1) Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly where the connections of the great arteries and ventricles are discordant. The right ventricle functions as the systemic ventricle and the tricuspid valve is the systemic atrioventricular valve. 2) The right ventricle is not well-suited to function as the systemic ventricle long-term, often leading to ventricular dysfunction, tricuspid regurgitation, and heart failure. Management options include physiologic repair, anatomic repair such as the double switch operation, or a Fontan pathway. 3) The ideal surgical approach remains debated and depends on the individual

1. CC- TGA
PRECEPTOR: DR. SAURABH GUPTA
SPEAKER: DR. ARVIND

2. Introduction
• Rare cardiac anomaly : 2 - 7 per
one lakh births
• 0.4% of all CHDs
• Discordant connections at both A -V
and V-A levels
• RV functions as the systemic
ventricle and Tricuspid valve is the
Systemic AV valve
Samanek M et al Bohemia survival study. Pediatr Cardiol. 1999

3. RV as the systemic ventricle
• Systemic pressure - Eccentric hypertrophy IVS shift
-> Dilated RV —->Increased wall stress
• Hypertrophied RV : not supported by adequate
proliferation of capillaries
• Myocardial Perfusion defects - Impaired flow reserve
• Low EF : does not increase with exercise

4. TV as systemic AV valve
ANATOMIC
ABNORMALITIES
OF TV
GEOMETRIC
CHANGES OF RV
ALTERED
LOADING
CONDITIONS
ANNULAR
DILATION
eester PD et al In The TV in congenital heart disease. (eds.). Springer-Verlag Italia 20

5. Overview of the Presentation
• Embryology
• Morphology
• Conduction tissue, Coronary anatomy
• Associated Lesions
• Clinical Presentation and Workup
• Natural History
• Management

6. Historical Perspective
• 1st described by Baron Rokitansky in 1875
• Term “Congenitally corrected TGA” was first used by
Schiebler in 1961
• Anderson and Lillehei performed the first surgical
repair in 1957
• Ilbawi and colleagues performed the first Double
Switch Operation ( Chicago , 1990)

7. Cardiac Looping - 3rd week

8. Abnormal looping in ccTGA
• TH

10. Morphology
• Sub- pulmonary infundibulum is absent (Pulmonary -
Mitral continuity )
• Aorta is supported by a infundibulum - anterior and
left, discontinuity between aortic valve and TV
• LVOT is deeply wedged between the AV valves
• IVS tends to a more sagittal : Malalignment between
atrial and ventricular septum

11. In Situs Inversus ?
• Situs Inversus : 5% of cc TGA
• 25 % of ccTGA have either Dextrocardia/ Mesocardia

12. Septal Malalignment
• Characteristic feature of AV
discordance
• The space is filled with a large VSD or
rarely by a perimembranous septum
• Affects the position of the conduction
system
• Less marked in hearts with a small or
atretic pulmonary trunk, Situs inversus
Hosseinpour AR et al Ann Thorac Surg 2004

13. Conduction tissue
• Monckeburg in 1913, described abnormal
conduction tissue in ccTGA
• Septal malalignment, Wedging of LVOT - contribute
to abnormalities in conduction tissue

14. Normal conduction tissue

15. Conduction tissue in ccTGA
The Normal AV node is
hypoplastic
Secondary AV node : below
the RAA orifice
The AV bundle runs
subendocardially, in the sub
pulmonary area
At IVS, turns inferiorly and descends
anteriorly along septum, before
branching into left and right bundles

16. Conduction tissue in situs inversus ccTGA

17. Clinical implications
• ECG : Reverse septal activation (from right to left)
• The long AV bundle prone to fibrosis with advancing
age - conduction disease progress over time
• CHB : 4% at birth, Lifetime incidence : 20-30% (
Risk increases @ 2% per year)
• Reentrant tachycardias, accessory pathways are
common

18. Surgical implications
• Asd
• Post OP CHB incidence : 30- 45 %

19. Coronary Arteries
• Coronary artery - Ventricular
concordance maintained
• Considerable variation in origins,
but epicardial distribution fairly
constant
• In a series of 46 patients , 35
had normal coronaries and most
common anomaly was single
coronary
Uemara H et al Eur J Cardiothorac Surg. 1996

20. Associated Lesions
• Isolated ccTGA (1 - 16%)
• Associated anomalies
• VSD (60-80%)
• LVOT Obstuction (30-50%)
• Tricuspid Valve abnormalities (14-56%)
• ASD (12%)
• Right Arch (18%)

21. VSD
• Associated with 80% of
ccTGA
• Most common is
perimembranous
• Conduction axis runs
Anterosuperior to this
defect

22. LVOT Obstruction
• 30% to 50% of ccTGA
(situs solitus)
• Isolated in 1/5th,
associated with VSD
4/5th
Anderson RH et al JTCS 1975

23. Morphologic TV abnormalities
• 90% demonstrated some TV
abnormality in pathologic studies
• Most common : TV Dysplasia, ±
Ebstenoid
• 75% associated with VSD
• Adversely affects surgical repair
• Rarely: Unguarded orifice,
Imperforate, Straddling
Anderson KR et al Circulation1978

