Congenital Heart Disease for the Adult Cardiologist

Transposition of the Great Arteries

Carole A. Warnes, MD, FRCP

Abstract—Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete
transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation)
performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure
and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node
dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial
arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch
procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the
pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected
transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological
RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the
tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block
occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for
a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration
of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All
patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and
hemodynamic assessment of adult congenital heart disease is available. (Circulation. 2006;114:2699-2709.)
Key Words: transposition of great vessels 䡲 heart defects, congenital 䡲 pediatrics 䡲 cardiovascular diseases

M any patients with transposition complexes have sur-

vived to adulthood. Those with complete transposition
(d-transposition) have survived because of prior radical repair
and are seen increasingly in cardiology practice. Residua and
sequelae are common, and morbidity and mortality are
ongoing. In contrast, although some patients with congeni-
tally corrected transposition (C-TGA) have had surgery in
childhood, others may present for the first time in adulthood,
and the diagnosis is often overlooked. This review will focus
on the presentation, evaluation, and management of these 2
complex lesions.
Glossary of Terms
Atrioventricular discordance: Inappropriate connections of
the morphological right atrium to the morphological left
ventricle and morphological left atrium to right ventricle.
Ventriculoarterial discordance: The pulmonary artery
arises from a morphological left ventricle, and the aorta arises
from a morphological right ventricle.
Transposition of the great arteries: Refers to ventriculoar-
terial discordance. The aorta arises from a morphological
right ventricle, and the pulmonary artery arises from a
morphological left ventricle.
D-loop: Refers to the normal rightward (dextro⫽D) loop or
bend of the embryonic heart tube and indicates that the inflow
portion of the right ventricle is to the right of the morpho-
logical left ventricle.
L-loop: Refers to a leftward (levo⫽L) loop or bend of the
embryonic cardiac tube resulting in the inflow portion of the
morphological right ventricle being to the left of the morpho-
logical left ventricle.
Cardiac chambers: Morphological right and left ventri-
cles refer to the anatomic characteristics of the chambers
and not their positions (eg, the morphological right ven-
tricle is on the left in congenitally corrected transposition).
Congenitally corrected transposition: Atrioventricular dis-
cordance and ventriculoarterial discordance. The right atrium
enters the left ventricle, which gives rise to the pulmonary
artery, and the left atrium enters the right ventricle, which
gives rise to the aorta. Thus, the circulation continues in the
appropriate direction but flows through the “wrong”
ventricles.
Complete Transposition of the Great Arteries
In this anomaly, the aorta arises from the morphological right
ventricle (RV), and the pulmonary artery arises from the
morphological left ventricle (LV) (ie, there is ventriculoarte-
rial discordance). Complete transposition of the great arteries

From the Division of Cardiovascular Diseases and Department of Internal Medicine, Mayo Clinic, Rochester, Minn.
Correspondence to Carole A. Warnes, MD, FRCP, Mayo Clinic, Gonda 5-368, 200 First St SW, Rochester, MN 55905. E-mail
warnes.carole@mayo.edu
© 2006 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org DOI: 10.1161/CIRCULATIONAHA.105.592352

2699
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2700 Circulation December 12, 2006

is also known as d-TGA; the “d-” refers to the dextroposition

of the bulboventricular loop (ie, the position of the RV, which
is on the right side). The aorta also tends to be on the right and
anterior, and the great arteries are parallel rather than crossing
as they do in the normal heart. Because the systemic and
pulmonary circulations run in parallel, there has to be a
communication between the 2, either with an atrial septal
defect, a ventricular septal defect (VSD), or at the great
arterial level (patent ductus arteriosus) to support life. These
connections allow systemic blood to enter the pulmonary
circulation for oxygenation and allow oxygenated blood from
the pulmonary circuit to enter the systemic circulation. The
most common associated lesions are VSD, which occurs in
almost half of the cases, pulmonary outflow tract obstruction,
and, less commonly, coarctation of the aorta (⬇5%).1
D-TGA is one of the most common cyanotic defects seen
in newborns, and when the ventricular septum is intact, it is
usually cyanotic in the first day of life.2 If circulatory mixing
occurs via a patent ductus, physiological closure of the ductus
causes abrupt cyanosis and clinical deterioration. Cyanotic
babies may be treated percutaneously with a Rashkind atrial
balloon septostomy to create a more sizable atrial septal
defect, which may dramatically improve their oxygenation3
until definitive surgery can be performed. Almost all patients
with this defect who reach adulthood have had prior repara-
tive cardiac surgery, although some with a large VSD and
pulmonary vascular disease sometimes survive with Eisen-
menger physiology.

