Anatomic correction of transposition

of the great arteries

Complete anatomic correction of transposition of the great arteries (TGA) and ventricular septal defect
(VSD) was performed on 2 infants with high pulmonary arterial resistance. Both patients were operated
upon under deep surface-induced hypothermia and limited cardiopulmonary bypass. Direct repair of the
anomaly was accomplished by switching the aorta and the pulmonary artery with reattachment of the
coronary arteries. The clinical result in the first patient was satisfactory. The postoperative cardiac
catheterization and angiogram demonstrated no gradient across either outflow tracts and normal
arrangement of the great vessels. The second patient died in the operating room, probably owing to
compression or kinking of the left coronary artery.

Guillermo Kreutzer, M . D . , Rodolfo Neirotti, M . D . , Eduardo Galindez, M . D . ,

Alberto Rodriguez Coronel, M . D . , and Eduardo Kreutzer, M . D . ,
Buenos Aires, Argentina

A s a result of Mustard's 1,2 report, the hemodynamic
correction of transposition of the great arteries (TGA)
by redirecting the systemic and pulmonary blood flow
has become a widespread procedure. However, several
complications (such as pulmonary venous obstruction,
obstruction of the venous channels, conduction prob-
lems, tricuspid incompetence, and right ventricular
malfunction) have demonstrated the lack of an ideal
operation for this malformation. 3 - 9
Recently, Jatene 10 reported the first clinical success
of the anatomic correction of TGA and VSD with the
use of a technique that allows normal anatomic and
physiological continuity of blood flow. This article will
report on two instances in which this technique was
used under profound hypothermia and limited car-
diopulmonary bypass.
Case reports
CASE 1: In an infant weighing 3.4 kilograms, a cardiac
murmur was detected shortly after birth. At that time, he was
acyanotic and without signs of heart failure. However, when
From the Unit of Cardiovascular Surgery, Hospital de Nifios, Buenos
Aires, Argentina.
Received for publication June 14, 1976.
Accepted for publication Sept. 24, 1976.
Address for reprints: Rodolfo Neirotti, M.D., Unit of Cardiovascular
Surgery, Hospital de Nifios, Gallo 1330, Buenos Aires, Argen-
tina.
admitted, at age 13 months, cyanosis and clubbing of the
digits were present. The first and second sounds were accen-
tuated and single. A Grade 1/6 systolic murmur was audible
to the left of the sternum, in the third and fourth intercostal
spaces. The chest roentgenogram demonstrated moderate
heart enlargement with an egg-shaped cardiac configuration.
The left atrium was also enlarged with posterior displacement
of the barium-filled esophagus. The hilar vessels were di-
lated, but the smaller peripheral vessels appeared normal.
Sinus rhythm with a QRS axis of +120 degrees and right and
left atrial hypertrophy with combined ventricular hypertrophy
were present on the electrocardiogram.
The clinical diagnosis of TGA with VSD and pulmonary
vascular obstruction was confirmed by cardiac catheterization
and angiocardiography (Table I). On Nov. 18, 1975, opera-
tion was undertaken with the use of deep surface-induced
hypothermia and limited cardiopulmonary bypass.11"13 The
anatomy of the heart was that of transposition, the aorta and
pulmonary artery being almost directly anteroposterior with
diameters of 10 and 18 mm., respectively. Circulatory arrest
was established at a nasopharyngeal temperature of 18° C ,
after a short period of cold perfusion. Both coronary arteries,
previously dissected, were detached from the aorta with a 6
by 4 mm. oval cuff of aortic wall and then anastomosed to the
posterior artery (pulmonary artery). The aortic orifices were
closed directly with continuous suture. The aorta and the
pulmonary artery were transected, contraposed, and then
anastomosed reducing the size of both ends of the dilated
pulmonary artery (Fig. 1). The VSD was closed with a Da-
cron patch through a right ventriculotomy during a second
period of circulatory arrest. Repeat cardiac catheterization
studies, performed 5 months after the operation, demon-
strated normal restoration of the blood flow continuity as
well as normal pressures and saturation in all heart chambers

538
 Volume 73
Number 4 Transposition of great arteries 539
April, 1977

Fig. 1. Surgical technique. A, Dissection of both coronary arteries. Dotted line shows the excision of the
coronary arteries with an oval cuff of the aortic wall. B, Detachment of the coronary arteries from the aorta. C,
The aortic orifice closed with direct suture. Both coronary arteries anastomosed to the posterior artery (pulmonary
artery). The aorta and the pulmonary artery transected, reducing the size of both ends of the dilated pulmonary
artery. D, The great vessels contraposed and anastomosed with restoration of the normal continuity of the blood
flow.

