MANAGEMENT OF TRANSPOSITION OF GREAT ARTERIES

1. TGA – MANAGEMENT STRATAGIES

4. Significant milestones in the evolution of surgery
for transposition of great arteries (TGA

5. The first surgical treatment of TGA-
Alfred Blalock- Rollins Hanlon
Johns Hopkins.
In 1948 - surgical palliation of TGA,
allowing effective mixing of both the parallel circulations
concluded that the presence of a VSD was favorable for survival,
followed by the presence of an ASD.
A combination of the two defects was the most favorable
situation
In 1950 - first reported the use of a surgically created atrial
septal defect as a treatment of TGA
‘Blalock–Hanlon Septectomy’

7. September 1953,
Walton Lillehei and Richard Varco
University of Minnesota –
surgical correction of TGA.
In the first four patients,
anastomosed the right pulmonary veins RA
Two - survived.
In the subsequent four patients, in addition, also
directed the inferior vena cava blood to the left
atrium –
none of these patients survived

8. Thomas Baffes
at the Children's Memorial Hospital Chicago.
Used aortic homograft to join the inferior vena cava to
the left atrium and the right pulmonary veins were
anastomosed to the right atrium
first successful procedure was performed on May 6,
1955 and reported in 1956
Originally planned to perform a second-stage
procedure that would switch the superior vena cava
with the left pulmonary veins, but this was never
performed clinically.
The Baffes operation remained the treatment option
for infants with transposition over the next 10 years

10. ‘Albert principle’
correction of TGA at the atrial level
using a baffle - Dr. Harold M Albert
Albert did not attempt this operation
clinically.
But the idea formed the basis of the
atrial level switch operation

11. (a)Creation of bilobed flaps of interatrial septum
(b)Each lobe used to cover the orifices of the
vena cava

12. 1956, Creech from New Orleans
attempted an atrial switch
9-month-old boy using a polyvinyl (Ivalon) baffle and
extracorporeal circulation procedure was a technical success.
But patient died 12 h later.
Autopsy - a nonobstructing prosthesis.
Death was attributed to respiratory failure due to bronchial
obstruction due to secretions and impaired ventilatory effort
secondary to bilateral thoracotomy.

13. 1957, Alvin Merendino,
from the University of Washington in Seattle,
attempted an atrial switch using a premolded
atrial septal prosthesis made of Ivalon using
cardiopulmonary bypass in two patients.
But both did not survive the operation

14. In 1957, Ake Senning
performed the first successful atrial switch using atrial flaps at
the Karolinska Hospital, Stockholm, Sweden
It was published in 1959
The procedure was unsuccessful in the first two patients. The
third case, a 9-year-old boy survived.

15. On May 16, 1963, at the Hospital for Sick Children, Toronto,
Mustard operated upon an 18-month-old girl who had
previously undergone a Blalock–Hanlon operation
He repaired the ventricular septal defect and then performed an
atrial switch using an autologous pericardial baffle

16. The Senning operation was perceived as
“ingenious, technically extremely difficult in the infant
or small child”.
It was practically abandoned and for a decade
Mustard procedure was used universally by surgeons
including Dr Senning himself.
However by 1975, the Mustard procedure revealed its
shortcomings including baffle obstruction and lack of
growth potential.
This led to a resurgence of the Senning procedure and
it remained a favorite for the next decade

17. Adib Dominos Jatene
a Brazilian surgeon of Lebanese origin performed
successfully for the first time,
Anatomical correction in the form of an ASO in 1975
at the University of Sao Paulo Heart Institute, Sao
Paulo, Brazil
Jatene et al stressed upon two technically important
points —
excising coronary buttons and
transecting the great vessels away from the valves

19. technically perfect coronary artery transfer - most
critical step in a successful ASO.
Jatene quoted “to divide, contrapose and then re-
anastomose the great arteries is not a surgical problem.
The major technical difficulty in this approach has
been the transfer of coronary arteries”

20. In 1981, Lecompte from Laennec Hospital, Paris, France
described a technically important modification of
surgically translocating the great vessels
‘Lecompte maneuver’.
This greatly simplified the method of right ventricular
outflow tract reconstruction during the ASO,
a better anatomical lie for the coronary arteries
and the newly reconstructed aortic anastomosis