Atrial Switch Procedure

The first atrial switch procedure was performed by Senning in
19584 and involves the creation of an atrial baffle from
autologous tissue to direct the venous return to the contralat-
eral atrioventricular (AV) valve and ventricle. Thus, deoxy-
genated blood from the vena cavae is directed to the mitral
valve and LV and thence to the pulmonary artery, and
pulmonary venous blood is directed into the morphological
RV and into the aorta. An alternative operation was subse-
quently developed by Mustard, who excised the atrial septum
and used synthetic material to create the baffle (Figures 1 and
2).5 Sometimes an atrial switch is preceded by either the
Rashkind balloon atrial septostomy or surgical atrial septec-
tomy (Blalock-Hanlon).6 Both atrial switch procedures pro-
vide excellent midterm clinical results but in the long term are
associated with important sequelae.
Arrhythmias
Late development of both atrial bradyarrhythmias and
tachyarrhythmias is a recognized late complication of atrial
baffle surgery and is more likely to occur with longer
follow-up.7–10 Sinus node dysfunction is common in adults,11
and the cause is probably damage to the sinus node and atrial
conduction tissue or interruption of sinus node blood flow at
the time of operation.12 In the series of 534 children by Gelatt
et al,13 sinus rhythm was present in 77% at 5 years and only
40% at 20 years. Fifty-three patients (11%) needed pace-
maker implantation. Pacemaker implantation in this setting
can be technically challenging because of the complex
anatomy.8 Atrial flutter was also fairly common, occurring in
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Figure 1. Schematic drawings of the Mustard operation. A, An
atrial baffle diverts blood from both the superior vena cava and
the inferior vena cava across to the mitral valve and LV, which
ejects blood to the pulmonary artery. B, The pulmonary venous
blood is returned to the tricuspid valve and RV, which ejects
blood into the aorta. IVC indicates inferior vena cava; LL, left
lower pulmonary vein; LU, left upper pulmonary vein; MV, mitral
valve; RL, right lower pulmonary vein; RU, right upper pulmo-
nary vein; SVC, superior vena cava; and TV, tricuspid valve.
14% of the survivors, and, as others have reported, is a
probable marker for sudden death.14 There were 77 late
deaths (16%), with unexpected sudden death occurring in 31
patients at a median of 3.2 years after the operation.13 Sudden
presumed arrhythmic death and myocardial failure were the
most frequent causes of late death, a finding that agrees with
other reports.15 Atrial arrhythmias can precipitate a signifi-
cant deterioration in systemic ventricular function, and, in
general, sinus rhythm should be restored when possible.
Judicious doses of antiarrhythmic drugs should be used
because aggressive therapy may precipitate heart block.
Radiofrequency ablation for atrial arrhythmias is technically
more challenging because of the complex anatomy, atrial
scar, and presence of artificial tissue. The reported success
 Warnes
Figure 2. Postoperative specimen of TGA after the Mustard
operation. The tomographic cut is comparable to an apical
4-chamber echocardiographic projection. The patch within the
atrium (arrowheads) directs pulmonary venous blood to the tri-
cuspid valve and into the RV. LA indicates left atrium; PV, pul-
monary vein; RA, right atrium; and VS, ventricular septum.
Reproduced, with kind permission of Springer Science and
Business Media, from Seward JB, Tajik AJ, Edwards WD,
Hagler DJ. Two-Dimensional Echocardiographic Atlas, Vol 1:
Congenital Heart Disease. New York, NY: Springer-Verlag; 1987.
rates are ⬇70%.16 Ventricular arrhythmias are uncommon in
the absence of severe ventricular dysfunction.
Systemic Ventricular Dysfunction and
Tricuspid Regurgitation
Late RV dysfunction is a recognized outcome after the
Mustard or Senning procedures because the RV is still the
systemic ventricle.10,17 Tricuspid regurgitation often coexists,
and both lesions increase the propensity for atrial arrhyth-
mias. Mild to moderate tricuspid regurgitation is very com-
mon in adult survivors and tends to progressively worsen.
Occasionally the tricuspid valve apparatus may be intrinsi-
cally abnormal or may have been damaged at the time of prior
VSD repair or by endocarditis. In this circumstance, tricuspid
