2. Course and prognosis
TGA is the most common CCHD in newborn
Untreated d TGA is a uniformly lethal lesion
9/10ths of patients expire by the first birthday
Natural history of these patients is bleak
without any intervention
Mortality within the first week is due to
hypoxia and metabolic acidosis
Within first month is due to CCF
Later on due to thrombo-embolic phenomena
and CVA’s.
3. Survival without treatment
1.D TGA IVS
1WEEK
2 MONTHS
1 YEAR
80%
17%
4%
2. D TGA VSD
1 MONTH
5 MONTH
1 YEAR
91%
43%
32%
3. D TGA VSD LVOTO
1 YEAR
5 YEARS
70%
29%
4. EVOLUTION OF MANAGEMENT
STRATERGY
• 1950 : Palliation of d-TGA started with the
Blalock-Hanlon operation who first described
1. Venous Switch- 9 patients –NO SURVIOVOR
& UNBALANCED CIRCULATION
2.Atrial Septectomy- a closed method of atrial
septectomy designed to provide mixing of pul
& sys venous at atrial level , results 12 pts-9
died
6. Venous Switch
• 1953: Lillehei and
Varco described
a “partial
physiologic
correction “ ( or
atrial switch )
consisting of
anastomosis of Rt
pul vein to Rt
atrium and IVC to
Lt atrium
• 4 PTS – 2 EARLY
DEATH
9. CONCEPT OF
PHYSIOLOGIC
CORRECTION
ALBERT - 1954 FIRST
PROPOSED 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
THIS CONCEPT WAS AMPLIFIED
BY MERENDINO AND
COLLEAGUES IN 1957
DESINGED INTRA ATRIAL
BAFFLE
SMOOTH CURVES & NO
SURFACE IR-REGULARITIES
STAGED CLOSURE OF PDA &
VSD
10. AKE SENNING -KAROLINSKA INSTITUTE,
STOCKHOLM, SWEDEN 1959
• The first successful operation of this type was
accomplished by Senning
• TRANS STERNAL INCISION –5 RIB BED
• AOXL –NO CARDIOPLEGIA
• RELEASE CLAMP EVERY 25 MIN
• DISSECT IA GROOVE
• U SHAPED FLAP
• 3 FIXATION SUTURES FROM BEHIND LA
• STAGED CLOSURE OF VSD
11. Senning Procedure
• Median sternotomy
• Right based or a midline pericardiotomy
• CPB by Aorto-bicaval cannulation(
extrapericardial)
• Circumference of the IVC and the svc are
measured
• AOXL,Root CBC
12. SENNING
PROCEDURE
DHCA 18 DEGREE
25 DEGREE -1.6
L/MIN/M*
Svc circumference ×
2/3
Dissect in interatrial
groove
Preserve pericardial
reflection between
Ivc &RIPV
Cut back c.s
Use ant & post. Lip
of c.s& Laa.
Imbricate svc & ivc
Preserve s.a node
Pvc catheters
through Laa & Raa
13.
14. THEORY OF LA & RA REDUCTION
• G.A. TRUSLER & W.T. MUSTARD TORONTO
CANADA
• RA VOLUME REDUCTION BELOW 65% & LA
VOLUME REDUCTION BELOW 50% HAS
DELITERIOUS EFFECT ON C.O
• BASIS OF MUSTARD TECHNIQUE
15. MUSTARD PROCEDURE
AT TORONTO SICK
CHILDREN`S HOSPITAL
1963
INDICATIONS SAME
AS SENNING
PROCEDURE
PERICARDIAL
PATCH HARVESTED
PATCH SHAPE MAY
VARY(BROM)
INCISIONS MADE
ALL RAW EDGES
OVER SEWN
LARGE PATCH WITH
REDUNTANCY(TORO
NTO TECHNIQUE)
SPECEFIC PATH OF
SUTURE LINE
C.S CUT BACK AV
NODE PROTECTION
16. MUSTARD PROCEDURE
• Excision of the IAS
• Cutting back of the Coronary sinus
• Suturing of all the raw areas
• Care should be taken to prevent damage to the SA
node and its supply
• Modifications of the Mustard procedure are based on
the nature of the patch used
• Material that can be used are
Autologous pericardium-preferable
allograft or xenograft pericardium
PTFE or knitted polyester
18. Suturing of patch
• Suturing of the patch started anterior to the
left PV’s from the LA wall
• Superiorly suturing proceeds laterally and
superiorly to the SVC orifice
• Inferiorly suturing proceeds along the anterior
lip of the CS ,lateral and then superior to the
IVC
• Followed by suturing along the excised region
of the IAS
19. Mustard procedure
• Till ASO was adopted the mustard procedure
provided excellent results with 95% survival in
most patients of d-TGA ,IVS
• The important advantage of this technique
was its simplicity and was reproducible with
many surgeons as compared to the Senning
procedure
20. REVIVAL OF SENNING
• J.M QUAEGEBEUR &
A.G BROM THORAX
1977
• MAINLY BECAUSE OF
PERSISTING PROBLEMS
WITH BAFFLE
OBSTRUCTION AND
ARRHYTHMIA
• NO DIFFERENCE IN
MUSTARD & SENNING
22. Complications of Atrial Switch
Procedures
• 1.Systemic and Pulmonary venous pathway
obstruction
• SVC obstruction 9% at 10 years
• More common in mustard than senning
• usually apparent in 12 months of operation.