21. In 1984, Castaneda and colleagues from the Children's
Hospital, Boston
introduced the concept of the neonatal arterial switch
describing their experience with 14 neonates
This emphasized the ability of the neonatal LV to
successfully handle the systemic circulation.
Today neonatal arterial switch remains the treatment of
choice

22. It was realized that the arterial switch did not give
acceptable results after the 1st month of life.
The inability of the LV to operate at a systemic pressure
after regression of neonatal pulmonary hypertension
was identified as the problem.
Sir Magdi Yacoub from Harefield Hospital,UK
proposed a
two-stage repair by performing PA banding first,
to train the LV followed by a second stage arterial
switch several months or a year later

23. The concept of rapid two-stage arterial switch for
patients with TGA with intact septum presenting
beyond neonatal period was introduced by Jonas and
colleagues in 1989.
Rapid two-stage procedure,
first stage
Pulmonary banding - after-load
a modified BTS - preload and improve the systemic
oxygen saturation.
This is followed by a corrective second stage arterial
switch 5-7 days later.

24. The availability of mechanical
circulatory support further allowed
pushing the age limits of performing
the arterial switch

25. Extending the boundaries of the primary arterial switch
operation in patients with transposition of the great arteries
and intact ventricular septum
If the period of temporary LV dysfunction is taken care of, using
mechanical support like extracorporeal membrane oxygenation
(ECMO)
the age limit can be pushed to as much as 6 months.
ECMO supported approach also offers advantages of
single surgery,
Improvement in LV function independent of the after-load
faster reconditioning period
over the PA band-BTS-based two-stage approach
Kang N, de Leval MR, Elliott M, Tsang V, Kocyildirim E, Sehic I, et
al.. Circulation. 2004;110:II123–7

26. • Correction of acidbase balance,
• maintenance of normothermia,
• prevention of hypoglycemia,
• Prostaglandin E1 is administered to increase
pulmonary blood flow and is usually followed
by atrial balloon septostomy to improve
mixing.

27. • high preoperative mortality rate - because of
absent or small atrial shunt
• And
• with institution of prostaglandin E1
and balloon atrial septostomy immediately
after birth could avoid a fatal outcome.

28. • Balloon atrial septostomy
• is performed on a semiurgent basis within
hours of the diagnosis of d-transposition of
the great arteries with no associated ASD or
VSD.

29. • The diagnosis of transposition is in itself an
indication for surgery.
• Arterial switch operation is the firmly
established as the procedure of choice.
•

30. ATRIAL SWITCH OPERATION-
SENNING TECHNIQUE

31. First, circumferences
of the SVC and IVC are determined (by compressing them with a
clamp, measuring length of clamp occupied
by compressed cava, and multiplying by 2).
Position and superior and inferior extent of
left atriotomy
junction of the left atrial–right pulmonary vein wall with the most
rightward aspect of the right atrial wall surface.

32. The proposed right
atriotomy incision is
visualized roughly
parallel to the left
atriotomy incision
The superior extent
is 3 or 4 mm anterior to
the sulcus terminalis,
thus anterior to the
sinus node,
is anterior to the
superior end of the
proposed left
atriotomy by 2/3RD of
the SVC circumference.

33. The inferior extent
of the proposed right
atriotomy is placed
anterior to the inferior
end of the proposed left
atriotomy by a 2/3RD of
the IVC circumference.
Further right-angled
anterior extensions
superiorly and inferiorly
so that later a right atrial
flap can be created

34. CPB and aortic cross clamp and cardioplegic arrest,
left and right atrial incisions are made.
Dashed line shows proposed incision in atrial septum.

35. Atrial septal flap
Coronary sinus is unroofed, with
incision shown along dashed
line.

36. septal flap - repositioned into left atrium
and connected to left atrial wall
Suture line is anterior and superior to left pulmonary vein and
anterior and inferior to left inferior pulmonary vein.

