D-Transposition, also known as dextro-Transposition of the great arteries (d-TGA), is a congenital heart defect where the ventricles are connected to the wrong great arteries. Specifically, the aorta arises from the right ventricle while the pulmonary artery arises from the left ventricle. This causes two parallel circulations instead of the normal series circulation. The basic embryological defect is abnormal development of the conus, which prevents normal septal formation between the great arteries. Untreated d-TGA is fatal in infancy due to lack of oxygenated blood to the body. Clinical presentation depends on the degree of mixing between the circulations.

1. D–Transposition

2. D -TRANSPOSITION
• Atrioventricular concordance with ventriculo-arterial
discordance
• aorta arises from a morphological RV, and the PA arises
from a morphological LV
• Abnormal spatial relationship of the great arteries
• two circulations in parallel

3. • dextroposition of the bulboventricular loop (ie, the
position of the RV, which is on the right side).
• aorta =right and anterior
• great arteries are parallel rather than crossing
•

4. Basic Embryological defect
• Abnormal development, growth, and
absorption of the distal infundibulum (conus)
• normal conus is subpulmonary, left sided, and
anterior= prevents fibrous continuity between
the pulmonary and tricuspid valve rings
• infundibulum is usually subaortic, right sided,
and anterior= prevents fibrous continuity
between the aortic and tricuspid valve rings

5. Embryology
1. Spiral aortico-pulmonary septum forms but does not
spiral or twist during its partitioning of the truncus
arteriosus
a. Aorta arises from right ventricle
b. Pulmonary trunk arises from the left ventricle
2. a. Systemic – unoxygenated – repeatedly re-circulated
b. Pulmonary - oxygenated - repeatedly re-circulated

8. • 4 truncus and 2 conal cushions develop.
• Dextro- sinistro cushions of both conus and truncus fuse to
form Conotruncal septum.
• Intercalated cushions play an role in formation of semi lunar
valves
Embryology - Septation of conus and truncus.

9. Embryology - Septation of conus and truncus.
• Because the cushions are
dextro-superior and sinistro
inferior in truncus and dextro-
dorsal and sinistro-ventral in
conus union forms a spiral
septum than true lineal
relation.

10. • Aorta will be in connection with RV and PA with LV.
• There are two rotations one at conoventricular junction
and other at Conotruncal junction.
• Both rotations are counterclockwise around 110º
Embryology - Rotation and absorption.

11. • Conoventricular rotation brings aorta in continuation with
LV and PA with RV.
• Conotruncal rotation brings the normal position of aorta in
relation to PA ( left and posterior to PA)
Embryology - Rotation and absorption.

13. • selective Resorption of conal septum
• coni which gets selectively resorbed during
development that respective artery is drawn
over LV .

16. 1. Absence of both conoventricular and Conotruncal rotation
2. Persistence of sub aortic and complete resorption of sub
pulmonic coni

17. • abnormal growth and development of the SA
infundibulum and the absence of growth of the SP
infundibulum.
• AV is protruded superiorly and anteriorly by the
development of the SA infundibulum, placing it above
the anterior RV
• Failure of development of the SP infundibulum
prevents the normal morphogenetic movement of the
PV from posterior to anterior and further results in
abnormal PV to MV ring fibrous continuity.

18. • Jatene =TGA and VSD.
• LECOMPTE = direct anastomosis of both great
arteries without interposition of a tube when
the pulmonary bifurcation is transferred in front
of the distal ascending AA.

19. Epidemiology
• 20.1 to 30.5 per 100,000 live births
• 60% to 70%= male
• 5% to 7% of all CHD
• males than in females =3:1

20. Pathophysiology

22. • physiologic L-to-R shunt =
volume of the PV blood
recirculating through the lungs
without having passed through
the body
• physiologic R-to-L shunt -
volume of SV blood reentering
the systemic circulation
without having passed through
the lungs.

23. • The net volume of blood
passing from the pulmonary
circulation (LA, LV, PA) to the
systemic circulation (RA, RA,
aorta) represents the
anatomic L-to-R shunt
• The effective systemic blood
flow (i.E., Oxygenated PV
return perfusing the
systemic capillary bed)

24. • The net volume of blood
passing from the systemic
circulation to the pulmonary
circulation represents the
anatomic R-to-L shunt
• The effective PBF (SV return
perfusing the pulmonary
capillary bed).

25. • deficient oxygen supply to the tissues
• excessive right and LV workload.
• incompatible unless mixing at some anatomic
level.