• 2.5% require reintervention
• IVC obstruction in 1-2%
23. SVC/IVC OBSTRUCTION Mx
• Management;
treatment is indicated if the patient is
symptomatic or if there is a progressive increase
in the size of the head in a child
• Options:
Balloon Dilatation with or without stenting
Re-operation
if PA pressure is low then a BD glenn can be
performed
24. • 1.2%
• more lethal
• Onset usually with in 6-12months
• Late onset up to 10 years
• Treatment-ASO Directly or after LV preparation
PULMONARY VENOUS OBSTRUCTION
25. Residual atrial shunting
• Trivial leak at baffle suture line occurs in 25 % of
patients
• Severe leak is uncommon
• 3% requires reoperation
• Most common site of leak- trabeculated upper portion
of atrias
JTCVS 1980 80 381
26. Electrophysiological disturbances
• Progressive loss of SR noted in 60% of patients
at mean 7 yr fu
• At 10 yrs Junctional rhythm in 29% of mustard
and 35% of senning
• Morphological basis
– Sinus node
– Sinus nodal artery JTCVS 1978 :75: 213 AJC 1972.30.526
JTCVS 1999 117 ,488-495
sarkar circn 1999:100 11 176-181
27. Arrhythmias in Late Follow up
• 23. yrs post mustard fu
– A Fib or Aflutter in 20% --seems to be a marker of
RV dysfunction
– 3% SVT
% free of pace maker insertion mustard and senning
AT 5 YRS 94%
AT 9 YRS 91%
IJC 2000:75: 129 AJC 1999.83.1080
28. SCD
• 5 % of hospital survivors over 10-20 years
• SCD is rare in patients with who remain in SR
post op and when pacemaker recovery time is
normal.
• Risk of SCD in patients with junctional rhythm
is 7%.
• Dysrhythmia that predispose to SCD is
unknown. AJC 1976 38 448
AHJ 1982,103,351
BHJ 1974 ; 36 ; 185
29. RVF
• 10% have symptomatic RV dysfunction at 10 yrs
• 60% have RV dysfunction that become evident on exercise at 10 yrs
• The probability of maintaining normal RV function declined rapidly after
10 yrs of follow up
• The probability of normal RV function after 14 yrs
– 52% simple TGA
– 37% Complex TGA
• In 64% of patients ,TR (15%)preceded the onset of RVD by mean interval
of 3.6 yrs
JTCVS 1999 117 ,488-495
Lawrence H Kohn
(Kirjavainen)
30. RVF (contd)
• It’s the MCC of late death
• Limited RV function has complex etiology
– Rigid atrial baffle
– Fibrosis and Reduced compliance due to RVH
– Myocardial fibre arrangement may differ from LV
– Mismatch between RV CBF and demand
31. RV Dysfunction
Options: 1.TV Repair
2.Staged conversion to ASO
LV pressure is high --- direct ASO
LV pressure is low -- PA band –LV training ---
ASO
3.Cardiac Transplantation
32. • Staged conversion starts with assessment of
LV function
if LV function is adequate due to LVOTO with a
LV peak systolic pressure >75% of RV pressure
–ASO is performed immediately
if LV function is inadequate –PA band is
performed.once the LV pressure reaches 75%
of systemic pressure
current recommendations are to leave the band
insitu for 1 year before proceeding to ASO
Staged conversion to ASO for late
failure of systemic RV
33. Staged conversion to ASO for late
failure of systemic RV
39 patients
35 PAB4 no PAB
1 early death
1 transplant
10 failed
training
25 anatomical
correction
4 early deaths
3 late death
2 transplantation 16 well doing
Roger mee et al JTCVS 2004
127 ,995
34. Staged conversion to ASO for late
failure of systemic RV
• Mean interval from atrial switch to RVF was 7 yrs.
The median duration of PAB 13 months(.5-5.4yrs)
• Median follow up of 8.2 years
Morphological LV function normal or mildly decreased in 89%patients
there were 3 cardiac deaths and 18 long term survivors are asymptomatic
Age>12 years
• greater probability of LVF and not completing the protocol
p =.02
• higher operative mortality at anatomic correction.p = .02
JTCVS 2004 127 ,995
3 cardiac
18 long term
are asym
35. Guidelines for the suitability of switch
conversion
• Peak LV pressure > 75% (LV pressure < 60%
indicate significant risk)
• A significant rise in peak LV pressure to supra
systemic level in response to isoproterenol
stimulation at catheterisation.