37. Posterior edge of right atrial
incision is approximated to
remaining edge of atrial
septum.
Most critical points in suture
line are areas over orifices of
superior (SVC) and inferior
(IVC) venae cavae. –
prevent narrowing of cavae at
their transition into surgically
created tunnel leading to
mitral valve.

38. Anterior edge of right atrial incision - advanced posteriorly
and attached to lateral free edge of left atrial incision.
Stay sutures are positioned to allow appropriate length of right
atrial flap overlying the two venae cavae.

39. ATRIAL SWITCH OPERATION –
MUSTARD TECHNIQUE

40. Atrial switch operation by Mustard technique.
proposed atrial incision.
Entire atrial septum is excised
coronary sinus is cut down

41. Shape
of the patch (intraatrial baffle) - oval,
a gradual waist created in its midportion along long axis.
Dimensions
of patch vary depending on size of infant.
For a newborn infant weighing less than 5 kg,
an initial oval patch - 7 cm × 3.5 cm
Width of patch at waist s- 2.5 cm.

42. Patch is sewn into place,
beginning within left atrium,
anterior to left-sided pulmonary
veins.
Suture lines –
superiorly and inferiorly around left
pulmonary veins
and toward superior and inferior
caval orifices on their posterior,
lateral, and then anterior aspects.
Suture line is then transitioned
from caval orifices onto cut edge of
atrial septum.

43. Baffle - after suture lines are
completed.
The four pulmonary veins are
visible
Atrial incision is closed

48. • DISADVANTAGES OF
SENNING OPERATION
• (1) postoperative
arrhythmias result from
direct injury of the sinus
node, its artery, or the A-V
node.
• (2) The Senning operation is
technically more difficult
than the Mustard
procedure, but with
increasing experience the
technical differences will be
more readily overcome.
• ADVANTAGES OF SENNING
OPERATION
• (1) The amount of foreign
material used is very small.
• new atria can be expected
to grow as the patient
grows
• (2) The geometry of the
operation is such that it is
nearly impossible to cause
stenosis of the venae cavae.
• stenosis of the pulmonary
veins is probably also
exceedingly rare.

49. ARTERIAL SWITCH OPERATION -
TRANSPOSITION OF THE GREAT
ARTERIES WITH USUAL GREAT ARTERY
AND CORONARY PATTERNS

50. • The aorta and pulmonary trunk are dissected apart and
the PDA dissected.
• The right and left pulmonary arteries are extensively
mobilized to their lobar branches and beyond if
needed.
• High aortic cannulation and bicaval cannulation done.
• After cannulation is completed, CPB is established and
cooling begun.
• PDA is ligated and divided.
• Aortic cross clamp and Cardioplegia.

52. Aorta is transected just above sinutubular junction,
Cronary buttons are harvested

53. Coronary buttons are completely mobilized.
Pulmonary trunk is transected
sites of coronary implantation are identified on proximal neoaorta

54. Coronary implantation is performed

55. The flap is achieved by creation of a J-shaped incision.
The trapdoor flap requires less rotation of the coronary buttons
and adds to the circumference of the proximal neoaorta.

56. Lecompte
maneuver
Anastomosis
between proximal
neoaorta and
ascending aorta

57. proximal neopulmonary trunk
reconstructed
fixed autologous pericardium.

58. The aortic cross
clamp is released.
Satisfactory
perfusion
of all areas
observed.
The pulmonary
anastomosis
using continuous
prolene suture

59. • If an ASD is present
• Commonly after aortic reconstruction is
complete, but before on pulmonary trunk
reconstruction.
• In this way, ASD is closed with the aortic clamp
still in place
• Aortic clamp is then removed prior to
performing pulmonary trunk reconstruction

60. MANAGEMENT OF HIGH RISK
CORONARY ARTERIES

61. Transposition of Great arteries with
Intramural coronary artery

62. 1
common form of Intramural coronary artery
left main coronary artery which has its ostium in the right
posterior facing sinus with the coronary vessel itself emerging
externally from the left posterior facing sinus.
The intramural segment of the artery frequently passes behind the
top of the posterior commissure of the original aortic valve.

63. separating the two closely spaced coronary ostia
and excising a larger than usual left coronary button
with detachment of the posterior
commissure of the neopulmonary valve.

