Вы находитесь на странице: 1из 139

SURGICAL

TECHNIQUES FOR
D -TGA : A REVIEW
Dr.Sashi kanth
D -TRANSPOSITION

 Abnormal origin of the Aorta and Pulmonary Artery


from the ventricular complex

 Atrioventricular concordance with ventriculo-arterial


discordance

 Abnormal spatial relationship of the great arteries

 Results in two circulations in parallel

SURGICAL TECHNIQUES FOR D TGA


Incidence & Prevalence

 5% to 7% of all congenital cardiac malformations

 The incidence is reported to range from 20.1 to


30.5/100,000 live births

 strong male preponderance

SURGICAL TECHNIQUES FOR D TGA


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. Result is two closed circuits

a. Systemic – unoxygenated – repeatedly re-circulated

b. Pulmonary - oxygenated - repeatedly re-circulated

SURGICAL TECHNIQUES FOR D TGA


 The morphogenesis of TGA can be hypothesized to result from the
abnormal growth and development of the subaortic infundibulum and
the absence of growth of the subpulmonary infundibulum.

 The aortic valve is protruded superiorly and anteriorly by the


development of the subaortic infundibulum, placing it above the
anterior right ventricle .

 Failure of development of the subpulmonary infundibulum prevents the


normal morphogenetic movement of the pulmonary valve from
posterior to anterior and further results in abnormal pulmonary to
mitral valve ring fibrous continuity.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Historical aspects
 1stmorphologic description of TGA
:Baillie in 1797.
 The term transposition of the aorta and
pulmonary artery was coined by Farre when
he described the third known case of this
anomaly in 1814, with transposition {trans,
across; ponere, to place) meaning that aorta
and pulmonary trunk were displaced
across the ventricularseptum

SURGICAL TECHNIQUES FOR D TGA


Historical aspects
 In 1953, Lillehei and Varco described a
"partial physiologic correction" (or atrial
switch) consisting of anastomosis of right
pulmonary veins to right atrium and inferior
vena cava (IVC) to left atrium.
 Baffes incorporated use of an allograft aortic
tube to connect IVC to left atrium

SURGICAL TECHNIQUES FOR D TGA


Historical aspects
 Palliation of TGA was revolutionized
when Rashkind and Miller in Philadelphia
introduced balloon atrial septostomy (BAS)
in 1966.

 A modification of this procedure was


introduced in 1975 by Park and colleagues
with their substitution of a blade rather
than a balloon at the end of the catheter.
SURGICAL TECHNIQUES FOR D TGA
Historical aspects
 Surgery of TGA commenced in 1950 when
Blalock and Hanlon at Johns Hopkins
Hospital described a closed method of atrial
septectomy,designed to provide mixing of
pulmonary and systemic venous return at
the atrial level.
 Edwards, Bargeron, and Lyons modified the
Blalock- Hanlon procedure in 1964 by
resuturing the septum so as to connect the
right pulmonary veins to the right atrium
SURGICAL TECHNIQUES FOR D TGA
Historical aspects
 Throughout the 1950s there were surgical attempts to correct TGA either
at the atrial or the great artery levels.

 The concept of a physiologic correction at the atrial level by switching the


atrial septum so that systemic venous return was directed to the LV and
pulmonary venous return to the RV was first proposed by Albert at a
meeting of the American College of Surgeons in 1954.

 This concept was amplified by Merendino and colleagues in 1957. The first
successful operation of this type was accomplished by Senning in 1959,
who refashioned the walls of the right atrium and the atrial septum to
accomplish atrial-level transposition of venous return.

SURGICAL TECHNIQUES FOR D TGA


Historical aspects
 The Mustard procedure, in which the atrial
septum is excised and a pericardial baffle
used to redirect systemic and pulmonary
venous flow, was devised in an attempt to
create larger atria than were produced by
the Senning procedure and was
successfully introduced at the TorontoSick
Children's Hospital in 1963

SURGICAL TECHNIQUES FOR D TGA


Historical aspects
 in 1969, Rastelli and colleagues combined
intraventricular tunnel repair (LV to
aorta) of the double outlet RV operation
with a rerouting valved extracardiac
conduit (RV to pulmonary artery) and
closure of the origin of the pulmonary
trunk from the LV to produce an
anatomic repair of TGA,VSD, and LVOTO

SURGICAL TECHNIQUES FOR D TGA


Historical aspects
 Jatene and colleagues in Brazil achieved a major breakthrough in
1975 with the firstmsuccessful use of an arterial switch
procedure {Jatenemprocedure), applying it in infants with TGA and
VSD.

 An important technical modification of the original Jatene procedure was


the demonstration by LECOMPTE and colleagues that direct
anastomosis of both great arteries without interposition of
a tube graft is possible when the pulmonary bifurcation is
transferred in front of the distal ascending aortic arch.
 Aubert and colleagues successfully used intraarterial baffling and creation
of an aortopulmonary tunnel to correct simple TGA by an arterial switch
in 1978, a technique currently used by some for intramural coronary
arteries.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
Anatomy

 The common clinical type - situs solitus of the atria,


concordant AV and discordant ventriculoarterial
alignments - complete TGA.