26. Anatomy
• TGA {S,D,D} –
• situs solitus (S)
• (D) looping of the ventricles
• anterior and rightward (D) aorta

27. Great artery relationship
• aortic root - anterior or anterior and to the right of
the pulmonary trunk in a slightly oblique relationship
(S,D,D)
• Less commonly- aorta may be positioned anterior
and to the left (S,D,L).
• rare - aorta is posterior

29. Atria
• normal internal anatomy.
• Right atria is larger , particularly when IVS is
intact.
• Almost always=PFO
• 5% - true secundum ASD
• sinus and AV nodes =usual locations.

30. Right Ventricle
• hypertrophied, and large
• 90% =subaortic conus (infundibulum)
• less wedging of the pulmonary trunk between mitral and
tricuspid valves
• atrioventricular (AV) valves may be at virtually the same level
• 10% of hearts with TGA and IVS= subaortic conus in the RV is
absent or very hypoplastic.

31. Left Ventricle
• infrequently contains an infundibulum (conus
• PV-MV fibrous continuity exists
• RV wall is considerably thicker than normal at birth
and increases in thickness with age.

32. • IVS is intact and no PS -- LV wall is of normal thickness at birth
• less than normal thickness within a few weeks of birth
• thin wall by age 2 to 4
• VSD is present = LV wall thickness increases slightly less than in
the normal heart but remains well within the normal range
during the first year of life
• LV cavity is the usual ellipsoid in shape at birth but soon
becomes banana shaped.

33. Conduction System
• AV node and bundle of His lie in a normal position,
although the AV node is abnormally shaped and may
be partly engulfed in the right trigone
• LBB originates more distally from the bundle of His
• Damage to the bifurcation of the bundle at VSD
closure – CHB

34. Coronary Arteries
• aortic sinuses that face the pulmonary trunk,
regardless of the interrelationships of the great
arteries
• LAD and LCx arise as a single trunk (LCA]) from aortic
sinus 1 and distribute in a normal manner
• RCA = sinus 2 and follows this artery's usual course.
• single coronary artery= sinus 2.

37. • Usual =single ostium in the center of the sinus
• may arise from a double-barreled ostium consisting
of two ostia immediately adjacent to each other and
constituting essentially a single ostium
• At times, the LCA or LAD passes forward between
aorta and pulmonary trunk in an intramural course
to emerge anteriorly.

38. • conus artery frequently arises separately and
from its own ostium in sinus 1. ==considerable
part of the anterior wall of the infundibulum of
the RV.
• sinus node artery =atrial switch (Mustard or
Senning) = arises from the RCA close to its origin
and passes superiorly and rightward, usually
partly embedded in the most superior portion of
the limbus of the atrial septum, where it can be
damaged if this portion of the atrial septum is
widely excised

41. Coexisting Anomalies
• 50% =no other anomaly except a PFO or a PDA.
• VSD =-40% to 45%.
1. - perimembranous (conoventricular 33%)
2. - AV canal (inlet septum 5%)
3. - muscular (27%)
4. - malalignment (30%)
5. - conal septal hypoplasia type (5%)

42. Ventricular Septal Defect
• Conoventricular defects of the several different
varieties
• some hearts with conoventricular VSDs=outlet
(conal, infundibular) septum is malaligned and fails
to insert within the Y of the septal band
• septum may be displaced leftward= LVOTO
• Rightward=n RV (subaortic) obstruction.

43. • conal septum is displaced to the right= pulmonary
trunk may be biventricular in origin and over a
juxtapulmonary VSD and may be associated with
subaortic stenosis or aortic arch obstruction (arch
hypoplasia, coarctation, or interruption).
• Occasionally the VSD is juxta-aortic and associated
with a malaligned but nondisplaced conal septum.
• The conal septum may be absent and the VSD is
then juxta-arterial (doubly committed).

46. LV Outflow Tract Obstruction
• subpulmonary obstruction
• dynamic or anatomic
• 0.7% of patients with TGA and intact ventricular septum
• 20% of patients born with TGA and VSD
• apparent or develop after birth in other patients,
• overall prevalence of 30% to 35%.
• Dynamic type of LVOTO, developing in patients with TGA and intact ventricular
septum= leftward bulging of the muscular ventricular septum secondary to
higher RV than LV pressure.