• Return to a normal LV wall thickness (indexed
for patient age and weight)
JTCVS 2004 127 ,995
37. Time related survival
JTCVS 1999 117 ,488-495
15 year survival1978-92
sarkar
77% mustard 94% senning
16 yr survival
QUAEGEBEUR
91%-simple
60%-complex
mustard
80% senning
30yr survival 1964-82
moons
80% senning
12yr survival 1978-91
kirjavainen
90% simple
TGA
78%complex
TGA
38. Late death
• 40 yrs f/u most common cause of death are
CCF and SCD(Toronto)
• Risk factors for late death
– sinus node dysfunction
– RV dysfunction
– Older age(>3years) of operation
JTCVS 1999 117 ,488-495
39. BALLON ATRIAL SEPTOSTOMY
• PALLIATION OF TGA WAS
REVOLUTIONIZED WHEN
WILLIAM .J. RASHKIND & W.
MILLER INTRODUCED BAS
• JAMA 1966
• 6 F BALLOON
• 2-6 ML NON IONIC CONTRAST
• RAPID WITHDRAWL
• REPEAT TILL NO RESISTANCE
• RAPID DEFLATION
• 31 PTS- 5 EARLY DEATH
• 1975 – PARK MODIFIED WITH
THEIR SUBSTITUTION OF
BLADE RATHER THAN BALLON
AT THE END OF THE CATHETER
40. STIMULUS FOR DEVELOPMENT OF
ARTERIAL SWITCH OPERATION
• TGA WITH LARGE VSD –
-DISAPPOINTING RESULTS OF ATRIAL SWITCH BECAUSE
THE RT (SYSTEMIC) VENTRICLE SOMETIMES FAILED POST-
OP
-RELATIVELY HIGH HOSPITAL MORTALITY AFTER REAPIR
-RAPID DEVELOPMENT OF PULMONARY VASCULAR
DISEASE
• HIGH POST BAS PREREPAIR MORTALITY BECAUSE IT
BECAME CONVENTIONAL TO DELAY ATRIAL SWITCH
PROCEDURE FOR 12-24 MONTHS
• ACCORDING TO NATURAL HISTORY THEY NOTED HIGH
MORTALITY IN TGA+IVS
41. INITIAL FAILED ATTEMPTS
• 1954- MUSTARD
ARTERIAL SWITCH USE OF
MONKEY LUNG AS THE
OXYGENATOR
• RCA FROM NEO PA WAS
COMPATIBLE WITH LIFE
• TECH . PROBLEM
• ECG CHANGES
• AIR EMBOLISM IN
CORONARIES
• 7 PT –NO SURVIVORS
42. INITIAL FAILED ATTEMPTS
• INITIAL EFFORTS BY BAILEY IN 1954
• KAY & CROSS IN 1955
• FAILURE DUE TO INABILITY TO SWITCH C.A
• POOR MYOCARDIAL MANAGEMENT
43. IDRISS PROCEDURE
• 1961 – IDRISS AND
COLLEAGUE
ATTEMPTED SUCH
PROCEDURE IN 2
PATIENTS WITH IVS
USING CPB ,
TRANSFERRING THE
GREAT ARTERIES AND
A RING OF AORTA
CARRYING THE
CORONARY ARTERIES
• 2 PATIENTS- BOTH
DIED ON OPERATING
TABLE
44.
45. ARTERIAL SWITCH
• 1975 – Adib Jatene in Brazil achieved major breakthrough
with the first successful arterial switch procedure applying
it in infants with TGA and VSD
• 1977 – This was presented in the henry ford symposium
• 1981-jatene published a survey of ASO from 14 centres
with 54 deaths in 89 patients lowest mortality was
25% and the highest was 100%
1982-Lecompte introduced his manoeuvre to directly
anastomose the PA’s to the neo-pulmonary root
this was termed “the french connection”
1978 – single coronary artery transfer by yacoub and radley
smith
1988 – mee and brawn –trapdoor technique of Coronary
artery transfer
46. ADIB . B . JATENE , SAO PAULO 1975
• CPB AT LOW FLOW / DHCA AT 16°
• CORONARY TRANSFER SITE SELECTED AT BEATING
HEART
• CORONARY BUTTON HARVESTING AND TRANSFER
• CONTINIOUS SUTURES TO REDUCE SIZE OF GREAT
VESSELS
• TRANS RV VSD CLOSURE
• NEO PA CLOSED WITH DURA MATER
• HIGH TRANSECTION OF AORTA / USE OF CONDUIT
(DACRON OR HOMOGRAFT)
• OBLIQUE TRANSECTION OF PA
• NO KINK IN LCA
47. HURDELS
• 1972- Yacoub attempts arterial switch
procedure in 3 infants with TGA and IVS –
unsucessful
• 1977 –Mauck and 1978 Abe indicated with
their work that such a repair is possible in
infancy
• The problem was low pressure LV not being
prepared for systemic pressure
48. SOLUTIONS
• Yacoub approached this problem of the low pressure
LV not being prepared for sustaining systemic pressure
by performing PA banding as a first stage
• He empirically waited an interval period of several
months to a year for ASO
• Disadvantage of long interval 2 stage
• Band caused scarring
• Neo-aortic valve in-competance
• Adhesions caused coronary transfer difficult
• Logistical difficulties
49. Cutting edge research
• Early 1980- Dr Bernardo Nadal Ginard his work
into the molecular biology of cardiac hypertrophy
, demonstrated not only that there were
important shifts in the isoforms of heavy chain
myosin in response to a pressure load , but in
addition those changes were apparent within
hours of the stress being imposed and max
synthesis was occuring by 48 hours, there fore
• Dr Nadal & Dr Castaneda reasoned the LV
preparation for ASO should be possible in days
rather than months
50. NEONATAL ASO IN TGA WITH IVS
• Castaneda and Norwood in Boston , Radley smith
and Yacoub in London, Quaegebeur in holland
took the bold approach of performing a primary
neonatal ASO who had IVS.