24. Clinical Presentation
• Increased Qp : CHF in infancy
• Decreased Qp : TOF physiology/ Ductus dependent
• Arrhythmia : SVT
• CHB
• Heart failure : RV dysfunction, progressive AVVR
• Asymptomatic

25. Clinical Examination
• Depends on associated lesions
• RV apex
• Loud and palpable single S2 (A2) in pulmonary area
- mistaken for PAH
• Systolic murmur of left AVVR - radiates to LSB

26. Chest X Ray
Normal ccTGA

28. ECG
• Absent q waves in V5, V6 > q in V1
• Deep q waves in III, aVF ( situs inverses : has deep q wave in II
Dr SKG )
• Left Axis Deviation , less likely when sub pulmonary LVSP
elevated
• Positive T waves in all precordial leads (> 80%): side-by-side
relationship of the inverted ventricles
• T wave inversion in I, aVL ( DrAS)
• Varying degrees of AV block - 75%
Okamura K et al. J Electrocardiol.6 (1) 3-10. 1973

29. 5 Year Boy, cc TGA ,PS

30. Echocardiogram
•

31. Systemic RV function assessment
• Conventional surrogates of RV function (TAPSE ,
MPI) - correlate poorly with MRI derived RVEF
• Reasons : Altered geometry, change in contractility
pattern
• Global STE-longitudinal - better correlation
Iriart X et al Archives of Cardiovascular Disease (2016) 109, 12-127

32. Systemic RV function parameters
Variable Cut-off
TAPSE (mm) < 14
Pulsed TDI at annulus (cm/s) < 10
Systemic RV GLS(%) >-10 to -14.5
FAC(%) < 29.5 - 33
3D RVEF (%) < 45
. Khattab K Am J Cardiol 2013; 111:908—13 .
Kalogeropoulos AP et al J Am Soc Echocardiogr 2012;25:304—12.

33. Cardiac Catheterization
• Indications
• Assessment of PS and shunt size
• Determination of PVR
• LV Hemodynamic data (prior to anatomic repair, adequacy of
training)
• Multiple VSDs
• MAPCA
• Postoperative : Conduit interventions

34. Angiocardiography
• LV pressure : 98 / 6. RV pressure : 100/10

35. Risk of CHB during cath

36. Catheterisation - Points to ponder
• Difficulty Entering PA : LVOT nearly 180 from the LV inflow
and is deeply wedged between AV valves
• Risk of Heart Block : especially while PA entry
• Mitral valve is more cephalad , difficulty entering the right-
sided LV is occasionally encountered
• Sagittal septum : Frontal and RAO 20-25 profiles the
septum, LVOT and mitral inflow

37. Natural History - Unmodified
• Although clearly not a benign condition, survival into
adulthood is not uncommon
• Impacted by the severity of associated lesions , RV
function, TR, CHB
• Left sided AVVR (TR) : consistently shown to influence
outcomes
• Systemic RV dysfunction accounts for 40-50% of deaths
Connelly et al,J Am Coll Cardiol 1996;27:1238-1243

38. Mortality
• Infant mortality : CHF
Huhta JC et al . Pediatr Cardiol. 1985

39. Freedom from RV dysfunction
• At 45 Years : 45% in ccTGA with associated lesions
and 68 % in isolated ccTGA free of RV dysfunction
Graham et al J Am Coll Cardiol. 2000

40. Effect of AVVR on survival
• 20 year survival 49% and 93% with and without AVVR
Rutledge JM et al Pediatr Cardiol. 2002 , Prieto LR et al Circulation. 1998

41. MANAGEMENT

42. Medical Management
• Neonates : Pulmonary atresia - pGE1
• Infants : Large VSD- Diuretic, ACEi
• Adult : RV dysfunction- Diuretic, ACEi, ARB, Beta
blockers
• Management of SVT

43. Surgical Management
• Conventional / Physiologic Repair : Associated
anomalies are repaired
• Anatomic repair
• One & half ventricle repair : Incorporating a BD Glenn
to partly unload the ventricles
• PA Band - protective/ LV training/ “open end”
palliation
• Fontan pathway

44. Physiologic Repair
• Essentially creates a state of “isolated ccTGA’
• Preferred in patients with
• Good RV and Tricuspid valve function
• Poor mitral valve function, Coronary anomalies,
Dextrocardia, Restrictive VSD, Inlet VSD
Karl TR et al ccTGA .In Pediatric Cardiac Surgery. 3rd
ed. 2003

45. Physiologic Repair - Outcomes
• Immediate surgical mortality
• VSD closure : < 5%
• VSD + LVOTO relief : 10 - 20%
• TV replacement : 15-25%
• Freedom from RV dysfunction : 88% at 1 year, 43% at
10 years.
• Freedom from SAVVR. : 91% at 1 , 52% at 10 years
Hraska V et al J Thorac Cardiovasc Surg. 2005

46. Postoperative Survival
Denktas et al J Thorac Cardiovasc Surg. 2001 Voskuil M et al Am J C
• Most common cause of death : RV failure