47. Subpulmonary Stenosis or LVOTO
• Fixed
-Circumferrential fibrous membrane /diaphragm
- Fibromuscular ridge
- Herniating tricuspid leaflet tissue
- Anomalous MV septal attachments
- Tissue tags from membranous septum
• Dynamic-associated with SAM

50. Subaortic Obstruction
• Rightward and anterior displacement of the infundibular
septum
- hypoplasia
- coarctation
- interruption
Asso. RV hypoplasia & tricuspid valve anomalies

51. Aortic Obstruction
• discrete (coarctation, or less often interrupted aortic
arch)
• distal arch hypoplasia.
• 7% to 10% of TGA and VSD.
• more frequent when the VSD is juxtapulmonary and
the pulmonary trunk is partly over the RV in
association with rightward and anterior displacement
of the infundibular septum and with some subaortic
narrowing.
• associated coarctation= underdevelopment of the RV
sinus is more common.

52. Patent Ductus Arteriosus
• PDA-more common
• Persistence of a large PDA for more than a few
months = increased prevalence of pulmonary
vascular disease.

53. • PDA is present at age 1 week =half
• thereafter the prevalence falls rapidly.
• When patent, the ductus is small (less than 3 mm in diameter)
in two thirds of patients =little influence on natural history.
• Large = LV output is increased and hypoxia lessens=heart
failure
• acute and often early closure of the ductus --sudden increase
in hypoxia and clinical deterioration = decreased mixing at
ductus level but also at atrial level because of the fall in left
atrial pressure that results from decreased pulmonary venous
return.

54. TV anomalies
 31%
 Functionally imp 4%
 Ratio of tricuspid to mitral anulus circumference is less than 1
in 50%
 normal hearts this ratio -greater than 1

55. TV anomalies
• Straddling/overriding of chordae
• Overriding of the tricuspid annulus
• Abnormal chordal atatchments
• Dysplasia
• Accessory tissue
• Double orifice

56. MV anomalies
20%
Functionally imp 4%
– Cleft anterior mitral valve leaflet
– anomalous papillary muscles and chordae
– Straddling
– redundant tissue tags

57. Juxtaposition of atrial appendages
• Both appendages or left + part of right are adjacent
• 2-6%
• dextrocardia, VSD, bilateral infundibulum, right
ventricular hypoplasia and tricuspid stenosis or
atresia.
• Imp in BAS

59. Right Aortic Arch
• 5%
• more common when there is an associated
VSD than when the IVS
• associated leftward juxtaposition of the atrial
appendages.

60. Bronchopulmonary Collateral Circulation
• > 30% of infants with TGA under 2 years of age
• functionally and freely communicate with the pulmonary vascular bed
proximal to the pulmonary capillary bed
• Potential intercirculatory (systemic-to-pulmonary) mixing pathway,
• accelerated and more widespread PVD
• Persistence of a significant BPC circulation after surgical repair - large
enough left-to-right shunt – CCF - warrant catheter embolization

61. • contribution to PBF from the BPC circulation
enters the pulmonary vascular circuit distal to the
usual catheter sampling sites
• true mixed PA saturation present at the
precapillary level cannot be sampled
• falsely high PA oxygen saturation and blood flow
calculation will result
• A modest BPC Circulatation(20%) to the pulmonary
precapillary blood flow can result in 30%
overestimation of PBF.

62. Fetal circulation
• compatible with normal fetal survival and relatively
normal gestational development.
• course of fetal circulation is modified
• right side of the heart ejects blood directly into the
ascending aorta
• RV ejects into the descending aorta via the PDA in normal
• cardiac and CNS structures similar in size and weight to
control values
• increased numbers and size of pancreatic islet cells
• increased weight of the adrenal cortex
•

63. Neonatal transition
• After birth, the PVR falls
• PBF and LA pressures increase
• more or less normal neonatal transitional physiology
• SVR increases because of removal of the low-resistance placental
circulation
• With TGA= RA pressures are increased( contrast to normal) and the
similarity of atrial pressures tends to keep the PFO open (incompetent
valve), with resulting bidirectional shunting
• TGA with IVS- ductus arteriosus is often widely patent after birth.
• Early after birth, when PVR is still high, there is bidirectional ductal flow:
• systole, LV–PA–ductus–-descending aorta
• diastole, aorta–ductus–PA.

64. Natural history
• 1st week-30%
• 1st month-50%
• 1st year-90%
• Depends on the degree of shunting
• Moderate PS improves survival

65. Survival
• all varieties
• 55% survive 1 month
• 15% survive 6 months
• 10% survive 1 year.
• Mean life expectancy = 0.65 year
• 4 years for those who survive to 12 months
• 6 years for the few who survive for 18
months
• Thereafter, life expectancy declines rapidly.

66. ]TGA and essentially intact ventricular septum
80% at 1 week
only 17% at 2 months
4% at 1 year
better when there is a true ASD.
TGA and important VSD
• 91% at 1 month
• 43% at 5 months
• 32% at 1 year
• lower when the patient has a very large Qp.