 TGA {S,D,D} - TGA with situs solitus (S) of the atria


and viscera, usual (D) looping of the ventricles and an
anterior and rightward (D) aorta.

SURGICAL TECHNIQUES FOR D TGA


Anatomy- Great artery relationship

 Situs solitus and intact ventricular septum - the aortic


root is directly anterior or anterior and to the right of
the pulmonary trunk in a slightly oblique relationship

 Less commonly, the aorta may be positioned anterior


and to the left or, rarely, posterior and to the right of
the pulmonary trunk.

SURGICAL TECHNIQUES FOR D TGA


Anatomy - Coexisting Anomalies

 Nearly half of the hearts have no other anomaly except a


PFO or a PDA.

 The VSD is the most frequent coexisting anomaly-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%)
SURGICAL TECHNIQUES FOR D TGA
VSD

 The subaortic stenosis caused by the anterior


malalignment of the infundibular septum is frequently
associated with aortic arch hypoplasia, coarctation or
even complete interruption of the aortic arch

 Posterior (leftward) malalignment is associated with


varying degrees of LVOTO–subpulmonary stenosis,
annular hypoplasia or even pulmonary valvar atresia

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Subpulmonary Stenosis 25%
 Fixed

-Circumferrential fibrous membrane /diaphragm

- Fibromuscular ridge

- Herniating tricuspid leaflet tissue

- Anomalous MV septal attachments

- Tissue tags from membranous septum

 Dynamic-associated with SAM


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Subaortic Obstruction

 Rightward and anterior displacement of the infundibular


septum

 Associated aortic arch anomalies

- hypoplasia

- coarctation

- interruption

Asso. RV hypoplasia & tricuspid valve anomalies

SURGICAL TECHNIQUES FOR D TGA


TV anomalies

Nearly 31%

Functionally imp 4%

Ratio of tricuspid to mitral anulus circumference is


less than 1 in almost 50% of cases, whereas in normal
hearts this ratio is always greater than 1

SURGICAL TECHNIQUES FOR D TGA


TV anomalies

 Straddling/overriding of chordae

 Overriding of the tricuspid annulus

 Abnormal chordal atatchments

 Dysplasia

 Accessory tissue

 Double orifice

SURGICAL TECHNIQUES FOR D TGA


MV anomalies

Nearly 20%

Functionally imp 4%

◦ Cleft anterior mitral valve leaflet

◦ anomalous papillary muscles and chordae

◦ Straddling

◦ redundant tissue tags

SURGICAL TECHNIQUES FOR D TGA


Juxtaposition of atrial appendages

 Both appendages or left + part of right are adjacent

 2-6%

 often additionally associated with major cardiac


pathology, including dextrocardia,VSD, bilateral
infundibulum, right ventricular hypoplasia and
tricuspid stenosis or atresia.

 Imp in BAS

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
Bronchopulmonary Collateral Circulation

 Bronchopulmonary anastomotic channels > 30% of


infants with TGA under 2 years of age

 Persistence of a significant bronchopulmonary collateral


circulation after surgical repair - large enough left-to-
right shunt – CCF - warrant catheter embolization

SURGICAL TECHNIQUES FOR D TGA


 The RV is normally positioned,
hypertrophied, and large in TGA
 In about 90% of cases, there is a
subaortic conus (infundibulum), and
the aorta is rightward and anterior
and ascends parallel to the posterior
and leftward pulmonary trunk

SURGICAL TECHNIQUES FOR D TGA


 The 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.
 The left bundle branch originates more
distally from the bundle of his than usual and
arises as a single cord rather than a sheath.
 Damage to the bifurcation of the bundle at
VSD closure is more likely to produce
complete heart block than in the normally
structured heart
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
CORONARY ARTERIES
 Usually arise from the aortic sinuses that face the
pulmonary trunk, regardless of the
interrelationships of the great arteries. Thus, the
noncoronary sinus is usually the anterior one.
 Most often the left anterior descending (LAD) and
circumflex (Cx) coronary arteries arise as a single
trunk {left main coronary artery [LCA]) from aortic
sinus 1 and distribute in a normal manner, although
the Cx system is often small
 The right coronary artery (RCA) arises from sinus 2
and follows this artery's usual course.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
 The course of the sinus node artery may be
important in the atrial switch (Mustard or
Senning) operation.
 This artery usually 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
atrialseptum, where it can be damaged if this
portion of the atrial septum is widely excised

SURGICAL TECHNIQUES FOR D TGA


 Development of LVOTO, which produces
subpulmonary obstruction, is part of the
natural history of many patients with TGA.
 The obstruction may be dynamic or
anatomic.
 LVOTO occurs in an important way at birth
or within a few days in only 0.7% of patients
with TGA and intact ventricular septum.
Obstruction is present in about 20% of
patients born with TGA and VSD

SURGICAL TECHNIQUES FOR D TGA


 Dynamic type of LVOTO, developing in patients with TGA and intact ventricular
septum, is the result of leftward bulging of the muscular ventricular septum
secondary to higher RV than LV pressure.

