This document provides information on the surgical management of transposition of the great arteries (TGA). It discusses the history, embryology, associated lesions, and surgical techniques for TGA. Some key points include: - TGA accounts for 5-7% of congenital heart defects and was first described in 1797. - The most accepted embryological theory is abnormal development of the bilateral subarterial conus. - Associated lesions include VSD, LVOT obstruction, and aortic arch anomalies. - Surgical techniques have evolved from atrial septectomy in the 1950s to the arterial switch operation (ASO) developed in 1975, which has high survival rates of over 98% today.

1. DR.ANKIT JAIN
PROF.A.SAXENA
SURGICAL MANAGEMENT OF
TGA

2. Prevelance
 D-TGA accounts for 5% to 7% of all congenital heart
defects
 Prevalence is 0.2 per 1,000 live births with male
preponderance of 2:1 to 3:1
 Sibling recurrence rates is 0.27% and 2%, in simple and
complex forms

3. HISTORY
 The TGA was first described by Mathew Baillie in 1797, in
book "The Morbid Anatomy of Some of the Most Important
Parts of the Human Body
 The term transposition was 1st used by Farre 1814
 Meaning that aorta and pulmonary trunk were placed (positio)
across (trans) the ventricular septum

4. EMBRYOLOGY
 Most accepted theory is abnormal development of the B/L
subarterial conus
 Normally both the subaortic conus and subpulmonary
conus are present initially and both great arteries are placed
above RV
 Subaortic conus resorb and aorta migrates inferior and
posterior to lie over LV

5. Contd..
 Subpulmonary conus persists so that PA remains stationary
over the right ventricle
 In D-transposition, the subpulmonary conus resorbs
 So pulmonary artery moves inferior and posterior with the
pulmonic valve in fibrous continuity with the mitral valve
 Failure of the septum to spiral
1. Straight septum
2. Parallel arrangement of RVOT and LVOT

6. d- and l- transposition
 Describe the spatial relationship between the aorta and the
pulmonary trunk
 In d-transposition, the aortic valve lies to the right of the
pulmonary valve
 This is the most frequent arterial arrangement In TGA
 Other possibilities of arterial distribution exist in this
setting, thus the two concepts are not synonyms

7. ARTERIAL RELATIONSHIP

8. ASSOCIATED LESIONS
 In 75% of the cases, the ventriculoarterial discordance is an
isolated finding simple transposition with PFO or ASD
 VSD (25% to 40%)
 LVOT Obstruction (25%)
 Aortic arch anomalies
 Coronary abnormalties
 Atrioventricular Valve Abnormalties

9. ASSOCIATED LESIONS
Data from series of 260 patients undergoing operation at GLH, 1964-1984

10. VSD
 VSD (20-25%)
 Anterior malaligned VSD , increases the probability that
the patient will have aortic arch anomalies
 Posterior malaligned VSD is associated with LVOT
obstruction
 Overridding and straddling of tricuspid valve is associated

11. LVOT OBSTRUCTION
 Present in up to 25 percent of patient
1.Dynamic LVOT obstruction
 Patients with IVS
 ASO alone is curative
 It is rare in neonates because of high PVR
2. Fixed LVOT obstruction
 TGA/VSD have high incidence of severe anatomical
LVOT obstruction

12. OTHERS
 PDA present in 50% at two weeks of life
 Aortic obstruction - rare in IVS, occurs in 7~10% with
VSD
 Right aortic arch 5%, more common with VSD
 Leftward juxtaposition of the atrial appendage 2.5%, more
common with underdevelopment of RV
 Right ventricular hypoplasia

13. HISTORY OF TREATMENT
 Surgery for TGA started in 1950 by Blalock and Hanlon at
Johns Hopkins,  closed method of atrial septectomy
 Edwards, modified it in 1964 by resuturing the septum to
connect the right pulmonary veins to the RA
 In 1953, Lillehei and Varco described a “partial physiologic
correction” (anastomosis of RPV to RA , and IVC to LA a
technique known as the “Baffes operation)

14. CONTD..
 Major step in Palliation of TGA was , BAS in 1966 by
Rashkind and Miller in Philadelphia
 Park introduced Blade atrial septectomy in 1975
 Switching the venous return at atrial level Senning in
1959
 The Mustard procedure, in which the atrial septum is
excised and a pericardial baffle used to redirect blood was
devised to create larger atria (1964)

15. CONTD..
 Rastelli procedure for anatomic repair of
TGA/VSD/LVOTO in 1969
 Jatene in Brazil introduce ASO in 1975 (TGA/VSD)
 1980 REV for TGA /VSD/LVOTO
 1977 Yacoub et al. two stage repair
 1983 Quaegebeur and Castaneda, primary repair in neonate
 1988 Boston group, rapid two-stage ASO
 1982-Lecompte introduced his manoeuvre to directly
anastomose the PA’s to the neo-pulmonary “the french
connection