67. • large VSD and aortic obstruction (coarctation, interrupted arch)
• Lethal
• all patients die within a few months with severe heart failure.
• In patients with TGA, VSD, and LVOTO, early survival is still
better, reaching 70% at 1 year and 29% at 5 years, because in
many LVOTO is only moderate initially.
• Leibman and colleagues found that PDA increased risk of early
death in all subsets of patients. This is particularly the case when
the ductus is large.

69. CLINICAL COURSE IN COMPLETE TGA
• CYANOSIS,
• HYPOXEMIC DETERIORATION,
• HEART FAILURE WITH EARLY DEATH
Inter circulatory mixing

70. CLINICAL FEATURES
• Symptoms and clinical presentation - degree of mixing
• high degree of mixing and large PBF- Qp, SaO2 may be near normal,
and unless there is pulmonary venous hypertension, symptoms are
minimal.
• When mixing is minimal= SaO2 is low and symptoms of hypoxia are
severe
• Adequate mixing =communications of reasonable size at atrial,
ventricular, or great artery levels
• Factors that reduce Qp, such as LVOTO and increased PVR= reduce
mixing and increase cyanosis.

71. • Reverse differential cyanosis
– TGA with a PDA and PA-to-aorta shunting
– complex TGA malformation including an aortic
arch anomaly, such as COA or IAA.
– TGA with suprasystemic PVR.

72. CLINICAL SPECTRUM
1. TGA (IVS or small VSD) with increased PBF
and small ICS
2. TGA (VSD large) with increased PBF and large
ICS
3. TGA (VSD and LVOTO), with restricted PBF
4. TGA (VSD and PVOD), with restricted PBF

73. Essentially TGA IVS
(Poor Mixing)
• infants with out a VSD or with a VSD 3 mm or less in
diameter.
• PFO or naturally occurring ASD +
• Cyanosis - half these infants within the first hour of life
• 90% within the first day and is rapidly progressive.
• critically ill with tachypnea and tachycardia and dies
from hypoxia and acidosis without appearance of frank
heart failure.

74. • rapid downhill course is usually obviated with a
naturally occurring ASD of adequate size because
cyanosis is less severe.
• surviving infants, appearance of moderate or severe
dynamic LVOTO -- increasing cyanosis and hypoxic
spells, even after an adequate atrial septostomy
• patients are of average birth weight and in good
general condition although with severe cyanosis
• Clubbing of fingers and toes is absent and generally
does not appear unless the infant survives to about age
6 months
• There is mild increase in heart and respiratory rates

75. • The heart is not hyperactive, and the liver is barely
palpable
• faint mid- systolic ejection-type murmur is present along
the midleft sternal edge in less than half these infants
• more prominent with organic or dynamic LVOTO, first
appearing at age 1 or 2 months with the dynamic form
and then gradually increasing in intensity.
• The second heart sound is unremarkable (often
apparently single or narrowly split).

76. ECG
• often normal at birth.
• By the end of the first week -- persistence of
an upright T wave in right precordial leads
indicates abnormal RV hypertrophy, and right-
axis deviation predominates.
• When important LVOTO is present or PVR
elevated, ECG evidence indicates biventricular
hypertrophy.

77. CXR
• • An oval- or egg-shaped cardiac silhouette with a
narrow superior mediastinum
• Mild cardiac enlargement
• Moderate pulmonary plethora
• first week of life-CXR may be normal, or occasionally
cardiac enlargement may be more marked.
• narrow mediastinum - great artery positions and by
shrinkage of the thymus, usually associated with stress,
and the plethora is caused by the increase in Qp.
• Plethora is less marked -LVOTO.

78. CXR
• TGA/IVS
(a) oval or egg-shaped cardiac silhouette with
narrow superior mediastinum
(b) mild cardiomegaly
(c) increased pulmonary vascular markings

80. D TGA
CHEST X RAY IN DIAGNOSIS OF CARDIAC
CONDITIONS

81. Large VSD, Large PDA, or Both (Good Mixing)
• Presentation -latter half of the first month
• mild cyanosis
• signs of HF -PVH and myocardial failure
• Tachycardia, tachypnea, important liver enlargement,
and moist lung bases
• heart is more active
• larger than in the poor-mixing group
• large VSD =moderate-intensity pansystolic murmur
along the lower left sternal edge that may not be
present initially.

82. • apical middiastolic murmur or gallop rhythm
• narrow splitting
• accentuation of p2
• large PDA-continuous murmur, bounding
pulses, and an apical middiastolic murmur are
=less than half the patients, even when the
ventricular septum is intact.