64. Transposition of the Great Arteries with
Origin of Circumflex Coronary Artery from
Sinus 2

65. 2
Cx coronary artery arises as a branch of
the
RCA, the ostium of which is in sinus 2
(right-facing sinus).
1L-2RCx
The Cx artery then passes leftward
behind the pulmonary
trunk and arborizes in the usual fashion
Less often the LCA arises from sinus 2
(an RCA from sinus 1)
1R-2LCx
and passes leftward behind the
pulmonary trunk to bifurcate
in the usual manner.

66. site of pulmonary trunk transection
is as far distal as possible, just
before bifurcation, to provide a
proper implantation site on
proximal neoaorta (native
pulmonary trunk) for coronary
button from sinus 2.
Proposed aortic transection site is
slightly more distal
to accommodate slightly shorter
distal pulmonary trunk segment at
time of pulmonary reconstruction.
myocardial area.

67. Coronary from sinus 1 - reimplanted in standard fashion.
Proximal neopulmonary trunk is retracted anteriorly, and tissue between
the two great arteries at their bases is fully dissected.
Incision to create “trapdoor” flap that serves to orient sinus 2 coronary
button after reimplantation
such that circumflex artery is neither kinked nor stretched.
Because pulmonary trunk was transected as distally as possible,
reimplanted coronary also is positioned more cephalad than in usual case.
This also minimizes chance of circumflex artery kinking.

68. Transposition of the Great Arteries with
Origin of All Coronary Arteries from Sinus 2-
SEPARATE OSTIA

69. Separate ostia too close together to allow safe mobilization of separate
buttons. Neither ostia shows an intramural course.
A single large button encompassing both ostia is mobilized.
Distal aspect of coronary button is then sutured to implantation site.

70. Proximal neoaorta to ascending aorta
anastomosis is performed,
completing entire circumference except for that
portion that contains coronary button.
A small hemisphere-shaped segment of
ascending aorta is excised in portion of
ascending aorta adjacent to implanted coronary
button.
A roof - over remaining opening in ascending
aorta and remainder of free edge of coronary
button

73. • The short- and mid-term results of the Jatene
operation performed at the Prince Charles
Hospital suggest that
• this option is more suitable than either the
Senning or the Mustard operation for the
correction of TGA, within the limits of
appropriate coronary artery anatomy.
• Mortality rates are acceptable and there appears
to be less morbidity compared to atrial repairs.

74. TGA-
SIDE-BY-SIDE GREAT ARTERIES

75. Side-by-side great arteries and aorta to right, with coronary
pattern of 1LR-2Cx.
Both arteries are transected higher

76. ecompte maneuver is not performed.
Coronary buttons- mobilized and coronary
implant sites on proximal neoaorta
developed.
Left-sided aspect of opening in distal
pulmonary trunk is partially closed with a
semilunar-shaped
patch of autologous glutaraldehyde-treated
pericardium. Right side of this opening is
enlarged into right pulmonary artery as
shown.

77. Coronary artery from sinus 2 is
particularly vulnerable.
Without Lecompte maneuver, proximal
neopulmonary trunk is
oriented somewhat posteriorly and can
compress posterior reimplanted
coronary artery (circumflex artery in this
case) over its proximal
extent.
For this reason, it is critical that proximal
neopulmonary trunk be implanted as far
right along transverse right pulmonary
artery as possible.

78. TGA IVS –
THE REGRESSED LEFT VENTRICLE

79. • Post natal LV growth in a normal heart occurs
• in response to a combination of volume and
pressure load,
• resulting in a rapid increase in LV mass.
• TGA - the absence of pressure load result in
retardation of this increase in LV mass

80. Geometry of the LV is an outcome of the differential load
conditions on both the left and the right ventricle (RV).
• Septum being the common wall between the two pumps is
• the first to undergo alteration in
• position (sphericalyDshapedybanana- shaped LV)
• and
• motion (moving with LV or RV)
• and
• earliest to reflect the histological changes.