47. Freedom from TV reoperation
• About 1–3% of the tricuspid valves need reoperation
per year.

48. Anatomic Repair
• LV made the systemic ventricle
• Requires LV to be Prepared
• Surgeries
• Atrial switch( Mustard/ Senning) + Arterial switch
(Double Switch)
• VSD + LVOTO : Senning - Rastelli

49. Double Switch Operation
• Unobstructed and equal
sized aortic and
pulmonary outflow tracts
• Ideal timing ( 7m - 3.2 Y)
• Early Hosp Mortality : 0 -
7.4%
• Event free survival at 10
years : 70- 85%
Murtuza B et al JTCS 2011
Quinn DW et al JTCS 2008

50. Senning - Rastelli
• In patients with
LVOTO, abnormal
pulmonary valves
• Requires a Sizeable
and routable VSD
Murtuza B et al JTCS 2011

51. LV retraining
• Median banding time for preparing LV reported to be
13-14 months
• Lesser success rates in patients > 12 years of age
(62% vs 20% p = 0.02)
• LV training with PAB beyond 16 yrs is doubtful
• Risk of late LV dysfunction in ccTGA patients who
undergo DSO after successful LV retraining
Poirier, N.C et al JTCS 2004
Quinn et al JTCS 2008, Brawn WJ et al Semin Thorac Cardiovasc Surg 2008

52. Criteria for LV Preparedness
Watanabe et al Ann Thorac Surg 2015

53. Timing of PA Band
• Retrospective Review, Boston (n=25)
• LV dysfunction developed in 4 of 6 patients ( PAB
> 2 yrs) vs 0 of 12 (< 2y)
• Early PAB associated with favorable LV function
after anatomic repair
• Prophylactic PAB in asymptomatic infants to
maintain rather than train the LV
Surg 2013 , Metton O et al Eur J Cardio

54. PA Banding and AVVR
• Decrease severity of TR
• Mechanism : LV became more spherical and the
interventricular septum shifted toward the
morphologic RV
• Dysplastic TV leaflets, Annular dilation, RV
dysfunction : decreased success rate
Kral Kollers et al Am J Cardiol. 2010 Mar

55. PA banding and AVVR
Post PA Band
Pre

56. anatomic repair- Outcomes
• Hospital Mortality : 0 - 15%
• Freedom from death or RV failure : 93% at 15years
(n=63)
Hraska V et al Ann Thorac Surg 2017
Hiramatsu T et al Eur J Cardiothorac Surg 2012
DSO Senning-Rastelli
20 year survival
83&
(n- 18)
76%
(n-72)
10 year freedom
of reoperation
75-85% 57-90%

57. Anatomic vs Physiologic repair
Lim H-G.et al Annals of Thoracic Surgery 2010

58. other surgeries involving septation
• 1½ Ventricular repair (Physiologic)
• When complete LVOTO relief is not possible
• Pulm valvotomy + BD Glenn
• Advantages :
• Avoids Conduit
• By retaining some LVOTO -IVS maintained in midline
- reduced TV distraction and TR
Malhotra et al JTCS 2011

59. Anatomic 1½ ventricular repair
• Hemi-Mustard + Rastelli + BD Glenn
• Especially in patients with small RV, SI, dextrocardia
• Advantages
• Lesser SVC, PV obstruction, arrhythmia
• Reduced ischemic time
• Increased conduit longevity
• Survial benefit
Malhotra et al JTCS 2011

60. Fontan Pathway
• In cases of difficult biventricular repair, Fontan has
shown lower operative risk
• Fontan mortality approaches zero in most centers (91%
survival and 87% freedom from reoperation at 10 years)
• Fontan vs biventricular repair : similar outcomes in
terms of late survival
• Reoperation rates : higher after biventricular repair than
that after Fontan (midterm followup)
Shinoka et al JTCS 2007
Horer et al JTCS 2008

61. Should all patients with ccTGA undergo anatomic repair ?
• Freedom from mortality
or Tx was 86.5% (PR),
79% (AR) and 100%
for Fontan, (P=0.18)
• No d/b DSO or SR
Al Omair M et al J Thorac Cardiovasc Surg 2017

62. Comparison of all strategies
Physiologic
Repair
Anatomic
Repair
Fontan
Complexity ++ +++ +
Operative mortality ++ ++ +
Atrial baffle problems na + na
Late TR +++ + ??
CHB +++ ++ +
Late Morbidity/ Mortality ++++ ++ +
Reoperation ++++ ++++ +
Karl TR et al Annals Ped Cardiol 2011

63. Management algorithm

64. take home messages
• ccTGA is a rare anomaly with very variable presentation
• RV and TV at the systemic position - ideally makes this
defect “uncorrected”
• The natural history is influenced by the associated
lesions and most importantly the RV function and TR
• The ideal surgical option still remains debatable and
should be individualised to the patient, as well as centers
expertise

65. take home messages
• Longterm outcomes of Anatomic repair are yet to be
known completely
• Prophylactic DSO for isolated ccTGA should be
viewed with caution
• With improved outcomes with Fontan pathway, and
the limits of Anatomic repair being pushed further,
the decision between these surgeries remain unclear

66. THANK YOU