83. • more cardiomegaly
• more plethora
• wider superior mediastinum than in the poor-mixing
group.
• ECG =BVH
• persistent large VSD=Q wave in V6.
• Isolated LV hypertrophy =RV hypoplasia with
tricuspid valve overriding
• Development of PVD = reduction in Qp and less
plethora, particularly in the peripheral lung fields, as
well as reduced heart size, but these features
generally appear after the neonatal period.

84. • coarctation with VSD and PDA= femoral
pulses are usually normal because the
coarctation is preductal and ductus arteriosus
large.
• Rarely, differential cyanosis can occur, with
cyanosis confined to the upper torso.

85. Large VSD and LVOTO (Poor Mixing without High PBF)
• least common of the three TGA groups
• decreased Qp and poor mixing
• PVH and associated symptoms and signs do not
develop
• Heart failure is -- not present.
• cyanosis is severe from birth.

86. • heart is not overactive
• pulmonary ejection murmur
• single S2
• apical gallop or middiastolic murmur
• near normal-sized heart with normal or
ischemic lung fields.
• ECG-biventricular hypertrophy.

87. TGA VSD Pulmonary Vascular Disease
• simple TGA= PVOD rarely develops in the first few months of life
• After 6 to 24 months=prevalence increases to 10% to 30%.
• TGA and moderate or large VSD= PVD develops more rapidly, as it
does in those with persistently large PDA
• Among those dying at about age 6 months, 25% have developed
severe pulmonary vascular disease (grade 3 or greater), and 50% of
infants dying by age 12 months have developed it.

88. Echocardiography
• dynamic LVOTO
• leftward deviation of the VS
• abnormal fluttering and premature closure of the PV
• SAM of the mitral leaflet
• prolonged diastolic apposition of the AML to the septum.
• coronary arteries=number, origin, major branching pattern, ,
intramural course.

89.  Coronary artery anatomy;
 Morphologic details of pulmonary or
subpulmonary obstruction;
 VSD number, site, and size;
 Great vessel alignments in relation to the
VSD and outlet septum;
 Type and severity of aortic arch
abnormalities
 Inter atrial communication

93. The RAA lies posterior and to the left of the GA and anterior to the LA.
The posterior portion of the atrial septum (a) is oriented normally; the anterior portion (b) is
oriented transversely and parallel to the anterior chest wall.
The atrial septum curves toward the right. The LA and RPV wrap around the posterosuperior
aspect of the RA

94. Cardiac Catheterization and
Cineangiography
• SBF and PBF and pressures, including those across the
LVOT.
• Using appropriate views, cineangiography demonstrates
the cardiac connections and great artery positions
position and number of VSDs site of any LVOTO the size
and function of AV valves, size and function of both
ventricles, and presence of other cardiac anomalies.

95. Indications of cardiac catheterization
• hemodynamically unstable and rquire BAS.
• physiologic and anatomic data is required
concerning coronary artery, the VSD, degree of
LVOTO.
• complex cardiac anomalies like CoA, interrupted
aortic arch.
• pulmonary vascular resistance in TGA with VSD

96. • oxygen saturation in the pulmonary artery is
always higher than in the aorta.

97. • TV - selective RV injection in the frontal or
RAO views by noting intra-atrial bulging of the
leaflets during ventricular systole and during
diastole by the negative silhouette of the
orifice as nonopacified blood enters the
ventricle
• Continuity of the anterior leaflet of the MV
with the PV = four-chamber long-axial or LAO
views in diastole when the anterior leaflet is
noted to form the posterior wall of the LVOT.

98. Catheterization
 PAP
 PBF(and PVR ); ( overestimate PBF and
underestimate PVR)
 Coronary artery anatomy;
 Morphologic details of pulmonary or
subpulmonary obstruction;
 VSD number, site, and size;
 Great vessel alignments in relation to the VSD
and outlet septum;
 Type and severity of aortic arch abnormalities

99. Coronary artery anatomy
• Selective transvenous CAG or antegrade aortic
root angiography with distal balloon occlusion of
the ascending aorta.
» balloon angiographic catheter introduced transvenously, is
positioned in the ascending aorta
» balloon is inflated with CO2 and stabilized in the ascending
aorta.
» 0.5 and 1.0 mL/kg of contrast medium are injected over 1
second
» balloon is deflated immediately after the injection.

100. • Balloon occlusion aortography
with extreme caudal
angulation of the anterior-
posterior camera, - laid-back
aortogram .
• visualization of both the
proximal ostia and distal
distribution of the coronary
arteries in the same plane of
view

101. Medical management
• hemodynamic stabilization
• correction of physiological aberrations caused
by cyanosis and poor perfusion.
• Correction of acid base balance, maintainance
of normothermia, prevention of hypoglycemia.