• The success of this procedure was based on the
premise that the LV was exposed to systemic
pressure prenatally in TGA because of patency of
the ductus arteriosus ( in the same way that the
RV is exposed to systemic pressure prenatally in
the child with NRGA
51. Surgery for d TGA
• Surgical palliation:
• 1.Creation of an ASD Blalock-Hanlon operation is only of
historical interest
Presently done in certain situations such as mitral atresia
and is done under CPB.
2.Palliation of associated anomalies.
-PDA ligation and repair of COA usually performed with
ASO
-VSD palliation with a PA band.
-BTS for those with LVOTO to allow for growth
Palliation is temporary and is only for immediate relief of
symptoms and no long term value therefore Definitive
procedures are necessary for continued amelioration of
symptoms
52. INDICATIONS FOR SURGERY
• Presence of malformation is an indication for
operation
• If cyanosis and symptoms are severe ---- BAS
• ASO < 1month of age
– in 1st week and at least in 1 month
– Increased PVR at this stage is not a CI
– LVOTO which is dynamic also is not a CI
– minimizing exposure time of brain to hypoxia of
uncorrected TGA
53. INDICATION
• Infant with IVS after the 1st month with low LV
pressure-immediate PA Band followed by ASO
within 7-10days
• Or Primary ASO +/- LVAD
TGA+VSD without LVOTO---ASO+VSD closure
within the 1st month of life
TGA+ VSD without LVOTO presenting after 1st
month of life ASO to be performed at the
earliest oppurtunity
54. D TGA+ VSD +LVOTO
• type and timing of definitive procedure is controversial
• When cyanosis and symptoms are important before 6
months of age either a systemic to PA shunt or a
primary Lecompte is indicated
• 6M – 5yrs of age Lecompte is the indicated treatment
• Children aged 3-5 yrs Lecompte or Rastelli has equal
results.
• all strategies aim at anatomical correction or to ensure
VA CONCORDANCE
55. ASO FOR d TGA + IVS
Conduction of CPB:
1.Continuous CPB
2. Continuous low flow CPB limiting
circulatory arrest time to a few minutes to
close the ASD
3.Total Circulatory arrest
56. • Anasthesia , parts prepared and draped
• Median sternotomy,Harvesting the
pericardium , it is used non-fixed/fixing
pericardium in 0.6% glutaraldehyde
• Inspection of anatomy of coronaries and great
vessels
• Dissection of PDA and mobilization of PA’s
from hilum to hilum
Preparation
57. Cannulation
• Ascending Aorta is cannulated as high as
possible
• For continuous CPB bicaval cannulation is
done
• For low flow CPB and TCA single venous
cannulation is done
• Placement of LV vent through the right
superior pulmonary vein
58.
59. Cooling initiated and the patient cooled to 25c
PDA is doubly ligated and divided
Site of Aortic transection is marked
Aortic cross clamp,Root CBC
Aorta transected just distal to the PA bifurcation
PA transected just proximal to the bifurcation
Semilunar valves are inspected as well as for LVOTO
Lecompte manoeuvre is performed
CPB
60.
61. Coronary Transfer
• Origin,Course and the presence of infundibular
branches are identified
• Coronary buttons are harvested with a large cuff
of aorta extending well into the SOV
• Proximal coronary arteries are mobilised to avoid
tension and distortion without sacrificing the
infundibular branches
• LM coronary artery is inserted into the left facing
sinus of neoaorta
• RCA is inserted into the rt facing sinus of
neoaorta
62.
63.
64.