(Table I). On angiography (Figs. 2 and 3), a rather normal Table I. Hemody namic data (Case 1)
arrangement of the great vessels was observed.
Eight months after the operation, the patient was acya- Oxygen sat. Pressure
notic, normally active, asymptomatic, and had gained 2 kilo- (per cent) (mm. HgA)
grams in weight. On auscultation, the first heart sound is nor-
mal and the second sound is slightly increased in intensity and Site Preop. Postop. Preop. Postop.
normally split. Sinus rhythm with a QRS axis of +90 de-
grees, incomplete right bundle branch block, and mild right Superior vena cava 29 70.5 - -
ventricular hypertrophy are present on the electrocardiogram. Right atrium 29 70.5 - -
Chest roentgenography shows mild heart enlargement. Right ventricle 55 69 70/0-12 30/0-5
CASE 2: Physical examination of a 13-month old girl, in Pulmonary artery 81 69 70/40 30/8
whom cyanosis had persisted since birth, disclosed moderate Left ventricle 79 97 70/0-12 75/0-5
cyanosis and a Grade 1/6 systolic murmur at the third inter- Aorta 55 97 70/40 75/30
costal space to the left of the sternum. The second heart sound QP 2.7 1.8
appeared to be single and increased in intensity. The liver was QS 2.5 1.8
palpable 1 cm. below the right costal margin. Chest roent- 1.1 1
QP/QS
genography demonstrated an egg-shaped heart with dilated RP (units sq. M.) 14 5
hilar vessels and constricted peripheral vessels. The electro- RS (units sq. M.) 15 23
cardiogram indicated sinus rhythms and mean frontal QRS 0.9 0.22
RP/RS
axis of +130 with combined ventricular hypertrophy.
The clinical diagnosis of TGA and VSD was confirmed by Legend: QP, Pulmonary flow. QS, Systemic flow. RP, Pulmonary arterial
cardiac catheterization and angiography. This examination resistance. RS, Systemic resistance. Pulmonary flow and systemic flow were
revealed severe systemic desaturation (38 per cent in the aor- calculated assuming the oxygen consumption.
 The Journal of
540 Kreutzer et al. Thoracic and Cardiovascular
Surgery

Fig. 2. Case 1: Postoperative selective angiography. A and 5, Right ventricular injection. The appearance of this
chamber differs somewhat from normal. C and/?, Left ventricular injection showing no evidence of obstruction
of the outflow tract. Arrow indicates the level of the pulmonary artery-aortic suture line.

ta) and showed no gradient between the left ventricle and the
pulmonary artery. The oxygen saturation in the latter was 81
per cent, indicating a decreased pulmonary blood flow with
pulmonary vascular obstruction (Table II). In December,
1975, surgical correction was performed with profound
hypothermia, limited cardiopulmonary bypass, and total cir-
culatory arrest. The external appearance of the heart was one
of transposition, with the ascending aorta anteriorly placed
and to the right of the pulmonary artery. After both coronary
arteries were dissected, the distance from the origin of the left
coronary to its first branch was found to be rather short.
Despite this finding, the anatomic correction seemed feasible,
and it was carried out by the technique described in Case 1.
After being rewarmed to 36°C, the heart could not take over
the load of the circulation because of poor contraction of the
left ventricle. Supportive measures were not effective, and
the patient died in the operating room.
Discussion
Since the early days of open-heart surgery, several
techniques for the idea of correcting the outflow
anatomy of patients with TGA have been pro-
posed. 1 4 - 1 7 However, the clinical experience was dis-
appointing, encouraging the hemodynamic correction
by intra-atrial inversion of venous return, following the
principle established by Albert 18 in 1955. Mustard's
modification of Albert's operation is now well known
and can be performed with low mortality rates, al-
though a number of postoperative complications have
been reported. The most important of these can be di-
vided into different groups: (1) arrhythmias secondary
to injuries of the sinus node, its artery, or the intra-
atrial conduction pathways 6 - 8 ; (2) baffle obstruction to
either pulmonary venous or caval return 3-5 ; and (3)
tricuspid incompetence. 9
The macroscopic anatomy of the tricuspid valve and
the right ventricular musculature of 67 specimens of
TGA was examined at the pathology division of our
hospital. The presence of congenitally malformed right
ventricular musculature and the high incidence of ab-
Volume 73
Number 4 Transposition of great arteries 5 41
April, 1977