102. Management
• Airway protection,
• Oxygen and ventilatory care
• Acid-base correction,
• PGE1 infusion,
– 0.01-0.02 microg/kg infusion
– Apnea, fever , hypotension
• Antibiotics,

103. • PGE1 infusion to maintain patencty of ductus
• BAS

104. Palliative procedures
• D-TGA/IVS –poor I.C.M.despite patent PDA-
• Balloon atrial septostomy,
• surgical septectomy
• partial venous correction,
• D-TGA/IVS/ICM depends on PDA-but it is
closing
• PGE1 infusion.
• D-TGA/IVS/LVOTO-
– systemic-to-pulmonary shunts.
• D-TGA/VSD(multiple)-
– pulmonary artery banding,

105. Balloon Atrial Septostomy
• profound hypoxemia
• when corrective surgery must be delayed.
• catheter should be advanced across the foramen ovale
into the LA or a PV and the position of the tip
established with certainty in the LA prior to proceeding
• posterior position of the tip in the lateral or LAO view or
entry of the catheter into a PV

106. • balloon is inflated with diluted angiographic
contrast medium to 12 to 15 mm diameter
• rapidly withdrawn across the atrial septum with
an abrupt, short tug
• balloon and interatrial septum are displaced
toward the inferior vena cava, and the septum
primum flap of the fossa ovalis is ruptured as
the balloon is carried in a single movement
from the left atrium to the right atrial-inferior
vena caval junction.

107.  The catheter should be advanced immediately and
the balloon pushed cephalad out of the IVC orifice
into the RA toward the SVC to verify crossing the
septum and to avoid obstruction to IVC return while
the balloon is being deflated.
 This same procedure should be repeated several
times with increasing balloon volumes so that
withdrawal of the balloon, inflated tensely to a
diameter of 15 mm, is achieved without much
resistance being perceived at the atrial septum level.

109. COMPLICATIONS
• Atrial wall, Pulmonary vein, inferior vena caval
perforation or tears or AV valve damage.
• Intracardiac rupture of the balloon.
• Air embolism
• CNS complications

110. Difficulties
• older infants, particularly those with
TGA/VSD=thickened interatrial flap =catheter equipped
with an extendable blade
• markedly thick atrial septum, a new defect (separate
from the foramen ovale) -transseptal needle and
dilated with an 8- to 15-mm-diameter balloon
angioplasty catheter (Brockenbrough angioplasty).

112. Surgical Creation of ASD
• Blalock & Hanlon operation or one of its
modifications –
• historical footnote
• excision of the posterior aspect of the IAS.
• oxygen saturation levels modestly higher than BAS
• mortality risks <3% to 5%.

113. Pulmonary Artery Banding
large VSD without LVOTO
To prevent
Heart failure
Pulmonary vascular disease
Present Indications
complex/multiple VSDs
Coexisting medical conditions that cause a delay in
surgery
To train LV before switch in TGA/IVS

114. Partial Venous Return Repair (Baffes)
• connecting the IVC to the LA with a homograft or
synthetic conduit and concurrently detaching the
right pulmonary veins and directly transferring them
to the RA.
• This results in an obligate, effective shunt at the
atrial level.
• Subsequently, some of these patients had a modified
Mustard type of atrial repair to achieve complete
physiologic correction.

115. Aims of surgery
1.To make the parallel circulations into series. So
that oxygenated blood goes to aorta and
deoxygenated blood goes to pulmonary trunk.
2.Correction of other cardiac anomalies like VSD,
PDA, TR, AORTIC OBSTRUCTION, LVOTO.
3.To provide a near normal functional status to
patients.

116. Definitive Repair
1. The atrial level : Senning or Mustard sx
2. Great artery level : Arterial switch operation or jatene
operation

117. Atrial switch
• Removal of the atrial septum
• redirection of the SV pathways to LV
• PV blood to RV
• Senning operation= rerouting by infolding of the atrial walls
• Mustard =synthetic or pericardial tissue
• modifications of the Mustard = trouser-shaped baffle, with
the legs anastomosed to the SVC & IVC inflows.

119. • early post-operative survival exceeding 95%.

120. PHYSIOLOGIC CORRECTION (ATRIAL
SWITCH)
• atrial switch operations may be delayed for a few
weeks to several months after birth (and balloon
atrial septostomy).