65. Closure of ASD and Deairing
• RA IS OPENED ASD CLOSED DIRECTLY OR
WITH A PATCH , RA CLOSED
• DEAIRING IS DONE BY
• RSPV VENT OFF/ LAST FEW ANTERIOR
SUTURE OF NEOAORTA IS NOT TAKEN /HEAD
LOW/AoXCL OFF /GENTEL MASSAGE OF THE
HEART /AFTER DEAIRING / LAST SUTURE OF
NEOAORTA TAKEN
66. NEO PULMONARY ARTERY
RECOSTRUCTION
• REWARMING STARTED
• AUTOLOGOUS PERICARDIAL PATCH IS USED TO
FILL IN THE DEFECT IN THE PROXIMAL NEO
PULMONARY TRUNK
• AFTER THE NEONATE HAS BEEN REWARMED
AND PROPER CONDITION ARE IN PLACE , CPB
IS WEANED AND DISCONTINUED
67.
68. • Circumflex artery from
sinus 2 with RCA or as a
separate ostium
• Implantation of the RCA
by the trap door
technique
CORONARY ANOMALIES AND THERE
TRANSFER TECHNIQUE
71. ALL CORONARY ARTERIES FROM
SINUS 2
• Management of 2LCx,R
Pattern 1-Lcx posterior to PA,RCA and LAD
anterior to Aorta
Pattern 2 –Intra mural LAD,with Lcx coursing
posterior to PA and RCA anterior to Aorta
72.
73. ALL CORONARY FROM SINUS 2
• All the coronary ostia
taken in a single patch for
anastomosis when there
is no intra-mural coronary
and minimal tissue
separating the ostia
• Defect in the ascending
aorta is filled with either
glutaraldehyde preserved
pericardium or tissue
from a pulmonary
homograft
74.
75. SIDE BY SIDE GA
• Transposition with more or less side by side
great arteries
• usual coronary pattern is 1L,R 2Cx
• Lecompte manoeuvre is not performed
• PA bifurcation is shifted on to the RPA ostium
• PA anastomosis is performed first prior to neo-
aortic reconstruction
76.
77.
78. dTGA+IVS > 1 month
• After the first month with the fall in PVR there is a
significant decrease in the LV mass
The effect of which is such that the LV is no longer able
to pump against the Systemic vascular resistance and
hence it fails
• As the LV is not prepared to take over the systemic
circulation after the first month it needs to be trained
• 1977Yacoub-PA band to increase the LV mass and a BTS
to relieve the cyanosis
• They waited several months before performing ASO
• In1994 boutin and jonas found that LVH occurs rather
rapidly and LV mass doubles within a week of PA
banding
79. TGA with IVS
first seen after 4 weeks
• GUIDE LINES
ASO
2 stage ASO following PAB
in 1 – 2 weeks
Atrial switch
Primary ASO
using LVAD( ECMO)
doubtful
LV prepared
J Th CVSurg 1993:106:111-5
80. Selection Criteria for 2nd stage
ASO(age 1- 14m )
• LV RV pressure ratio greater than 65%
• LVEDV >90% of normal.
• LVEF >50% or may be greater than 40%
• LVEDPWT >4 mm or safely >4.5mm
(BSA<.5M2)
• Predictive LV wall stress <120 x 103 dynes/cm
• LV MASS > 70 GM/M²
Nakazawa circulation 1988 ,78, 124-131
81. Procedure of the two stage ASO
• right thoracotomy or a midline sternotomy
• BTS performed with a 3.5-4mm PTFE graft and
followed by a PA band so as to achieve 75% of
the systemic pressure
• Second stage-shunt take down and debanding
done
• Adhesions usually not a problem as the
procedure is being performed within 7 days
82. Tentative criteria for primary ASO >
1month
• LV RV pressure ratio >60%
• LVEDPWT >4.5 mm (BSA<.5M2)
• Absence of septal bulging into LV on 2D echo
83. d-TGA,VSD,LVOTO
• Primary palliation by a BTS if LVOTO is not
resectable
• If resectable then ASO with LVOT resection
should be performed
• Where LVOT is not resectable –options
Rastelli
REV or Lecompte
84. Rastelli procedure
• It is done for d-TGA ,LVOTO and a large sub-
pulmonic VSD
• Not suitable for non-comitted VSD’s
• VSD is closed routing the Aorta to the LV with
or without VSD enlargement
• PV is closed from the RV or is transected and
suture ligated
• RV-PA extra-cardiac valved conduit is placed
85.
86. Rastelli procedure
• PA TRANSECTED AND
PROXIMAL PUL TRUNK
OVERSEWN
• INCISION ON
VENTRICULAR SEPTUM
IF VSD IS <60% OF
AORTIC DIA
• LV TO AORTIC BAFFLE
89. REV or Lecompte procedure
• VSD is closed routing Aorta to the LV after
excising the outlet septum
• Pulmonary valve is closed through the RV or is
transected and ligated
• PA’s are extensively mobilised
• Lecompte manoeuvre is performed
• PA’s brought down to anastomose to the
ventriculotomy posteriorly
• Anteriorly augmented with a pericardial patch
91. Damus-stansel-Kaye procedure
Performed for d-TGA with a large vsd
• PA transected .proximal PA to Ascending Aortic
anastomosis performed
• VSD closure followed by a RV-PA conduit is placed
• Coronaries perfused retro-gradely
• This procedure is use full in pt who are
undergoing staged conversion from atrial baffel
to systemic correction, because dense adhesion
prohibit coronary transfer and ASO
92.