valve replacement may be warranted, but in most cases the
regurgitation is secondary to annular dilatation from RV
failure, and tricuspid valve replacement is not helpful.
The treatment of systemic ventricular dysfunction is chal-
lenging. Although it is tempting to extrapolate the use of
angiotensin-converting enzyme inhibitors and ␤-blockers
from trials of acquired heart disease to patients after an atrial
switch procedure, convincing data that they increase exercise
time, ventricular function, or survival do not exist.18 None-
theless, angiotensin-converting enzyme inhibitors are often
used empirically. Caution should be exercised with the use of
␤-blockers, which might exacerbate AV block and precipitate
bradycardia.
Many young patients have survived to their 20s and 30s,
and an actuarial survival of 80% at 20 years has been reported
from a single-center experience.19 Late attrition from RV
failure and arrhythmias is an ongoing issue, however, that
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Transposition of the Great Arteries 2701
warrants continued follow-up.13 The long-term prognosis
must be guarded, and in later decades it is possible that many
will require cardiac transplantation.20
The assessment of RV function is challenging, and the most
commonly used modality is still 2-dimensional echocardiogra-
phy, albeit qualitative rather than quantitative because of the
complex geometry of the RV. Other echo-Doppler parameters
can also be followed, such as the index of myocardial perfor-
mance21 and the dP/dT with the use of the tricuspid regurgitant
velocity. More recently, a tissue Doppler measurement of
myocardial acceleration during isovolumic contraction, “iso-
volumic myocardial acceleration,” has been reported and may be
a sensitive, noninvasive method of assessing RV contractility,
which is less load-dependent than other parameters.22 Other
imaging modalities include radionuclide methods,23 angiogra-
phy,24 or magnetic resonance imaging (MRI).25
Although the functional status of many young adults is
good, reduced exercise capacity is the norm.26 This may
relate to chronotropic incompetence, impaired systemic ven-
tricular function, or impaired capacitance and conduit func-
tion of the intra-atrial conduit, which may be fibrotic or
calcified and produce fixed ventricular filling rates.1,27
Late Surgical Strategies
As an alternative to medical therapy for the failing RV,
consideration has been given to the restoration of the RV to
the subpulmonary position in the hope that, in the face of
reduced afterload, its function will improve. This involves
“retraining” of the LV, however, before it can assume
function as a systemic pump, and therefore the pulmonary
artery must be banded to facilitate hypertrophy of the LV. If
successful, the atrial baffle may subsequently be taken down
and an arterial switch operation performed.28,29 This approach
has been successful in some young patients but is more
problematic in adults.30 In addition to the technical challenges
of the extensive surgery, the LV appears less able to respond
to the increased afterload, and ventricular failure occurs after
the pulmonary banding. In a series of 35 patients with
pulmonary banding, 10 failed retraining, and this was more
common in patients aged ⬎12 years.31 Whether a slower,
more physiological banding process might be more effective
in retraining the LV in adults remains to be seen.
Because pulmonary banding increases the LV pressure, a
rightward “septal shift” may occur with improved coaptation
of the systemic AV valve and reduction in systemic AV valve
regurgitation. Some authors have advocated pulmonary band-
ing as a therapy for the failing RV in its own right, but
whether this is an effective palliation has yet to be deter-
mined.32 In many centers, these surgical approaches have
been abandoned in adults in favor of cardiac transplantation.
Atrial Baffle Obstruction and Leaks
Scarring and narrowing of the atrial baffle are infrequent late
complications but should always be investigated. Obstruction
of the superior vena cava is more common than that of the
inferior vena cava and may produce “SVC syndrome.”
Imaging of the superior limb before the implantation of an
endocardial pacing system will ensure that the lead will not
cause obstruction and possible thrombosis. This imaging can
2702 Circulation December 12, 2006