 Dynamic LVOTO is particularly likely to occur if the aorta lies anterior and
more to the left than usual, with increased wedging of the subpulmonary
area.

 The septum impinges against the anterior mitral leaflet in combination


with abnormal systolic anterior leaflet motion (SAM).

 In patients with TGA and intact ventricular septum,rarely a subvalvar fibrous


ridge may produce LVOTO

SURGICAL TECHNIQUES FOR D TGA


 In patients with TGA and VSD, stenosis is
usually subvalvular and valvular.
◦ Subvalvar stenosis is in the form of a
◦ localized fibrous ring,
◦ long tunnel-type flbromuscular narrowing,
◦ muscular obstruction related to protrusion of
the infundibular septum into the medial or
anterior aspect of the LV outflow tract

SURGICAL TECHNIQUES FOR D TGA


Mortality

 1st week-30%

 1st month-50%

 1st year-90%

 Depends on the degree of shunting

 Moderate PS improves survival

SURGICAL TECHNIQUES FOR D TGA


LALL-ALL TGA
L IVS- TGA WITH INTACT SPTUM
T1-MUSTARDS(1963-73)
T2-MUSTARDS(1974-85)
K-ARTERIAL SWITCH. SURGICAL TECHNIQUES FOR D TGA
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,

SURGICAL TECHNIQUES FOR D TGA


Balloon Atrial Septostomy
 Only in cases of profound hypoxemia or in cases when corrective surgery must
be delayed.

 The catheter should be advanced across the foramen ovale into the left atrium
or a pulmonary vein and the position of the tip established with certainty in the
left atrium prior to proceeding.

 Once the position is verified, the balloon is inflated with diluted angiographic
contrast medium to 12 to 15 mm diameter and then rapidly withdrawn across
the atrial septum with an abrupt, short tug.

 The 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.

SURGICAL TECHNIQUES FOR D TGA


 The catheter should be advanced immediately and the
balloon pushed cephalad out of the inferior vena caval
orifice into the right atrium toward the superior vena cava
to verify crossing the septum and to avoid obstruction to
inferior vena caval 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.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
COMPLICATIONS

 Atrial wall, pulmonary vein, or inferior


vena caval perforation or tears or AV
valve damage.
 Intracardiac rupture of the balloon.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
Difficulties
 In older infants, particularly those with TGA/VSD, a thickened
interatrial flap may preclude successful balloon catheter septal
rupture.

 If necessary, a catheter equipped with an extendable blade may be


used to enlarge the interatrial communication prior to
septostomy.

 In patients with a markedly thick atrial septum, a new defect


(separate from the foramen ovale) can be created with a
transseptal needle and dilated with an 8- to 15-mm-diameter
balloon angioplasty catheter (Brockenbrough angioplasty).

 A second hole is recommended because long-term dilation of the


true foramen ovale has been unsuccessful in most cases.

SURGICAL TECHNIQUES FOR D TGA


Surgical Creation of Atrial Septal
Defect
The Blalock & Hanlon operation or one of
its modifications - now assumes the
position of a historical footnote.
 excision of the posterior aspect of the
interatrial septum.
The Blalock Hanlon operation (closed heart)
and atrial septectomy (open heart) produced
systemic arterial oxygen saturation levels
modestly higher than balloon atrial septostomy;
and these increases were well maintained for
long periods.
Early , mortality rates approached 30%,
later series reported mortality risks <3%
to 5%.
SURGICAL TECHNIQUES FOR D TGA
Partial Venous Return Repair
(Baffes)
 Achieved by connecting the inferior vena
cava to the left atrium with a homograft or
synthetic conduit and concurrently
detaching the right pulmonary veins and
directly transferring them to the right
atrium.
 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.
SURGICAL TECHNIQUES FOR D TGA
Pulmonary Artery Banding
Transposition associated with large VSD without LVOTO
To prevent
Heart failure
Pulmonary vascular disease
Present Indications
Presence of complex/multiple VSDs
Coexisting medical conditions that cause a delay in
surgery
To train LV before switch in TGA/IVS

SURGICAL TECHNIQUES FOR D TGA


Pulmonary Artery Banding

 The pulmonary artery in infants with TGA


in general banded more loosely than in
infants with normally related great
arteries.