124. The patch should not
touch the lateral atrial wall
7/22/2018

125. Final
7/22/2018

126. Results and Sequelae of Physiologic
Correction
• 10-year survival rates of 85% to 90%.
(a) residual intra-atrial shunts,
(b) caval and pulmonary venous obstructions,
(c) right ventricular dysfunction,
(d) tricuspid valve insufficiency, and
(e) arrhythmia.

127. • Trivial leaks -10% to 20% of patients,
• Significant leaks requiring reoperation -(1 to 2%).
• RV dysfunction to some extent and dysrhythmias
=late concerns

128. • Progressive loss of NSR and increase in atrial
rhythm disturbances
• Gradual time-related decrease in sinus rhythm
• sinus rhythm at 1 year was 72%, at 5 years
56%, at 10 years 50%, and at 13 years 43%

129. Arterial switch operation (Jatene operation)
Advantages
• Fewer long-term complications
– Arrhythmias
– RV dysfunction
– Baffle stenosis
– Tricuspid regurgitation (TR).
– LV as systemic ventricle & MV as Systemic AV valve

131. • functional adequacy of the LV??
• Adequate LV muscle mass .
1. Early infancy
2. Nonrestrictive PDA,
3. Surgically remediable or dynamic LVOTO
4. Delayed decrease in PVR and persistent PAH
5. A large, nonrestrictive VSD.
• Definitive early (neonatal) one-stage arterial
repair >> early palliation with PA banding and
later arterial switch surgery.

132. Pre requisite
• LV pressure should be near systemic levels
• switch should be performed shortly after birth
(i.e., before 2 weeks of age).
• LV pressure is low= PA banding, either with or
without a shunt, for 7 to 10 days ( rapid, two-
stage switch operation) or for 5 to 9 months

133. 1. absolute LV systolic pressure that is
appropriate for age,
2. A LV pressure at cardiac catheterization = >70%
systemic levels (left to RV ratio >0.7), or
3. LV muscle mass that is within the normal range
for BSA
EMPIRICAL CRITERIA FOR LV SIZE

135. Pre-op
• Coronary artery pattern amenable to transfer to the
neoaorta without distortion or kinking.
• Risk is high when the left main or LAD coronary
artery passes anteriorly between the aorta and the
PA.
• LV inflow and outflow tracts must be free of
significant structural abnormality
• RVOT should be free of significant stenosis.

136. Anatomic variants that may impact operative
mortality include
– intramural course of a coronary artery
– retropulmonary course of the left coronary
artery
– Multiple VSDs
– Coexisting abnormalities of the aortic
– Straddling AV valves
– Longer duration of global myocardial ischemic
(cross-clamp)
– prolonged circulatory arrest times

137. • great arteries are transected
• reanastomosis of the distal aortic segment to the
proximal pulmonary artery (neo aortic root).
• Transfer of the coronary arteries to this pulmonary
segment =excision from the aortic sinus with a cuff of
adjacent aortic wall
• proximal aortic segment (neopulmonary root) connected
to the distal PA segment
• Maneuver of lecompte - passes the anterior aorta
posterior to the bifurcation of the pulmonary artery

138. • aorta is transected, pulmonary trunk is
transected just proximal to its bifurcation
• aortic button around the orifice of the left
main coronary artery is excised from its sinus,
and this is inserted into the left facing sinus of
the neoaorta (originally,pulmonary trunk).

140. Arterial switch

142. Complications
• PA stenosis at the site of reconstruction - 5% to 10%
• CHB- 5% to 10%.
• AR
– late complication > 20% of patients especially PA banding
– unequal size of the pulmonary cusps that leads to eccentric
coaptation
• Coronary artery obstruction
– myocardial ischemia, infarction, and even death.

144. TGA with Low LV Pressure
PA banding ..
Long preparation period.
Rapid two stage switch.
 LV function may be extremely impaired following
banding.( systemic-to-PA shunt is frequently placed to
ensure adequate PBF )
interval period between banding and correction = low
output syndrome.
Clinical improvement coincides with improvement in LV
function such that anatomic correction can be performed
within 7 to 10 days in most cases.

145. OTHER INNOVATIVE APPROACHES
• Percutaneously adjustable band
• Partial balloon occlusion of the MPA with a
percutaneously placed balloon-tipped catheter
• Systemic-to-PA shunting alone
• Primary arterial switch with LV assist in the
perioperative period.

146. Anatomic Correction without Coronary
Translocation
• DAMUS, KAYE, AND STANSEL
• arterial level repair without coronary
translocation.
• children with TGA and coronary artery
patterns not suitable for transfer
• patients with DORV (Taussig bing type) with
severe subaortic stenosis.