be accomplished by transthoracic or transesophageal echo-

cardiography,33 angiography, or MRI. Inferior vena cava
obstruction is less common but in rare cases may cause
hepatic congestion or cirrhosis. Both limbs can often be
assessed by transthoracic Doppler echocardiography.34 Mild
stenosis can be treated conservatively, but significant stenosis
may need percutaneous balloon and stent placement35 or even
surgical intervention.
Pulmonary vein stenosis is much less common but may
cause pulmonary hypertension. It can be treated percutane-
ously or surgically.
Small baffle leaks are more common than obstruction and are
often visualized only by transesophageal echocardiography.
They are usually small and hemodynamically insignificant but
pose a risk of paradoxical embolus and cerebrovascular event in
the setting of tachyarrhythmias or an endocardial pacemaker. It
is the author’s preference either to close the defect before
implantation of a pacemaker or to place the leads epicardially
because stroke may occur even with concomitant anticoagula-
tion. Patients with a large shunt will require closure, performed
either percutaneously36 or surgically.

Pulmonary Hypertension
Pulmonary hypertension is a serious complication of atrial
switch repairs for d-TGA and occurs in ⬇7% of those who
survive to adulthood.37,38 The exact cause is not completely
clear, but patients appear more likely to develop pulmonary
vascular disease when undergoing operation at ⬎2 years of
age.39 Other risk factors include having had shunts at the
ventricular or great artery level before repair.40 Patients with
mild elevation of pulmonary pressures at early postoperative
catheterization appear at increased risk for developing pul-
monary vascular disease.37 Pulmonary hypertension may also
be caused by pulmonary venous baffle obstruction, which
should always be excluded.
Pregnancy
Pregnancy may be well tolerated after an atrial switch
procedure,41 but careful prepregnancy counseling and evalu-
ation, including a frank discussion about the patient’s long-
term prognosis, are critical. Attention should be paid to the
function of the systemic RV and degree of tricuspid regurgi-
tation because pregnancy poses a significant volume load that
may increase RV dimensions and is sometimes irreversible.42
Pregnancy poses a definite risk of deterioration of functional
class, even if the patient is reportedly asymptomatic before
pregnancy.42 In 1 series, 2 of 16 pregnant women required
heart failure therapy during or soon after pregnancy.42 The
recurrence risk of congenital heart disease in the offspring, in
the absence of a family history, is probably ⬍5%, although
no large series have been reported.41
Arterial Switch Procedure
This operation restores the normal anatomic arrangement of
the circulation and, as such, is a more attractive physiological
long-term option. From its first description by Jatene in
1976,43 it has steadily become the procedure of choice when
the anatomy is appropriate and is usually performed in the
first month of life. It involves transection of the great
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Figure 3. The Rastelli operation. The VSD is patched to tunnel
blood from the LV into the aorta (1). The pulmonary valve is
oversewn, and a valved conduit is inserted from the RV to the
pulmonary artery (2) to bypass the pulmonary stenosis. Repro-
duced from Hornung et al1 with permission from Elsevier.
arteries above the sinuses and detachment of the coronary
arteries along with a “button” from the aortic wall. The
great arteries are then switched into their new position,
with the pulmonary artery brought forward anterior to the
aorta and the coronary buttons sutured into the “neoaorta.”
Adult survivors of this procedure are only now appearing
in adult congenital heart disease clinics. Complications in-
clude distortion of the RV outflow tract and pulmonary
arteries and dilatation of the neoaortic root with aortic
regurgitation. Coronary stenoses may also occur and cause
sudden death or myocardial infarction.44,45 Other coronary
stenoses may require surgical or catheter intervention.46,47
Lifetime follow-up is clearly warranted.
Rastelli Procedure
This operation is used when d-TGA coexists with a large
subaortic VSD and pulmonary stenosis.48 A patch is placed to
direct blood from the LV through the VSD to the aorta. The
pulmonary valve is oversewn, and continuity is established
between the RV and the pulmonary artery by means of a