 Hemodynamically important subaortic


stenosis may develop following pulmonary
artery banding.
SURGICAL TECHNIQUES FOR D TGA
Systemic-Pulmonary Anastomosis
 Infants with TGA/VSD and severe
LVOTO have been successfully
palliated with systemic-to-pulmonary
anastomosis.
 The higher risk for developing
pulmonary vascular obstructive
disease in patients with TGA makes
continuing reassessment of
pulmonary vascular resistance
imperative during follow-up.
SURGICAL TECHNIQUES FOR D TGA
Definitive Repair

AT THREE LEVELS:
 The atrial level : Senning or mustard sx
 Ventricular level : Rastelli operation
 Great artery level : Arterial switch operation or
jatene operation

 Damus-Kaye-Stansel operation in conjunction with


the Rastelli operation can be used in patients with
VSD and subaortic stenosis
 Lecompte Operation-VSD+subpulmonary stenosis

SURGICAL TECHNIQUES FOR D TGA


SURGICAL OPTIONS
Anatomy Surgical options Comments
TGA/IVS Physiologic repair Usually elective, neonatal-1 yr
Senning or Mustard
Anatomic repair (primary) Neonatal period, usually within 2 wk of age
Arterial switch (Jatene)
TGA/IVS with prolonged low LV pressure Physiologic repair Usually elective, 1 mo to “1 yr
Senning or Mustard

Anatomic repair (delayed) Long preparation period (Yacoub)


Two-stage arterial switch Rapid two-stage switch (Jonas)

TGA/VSD Physiologic repair Poor long-term results


Senning or mustard with VSD closure
Anatomic repair Usually neonatal repair; PAB occasionally
Arterial switch with VSD closure (multiple VSDs)
Interventricular baffle repair Not all VSDs suitable

Damus-“Kaye-“Stansel: VSD closure Used when coronary translocation impossible


(LVto’PA); proximal PA to Ao anastomosis; aortic valve closure
RV to distal PA conduit
SURGICAL TECHNIQUES FOR D TGA
TGA/VSD/PS VSD closure (LV to Ao), RV to PA Palliative systemic-to-pulmonary shunt
conduit (Rastelli) frequently performed
Conduit replacement frequently
necessary
VSD closure (LV to Ao), anterior Long-term pulmonary regurgitation
translocation of PA with direct
connection to RV: REV procedure
(Lecompte)
TGA/PVOD Physiologic repair, palliative Symptomatic improvement
Anatomic repair, palliative

SURGICAL TECHNIQUES FOR D TGA


Atrial switch
 These procedures involve removal of the atrial septum, and redirection of the systemic
venous pathways to the sub pulmonary morphologically left ventricle, and pulmonary
venous blood to the systemic morphologically right ventricle.

 In the Senning operation, the rerouting of systemic venous blood was achieved by means
of infolding of the atrial walls, whereas in the Mustard operation this was achieved using
synthetic or pericardial tissue.

 One of the most popular modifications of the Mustard operation involved creation of a
trouser-shaped baffle, with the legs anastomosed to the superior and inferior caval venous
inflows.

 Bythe 1980s, such atrial redirection procedures were associatedwith early post-operative
survival exceeding 95%.

SURGICAL TECHNIQUES FOR D TGA


PHYSIOLOGIC CORRECTION
(ATRIAL SWITCH)
 The atrial switch repair imposes a
discordant AV connection on the existing
discordant atrioventricular connections; this
double negative results in a normal (i.E.,
Series) circulation.
 In contrast to the arterial switch, atrial
switch operations may be delayed for a few
weeks to several months after birth (and
balloon atrial septostomy).

SURGICAL TECHNIQUES FOR D TGA


PHYSIOLOGIC CORRECTION (ATRIAL
SWITCH

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Results and Sequelae of Physiologic
Correction
 10-year survival rates of 85% to 90%.

 complications following physiologic


repair and include
(a) residual intra-atrial shunts,
(b) caval and pulmonary venous obstructions,
(c) right ventricular dysfunction,
(d) tricuspid valve insufficiency, and
(e) arrhythmia.

SURGICAL TECHNIQUES FOR D TGA


 Residual intra-atrial baffle shunts
 Trivial leaks -10% to 20% of patients,
 Significant leaks requiring reoperation -
(1 to 2%).

SURGICAL TECHNIQUES FOR D TGA


 More recent series of either the Mustard
or Senning repairs report a significantly
reduced incidence of baffle leaks or
venous obstruction, but right ventricular
dysfunction to some extent and
dysrhythmias in particular remain
important late concerns
SURGICAL TECHNIQUES FOR D TGA
Systemic and pulmonary venous
pathway obstructions
 at greater risk for venous obstruction
because of the limited anatomic spaces
involved.
(A) improper baffle geometry and suture line
placement,
(B) contraction of the pericardial or synthetic
material baffle,
(c) scar tissue or adhesions involving the baffle
and excised margins of the atrial septum.

