Congenital
Heart Disease
Colin J. McMahon,
MB, BAO, BCh
Christopher S. Snyder, MD
Shannon M. Rivenes, MD
Charlie J. Sang, Jr., MD
Charles D. Fraser, Jr., MD
Key words: Arterial switch
operation; dextrocardia;
situs inversus; transposition
of the great vessels
From: Departments of
Pediatric Cardiology and
Cardiovascular Surgery,
Texas Children’s Hospital
and Baylor College of
Medicine (Drs. McMahon,
Snyder, Rivenes, and
Fraser), Houston, Texas;
and Department of
Pediatric Cardiology,
Texas Tech University Health
Science Center (Dr. Sang),
Lubbock, Texas
Address for reprints:
Charles D. Fraser, Jr., MD,
Chief, Division of Pediatric
Cardiovascular Surgery,
Texas Children’s Hospital,
MC 2-2280,
Houston, TX 77030
© 2000 by the Texas Heart ®
Institute, Houston
Texas Heart Institute Journal
Neonatal Arterial Switch
Operation for Transposition
of the Great Arteries
in a Patient with Mirror Image Dextrocardia
and Situs Inversus Totalis
The neonatal arterial switch operation has become the standard therapy for D-transposi-
tion of the great arteries in the absence of left ventricular outflow tract obstruction. We
describe our experience of successful arterial switch operation after balloon atrial sep-
tostomy in a 5-day-old infant girl who had atrial and visceral situs inversus totalis, mirror
image dextrocardia, and D-transposition of the great arteries. To our knowledge, ours is
the first report of this operation in a patient with this anatomy. (Tex Heart Inst J 2000;
27:193-5)
S
urgical management of D-transposition of the great arteries (D-TGA) has
evolved over several decades under the influence of a few key surgical pio-
neers.1-3 Definitive anatomical correction, however, was not described until
1975 with the advent of Jatene’s arterial switch operation (ASO).4 Anatomical cor-
rection was intended to avoid the long-term complications of a systemic right ven-
tricle and the complex atrial arrhythmias inherent to the Mustard and Senning
operations.5 The arterial switch operation has been well described in neonates with
atrial and visceral situs solitus.6 We describe a patient with atrial and visceral situs in-
versus totalis, mirror image dextrocardia, and D-TGA who underwent successful
neonatal arterial switch operation following balloon atrial septostomy. To the best of
our knowledge, the arterial switch operation has not been described previously in a
patient with this anatomy.
Case Report
In March 1999, 3.06 kg girl was born at term to a 25-year-old mother (gravida 2,
para 2) via spontaneous vaginal delivery. Apgar scores were 9 and 9 at 1 and 5 min-
utes. Physical examination revealed a moderately cyanosed neonate with baseline
oxygen saturation of 70%. Vital signs were otherwise normal. The lungs were clear
to auscultation, and respiratory effort was unlabored. Palpation of the precordium
revealed a ventricular tap at the right lower sternal border and the apex beat at the
5th intercostal space in the right mid-clavicular line. Auscultation of the right chest
revealed a normal 1st heart sound and a single loud 2nd heart sound. No murmurs,
clicks, or gallops were noted. The liver was palpable 1 cm below the left costal mar-
gin. The pulses were normal in volume and character.
Chest radiography demonstrated dextrocardia, cardiomegaly (cardiothoracic ratio
0.75), a right aortic arch, and clear lung fields with normal pulmonary vascular
markings (Fig. 1). A gastric air bubble was noted on the right side, and a liver shad-
ow was visible on the left side. The electrocardiogram revealed a low left atrial
rhythm and dextrocardia (Fig. 2). Abdominal ultrasound demonstrated the stomach
and spleen on the right side and the liver on the left. The aorta and the inferior vena
cava were inverted. Echocardiography demonstrated dextrocardia, atrial situs inver-
sus, atrioventricular concordance, and ventriculo-arterial discordance (I,L,L). There
was bidirectional shunting through a moderate-size (5-mm) nonrestrictive perimem-
branous ventricular septal defect, and there was left-to-right shunting through a
patent foramen ovale. Continuous low-velocity left-to-right shunting due to a large
Arterial Switch Operation for D-TGA in Situs Inversus 193
patent ductus arteriosus was also present, and the
aortic arch was on the right. The coronary artery pat-
tern was mirror image Yacoub type A.
The patient was electively intubated and a pros-
taglandin infusion was initiated. On day of life 2, she
underwent balloon atrial septostomy to improve mix-
ing at the atrial level and to reduce left atrial hyper-
tension (Figs. 3 and 4). Oxygen saturation improved
to 85% in room air following the procedure, and the
prostaglandin was discontinued without change in
oxygen saturations. At this point, she was transported
to Texas Children’s Hospital for further management.
On day of life 5, the patient underwent arterial
switch operation. Under full-flow cardiopulmonary
bypass, the aorta and pulmonary artery were tran-
sected, a Lecompte maneuver was performed, and
Fig. 3 Fluoroscopy shows balloon atrial septostomy. The
balloon is inflated in the right-sided left atrium.