valved conduit (Figure 3). This operation has the advantage
that the LV functions as the systemic ventricle, but conduit
degeneration and stenosis are inevitable, necessitating reop-
eration with all of its attendant risks. Atrial and ventricular
arrhythmias are frequent, and sudden death may occur,
particularly when significant conduit obstruction exists. RV
and LV failure also may occur. Regular clinical and echocar-
diographic surveillance is necessary, and the development of
 Warnes
Figure 4. Schematic drawings to demonstrate normal AV and
ventriculoarterial relationships (left) and those in C-TGA (right), in
which both AV and ventriculoarterial discordance exist. In
C-TGA, the normal great arterial relationships are lost, and the
pulmonary trunk (PT) is no longer anterior to the aorta. Ao indi-
cates aorta; LA, morphological left atrium; LV, morphological LV;
RA, morphological right atrium; and RV, morphological RV.
arrhythmias should prompt a detailed evaluation of the
hemodynamics.
Follow-Up
All patients, regardless of the type of prior surgical repair,
should have a clinical evaluation yearly or every 2 years.
Imaging by echocardiogram and/or MRI permits an anatomic
and hemodynamic assessment, and exercise testing facilitates
detection of arrhythmias, occult coronary disease, and serial
functional capacity. Periodic Holter monitoring allows detec-
tion of sinus node disease and atrial arrhythmias, although it
may be of little help in identifying those at risk of sudden
death.14 Most patients need endocarditis prophylaxis unless
they have had an atrial switch procedure and have no residual
valve dysfunction or outflow tract disturbance.
Congenitally Corrected Transposition
C-TGA is a rare anomaly and comprises ⬍1% of all forms of
congenital heart disease. In adults, it represents a very
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Transposition of the Great Arteries 2703
heterogeneous patient population, some of whom may not
have been identified until adulthood. Surprisingly, the diag-
nosis is often overlooked.49 In this anomaly, the right atrium
enters the morphological LV, which gives rise to the pulmo-
nary artery, and the left atrium communicates with the
morphological RV, which gives rise to the aorta. Thus, AV
and ventriculoarterial discordance exists, and although blood
flows in the normal direction, it passes through the “wrong”
ventricular chamber (Figure 4). Hence, this “double discor-
dance” results in the term C-TGA, which is, in essence, a
misnomer.50 It is also called L-transposition because the
morphological RV is in the levoposition. The aorta is also
usually, but not universally, anterior and to the left, and the
great arteries may be side by side. Because the tricuspid valve
always enters a morphological RV, it too is on the left side in
the systemic circulation and is more appropriately termed the
systemic AV valve (Figure 5).
Associated Anomalies
Most patients have 1 or more associated cardiac anomalies,
and the presence or absence of these markedly alters the
natural history.
Ventricular Septal Defect
A VSD occurs in 70% of patients, usually in the perimem-
branous location. Patients with a large VSD usually present in
infancy or childhood with congestive heart failure.
Pulmonary Stenosis
Pulmonary stenosis occurs in ⬇40% of patients and is
commonly subvalvular. It may result from an aneurysm of the
interventricular septum or may be associated with fibrous
tissue tags or a discrete ring of tissue in the subvalvular area.
Associated valvar pulmonary stenosis also occurs. Patients
who have both a VSD and pulmonary stenosis may be
hemodynamically well balanced or have varying degrees of
cyanosis.
Abnormalities of the Systemic (Tricuspid) AV Valve
Some abnormality of the systemic AV valve occurs in up to
90% of patients. Ebstein’s anomaly of the systemic AV valve
Figure 5. Necropsy specimens showing
AV concordance (left) and AV discor-
dance (C-TGA, right). The crux anatomy
facilitates recognition of AV morphology
because the tricuspid valve is always
lower (arrow) than the mitral valve and
always enters a morphological RV.
Although the ventricular morphology may
be suggested by the more trabeculated
pattern of the RV, this is not always con-
sistent or easily identified. Reproduced,
with kind permission of Springer Science
and Business Media, from Seward JB,
Tajik AJ, Edwards WD, Hagler DJ. Two-
Dimensional Echocardiographic Atlas, Vol
1: Congenital Heart Disease. New York,
NY: Springer-Verlag; 1987.
2704 Circulation December 12, 2006