SURGICAL TECHNIQUES FOR D TGA


 The usual location of the obstruction is distal to
the superior vena caval entrance, within the
systemic venous atrium at the site of excision of
the superior remnant of the atrial septum .
 Surgical revision,
 Balloon catheter dilation,
 Stent placement,
 Innominate vein to left (physiologic right) atrial
appendage shunt all have been used successfully
to achieve temporary relief for both acute and
chronic superior vena cava obstruction

SURGICAL TECHNIQUES FOR D TGA


 Pulmonary venous obstruction, :lethal type of
obstruction
 A low frequency (about 2%) of pulmonary venous
obstruction has been reported for both the Mustard
and Senning types of atrial repair in two series
representing the original surgical development teams.
 Prompt suspicion should be aroused by a
postoperative chest roentgenogram with pulmonary
venous congestion or unexplained arterial
desaturation in the postoperative period.
 Reoperation with atrial baffle revision should be
considered for hemodynamically severe obstructive
complications of the atrial switch repair

SURGICAL TECHNIQUES FOR D TGA


 Tricuspid valve regurgitation has been recognized
postoperatively, but hemodynamically important
tricuspid regurgitation (TR) is rare (1% to 2%) in
most series concerned with TGA/IVS.
 In contrast, moderate and severe TR is more
prevalent (about 5% to 10%) in patients with
TGA/VSD.
 Manipulation and damage to the tricuspid valve
and its support apparatus, as well as the
occurrence of a right bundle branch block pattern
during repair of the defect, are considered
responsible for the TR.

SURGICAL TECHNIQUES FOR D TGA


 The right ventricle is functionally diminished
as a systemic ventricle compared with the
left ventricle because it contains a
hypokinetic segment (the infundibulum).
 Prerepair hypoxemic coronary perfusion
may cause sufficient myocardial fibrosis and
functional damage to preclude normal
systemic right ventricular function, but
normal pulmonary right ventricular function
usually returns after late second-stage
arterial switch repair.
SURGICAL TECHNIQUES FOR D TGA
 abnormal resting and exercise responses for right ventricular
ejection fraction (decreased) and abnormal resting
measurements for right ventricular volumes (increased) in
most patients
 The progressive increase in right ventricular volume also may
be due to increased flow from persistently enlarged
bronchial arteries, producing a silent volume overload to the
systemic ventricle
 Exercise performance in this patient population is uniformly
diminished, which appears to be a consequence of both
decreased heart rate and stroke volume response.

SURGICAL TECHNIQUES FOR D TGA


The following explanations for postnatal and post atrial
repair subnormal right (systemic) ventricular function

 Right ventricular myocardial fiber arrangements may not be optimum for systemic
function, and there may be a mismatch between right ventricular coronary blood supply
and systemic ventricular work demand. In this regard, the anatomic structure of the
ventricular free walls is dissimilar. The left ventricular free wall is composed predominantly
of stratum compactum (coronary-supplied myocardium), whereas the right ventricular
free wall consists predominantly of stratum spongiosum (trabeculae carneae).

 The right ventricle can achieve only suboptimal pumping function because the transposed
ventricular pressure relationships force the ventricular septum to bulge posterior-leftward
and to present a concave septal surface during contraction.

SURGICAL TECHNIQUES FOR D TGA


 Abnormal function of the mitral valve left
ventricular outflow tract subunit
◦ characterized by abnormal systolic movement
and coarse diastolic flutter of the anterior mitral
valve leaflet, systolic bulging of the basal segment
of the ventricular septum into the outflow tract,
and systolic pressure differences across the
outflow tract
 Abnormal distribution of pulmonary blood
flow to the right lung and hypoplastic
changes in the left pulmonary vascular bed.

SURGICAL TECHNIQUES FOR D TGA


 Progressive loss of NSR and increase in
atrial rhythm disturbances
 Gradual time-related decrease in sinus
rhythm
 In one series concerned with the mustard
repair of TGA (without significant VSD),
the probability of being in sinus rhythm at
1 year was 72%, at 5 years 56%, at 10
years 50%, and at 13 years 43%
SURGICAL TECHNIQUES FOR D TGA
The predominant dysrhythmias
include
1. Marked sinus bradycardia,
2. Ectopic atrial rhythm,
3. Slow junctional rhythm,
4. Supraventricular tachycardia, especially
atrial flutter.
5. Av conduction disturbances,
6. Surgical complete heart block,
7. Premature ventricular beats

SURGICAL TECHNIQUES FOR D TGA


 Intraoperative damage to the sinus node or sinus

artery

 Interruption of intra-atrial conduction by damage to

internodal pathways.

 The various extensive surgical incisions and suture

lines in atrial switch procedures result in marked delays

in intra-atrial conduction throughout the atria.

 These local areas of conduction delay are significant in

providing the substrate for the development of intra-

atrial reentry types of tachycardia, and late

postoperative electrophysiologic studies indicate a high

frequency of inducible sustained atrial flutter

SURGICAL TECHNIQUES FOR D TGA


Beyond atrial repair?
 An increasing frequency of important
dysrhythmic complications, including sudden,
unexplained death;
 The specter of late right ventricular
dysfunction and major TR in a ventricle most
likely unsuited for a lifetime of systemic
function
 Dissatisfaction with the operative mortality
and results in the subgroup of infants with
TGA and large VSD

SURGICAL TECHNIQUES FOR D TGA


Arterial switch operation (Jatene operation)
Advantages
 Fewer long-term complications

◦ Arrhythmias

◦ RV dysfunction

◦ Baffle stenosis

◦ Tricuspid regurgitation (TR).