82. • A prepared LV -
• the LV geometry is maintained (sphericalyD-
shaped)
• Septal motion with the LV.
• Regressed LV-
• When the LV geometry is altered (banana-
shaped),
• with septal motion with RV,

83. • Children with TGA and
prepared LV - EM features:
• – prominent Z bands
• – uniform round
mitochondria
• – few fat vacuoles and
glycogen granules
• – minimal sarcoplasmic
vacuolation and
• – minimal collagen in the
background.
• Children with TGA and
regressed LV - EM features:
• – Z-band disruption
• – non-uniform elliptical
mitochondria
• – myofibrillary disarray
• – an abundance of fat
vacuoles
• – sarcoplasmic vacuolation
and
• – abundant collagen in the
background.

84. With the gradual involution of pulmonary vascular
resistance following birth,
there is a progressive reduction in afterload to the left
ventricle in dTGA IVS;
left ventricular mass does not progress in a normal
manner.

85. • Children with TGA and intact ventricular
septum (TGA-IVS)
• presenting for surgery beyond the first few
weeks of life -
• considered at high risk for left ventricular
failure after an ASO
• Concern about the ability of the left ventricle
(LV) to support the systemic circulation.

87. • The cardiac myosite of the infant is capable -
Of a hyperplastic response to pressure and
volume loading
• Angiogenesis accompanies myosite
proliferation up to 3 to 6 months of age.
• A more physiologic type of accomodative
response than one of pure hypertrophy that
results only in increase in cytoplasmic volume
of the myosite.

89. • Primary arterial switch in the first month of life is the preferred
treatment for TGA .
• For patients presenting beyond the neonatal period, anatomic
correction - in two stages.
• disadvantages of 2 STAGE –
• band migration,
• proximal pulmonary artery distortion- RVOTO
• pulmonary annular dilatation resulting in neoaortic valve
regurgitation.
• need for a prosthetic tube to bridge the gap in the neopulmonary
artery.
• The demonstration of the rapidity of cardiac myosite hypertrophy in
the biochemical laboratory followed by successful application of
the concept to the clinical setting has resulted in the
• rapid two-stage arterial switch operation

90. • TGA-IVS up to 21 days of age, the LV can sustain the
• systemic circulation.
• late diagnosis and referral for ASO is a major problem.
• In older children with TGA-IVS alternative strategies, such
as the rapid two-stage arterial switch have generally been
• used,
• It has been demonstrated that primary ASO has a better
outcome in comparison to rapid two-stage ASO

91. • The shape of the left ventricle and IVS motion is a
reversible phenomenon
• and can be reversed by pharmacological means and/or
by the use of ECMO.
• Postoperative echocardiography followup
• interventricular septal motion along with the left
ventricle within a few weeks time (mean 1.5 months).
• Rapid left ventricular hypertrophy in the early
postoperative period has also been documented.

95. • There is no exact age cutoff for primary ASO.
• Banana-shape of the LV in echocardiogramm
• and low LV-pressure are obviously not absolute contraindications
for an ASO in simple TGA at least up to 3 months of age.
• Trial of pulmonary banding seems to be a reliable indicator as to
whether primary ASO will be tolerated or not.
• If LV failure does not occur after 15–30 min, a ‘one-stage’ ASO
appears to be possible.
• If however, a two-stage procedure should become necessary,
• the secondary ASO should be done early within the second week
after the
• banding in order to operate before the development of severe
adhesions.

103. Repair of Left Ventricular Outflow Tract
Obstruction

104. When obstruction is dynamic and LV systolic pressure is
similar to or less than that in the right (systemic) ventricle,
nothing is done directly to LVOTO.
When LV systolic pressure is considerably higher, surgical relief of
LVOTO is required.

105. When the LVOTO is in the form of localized or diffuse
fibromuscular obstruction, the obstructive tissue is resected.
One approach is through the mitral valve
after creating the septal flap (Senning repair)
or
excising the atrial septum (Mustard repair).
Alternatively, resection is performed through the pulmonary
trunk and valve.
In the uncommon circumstance of valvar obstruction, valvotomy
through the pulmonary trunk is performed.
When LVOTO is severe and cannot be relieved by resection,
placing an LV–pulmonary trunk allograft valved conduit is
required.