Figure 6. Chest radiographs of a patient with C-TGA and levocardia (A) and dextrocardia (B). A, The vascular pedicle on the left, where
the usual rounded convexities of the descending aorta and pulmonary artery are seen in a normal heart, is unusually straight. In addi-
tion, the ventricular border has a “humped” appearance and is more vertical than usual. B, The apex of the heart points to the right,
but the gastric bubble is on the left. This should always raise the suspicion of C-TGA because dextrocardia occurs in ⬇20% of cases.
The gross cardiomegaly is the result of profound heart failure secondary to severe AV valve regurgitation.

also occurs but is different from the typical right-sided
Ebstein’s anomaly, the only real similarity being that the
valve is displaced inferiorly closer to the cardiac apex.51 No
large “saillike” anterior leaflet exists, adherence of the septal
and posterior leaflets is limited, and the atrialized portion of
the RV inflow is also relatively small. In addition, this
systemic tricuspid valve can rarely be surgically repaired with
success. These delicate valves are inherently vulnerable to
developing regurgitation, which is an additional burden for
the systemic morphological RV. Sometimes the tricuspid
valve may straddle the ventricular septum across a VSD,
making a biventricular repair much more difficult and some-
times impossible. Such patients may need single ventricle
repair or palliation with a Fontan operation or bidirectional
cavopulmonary anastomosis.
Conduction System
The AV node and His bundle have an unusual position and
course, and many patients have dual AV nodes.52,53 The
second anomalous AV node and bundle are usually anterior,
and the long penetrating bundle is vulnerable to fibrosis with
advancing age. This makes the conduction system somewhat
tenuous, with a progressive incidence of complete AV block
occurring at ⬇2% per year. Tricuspid valve or VSD surgery
may also precipitate heart block.
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Diagnostic Evaluation
Chest Radiograph
With mesocardia or levocardia, the diagnosis may be sus-
pected from the chest radiograph. The vascular pedicle
appears abnormally straight because the normal arterial rela-
tionships are lost. The ascending aorta is not visible on the
right side, and the convexities from the descending aortic
knob and pulmonary artery are absent on the left side. The
ventricular border on the left may also appear more vertical
than usual (Figure 6A). C-TGA is one of the most common
anomalies associated with dextrocardia and should be sus-
pected when there is abdominal situs solitus (gastric bubble
on the left) and dextrocardia (Figure 6B).
Electrocardiogram
The ECG may be misinterpreted as inferior myocardial
infarction. Because of the ventricular inversion, the right and
left bundles are also inverted, causing septal activation to
occur from right to left. This produces Q waves in the right
precordial leads and absent Q waves in the left precordial
leads (Figure 7). Varying degrees of AV block are also
common.
Two-Dimensional Echocardiography
Echocardiography can be difficult for those without experi-
ence of congenital heart disease, particularly because dextro-
 Warnes
Figure 7. ECG of a patient with C-TGA. Septal activation occurs
from right to left, and therefore Q waves are seen in the right
precordial leads II and III, but no Q waves are seen in V5 and V6.
cardia and mesocardia may occur. Subcostal imaging facili-
tates detection of atrial situs and position of the cardiac apex
and thus determines the presence or absence of dextrocardia
or mesocardia. Ventricular morphology can best be deter-
mined from short-axis and apical 4-chamber views. The
morphological RV is on the patient’s left (l-loop), and
although it has prominent trabeculations, it may be mistaken
for a LV.49 Because the tricuspid valve is always more
inferior (closer to the cardiac apex) than the mitral valve and
always enters the morphological RV, an examination of the
cardiac crux facilitates detection of the anomaly (Figure 8).
Mitral-pulmonary fibrous continuity can be seen on the
long-axis and 4-chamber views along with the abnormal
ventriculoarterial connections. A high parasternal short-axis
view may show the abnormal arterial relationships, with the
aorta usually anterior and to the left of the pulmonary artery.
Cardiac Catheterization
Cardiac catheterization permits evaluation of systemic AV
valve regurgitation and systemic ventricular function.54 A
hemodynamic assessment of associated anomalies can be
performed, including measurement of left and right heart
pressures and pulmonary resistance. In older patients, coro-
nary angiography is necessary before any operative
intervention.
MRI/Radionuclide Imaging
MRI may help to define ventricular function and volumes,55
although it requires some expertise in imaging congenital
heart disease. Because some patients have cardiac pacemak-
ers, it is not always a feasible imaging modality. Radionu-
clide angiography may also be used.56
Natural History/Long-Term Sequelae
The minority of patients may be relatively normal from a
functional standpoint, and survival to the seventh and eighth
decades has been reported when no associated anomalies
exist.57–59 Failure of the systemic ventricle is much more
common earlier in life, usually with concomitant tricuspid
regurgitation.
Systemic Ventricular Failure and AV
Valve Regurgitation
There has been much debate about the cause of systemic
ventricular failure. The coronary anatomy is concordant, and
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Transposition of the Great Arteries 2705
Figure 8. Apical 4-chamber view of a patient with C-TGA in the
same tomographic plane as Figure 5. Pulmonary veins (dashed
arrows) enter the left atrium (LA). The cardiac crux has a mirror-
image appearance with the right-sided AV valve clearly inserting
higher than the left-sided valve (arrows). The lower valve is the
tricuspid valve, which enters the morphological RV, which has
prominent trabeculations (arrowheads). AS indicates atrial sep-
tum; LV, morphological LV; and RA, right atrium. Reproduced,
with kind permission of Springer Science and Business Media,
from Seward JB, Tajik AJ, Edwards WD, Hagler DJ. Two-
Dimensional Echocardiographic Atlas, Vol 1: Congenital Heart
Disease. New York, NY: Springer-Verlag; 1987.
therefore the morphological RV is perfused by a single right