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
 A critical physiologic challenge to anatomic repair
concerns the functional adequacy of the left ventricle.
 Adequate left ventricular muscle mass for successful
systemic function is usually present in
1. Early infancy
2. Nonrestrictive PDA,
3. Surgically remediable or dynamic left ventricular
outflow obstruction,
4. Delayed decrease in pulmonary vascular resistance and
persistent pulmonary hypertension
5. A large, nonrestrictive VSD.
 Definitive early (neonatal) one-stage arterial repair is
presently preferable to early palliation with pulmonary
artery banding and later arterial switch surgery.

SURGICAL TECHNIQUES FOR D TGA


Pre requisite
 An LV that can support the systemic circulation after
surgery

 The LV pressure should be near systemic levels at the


time of surgery, or the switch should be performed
shortly after birth (i.e., before 2 weeks of age).

 In patients whose LV pressure is low, it can be raised


by PA banding, either with or without a shunt, for 7
to 10 days (in cases of a rapid, two-stage switch
operation) or for 5 to 9 months before undertaking
the switch operation.

SURGICAL TECHNIQUES FOR D TGA


EMPIRICAL CRITERIA FOR LV SIZE

1. An absolute left ventricular systolic pressure that is


appropriate for age,
2. A left ventricular pressure at cardiac
catheterization that is >70% systemic levels (left to
right ventricular ratio >0.7), or
3. Left ventricular muscle mass that is within the
normal range for body surface area.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
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.

SURGICAL TECHNIQUES FOR D TGA


Pre-op
 The left ventricular inflow and outflow tracts must be
free of significant structural abnormality.

 The right ventricular outflow tract should be free of


significant stenosis.

SURGICAL TECHNIQUES FOR D TGA


Anatomic variants that may impact operative mortality
include

◦ An intramural course of a coronary artery


◦ A 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

SURGICAL TECHNIQUES FOR D TGA


 The great arteries are transected in a manner that allows eventual
reanastomosis of the distal aortic segment to the proximal pulmonary
artery (neoaortic root).

 Transfer of the coronary arteries to this pulmonary segment is facilitated


by their excision from the aortic sinus with a cuff of adjacent aortic wall.

 The proximal aortic segment (neopulmonary root) may be connected to


the distal pulmonary artery segment by an end-to-end anastomosis

 Maneuver of lecompte et al: passes the anterior aorta posterior to the


bifurcation of the pulmonary artery

SURGICAL TECHNIQUES FOR D TGA


Arterial Switch Operation
Simple Transposition of the Great Arteries
with Usual Great Artery and Coronary Patterns

 The aorta and pulmonary trunk must be


dissected apart,
 The right and left pulmonary artery
dissected out to their first branch, and the
ductus arteriosus dissected.
 The aortic purse-string stitch is placed as
far downstream aspossible to facilitate
work on aortic root and ascendingaorta

SURGICAL TECHNIQUES FOR D TGA


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

SURGICAL TECHNIQUES FOR D TGA


 The aortic button around the orifice of the right
coronary artery is excised, and inserted into
theright facing sinus of the neoaorta
 After the Lecompte maneuver the neoaorta is
constructed by anastomosing the proximal
segment of the original pulmonary trunk to the
distal aorticsegment.
 The stretched or torn foramen ovale (or ASD) is
closedthrough an incision in the right atrium,
usually with a running stitch. Separate autologous
pericardial patches are used to fill in the defects
in the proximal neopulmonary trunk. The
neopulmonary trunk is then constructed

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
Arterial switch

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
 The aorta is transected
just above sinotubular
juncture, and the
coronary arteries are
carefully examined to
confirm their positions
and to rule out the
possibility of any
unusual variations,

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Complications
 PA stenosis at the site of reconstruction - 5% to 10%
 complete heart block - 5% to 10%.

 Aortic regurgitation (AR)

◦ late complication > 20% of patients especially PA banding


◦ An important cause of AR may be unequal size of the pulmonary
cusps that leads to eccentric coaptation
 Coronary artery obstruction
◦ myocardial ischemia, infarction, and even death.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
TRANSPOSITION OF THE GREAT ARTERIES
WITH LOW LEFT VENTRICULAR PRESSURE

SURGICAL TECHNIQUES FOR D TGA


Transposition of the Great Arteries
with Low Left Ventricular Pressure
 Pulmonary artery banding can produce adequate muscle
retraining .
 Long preparation period.
 Rapid two stage switch.

 left ventricular function may be extremely impaired following


banding.( systemic-to-pulmonary artery shunt is frequently
placed to ensure adequate pulmonary blood flow )
 The interval period between banding and correction is
frequently characterized by a low output syndrome.
 Clinical improvement coincides with improvement in left
ventricular function such that anatomic correction can be
performed within 7 to 10 days in most cases.