93. • IT IS FOR TGA+ VSD + LVOTO
• IT CONSISTS OF AORTIC TRANSLOCATION
WITH SELECTED INDIVIDUAL CORONARY
ARTERY TRANSFER AND BIVENTRICULAR
OUTFLOW RECONSTRUCTION
NIKAIDOH -BEX
96. CLINICAL OUTCOMES AFTER ASO FOR
SIMPLE & COMPLEX TGA
• MULTIINSTUTIONAL PROSPECTIVE STUDY
,INCLUDED 513 NEONATES
• RISK FACTORS FOR DEATH
1.PATIENT
2.SUPPORT
3.PROCEDURAL
4.INSTITUTIONAL
KIRKLIN et al
CIRCULATION 1992;86:1501-1515
1.LCA,LAD or Cx arising from sinus 2
without an intra-mural course
with an intra-mural course
2.Multiple VSD’s
3.Co-existing non-cardiac anomalies
4.PA banding >1month previously
5.Older age at repair for simple TGA
longer global myocardial
ischemia time1.Aorta transected Distally
2.PA transected proximally or in
the mid portion
3.No Lecompte manoeuvre
4.Coronary implantation not at
the transection site
High risk instituitions-mortality greater
than 13%
low risk instituitions-motality 6-13%
Shorter interval since first ASO
97. Multi-institutional prospective study
• No of patients-513
• ASO for simple & complex TGA in <15 days of
age
• Survival
• Hazard function for death
• Mortality
• Functional class
1 month 84%
1 year 82%
5 years 82%
Approached
zero by 12
months
8 pts died > 3months after
operation , 4 had sev LV dys
Probably related to imperfect
coronary transferAll patients have physical strength and activity
expected of normal infants and children of their age
except 1 who is in NYHA class 1
98. Functional status
J Th CVSurgery 2002,124,790-797
Class 1 Class 2 class3
N=195 25 yrs
1977-2000
JTCVS 2002
96.6% 2.7% 0.7%
Wernovsky
N=412 10yrs
1983-92
97.6 2.2 .2
100. RVOTO
• 17% reintervention for RVOTO at 10 yrs(n=514)
• Peak incidence at 9 months ,then declined to
merge with a constant risk of 1% per year by
about 2 years.
• Site of obstruction
– Pulmonary trunk(most common)
– Bifurcation of pulmonary trunk
– RV pulmonary trunk junction
– RV infundibulum.
J Th CVSurgery 1997,114,975
J Th CVSurgery 2002,124,790-797
101. LVOTO
• 2% reintervention for LVOTO at 10yrs(N=514)
• Peak incidence at 6 months ,then declined to
merge with a constant risk of 0.1% per year
by about 1 year.
J Th CVSurgery 1997,114,975
102. Neo aortic valve regurgitation
• 173 patients study 8.2 yrs median follow up
• 61 (35%)had angiographic or doppler evidence of neo AR
• 11 (18%) had trivial – mild valvular incompetence early post
operative phase.
• 80% were free from neo AR at discharge and developed
regurgitation between 2 & 5 years after the operation.
• 1 patient had valvular dysfunction after 10 years of operation
Formigari J Th CVSurgery 2003,126,1753-9
Onset of Aortic regurgitation
103. Neo aortic valve regurgitation
follow up
• Stable in 47 (77%)
– through out the entire follow up
• trivial in 30
• mild in 17.
• Progressive in 14(23%)
• trivial – mild in 11(18%)
• mild- moderate in 1(1.6%)
• trivial to severe in 2 (3.3%)
• 2 children in this group required
reintervention.