coronary artery. In such a situation, there may be limitations
of myocardial perfusion and a mismatch between oxygen
supply and demand. In a study of 20 patients in which
exercise sestamibi imaging was used, all 20 had perfusion
defects at rest, and in 17 these defects worsened with
exercise.60 In a study in which positron emission tomography
imaging was used, myocardial blood flow in the systemic
ventricle was normal at rest, but the hyperemia resulting from
adenosine-induced vasodilatation was significantly less than
in the controls, suggesting that coronary flow reserve is
greatly attenuated.61
A retrospective multi-institutional study clearly demon-
strated an increasing incidence of systemic ventricular dys-
function and clinical congestive heart failure with advancing
age.62 Even in patients with C-TGA and no significant
associated lesions, more than one third had congestive heart
failure by the fifth decade. In patients with significant
associated defects and prior open heart surgery, two thirds of
patients had congestive heart failure by the age of 45 years.62
In addition to the morphological RV’s inherent vulnerabil-
ity to failure, it has a complex relationship with systemic AV
valve regurgitation, and controversy exists as to which is the
“chicken” and which is the “egg.” In general, however, it
appears that primary RV failure, while uncommon, is a
frequent sequel to systemic AV valve regurgitation.63 Thus,
when systemic ventricular function deteriorates, AV valve
regurgitation should be considered the explanation until proof
of a different explanation can be provided, and surgery should
2706 Circulation December 12, 2006
be considered early, before irreversible ventricular function
ensues.
Late referral is common. In the Mayo Clinic series of 44
patients aged 20 to 79 years with no prior cardiac surgery, 26
patients (59%) were referred with significant (⬎3/4) AV
valve regurgitation.49 Of 30 patients who needed systemic
AV valve replacement, 16 were referred late with clinical
ventricular dysfunction ⬎6 months. The mean preoperative
ejection fraction was 39%, which would be considered
unacceptably late in patients with normal ventricular anatomy
and mitral regurgitation. Although there was no early mor-
tality, 4 patients ultimately needed cardiac transplantation for
poor ventricular function, and the only marker for poor
survival was a poor preoperative ejection fraction.49
Because complete heart block is common, it should be
noted that implantation of an endocardial pacemaker may
also precipitate deterioration in systemic ventricular function
and worsening of AV valve regurgitation. This procedure
presumably alters the position of the ventricular septum,
inducing “septal shift” and failure of tricuspid valve coapta-
tion64 (Figure 9).
Surgical Repair
Systemic AV valve replacement is relatively common in the
adult age group. Surgery can be accomplished with accept-
able perioperative mortality in experienced centers. The
outcomes of 40 patients aged 5 months to 70 years who
underwent systemic AV valve replacement at the Mayo
Clinic between 1964 and 1993 were reported by van Son.59
Of these 40 patients, 36 had severe regurgitation, and 29
(72.5%) had associated cardiac anomalies. The preoperative
ejection fraction ranged from 20% to 60% (mean, 48%). The
in-hospital mortality in this high-risk group was 10%, but
with follow-up to 26 years (mean, 4.7), another 8 patients
died. The survival was 78% at 5 years and 61% at 10 years.
The cause of death in all 12 patients in this series was
systemic ventricular failure, an outcome which emphasizes
the need for early operation before the development of
ventricular dysfunction. Functional status improved in the 28
survivors, with 27 patients in New York Heart Association
class I or II. Survivorship correlated with a preoperative
ejection fraction ⬎44%.
Rutledge et al65 reported a pediatric series of 121 patients
seen at Texas Children’s Hospital between 1952 and 1999
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Figure 9. A, Chest radiograph of a
16-year-old young man with C-TGA just
after implantation of an endocardial
pacemaker. Reportedly, his ejection frac-
tion was 40%, and he had moderate
systemic AV valve regurgitation. B, Chest
radiograph 18 months later at the time of
referral. His systemic ventricular ejection
fraction was 15%, and he had severe
psystemic AV valve regurgitation. Car-
diac transplantation was the only viable
surgical option because referral was too
late for conventional operation. Repro-
duced from Warnes64 with permission
from the American college of Cardiology
Foundation.
with a median age at diagnosis of 1 month. Asymptomatic
patients had isolated C-TGA, a small VSD, or mild pulmo-
nary stenosis, or they were hemodynamically well balanced
with a VSD and pulmonary stenosis. In contrast, patients with
heart failure had a large VSD, a VSD with mild pulmonary
stenosis, or a regurgitant systemic AV valve. Those with
cyanosis had a VSD and either pulmonary stenosis or
pulmonary atresia. In a median follow-up of 9.3 years, the 5-,
10-, and 20-year survival rates were 92%, 91%, and 75%,
respectively. There were 20 deaths (16.5%) at a median age
of 13.2 years and 5 cardiac transplants (4%). Surgery was
performed in 86 patients with an operative mortality of 2.5%.
Biventricular repair was performed in 47 patients and varied
according to the underlying cardiac anatomy. Risk factors for
mortality included age at biventricular repair, moderate or
severe systemic AV valve regurgitation, and poor RV func-
tion. In this study, as in others, most patients undergoing
surgical repair had worsening systemic AV valve regurgita-
tion to a moderate or severe degree. Implicated factors
include the “insult” of cardiopulmonary bypass leading to
annular dilatation,66 annular distortion caused by VSD clo-
sure,67 changes in the position of the ventricular septum, and
annular dilatation after relief of pulmonary stenosis.68,69 In
addition, poor tolerance of the systemic AV valve to systemic
pressure after VSD closure is possible because afterload is
increased, and postoperative complete AV block may com-
pound the adverse hemodynamics.59,69 –71 The observation of
progressive systemic AV valve regurgitation after operative
intervention has prompted some authors to recommend sys-
temic AV valve replacement at the time of intracardiac repair
if the systemic AV valve regurgitation is more than mild at
the time of surgery.66