Fig. 4 Following pullback of the Rashkind balloon, the balloon

is located at the junction of the inferior vena cava and the left-
sided right atrium.

Fig. 1 Chest radiography demonstrates dextrocardia,

cardiomegaly, and a gastric air bubble on the right side.
the coronary arteries were re-anastomosed to the
aorta in the standard fashion. The atrial and ventric-
ular septal defects were patched using a transatrial
approach. Postoperative transesophageal echocardio-
graphy demonstrated no new obstruction in the
aorta or pulmonary artery, and no residual shunting
at the atrial or ventricular level. The patient returned
from the operating room on dopamine (3 µg/kg/min)
and minimal ventilation settings. She had an un-
eventful postoperative course, during which there
was no disturbance in cardiac rhythm, blood pres-
sure, or perfusion, and no ST segment change. She
was extubated within 36 hours and weaned from
hemodynamic support by postoperative day 2. On
Fig. 2 Electrocardiogram demonstrates low left atrial rhythm
and dextrocardia.
postoperative day 5, she was discharged to her refer-
ral hospital.

194 Arterial Switch Operation for D-TGA in Situs Inversus Volume 27, Number 2, 2000
 Discussion
D-transposition of the great arteries is the most com-
mon cyanotic congenital heart lesion to present in
the 1st week of life and affects between 2.6% and
7.8% of infants who have congenital heart disease.6
It is commonly associated with ventricular septal de-
fect (50% of D-TGA cases), atrial septal defect
(20%), atrioventricular valve abnormalities (10%),
arch obstruction (10%), abnormal coronary artery
patterns (5%), and subaortic or subpulmonary steno-
sis (5%). 7
Transthoracic echocardiography alone is generally
sufficient to delineate the anatomy and coronary ar-
tery branching pattern, thereby avoiding the need for
a diagnostic catheterization. Balloon atrial septosto-
my, introduced by Rashkind8 in 1966, revolutionized
management of these patients and can be performed
in the catheterization laboratory or by the bedside.
The arterial switch operation has become the stan-
dard surgical repair for D-TGA in the absence of left
ventricular outflow tract obstruction. The current
mortality risk for neonatal ASO is less than 5%, even
in the presence of a complex coronary arrangement.9
At Texas Children’s Hospital, we recommend that all
patients undergo balloon atrial septostomy prior to
surgery in order to achieve optimal atrial level mix-
ing, reduce left atrial hypertension, and minimize
preoperative mortality. The optimal timing of arteri-
al switch operation is between day of life 7 and 28,
before the pulmonary vascular resistance falls and
while the left ventricle remains capable of sustaining
a systemic workload.
Establishment of unimpeded coronary blood flow
remains the most crucial intraoperative factor in
achieving successful arterial switch. Adverse outcome
in the early experience with arterial switch was often
attributed to impeded flow. As surgeons have gained
experience with coronary artery transfer and greater
awareness of coronary artery patterns over the past
10 years, morbidity and mortality rates have fallen
substantially. 10 The highest mortality rates have tradi-
tionally been seen in patients who have intramural
coronary patterns. Recently, transferring a single
coronary button that houses both coronary ostia—
and unroofing the intramural coronary to enable a 2-
button repair—have greatly improved outcome in
this group. 9,11 Interestingly, coronary artery pattern
has become less of a concern, and poor outcomes
have been attributed to poor preoperative ventricular
function.9
In conclusion, the success of early neonatal arterial
switch operation for D-TGA has been replicated
with low mortality at several centers.6,10,12 We have
presented a report of successful neonatal arterial
switch in a patient with atrial and visceral situs in-
Texas Heart Institute Journal
versus totalis, dextrocardia, atrioventricular concor-
dance, and ventriculo-arterial discordance (I,L,L). To
our knowledge, ours is the first report of this opera-
tion in a patient with this anatomy.
References
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Arterial Switch Operation for D-TGA in Situs Inversus 195