presented in LPS institute of cardiology on 15.2.17 by Dr Dibbendhu Khanra 2nd year DM resident

1. Surgical Approach of
Cyanotic CHD
Dr. Dibbendhu Khanra

2. Disclaimer
• No cong Acyanotic Heart diseases
• No Eisenmenger’s
• No clinical or Echo diagnosis
• No medical management
• No surgical details
2

3. Parts of Discussion
• Introduction
• History
• Fetal and Adult circulation
• Pulmonary artery and PBF
• Shunt
• Fontan & complications
• PAB and BAS
• ICR & ASO
• Surgeon’s perspective
• Individual defect and m/n
3

4. Cyanotic CHD
PULMONARY
STENOSIS
Pulm ESM
NO
PULMONARY
STENOSIS
NO VSD VSD PULMONARY
HYPERTENSION
NO
PULMONARY
HYPERTENSION
INCREASED
PBF
DECREASED
PBF
PULMONARY
VENOUS
HYPERTENSION
ASD+PS
(Triology)
1
Fallot’s
Physiology
2
Transpositio
n physiology
3
Eisenmenger’s
physiology
4
Obstructive
TAPVC
5
PAVF
SV to LA
6
4

5. So many surgeries!
• ICR/ ASO
• Blalock-taussig
• Glenn/ Fontan
• Banding/ TCV repair
• Mustard/ senning
• Norwood- sano
5

6. Rome was not built in a day
6

7. 1945: BT shunt 7

8. 1958
Glenn shunt 1971
Fontan surgery
1973
Kreutzer
1983
Kawashima 8

9. 1954
Lillehei: TOF
1957
Kirkin: DORV
1959
Senning: TGA
1959
Mustard: TGA
1966
Rashkind: TGA
1975
Jatene: ASO
1958
Carpentier:
TC repair
1983
Norwood
HLH
2003
Sano
HLH
9

10. 10

11. What we already know
Disease Types Surgery Timing
TGA NO VSD Rashkind/ BAS If switch delayed
Artreial switch 3-4 wk
TGA VSD LV inadequate Atrial switch 3-6 m
LV adequate Arterial switch 3 m
TOF Uncontrolled spells BT shunt <3 m
Stable Total repair 1-2 yrs
TOF PA severe cyanosis BT shunt <3 m
Post-shunt Total repair
RV – PA conduit
3-4 yrs
TAPVC Obstructive Total repair Urgently
Non obstructive Elective repair 1-2 yr
11

12. What we already know (cont.)
Disease Types Surgery Timing
PTA CHF Total Repair
If delayed
Urgently
PA banding
NO CHF Total Repair 6-12 wks
Ebstein Deep cyanosis
RV inadequate
Fontan pathway
ASD enlargement
Good RV TCV repair>
replacement
HLH Norwood
Fontan pathway
3m
1-2 yr
TOF like conditions
Two ventr repair not possible
Mild cyanosis Direct fontan
Glenn
3-4 yrs
3-4 yrs
TA, SV
TGA OR DORV
With non-routable VSD
Significant
cyanosis
Glenn
Fontan
< 6m
> 6m
12

13. A gap in understanding
Guidelines
• What?
• When?
GAP
• Why?
Textbooks
• How ?
Philosophy behind the surgeries
13
Surgeon’s perspective

14. Necessity
Innovation
14

15. The normal structure
• Two filling chambers
• Two pumping chambers
• Two septum
• Two great vessels
• Two coronary arteries
15

16. The fetal circulation
% Cardiac output % saturation Pressure
16
RV is the main pump in Fetal life

17. Fetal vs adult heart
Points Fetal heart Neonatal
heart
Implications
Lungs Immatured Matured PBF not mandatory in fetus
MPA Small Large PBF less in fetus
PVR Very high less PVR falls with first cry
RV Main pump Smaller RV large and thick in fetus
PDA R-L L-R PDA closes by 2 wks
FO R-L L-R PFO closes by birth
Circulation parallel series Better O2 pickup & delivery
17
RV is well trained in Fallot

18. Normal relation
18
• SVC/IVC – PA, PV – AO (CPB)
• PA – both Lung (collaterals, shunts)
• LV-AO, RV-PA (VSD routing/ switch)
• PA anterior and to the left of aorta (Le Compte)
• Coronaries from Aorta (TGA, TOF)