Hraska et al72 recently reported the outcomes of 123
patients with C-TGA who underwent a variety of surgical
procedures. The 10-year survival was only 67%, and systemic
ventricular dysfunction occurred in 44% of patients. Ventric-
ular dysfunction was again closely linked to systemic AV
valve regurgitation, particularly when Ebstein’s anomaly of
the systemic AV valve was present. The authors also specu-
lated that the change in RV geometry precipitated more
tricuspid regurgitation, which in turn led to more annular
dilatation and then more tricuspid regurgitation. They too
were disappointed with the results of classic biventricular
 Warnes
Figure 10. “Double-switch” operation for C-TGA using the Mus-
tard atrial baffle technique and arterial switch procedure. The
VSD has been closed with a patch. Venous blood from the
superior and inferior vena cava (SVC, IVC) is directed to the RV
and then to the pulmonary trunk, and pulmonary venous blood
is directed to the LV and then to the aorta.
repair and suggested that alternative approaches should be
explored, such as banding the pulmonary artery to increase
LV pressure, which might maintain the geometry of the
morphological RV and prevent progression of systemic AV
valve regurgitation.
Interest has recently been expressed in a more anatomic
repair that permits the morphological LV to function in the
systemic circulation. This strategy would have the theoretical
advantage of relieving the hemodynamic burden on the RV
and tricuspid valve, potentially improving surgical results and
longevity.73,74 Anatomic repair can be accomplished by using
a venous switch procedure (either the Mustard or Senning
technique), and, in those with a normal LV outflow tract, the
arterial switch operation (Figure 10). For patients with a
VSD, a patch can be inserted to tunnel the LV flow into the
aorta, and the morphological RV is connected to the pulmo-
nary artery via a conduit (Rastelli technique). Before ana-
tomic repair, the LV needs to be “prepared” to function as a
systemic ventricle, and therefore, in the absence of pulmo-
nary stenosis that would have “trained” the LV to hypertro-
phy and function at a higher pressure than a venous ventricle,
pulmonary banding may be performed. Concomitant tricuspid
valve surgery can also be performed if necessary. Langley et
al75 reported the results of this approach in 54 patients aged 7
weeks to 40 years (median, 3.2 years) and noted a surgical
mortality of 5.6% and 2 late deaths. Those with tricuspid
regurgitation, however, still tended toward a poorer outcome:
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Transposition of the Great Arteries 2707
Of 18 patients with moderate to severe tricuspid regurgitation
at the time of operation, 2 died early, 2 died late, and 1 had
cardiac transplantation. Given the complexity of these oper-
ations, however, the early mortality appeared encouraging
(and better than many other centers have experienced), but
several reinterventions had to be performed. Seven patients
with the Rastelli procedure needed a repeat conduit, aortic
valve replacement, or transplantation. Percutaneous balloon
dilatations for baffle obstruction or pulmonary stenosis were
also needed. Survival at 9 years was 77%. Although most
survivors are in New York Heart Association class I, aortic
regurgitation in those with a double switch and residual LV
dysfunction are among the ongoing concerns. In addition,
atrial arrhythmias are common after venous baffle procedures
and increase with longer follow-up. Whether older patients
are suitable candidates for these procedures remains to be
determined because the idea that the LV can be “trained” after
childhood remains controversial. Some authors have reported
failure of the LV after pulmonary banding even before
adolescence.31,76 Late follow-up will be necessary to deter-
mine whether this management strategy confers a survival
advantage.
Pregnancy
Successful pregnancy can be achieved in most women with
C-TGA, but careful prepregnancy evaluation is mandatory.77
A comprehensive cardiovascular examination should include
an assessment of cardiac rhythm, ventricular function, AV
valve regurgitation, associated lesions, and postoperative
sequelae.78 In general, those women with a systemic ventric-
ular ejection fraction ⬍40% or significant systemic AV valve
regurgitation should be counseled against pregnancy because
the added volume load of pregnancy will not be well
tolerated. Because angiotensin-converting enzyme inhibitors
are contraindicated in pregnancy, the assessment of ventric-
ular function should be performed with the patient not taking
these drugs. Patients should be advised that the rate of fetal
loss and maternal cardiovascular morbidity is increased. In
the series of Connolly et al,78 22 women had 60 pregnancies
resulting in 50 live births (83%). None of the offspring had
congenital heart disease. There were no pregnancy-related
deaths, but 1 woman with important AV valve regurgitation
developed congestive heart failure, and another woman who
had 12 pregnancies had multiple complications, including
endocarditis and congestive heart failure.
If pregnancy is undertaken, the patient should be monitored
throughout, and delivery should be managed by a multidis-
ciplinary healthcare team including obstetricians, cardiolo-
gists, and obstetric anesthesiologists, preferably in a center
where the cardiologists have expertise in congenital heart
disease and high-risk pregnancy care.
Follow-Up
All patients, whether operated on or not, should have a
periodic evaluation by a specialist in adult congenital heart
disease. The evaluation should include a detailed imaging
study by echocardiography and/or MRI. Declining systemic
ventricular function should prompt a search for worsening
AV valve regurgitation. Exercise testing facilitates detection
2708 Circulation December 12, 2006
of subtle deterioration in exercise capacity in patients who
may believe that they are asymptomatic and permits exercise
guidelines to be prescribed. Symptoms or signs of arrhythmia
warrant detailed investigation and a review of underlying
hemodynamic problems. Most patients require endocarditis
prophylaxis unless they have no valvular dysfunction, out-
flow obstruction, or VSD.
Disclosures
None.
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Circulation. 2006;114:2699-2709
doi: 10.1161/CIRCULATIONAHA.105.592352
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