SURGICAL TECHNIQUES FOR D TGA


OTHER INNOVATIVE
APPROACHES
 Percutaneously adjustable band ,
 Partial balloon occlusion of the main
pulmonary artery with a
percutaneously placed balloon-tipped
catheter,
 Systemic-to-pulmonary artery
shunting alone,
 Primary arterial switch with left
ventricular assist in the perioperative
period.
SURGICAL TECHNIQUES FOR D TGA
Anatomic Correction without
Coronary Translocation
 In 1975, DAMUS, KAYE, AND STANSEL in
independent reports proposed an arterial
level repair without coronary
translocation.
 reserved for children with TGA and
coronary artery patterns not suitable for
transfer or for patients with double-
outlet right ventricle (DORV) (Taussig
bing type with severe subaortic stenosis.

SURGICAL TECHNIQUES FOR D TGA


◦ The main pulmonary
artery is transected and
anastomosed in an end-
to-side fashion to the
ascending aorta.
◦ The coronary arteries
are perfused in a
retrograde fashion.
◦ The native aortic valve
may be left intact.
◦ A VSD (if present) is
closed to direct left
ventricular blood to the
native pulmonary
(neoaortic) valve.
◦ a right ventricular to
pulmonary artery
conduit is placed to
establish a normal series
circulation
SURGICAL TECHNIQUES FOR D TGA
Native aortic valve regurgitation is
common in this group of patients,
SURGICAL TECHNIQUES FOR D TGA
INNOVATIVE TECHNIQUES FOR ANATOMIC
CORRECTION WITHOUT CORONARY
TRANSLOCATION
 Creation of aortopulmonary tunnel
(aubert procedure)
 Baffling the left ventricular 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.
SURGICAL TECHNIQUES FOR D TGA
AUBERT OPERATION.
AN AORTOPULMONARY WINDOW IS CREATED (A) AND THE
CORONARY ARTERY IS DIVERTED INTO THE PULMONARY
ARTERY (PA) THROUGH THE WINDOW (B).
THEN, THE GREAT ARTERIES ARE SWITCHED (C AND D).

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
Surgery for Transposition of the Great
Arteries with Associated Left Ventricular
Outflow Tract Obstruction
 In the infant with TGA/large VSD and severe
LVOTO, there may be markedly restricted
PBF and severe hypoxemia.
 In some neonates, a palliative systemic-to-
pulmonary arterial shunt (Gore-Tex
interposition shunt or classic Blalock Taussig
shunt) may be performed, with intracardiac
correction carried out at a later age.
 Alternatively, corrective surgery can be
performed in early infancy

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
Surgery for Transposition of the Great
Arteries with Associated Left Ventricular
Outflow Tract Obstruction
RASTELLI OPERATION
 Combination of intraventricular repair and placement of an extracardiac right
ventricle to pulmonary artery conduit.

 Most appropriate operation for TGA with large VSD and extensive LVOTO
because it achieves complete bypass of the LVOTO and an anatomic correction
of the transposition pathology.

 Recently operative survival of about 95% and midterm survival of about 90% are
reported with good results, particularly in children older than 1 to 2 years of
age.

SURGICAL TECHNIQUES FOR D TGA


◦ The proximal main pulmonary artery is functionally

divided either by pledgeted mattress suture closure

of the subvalvar obstruction or by over sewing a

stenotic pulmonary valve orifice.

◦ The left ventricular output is directed to the aorta

by placement of an intraventricular patch-tunnel

technique.

◦ The right ventricle is connected to the proximal

main pulmonary artery by means of a valved

extracardiac conduit.

◦ The VSD must be of adequate size to permit

unobstructed outflow from the left ventricle, and

enlargement of the defect by anterior excision of

septal muscle may be necessary.

SURGICAL TECHNIQUES FOR D TGA


complications
1. Unfavorable anatomic variants,
• restrictive VSD
• anomalous tricuspid valve connections to the
infundibular septum that prevent baffling the left
ventricle to the anterior aorta.
2. Residual VSD,
3. Late unexpected death,
4. myocardial dysfunction .
5. ??Functional longevity of the valved conduits.
6. Improved results are noticeable with fresh or
cryopreserved homograft-valved conduits
compared with the previously used dacron
heterograft structures
SURGICAL TECHNIQUES FOR D TGA
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.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
REV
 APPLICATION IN YOUNGER PATIENTS,
 AVOIDANCE OF PROSTHETIC EXTRACARDIAC
CONDUIT,
 AVOIDANCE 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

SURGICAL TECHNIQUES FOR D TGA


 This operation involves
1. Performing a high, anterior right ventricular
incision
2. Radical excision of the outlet septum to create
an unobstructed anterior right ventricular
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) pulmonary artery
directly onto the right ventricular outflow cavity
without a prosthetic conduit
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
SURGICAL TECHNIQUES FOR D TGA
NIKAIDOH PROCEDURE

SURGICAL TECHNIQUES FOR D TGA


Surgery for TGA with Associated
RVOTO
 Anterior malalignment of the infundibular
septum, subaortic narrowing/stenosis, aortic
annular hypoplasia, a small hypo-plastic arch,
and coarctation or interruption of the distal
aorta
 significant proximal arch hypoplasia in
association with coarctation or interruption:
◦ Arch reconstruction +lecompte maneuver to bring the
pulmonary arteries anterior to the reconstructed
aorta, and an arterial switch operation with VSD
closure

SURGICAL TECHNIQUES FOR D TGA


Surgery for TGA with Associated
RVOTO
 Unusual coronary artery patterns :
 the anterior-facing sinus gives rise to the right coronary
artery and the left anterior descending, and the
posterior sinus of valsalva gives rise to the circumflex.
 The anterior course of the right coronary
artery may complicate the repair;
◦ Because an anterior malalignment VSD with
subaortic narrowing frequently coexists in these
patients, the coronary artery course may prevent
or limit the size of the ventriculotomy necessary
to relieve subaortic stenosis or to expose and
close the VSD.