16. WHEN TO OPERATE
 There is no clear guidelines to inform the surgical decision
regarding the timing of complete repair
 A recent study by anderson et al tried to find out the
optimal timing of ASO

17. METHODS
 In a Study of 140 selected infants with D-TGA undergoing
ASO
 The authors analyzed the influence of age at surgery on
early “major” morbidity
 Major morbidity was defined as
 Cardiac arrest, ECMO support, delayed sternal closure,
infection, CNS insult, reoperation or readmission at 30
days
 The median age at operation was 5 days (range: 1 to 12
days)
Anderson et al arterial switch operation improves outcomes and reduces costs for neonates with
transposition of the great arteries. J Am Coll Cardiol 2014;63:481

18. RESULTS
 Decreasing probability of major morbidity between 1 and 3
days with increase after 3 days
 ASO between 1 and 3 days had an incremental benefit of
decreasing morbidity (46%) for every day later that surgery
was performed
 Opposite was true for neonates undergoing switch after 3 days
of age

19. CONTD..
 In the “older” cohort, there was an incremental increase in
major morbidity (47%) for every day later that surgery was
performed.
 The authors inferred that, ASO is ideally performed on day
of life 3

20. ADVANTAGES OF DELAYING ASO
 1) Transition from fetal to neonatal circulation
 2) Reduction in pulmonary vascular resistance
 3) Kidney and liver function improvement
 4) Initiation of enteral nutrition
 5) Evaluation for other congenital anomalies
 6) Family preparation for surgery

21. Harms
 A good Spo2 may be associated with paradoxically low
cerebral oxygen delivery
 Cerebral SvO2 is significantly lower than predicted from
the Spo2 in neonates with a run-off lesion
 Even a few days delay in ASO may increase CNS injury

22. ASO
 The great arteries are divided
 Coronary button transfer
 Lecomptes maneuver
 Great artries reconstruction
 Closer of any intracardiac communication

23. Considerations in ASO
Coronary anatomy
 Usual course
 Intramural course
 Anterior/ posterior looping
 Single coronary
Relationship and size of the great arteries
Associated cardiac defects
 –VSD
 –Atrioventricular valve anomalies
 –Aortic arch obstruction
 –Subaortic stenosis
 Left ventricular regression

24. GREAT ARTERIES TRANSECTION
 Transection of arterial trunks
 –Aorta
Transacted just above ST junction
 –PA
Transacted as far distal near bifurcation to
accommodate coronaries

26. 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 is inserted into the left facing sinus
 RCA is inserted into the rt facing sinus

27. COMMON CORONARY PATTERNS

28. CORONARY ANOMALIES
 The most common pattern (67% of cases) LMCA
arising from the left facing sinus and branching
into LAD/LCx
 The 2ND MC (16% of cases) is the LAD arising
from the left facing sinus and the RCA /LCx
arising from the right sinus
 So the LCx courses posterior to the pulmonary
artery and there are chances of kinking of LCx

29. TRAPDOOR CORONARY TRANSFER
 Pulmonay trunk transected as distally as possible
 Coronary artery will be transferred by creating a trapdoor
flap in neoaorta
 This will prevent too far implantation of coronary button
on right-lateral aspect of neoaorta
 Coronary button is also positioned more cephalad than in
usual case

30. TRAPDOOR CORONARY TRANSFER

31. INTRAMURAL CORONARY

32. ASO IN PATIENTS WITH
INTRAMURAL CORONARIES

33. LECOMPTES MANEUVER

34. MORTALITY
 Two recent studies demonstrate a hospital
survival rate of >98%
 30-day mortality rate for the ASO at <3% with a
1-year survival rate of >96%
 University College London. National Institute for Cardiovascular Outcomes
Research, June 20, 2014

35. Early mortality after ASO
 Early mortality is always due to difficulty with coronary
artery transfer
 Coronaries are transferred with an margin of 2-3 mm sinus
aorta known as coronary button
 Preoperative knowledge of course of coronary is important
to prevent coronary damage during button excision
 Metton O Intramural coronary arteries and outcome of neonatal arterial switch operation. Eur J
Cardiothorac Surg 2010;37:1246-

36. OUTCOME AND PREDICTORS OF
EARLY MORTALITY

37. RISK FACTORS

38. THE “LATE” ASO

39. TGA+IVS > 1 month
 LV is regressed
 1977Yacoub introduce PA band to increase the LV mass
and a BTS to relieve the cyanosis
 They waited months after procedure
 In1994 boutin and jonas found that LVH occurs rather
rapidly and LV mass doubles within a week of PA banding