19. Target for surgery
Priority wise
• Systemic blood flow (Norwood, VSD routing)
• PA maturation/confluence (AP shunt, RV-PA conduit, PDA stent)
• Pulmonary blood flow (BDG/ Fontan) (PA banding)
• Managing collaterals (embolization/ unifocalization)
• VA switch (atrial/ ventricular/ artreial)
• Aorta/ PA relation (Le Compte)
• Shunt repair/ closure (ASD/ VSD/ PDA/ AP shunt/ conduit)
• Take care of coronaries
19

20. The right heart
• SVC – RA (passive)
• IVC – RA (passive)
• RA – RV (RA = flowing reservoir)
• RV – RVOT (active pump)
• RVOT – MPA
• MPA – LPA – LT LUNG
• MPA – RPA –RT LUNG
L/O
ENERGY
20
Classic Fontan
Bypasses RV
With Intact RA

21. PBF
PA growth
• PA in-confluent
• In Pulm atresia/ absent PA
• P annular hypoplashia
• Collaterals
• Aorto-pulmonary shunt (few wks)
• PDA stenting
• RV – PA conduit
• Active flow
• Lung maturation
• Makes PA adequate
Complete venous drainage
• RV not functional
• TA
• SV
• PA IVS small RV
• Ebstein with small RV
• Cavo-pulmonary shunt
SVC – PA = Glenn (3-6m)
IVC – PA = Fontan (1-2yr)
• Passive flow/ PVR low
• Only when PA adequate 21

22. Aortopulmonary shunt
Central shunt:
- CHF
- PAH
- Distorted PA
- Difficult to close
Classical BT Modified BT
Connection End to side Side to side
Material Rt SA Gore tex (Lt SA)
Upper limb Less Growth Normal growth
PA Rt PA (I/L) Lt PA (I/L)
Arch Opposite side Same side
Age >3m <3m
Thrombosis High in <3m Common
Size mismatch - +
22
Surgeon’s choice:
Mod BT shunt
Side which PA is smaller
Aspirin for 3-6m
Size mismatch
Thrombosis/ obstruction If IL Subclavian if <2.5mm
Common carotid can be used

23. Cavopulmonary shunt (SVC)
Classic Glenn Modified Glenn (BDG) Hemi Fontan
Classic Glenn BDG /BDCPA
Connection End to end End to side
Flow unidirectional Bidirectional
Left lung Deprived Normal growth
Cavopulmonary shunt
-IVC blood bypasses lung
- No Hepatic vasoconstrictor PG
-PAVF
- remain cyanotic
Passive
(low PVR)
23
Surgeon’s choice:
BDG
If VSD not repairable

24. Cavopulmonary shunt (IVC)
BDG
To
Fontan
HemiFontan
to Fontan
Passive
(low PVR)
Fontan patient:
Swollen face
Pulsations in head / neck veins
PAVF
IJV approach not possible
24
Surgeon’s choice:
BDG to Fontan
Fenestration relieves RA pressure
At the cost of cyanosis

25. Fontan (TCPC)
• Total cavo-pulmonary connection
• Physiologically flawed
• Cyanosis
• RA overloaded
• Chronic low CO
• Syst ven congestion
• Exercise intolerance
• Arrythmia
• Thromboembolism
• Pulm vein compression
• PLE
• CLD
• No Heart transplant
• Obstructed FONTAN
25

26. Complications Prevalence Timing Reasons Prevention
Thrombo
embolism
(rarely PVOD)
20% 1st yr
After
10 yrs
Dilated RA
Stasis in RA
Low CO
Arryhtmia
Aspirin
preferred
+ Warfarin
(INR >2)
(high risk cases)
Arrythmia
SVT
20-35%
MC A flutter
As
long as
20 yrs
surgical scar
High RA pressure
RA distension
sinus node injury
Acute
DC shock
Chronic
Amiodarone
Chronic
Fatigue
Exercise
Intolerance
Low CO
Arrythmia/ CMP
Syst congestion
Myo remodelling
PLE
ACEI
Digoxin
Avoid
–ve ionotrops
LVF Pulm vein compression
by dilated RA
More in classic Fontan
Fontan
conversion
TCPC
Fontan complications
26