Formigari J Th CVSurgery 2003,126,1753-9
104. Aortic regurgitation
25 years experience with ASO
(n = 150)
• 145 Absent or trivial
• 3 mild AR
• 1mod AR
• 1 severe AR
P.A Hutter ,Bennink J Th CVSurgery
2002,124,790-797
105. Aortic regurgitation after ASO
1156 hospital survivors from 1982-2000
Incidence
Risk factors
Out come
J Losay et al JACC 2006 ;47:20
14.9% (172 pts )
Residual Risk
VSD 2
AR 4
Freedom from AR-77.9% at 10 yrs & 69.5% at 15 yrs
Reoperation from AR was done in 16 pts with 1 death
Freedom from reoperation for AR 97.7% at 10 yrs & 96.8% at 15
yrs
Hazard function for AR declined rapidly and slowly increased
from 2 to 16 yrs
106. Neuro developmental status at 4 years (n=74)
• At 4 yrs 20% had definite mild neurological
abnormality
• At 8 year follow up over all physical and
psycho social status was similar to that of
general population
Karl JTCVS 2004 ,127.1,213
107. Neuro developmental status at 4 years (n=74)
Neuro developmental sequelae patients controls P value
WPPS IQ 101.9 108.6 .0007
Speech problem worse better .002
Language expression problem worse better .001
Language comprehension
problem
worse better .033
inattentive worse better .033
Karl JTCVS 2004 ,127.1,213 1988-1994
108. Neuro developmental sequelae (contd)
Neuro developmental sequelae P value
vision .405
hearing .317
movement .074
Manual dexterity skills & balance .07
Karl JTCVS 2004 ,127.1
109. Risk factors for neurodevelopmental
outcomes
Pre op factors Severe pre op acidosis & sepsis <.04
Peri op Duration of circulatory arrest .03
JTCVS 2002 124 448
110. Late coronary problems after ASO
• Bonhoeffer et al 1996
• Tanel et al 1995
• Bonnet et al 1996
12 coronary occlusion were
identified in 165 children of ASO at
an avg age of 6 yrs
13 pts ( 3%) of 366 ASO were having coronary
problems
N = 64 Angiographic f/u at the mean age
of 7 yrs(4 – 11 yrs)
5 had occlusion or stenosis of coronary
artery
3 patients with occlusion had peri operative
ischemic complications
2 with stenosis had normal execise ECG and
111. Pulmonary hypertension Post -op in dTGA
IVS
• Surgery performed in 1st 3 months no
instances of progressive PVD
• surgery performed after 3 months in those with
normal PVR,5-10% develop PVD post
operatively.
• This often progresses and causes death.
AJC 1975 35 107
Circulation 1979 59 525
112. LV is better suited as pulmonary ventricle in
simple TGA with severe PAH.
Dr R. K. Sharma, S.K. Choudhary, Dr. A Bhan and
Dr P. Venugopal
10 pt. 3-6 months age with prepared LV
4 underwent arterial switch
Uneventful surgery
Prolonged with ICU stay & ventilation
SEV PAH with acidosis on weaning
Only 1 pt. Could be extubated and discharged.
113. 6 pt. Had senning repair
no early mortality
ventilation only for 48 hrs.
F.U. at 4 year
1 child with ASO had PA press 50% of arterial
pressure.
In Senning Group 2 pt –PA press >60% of
systemic press.
4 pt. Normal PA press.
114. 9 yr survival with out eliminating
high risk group 1983-92
(wernovsky)
91%
10 yr survival with out eliminating
high risk group (1990-2000)
90%
15 yr survival with out eliminating
high risk group (1982-1999)Losay
88%
LATE SURVIVAL
J Th CVSurgery 2002,124,790-797
J Th CVSurgery 1995,109,289-302
Circulation . 2001 :104
115. LATE SURVIVAL
• Death rate is extremely low by 6- 12 months
of age.
• Causes of late death after ASO
– VF due to coronary artery stenosis
– Right ventricular dysfunction due to severe PVOD
J Th CVSurgery 2000,48,228
J Th CVSurgery 2002,124,790-797
116. REINTERVENTIONS
10 yrs 9.3%
(n= 68753)
Procedure no. Patient no.
PS 42 25
PS VSD 1 1
PS mod supra valv AS 1 1
PS PR 1 1
Re Coarctation 4 3
Diaphragm paralysis 3 3
AR 2 1
VSD 2 2
Supra valv AS 1 1
Sub valv AS 1 1
AICD 1 1
Pacemaker 1 1
Serraf J Th CVSurgery 1995,110,892-99
117. Re-operation
• Risk factors for reoperation for RVOT
obstruction
– non neonatal repair (P<.01)
– long standing PAB (P<.01)
Serraf J Th CVSurgery 1995,110,892-99
118. Rapid Two Stage ASO for TGA IVS
R.A Jonas , AR Castaneda – Boston Children Hospital
Circulation 1989
• A subgroup present late for ASO
– Sickness
– Size
– Late referral
• 11 pt – mean age 4.5 months
• LV prepared in 9 days –band with or without shunt
• L.v mass increased by 85 %
• P LV/RV – increased from 0.5 to 1.04
• One patient had senning due to intramural coronary artery
• 10 patient had second stage
• No early death
• Mean hospitalization – 8 days
• FU
– One late death at 5 months
– No LV dysfunction
– Mild to trival AR was present
119. • 1991-2001 ( 299 patients )
• GR A– TGA IVS—169 PT (2DAYS-18 YRS)
• GR B—TGA VSD—130 PT(4 DAYS-4 YEARS)
• GR A – 141 PT –ASO
• 28 PT RAPID TWO STAGE
• 23 PT WITH LVOTO
• Mortality
• Causes
AIIMS RESULTS Dr RAJESH SHARMA ,Dr ANIL BHAN ,Dr
S.K CHOUDHARY & Dr P VENUGOPAL(IHJ 2002)
GR A-8.8%
GR B-33%PAH 21 pt
SEPSIS 16PT
LV FAILURE –8 PT
120. Continued
• 3 PT –LIMA TO LAD
• 3 REQUIRED LVAD
• 1-10 Y FU –87 % COMPLETE
• 3 LATE DEATH
• 1 LV DYSFUNCTION
• 1 PACE MAKER DYSFUNCTION
• 1 PAH
• 5 RE OPERATION
• 91% PT . FREE OF MEDICATION
121. Cont
• MEAN DURATION OF VENTILATION-4 DAYS
MEAN DURATION OF ICU STAY-8 DAYS
• HOSPITAL STAY—15 DAYS
• EARLY REOP-
BLEEDING ;5.5%
RVOT REVISION;0.7%
PACEMAKER;1%
DIAPHRAMATIC
PLICATION;0.7%
122. Continued
• RAPID TWO STAGE-
• 28 PT
• 22 SUCESSFUL CONVERSIONS
• MEAN DURATION OF CONVERSION--10±4
DAYS
• 11 % MORTALITY(3PT)
• 3 PT CONVERTED TO SENNING
124. TGA IVS –MARC.R .DE LEVAL 2004
CIRCULATION
• TGA IVS 4 WEEKS & 8 WEEKS
• 275 PT < 1 MONTH
• 105 PT >1 MONTH (21-185 days )
• MORTALITY– 5% / 4%
• NEED FOR MECH. SUPPORT– 5% / 7%
• LENGTH OF POST OP STAY-- 13% / 29%
• LENGTH OF VENTILATORYSUPPORT 5%/57%
• SO NEED FOR VENTILATORY SUPPORT MAY LIMIT
ITS APPLICATION .