40. CONTD..
 LV mass increases most rapidly in the first 2 days after
band placement, with an exponential decrease in the growth
rate after that
 Disadvantage of long interval 2 stage
1. Band caused scarring
2. Neo-aortic valve incompetance
3. Adhesions caused coronary transfer difficult

41. LV PREPRATION INDICATORS
 LV RV pressure ratio greater than 65%
 LVEDV >90% of normal.
 LVEF >50%
 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

42. Procedure of the two stage ASO
 BTS performed followed by a PA band 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

43. LATE PRIMARY ASO
 ASO has been successfully performed beyond the neonatal
period up to age 9 month in patients with TGA and IVS
 Such patients are more likely to require postoperative
mechanical support
 Kang N, de Leval MR, Elliott M, Tsang V, Kocyildirim E, Sehic I, et al. Extending the boundaries of the
primary arterial switch operation in patients with transposition of the great arteries and intact ventricular
septum. Circulation 2004;110:II123-7

44. AIIMS DATA –A.K BISOI ET AL
IJCTVS 2006
 Favoured primary aso >21 days
 Age 25 – 70 days
 Gr A (RTS ASO)-11pt
 Gr B(ASO)-- 15 pt
 Gr A–3/11 died after first stage
1. 8/11(73%) sucessfully trained
2. 5/11 survival( 45%)
 Gr B –13/15 survived(86%)
 2 deaths due to cardiac failure

45. Drawbacks of RTS
 First stage puts lot of strain on the ventricle
 Potential problems can arise of tightness of PA
band and overflow/blockage of the shunt.
 In case of any such event there is danger to life
 Evident from the 27% mortality after 1ST Stage

47.  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

48. ADVANTAGES
 1.No time lag to initiate ECMO
 2.Enable LV Retraining Under normoxemia condition
 3.Early initiation of ECMO prevent end organ damage

49. 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

50. Rastelli procedure
 It is done for d-TGA ,LVOTO and a large S/A 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

51. CONTD..
 For success of this operation, the VSD must be both large
and free of obstruction from AV valve tissue, so that the neo
LVOT is patent
 Surgical enlargement of small VSD can be done to
complete the Rastelli repair
 Straddling of the tricuspid valve often precludes this type of
repair

52. Rastelli procedure

53. 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

54. REV

55. ADVANTAGES OF REV
 Avoides the use of an Extracardiac conduit
 It involves the resection of the muscular outlet septum,
providing better alignment of aorta and LV
 Rastelli operation is associated with more risk of
reintervention due to LVOT obstruction, and extracardiac
conduit problems

57. REV VS RASTELLI
Surgery for malposition of the great arteries:the REV procedur Duccio Di Carloadoi:10.1510/mmcts.2007

58. Nikaidoh Procedure, or Aortic
Translocation
 Unsuitable for the rastelli or REV procedure
1. Inlet or restrictive VSD
2. Straddling or overriding of the AV valves
3. Coronary artery crossing the right ventricular
outflow tract

59. Nikaidoh Procedure

60. Damus-stansel-Kaye procedure
 It is for TGA and coronary artery patterns not suitable for
transfer .
 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 ,VSD is closed
 RV to PA conduit is placed

61. Damus-stansel-Kaye procedure
 Useful in patients who are undergoing staged conversion from
atrial baffel to systemic correction
 In these patients dense adhesion prohibit coronary transfer and
ASO

62. Damus-stansel-Kaye procedure

64. TIMING OF SURGERY
 These surgeries can be performed in infants >6 months of
age
 When cyanosis and symptoms are important before age 6
months
1. BT shunt, followed by a REV within 6 to 18 months
2. Primary REV

65. SURGICAL OPTIONS
Anatomy Surgical options Comments
TGA/IVS
Arterial switch (Jatene)
Neonatal period, usually within 2 wk of age
Physiologic repair
Senning or Mustard
Usually elective, neonatal-1 yr
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 anastomosis;
RV to distal PA conduit
Used when coronary translocation
impossible aortic valve closure

66. 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

67. PULMONARY VASCULAR DISEASE
 When TGA occurs as an isolated lesion PVD occur in 10%
to 30% at 24 months
 In patients with TGA and moderate or large VSD /PDA ,
PVD devlopes rapidly
 At 6 months, 25% have developed severe pulmonary
vascular disease (≥grade 3), and 50% at 12 months

68. Palliative Surgery for Patients with
Severe PAH
 Palliative operations may be indicated when PVR> 10
woods
 If the saturation in the PA is higher than the aorta, an atrial
redirection procedure, will improve streaming and improve
systemic oxygenation
 When the ventricular septum is intact, a large VSD is
created in the apex of the ventricular septum