SURGICAL TECHNIQUES FOR D TGA


Surgery for Right Ventricular Failure
Following Physiologic Correction
1. Tricuspid valvuloplasty/replacement,
2. Cardiac transplantation
In cases of significant, symptomatic
ventricular dysfunction. TGA is the single
most common anatomic diagnosis for
patients with congenital heart disease listed
in most cardiac transplantation registries

3. Anatomic correction.

SURGICAL TECHNIQUES FOR D TGA


Surgery for Right Ventricular Failure
Following Physiologic Correction
 An approach of retraining and using the left ventricle (anatomic
repair) together with takedown of the atrial repair was advocated
by Mee et al
 Anatomic correction can be arterial switch operation with
coronary translocation or, alternatively, transection of the main
pulmonary artery with an end-to-side anastomosis and right
ventricular “to“pulmonary artery conduit placement (Damus Kaye
Stansel operation)

 DISADVANTAGES.
◦ ASO may be technically more difficult in the older patient with a prior
physiologic correction because frequently dense adhesions are present
that may restrict anterior mobilization of the branch pulmonary arteries
and limit precise identification and adequate mobilization of the
coronary arteries.
◦ A Damus Kaye Stansel operation may be technically easier to perform,
but it requires the use of a prosthetic conduit from the right ventricle
to pulmonary arteries.

SURGICAL TECHNIQUES FOR D TGA


Surgery for TGA/VSD and
Pulmonary Vascular Obstructive
Disease
 Palliative atrial switch repairs (without closure of the VSD) have
been accomplished with low risk and substantial benefit.
 In one series concerned with 41 palliative Mustard procedures, operative mortality
was 7%, late mortality was 5%, and actuarial survival was 92% at 7 years
 After palliative atrial switch repair, the effective systemic blood flow is markedly
increased. Mean systemic arterial oxygen saturations were increased in two series
from preoperative levels of 68% to 74% to postoperative levels of 87% to 90%.
 Reserved for patients in whom peripheral desaturation is a major
cause of symptomatology because there is no favorable
postoperative change in calculated PVR.
 The concept of the palliative Mustard operation also has been
applied successfully to patients with pulmonary vascular disease
with intact ventricular septum by creating concurrently a VSD in
the apical segment of the muscular septum

SURGICAL TECHNIQUES FOR D TGA


Surgery for TGA/VSD and
Pulmonary Vascular Obstructive
Disease
 Elevated PVR markedly increases the risk
of surgery with closure of the VSD.
 Advanced PVD characterized by
calculated PVR>10 U or grade 4 (H-E)
histologic changes is a CI to VSD closure.

SURGICAL TECHNIQUES FOR D TGA


SURGICAL TECHNIQUES FOR D TGA
SURGICAL OPTIONS AVAILABLE TO THE INFANT WITH
D TRANSPOSITION OF THE GREAT ARTERIES
Anatomy Surgical options Comments
TGA/IVS Physiologic repair Usually elective, neonatal-1 yr
Senning or Mustard
Anatomic repair (primary) Neonatal period, usually within 2 wk of
Arterial switch (Jatene) age
TGA/IVS with prolonged low LV Physiologic repair Usually elective, 1 mo to “1 yr
pressure Senning or Mustard

Anatomic repair (delayed) Long preparation period (Yacoub)


Two-stage arterial switch Rapid two-stage switch (Jonas)

TGA/VSD Physiologic repair Poor long-term results


Senning or mustard with VSD closure
Anatomic repair Usually neonatal repair; PAB occasionally
Arterial switch with VSD closure (multiple VSDs)
Interventricular baffle repair Not all VSDs suitable

Damus-“Kaye-“Stansel: VSD closure Used when coronary translocation


(LVto’PA); proximal PA to Ao impossible aortic valve closure
anastomosis; RV to distal PA conduit
SURGICAL TECHNIQUES FOR D TGA
TGA/VSD/PS VSD closure (LV to Ao), RV to PA Palliative systemic-to-pulmonary
conduit (Rastelli) shunt frequently performed
Conduit replacement frequently
necessary

VSD closure (LV to Ao), anterior Long-term pulmonary regurgitation


translocation of PA with direct
connection to RV: REV procedure
(Lecompte)

TGA/PVOD Physiologic repair, palliative Symptomatic improvement


Anatomic repair, palliative

SURGICAL TECHNIQUES FOR D TGA