted.
Fig. 10 Constricted foramen ovale (indicated by the arrow)
in a fetus with transposition of the great arteries noted at 35
weeks’ gestation. The ventricular septal defect was also nearly
closed before birth, prompting emergency surgery during the
first day of life.
J.-C. Shih et al.
Genetic counseling of TGA diagnosed in the fetus
The morphogenesis of the fetal heart concerns complex
developmental changes in the first few weeks of embryonic
life. Many chromosomal and genetic factors may contribute
to the development of congenital cardiac defects. Based on
a population-based case-control study, the prevalence of
chromosome aberrations in fetuses with CHD is approxi-
mately 13% [41]. Most of these aberrations are trisomies 21,
18, and 13. However, the incidence of chromosome
anomaly in fetuses with TGA is 0.9% [42], which is identical
to that of the normal population. DiGeorge syndrome
results from the microdeletion of chromosome 22q11,
which occurs in 1 per 4000 live births [43]. Phenotypic
manifestations include cardiac defects, characteristic
facies, and thymic hypoplasia. This condition reportedly is
associated with tetralogy of Fallot or other conotruncal
malformations. However, it rarely occurs in the fetus with
TGA. Extracardiac defects usually are associated with other
congenital cardiac defects, but the incidence in TGA is low.
Approximately 9% of neonates with TGA are reported to
have associated extracardiac malformations, and no
specific type of malformation is described [42].
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