106. RASTELLI OPERATION

107. Rastelli operation for transposition of great arteries, ventricular
septal defect (VSD), and left ventricular outflow tract
obstruction.
A conduit is prepared using an estimate of the largest size of
extracardiac conduit that can be comfortably placed within the
patient’s thorax.
A valved conduit is preferred,
and options include
pulmonary or aortic valved allografts and
composite grafts using either woven polyester or PTFE conduits
with bioprosthetic valves

108. After standard median sternotomy incision,
pulmonary trunk and left and right pulmonary arteries are
completely dissected away from aorta and surrounding
structures.
site of proposed right ventricular infundibular incision, which is
in line with most anterior aspect of aorta.

109. Standard cardiopulmonary bypass and
myocardial protection techniques are used.
Right ventricular infundibular
incision is made and retraction sutures
placed.
Through this incision, rightward and
anterior ascending aorta can be seen and
leftward and posterior pulmonary valve can
be visualized through VSD.
Dashed line on rim of VSD shows site of
incision on ventricular
septum where VSD is enlarged in
preparation for left ventricular to aortic
baffle. This incision is necessary only when
VSD is small (less than
60% aortic diameter).
Dashed line on pulmonary trunk indicates
proposed site of transection at sinutubular
junction.

110. Pulmonary trunk
has been transected and proximal pulmonary trunk
oversewn at level of valve.
VSD, which has been enlarged by incision, is shown
with tunnel from left ventricle to aorta partially
constructed.
Material for tunnel is fashioned from a tube of
polyester with a diameter approximately
the size of ascending aorta. After tailoring, this results
in a naturally curved baffle that is positioned with
convex aspect of baffle facing into right ventricle.
Lower aspect of baffle is sewn around rim of VSD,
taking standard precautions with respect to inlet valves
and conduction system
Upper aspect of baffle is sewn into place by
transitioning suture line away from rim of VSD as patch
approaches aortic valve anulus on each side.
Baffle is then sewn to immediately subaortic region
along lateral aspects, and then finally anterior aspect,
of circumference of aorta.

111. Left ventricular to aortic baffle suture line completed.
A valved allograft conduit (or other composite conduit) is used to reconstruct
right ventricular outflow tract.
Conduit is tailored to appropriate length and is sewn end to end to pulmonary
trunk with a running suture.

112. Proximal end of valved conduit is connected to distal aspect of right ventricular
infundibulum incision with a running monofilament suture.
This suture line covers approximately 30% of circumference of conduit along its
posterior aspect.
A roughly triangular patch of polyester (or allograft arterial wall) is used to close
remainder of right ventricular to pulmonary trunk connection.
This is sewn into place around lateral and anterior aspects of circumference of
proximal edge of conduit, and then around cut edges of infundibular incision.

113. AORTIC ROOT TRANSLOCATION
(NIKAIDOH)

114. For patients with TGA and LVOTO with or without VSD,
aortic root translocation in view of
concerns about long-term outcomes of the Rastelli
procedure.
aortic root is detached
from the RV along with the coronary arteries and translocated
posteriorly after making a septal incision or enlarging
the VSD, then patching it, thereby relieving the LVOTO.
pulmonary outflow tract
is then reconstructed with an allograft valved conduit or a
valveless patch.

115. Ventricular and aortic incisions required
for aortic autograft excision. infundibular
incision is circumferential just below
aortic anulus
Coronary ostia are excised as circular
buttons from respective sinuses of
Valsalva.
Once aortic autograft is excised and
coronaries mobilized, pulmonary trunk is
transected and an incision is extended
across pulmonary valve anulus and
septum connecting to ventricular septal
defect (VSD), if present.
Enlargement of left ventricular outflow
tract is then accomplished by inserting a
triangular-shaped VSD patch.

116. Aortic autograft is reinserted into
left ventricular outflow tract. It is
then rotated 180 degrees so that
defects from coronary buttons face
anteriorly. Coronaries are then
reimplanted.
(Lecompte maneuver) is done prior
to this anastomosis in preparation
for right ventricular outflow
reconstruction.
Right ventricle (RV) to pulmonary
trunk continuity is
achieved by inserting an
interposition allograft connecting RV
infundibulum to pulmonary trunk.