normalities of the tricuspid valve mechanism found in

this study may be strong arguments in favor of continu-
ing research aimed at correcting the outflow anatomy.19
Recently, Jatene10 reported the first successful ana-
tomic correction of TGA and VSD by switching the
aorta and the pulmonary artery and reattaching the
coronary arteries. Both case reports described above
used a similar technique. The rearrangement of the
great vessels results in a more physiological correction
and eliminates the need for a morphologic right ventri-
cle and a tricuspid valve to support the systemic flow
and pressure work.
At the present time, it is believed that patients with
VSD and no left ventricular obstruction are the best
Fig. 3. Case 1: Postoperative aortogram showing thefillingof
candidates for the Jatene technique. The constraint
both coronary arteries.
against the presence of pulmonary stenosis will proba-
bly prove to be valid and long lasting. However, it is
not clear whether or not those patients with an intact Table II. Hemodynamic data (Case 2)
ventricular septum might be suitable candidates for the
Oxygen sat. Pressure
anatomic repair early in life, before the onset of sig-
(per cent) (mm. Hg)
nificant atrophy of the left ventricle.
With more experience being gained, preoperative Site Preop. Preop.
hemodynamic and angiographic studies should provide Superior vena cava 27.9 _
valuable information about the proper selection of pa- Right atrium 27.9 7.5
tients for anatomic correction of transposition, in re- Right ventricle 38 80/0-8
gard to the anatomy of the outflow tract of the left Pulmonary artery 81 84/52
ventricle and the coronary arteries. Left ventricle 86.5 84/0-8
Aorta 38 84/52
The coronary artery anatomy will probably limit the
indications for the arterial correction of TGA, because QP 2.2
QS 3.5
the distance from its origin to its first branch might not
QP/QS 0.63
be long enough to reach the new proposed site on the RP (units sq M.) 23
pulmonary artery without resulting in kinking, twist- RS (units sq. M.) 15
ing, or compression.20 The case report on the second RP/RS 1.5
patient indicates that she died soon after surgery due to Legend: QP, Pulmonary flow. QS, Systemic flow. RP, Pulmonary arterial
poor contraction of the left ventricle. This poor contrac- resistance. RS, Systemic resistance. Pulmonary flow and systemic flow were
tion resulted most probably because the left coronary calculated assuming the oxygen consumption.

artery was compressed by the "new pulmonary ar-

tery." The anatomic characteristic of the left coronary
artery may well impair the normal blood flow through
this vessel, more often that the right, after the direct
reimplantation of its coronary ostium. In TGA, the
anatomy of the left ventricular outflow tract is some-
what different from normal. The transposed pulmonary
artery literally does arise above the left ventricular cav-
ity and is located distinctly further to the left than is the
normally located aortic valve. The plane of the trans-
posed pulmonary valve is approximately horizontal,
whereas the plane of the normally located aortic valve
is oblique, tending to occupy a semivertical position.21
This peculiar geometry of the outflow tract might favor
the development of progressive obstruction and com-
promise the long term result of the arterial correction of
d-TGA. Late complications related to the surgical
technique, such as stenosis of the new coronary ostia
and "coarctations" of the aorta and pulmonary artery
at the suture lines, may occur eventually. The post-
operative cardiac catheterization of the patient that sur-
vived demonstrated no gradient across either outflow
pathways. The marked drop in pulmonary resistance
would not have been anticipated in a patient with a
normal preoperative hematocrit value. Despite the
normal pressure and resistance, the angiographic pat-
terns of pulmonary vascular disease were still present.
Because the aspect of the left ventricular outflow tract
was normal; the late development of significant subaor-
tic stenosis seems unlikely. The anatomic correction of
TGA may prove to be the best procedure. The indica-

5 42 Kreutzer et al.
tions will probably become broader than they presently
are, possibly including patients with intact ventricular
septum early in life.
Addendum
The course and direction of the first part of the coronary
arteries may have a decisive effect on the outcome of the
"switch" operation. When the coronary orifices are in the
anterior or anterolateral part of the sinuses and the initial
course of the coronary vessels is toward the anterior or an-
terolateral aspect of the heart, reimplantation into the front of
the pulmonary root is relatively simple. However, if the
coronary orifices are truly lateral and the main coronary ves-
sels initially course laterally or even posterolaterally, the
coronary arteries after extirpation from the aorta may have to
be turned back to implant the orifices into the anterior surface
of the pulmonary root. This may result in axial twisting and
consequent obstruction of a main coronary artery and lead to
infarction and death, as in the second case.
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