125. AIIMS DATA –A.K BISOI ET AL
IJCTVS 2006
• FAVOURED PRIMARY ASO >21 DAYS
• AGE 25 – 70 DAYS
• WT & OTHER PARAMETER MATCHED
• GR A (RTS ASO)-11PT
• GR B(ASO)-- 15 PT
• GR A –3/11 DIED AFTER FIRST STAGE
• 8/11(73%) SUCESSFULLY TRAINED
• 5/11 SURVIVAL( 45%)
• GR B –13/15 SURVIVED(86%)
• 2 DEATHS DUE TO CARDIAC FAILURE
• 2 PT ELECTIVE ECMO
126. • SURGICAL OUTCOME OF PRIMARY ASO > 6
WEEKS
• JAN 2003- JUNE 2009
• 55 CHILDREN ( 42 DAYS TO 9 YEARS )
• MORTALITY – 7 PTS ( 13% )
• CHILDREN WHO HAD SEVERLY REGRESSED LV (
BANANA SHAPED ) WERE OPERATED WITH
INTEGRATED ECMO-CPB
• CHILDREN WITH REGRESSED LV REQUIRED
LONGER VENTILATORY TIME AND INOTROPES
AIIMS DATA –A.K BISOI ET AL
EJCTVS 2010
ADVANTAGES
1.NO TIME LAG TO INITATE ECMO
2.ENABLE LV RE-TRAINING UNDER
NORMOXAEMIA AND CONTROLLED
LOADING
3.EARLY INNITIATION PREVENTS END
ORGAN DAMAGE
4. SURGICAL ASEPSIS
5. COST EFFECTIVENESS
127. TGA VSD with LVOTO
Results of Rastelli & Lecompte procedure
EJCTVS –2004 may seoul National University
35 pt between –1990- 2002
Lecompte Rastelli
25 pt (71%) 10 pt. (29%)
23 month 39 months
1 early death 3%
F.U. 5.9 years No late death
Re- operation 6/24 (25%) 8/10 reoperation (80%)
Freedom from reoperation
95.7 at 5 yr 40% at 5 year
63.5% at 10 yrs 26% at 10 year
So both procedure provide satisfactory early and late result buy late
morbidity & reoperation are more with Rastelli.
128. ATS 2002
Dr. R. Sharma, Dr. S.K. Choudhary, A Bhan
TGA with LVOTO
• 299 patient of ASO 1991-2001
• 23 had LVOTO
• Age 4 days-18 years
• Sx- ASO + VSD closure + excision of LVOTO
– 2 early deaths
– 8 patient had mild neo aortic AR at discharge
– 1 patient mild MR
• FU – mean 60 months-(8months-9 years)
• 4 patient of AR – progressed to mod to severe AR , 1 pt developed sev MR
in 72 months , DVR was done
• So LVOTO – excision has high incidence of AR
129. ASO + AORTIC Arch anomalies
JACC 2005 Oct – Pocar et al – Paris ,France
• Single stage correction through median
sternotomy is treatment of choice
– 1996-1998
– 38 pt
• DHCA with aortic arch enhancement using
autologous pericardial patch.
130. • Porcar et al (cont)
• 9 hospital death – 24%
– None related to aortic arch but with associated TGA abnormalities like
• RV hypoplasia
• Complex coronary anatomy
• Sev PAH
– No late death
• 4 reoperation – all cardiac
• 3 pt – recurrent COA – balloon dilatation
• Though operative mortality is significant – FU operative survival
is favourable.