147. MPA is transected and
anastomosed to the
ascending aorta.
coronary arteries are perfused
in a retrograde fashion
native aortic valve may be left
intact
VSD (if present) is closed to
direct lv blood to the native
pulmonary (neoaortic) valve
RV to PA conduit is placed to
establish a normal series
circulation

149. Innovative Techniques for anatomic correction with out
Coronary Translocation
• Creation of aortopulmonary tunnel (aubert
procedure)
• Baffling the LV outflow to the nontranslocated
coronary ostia with a patch of native aorta or
pericardium.
• The entire aortic root may be translocated to the
left ventricle with biventricular outflow tract
reconstruction.

150. Transposition of the Great Arteries (VSD and LVOTO)
with Restricted Pulmonary Blood Flow
• Neonates with TGA, VSD, and severe PS or
atresia have diminished PBF.
• They represent a relatively small proportion
(5% to 8%) of the neonatal TGA population.
• Clinical findings are similar to those in the
infant with TOF with severe PS or atresia, and
the cyanosis is extreme from birth

152. Surgery for TGA with Associated LVOTO
• In some neonates, a palliative systemic-to-PA
shunt (Gore-Tex interposition shunt or classic
BT shunt) may be performed, with intracardiac
correction carried out at a later age.
• Alternatively, corrective surgery can be
performed in early infancy

154. RASTELLI OPERATION
• intraventricular repair ++ extracardiac RV to PA
conduit.
• TGA with large VSD and extensive LVOTO -
complete bypass of the LVOTO and an anatomic
correction of the transposition pathology
• operative survival =95%
• midterm survival =90%

157. complications
1. Unfavorable anatomic variants,
-- restrictive VSD
– anomalous TV connections to the infundibular septum that
prevent baffling the LV to the anterior aorta.
2. Residual VSD,
3. Late unexpected death,
4. myocardial dysfunction .
5. ??Functional longevity of the valved conduits.
Improved results are noticeable with fresh or cryopreserved
homograft-valved conduits compared with the previously
used dacron heterograft structures

158. Complications
• conduit obstruction (especially in those containing porcine
heterograft valves)
• complete heart block (rarely occurs).
• This conduit needs to be replaced as the child grows.

160. REV
 Applicable to younger patients ,
 Avoidence of Prosthetic extrcardiac conduit,
 Avoidence of intracardiac Tunnel Obstruction.
• REV approach allows
1. Complete repair earlier in infancy,
2. Is feasible in patients with anatomic
contraindications to the rastelli operation,
3. reduce the need for reoperation and the prevalence
of residual pulmonary outflow tract obstruction
4. The lifelong implications of pulmonary regurgitation
following this newer operative approach require
continued investigation

163. • This operation involves
1. Performing a high, anterior RV incision
2. Radical excision of the outlet septum to create
an unobstructed anterior RV cavity;
3. Establishing a short and direct intraventricular
tunnel from the LV to the aorta
4. Closure of the pulmonary artery orifice;
5. Reimplantation of the transected (and usually
anteriorly translocated) PA directly onto the RV
outflow cavity without a prosthetic conduit

164. Surgery for TGA/VSD and PVOD
• PVR>10 U or grade 4 (H-E) histologic changes is a
CI to VSD closure.

166. SURGICAL OPTIONS DTGA
Anatomy Surgical options Comments
TGA/IVS Physiologic repair
Senning or Mustard
Usually elective, neonatal-1 yr
Anatomic repair (primary)
Arterial switch (Jatene)
Neonatal period, usually within 2 wk
of age
TGA/IVS with prolonged low
LV pressure
Physiologic repair
Senning or Mustard
Usually elective, 1 mo to “1 yr
Anatomic repair (delayed)
Two-stage arterial switch
Long preparation period (Yacoub)
Rapid two-stage switch (Jonas)
TGA/VSD Physiologic repair
Senning or mustard with VSD
closure
Poor long-term results
Anatomic repair
Arterial switch with VSD closure
Usually neonatal repair; PAB
occasionally (multiple VSDs)
Interventricular baffle repair Not all VSDs suitable
Damus-“Kaye-“Stansel: VSD closure
(LVto’PA); proximal PA to Ao
Used when coronary translocation
impossible aortic valve closure

167. TGA/VSD/PS VSD closure (LV to Ao), RV to
PA conduit (Rastelli)
Palliative systemic-to-pulmonary
shunt frequently performed
Conduit replacement frequently
necessary
VSD closure (LV to Ao),
anterior translocation of PA with
direct connection to RV: REV
procedure (Lecompte)
Long-term pulmonary
regurgitation
TGA/PVOD Physiologic repair, palliative
Anatomic repair, palliative
Symptomatic improvement