69. CONTD..
 SaO2 in TGA depends on the relative proportions of
systemic venous and pulmonary venous blood reaching the
aorta, and on SvO2
 After palliative switch repair, the effective systemic flow is
greatly increased
 Decrease in the proportion of systemic venous blood
entering the aorta is also influenced by the rise in SVR that
follows the rise in SaO2

70. CONTD..
 There is an absolute increase in SaO2 of approximately
20%
 The only preoperative variable that correlates with
postoperative SaO2 is pulmonary AV difference
 A higher AV difference is associated with a higher
postoperative SaO2

71. POST OPERATIVE SEQUELE
Wernovsky G et al Guidelines for the outpatient management of complex congenital heart disease. Congenit Heart Dis 2006

72. MYOCARDIAL ISCHEMIA
 Obstructed coronary arteries are present in 5% to 7% of
survivors
 Most common cause of morbidity and mortality following
ASO
 Commonest in first 3 months after ASO
 Coronary obstruction late after the ASO is uncommon
 In a long-term study, freedom from coronary events was
88.1 %at 22 years
Khairy P et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries.Circulation
2013;127:331

74. NEOAORTIC ROOT DILATION AND
AORTIC REGURGITATION
 Freedom from N-AR of grades IV, III, and II at 23 years
was 90.2%,70.9% and 20.3 %
 Usually mild
RISK FACTORS
 Older age at time of ASO
 Presence of VSD
 Bicuspid pulmonic valve,
 Previous PA banding
 Higher neoaortic root/ascending aorta ratio
 LVOTO
 Taussig-Bing anomaly
Meshkishvili V. Fate of the aortic valve following the arterial switch operation. Card Surg 2010;25:730

75. CONTD..
 Severe AR requiring intervention is less then 2% in long
term follow up
 McMahon CJ et al. Risk factors for neo-aortic root enlargement and aortic regurgitation following
arterial switch operation. Pediatr Cardiol 2004;25:329-35.

76. Right Ventricular Outflow Tract
Obstruction
 RVOTO has occurred with sufficient severity to require
reintervention in about 10% of patients
 Peak incidence about 6 months after the ASO
 In one analysis, freedom from reintervention for RVOTO
was 94% at 1 year, and 79% at 5 years
 Swartz et al.Decreased incidence of supravalvar PS after ASO . Circulation 2012;126(11
Suppl 1):S118–22

77. RVOT OBSTRUCTION
 The obstruction can occur at multiple levels
 Diffuse hypoplasia of the pulmonary trunk commonly
results from inadequate mobilisation of the pulmonary
arteries

78. FREEDOM FROM SURGERY FOR
RVOTO From Norwood WI Congenital Heart Surgeons Society: personal communication; 1992

79. REOPERATION STUDY
 The ASO reoperation study revealed that pulmonary artery
reconstruction was required earlier than neoaortic
intervention ( 6.8 years vs. 13.8 years, p < 0.001)
 Raju V et al. Reoperation after arterial switch: a 27-year experience. Ann Thorac Surg
2013;95:2105–12.

80. ARRYTHMIAS
 Chronotropic impairment found consistently post-ASO
 Associated with residual hemodynamic lesions or CAD
 Late post-operative atrial flutter or fibrillation, is
associated with RVOT obstruction
 Khairy P et al. Cardiovascular outcomes after the arterial switch operation for D-
transposition of the great arteries. Circulation 2013;127:331–9.

81. SUDDEN CARDIAC DEATH.
 Most deaths occur 1 to 5 years after the ASO
 Probably related to exercise-induced external compression
of unusually distributed coronary arteries
 Incidence is 0.3% to 0.8%
 VF and late SCD are usually associated with myocardial
ischemia or infarction
 Khairy P et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the
great arteries. Circulation 2013;127:331–9

82. High-risk patients
 History of atypical, intramural, or problematic coronary
transfer require screening prior to engaging in high-level
physical activity

83. Neurodevelopmental Status
 Significant hypoxemia, acidosis ,long CPB, and low
cardiac output are correlated with abnormal ND and
behavioral testing
 In a study behavior, speech, and language delays at 4 and 8
years, with significant deficits in visual-spatial and -
memory skills
 Bellinger DC et al. Neurodevelopmental status at eight years in children with dextro-
transposition of the great arteries: The Boston Circulatory Arrest Trial. J Thorac Cardiovasc
Surg 2003;126:1385–96

84. 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

85. 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

86. LIFESTYLE CHOICES, CHOLESTEROL, HTN, AND
EXERCISE
 Neonatal coronary manipulation, potential endothelial
stress, and ongoing aortic root pathology may increase the
CAD risk
 These individuals have limited aerobic capacity on exercise
testing
 Atypical coronary anatomy, pulmonary artery stenosis, are
associated with decreased aerobic capacity

87. THANK YOU