27. Fontan complications
Complications Prevalence Timing Reasons Prevention
Prolonged pl eff
PLE/ ascitis
Neutr deficinecy
Immuodeficiency
Thrombogenecity
3%
Bronchitis
1%
3 yrs High SVC pressure
Lymphatic drainage
impaired
Interstitial Leakage
L/o α1AT in stool
Loss of ATIII
High protein
diet
AB/ vaccine
MLCFA
Somatostatin
Octeotride
Heparin
Hepatopathy
Ascitis
ALI
CLD
Diuretics
Spiranolactone
NO heart
transplantation
Cyanosis Fenestration leak
Microemboli PVOD
PAVF
Pulm dis
Abnormal SVC 27

28. 1. Age above 4 years
2. Adequate size of right atrium
3. Normal systemic venous return
4. mean pulmonary artery pressure (below 15 mmHg)
5. Low PVR
6. No atrio-ventricular valve regurgitation
7. Normal ventricular function
8. No distortion of pulm art from prior shunt/ band
9. Normal sinus rhythm
10. Adequate pulmonary artery size
Ten commandments
(Fontan and Baudet)
28

29. Fontan Evolution
Classic Fontan
1. SVC – RPA (end to end)
2. RAA – RPA (outlet Valve)
3. IVC-RA (inlet valve)
4. ASD closure
5. MPA ligated
Kreutzer
modification
1. RAA – MPA
2. ASD closure
Bjork modifications
1. RAA – RVOT
2. ASD closure
1. RAA – RPA
2. ASD closure
No valveinlet/ outlet valve
RA
RV
RA
29
No RV

30. Fontan Evolution
Kiwoshima modifications
IVC cont of
hemiazygous vein
Total venous return into RPA
NO RA
Classic Glenn
BD Glenn
Modified
Fontan
30

31. Fontan Evolution
Intracardiac tunnel Extracardiac conduit
Fenestration
31

32. Fontan
Classic Fontan
RAA - RPA
Lateral Tunnel
Intra-atrial Baffle
PTFE
Extra cardiac conduit
Intracardiac
baffle
Extracardiac
conduit
Pleural effusion ++ +++
Thromboembolism ++ +
SVT +++ +
Age I year > 3yr
Exercise intolerance ++ +++ 32
Surgeon’s choice:
1-3 yr: intracardiac
>3 yr: extracardiac

33. Fenestration
 right-to-left shunt
 pop-off valve
◦ prevent rapid volume overload to
the lungs
◦ Limit caval pressure
◦ Increase preload to the systemic
ventricle
◦ Increase cardiac output
 Cyanosis
 decrease pleural effusions
 Less hospital stay
 Can be closed (if required)
33
Surgeon’s choice:
Fenestrated Fontan

34. The left heart
• PV – LA
(abnormality=TAPVC)
• LA – LV
• LV – LVOT
• LVOT – AO
(active pump: high pressure)
• AO – BRAIN/ ARMS/ LEGS
Late presenting TGA
LV is not trained
34
BT shunt
Upper limb is deprived
Surgeon’s choice
PAB

35. Cyan CHD with increased PBF
PAB
VSD
repair
-Anatomical repair
- overcomes RV failure
- Qp:Qs = 1:1
35

36. PA banding
Too
loose
Too
tight
- PBF/ CHF
- PAH/ PVOD
- IPPR/ NO CPB
- Pulm Dysfunction
- cyanosis
- anatomic distortion
-Asym LVH
36

37. PA banding
How tight?
• Diamater 50% reduction
- TRUSLAR FORMULA
NRGA : 20mm+1mm/KgBW
TGA: 24mm+1mm/KgBW
• mPAP 50% reduction
• Maintaining SPO2 to 93%
Where to band?
• MPA (not annulus)
• If too high
- branch PA stenosed
• If too low
- coronary reimplntation difficult
Not reliable in TGA
Needs multiple banding
37
Surgeon’s choice
Proper size hegar should pass
Often PBF reduces
At the cost of
Asymmetric LVH
Subaortic AS

38. PA banding: Indicatons
• Very sick neonate on IPPR
can not tolerate CPB
chance of early PVOD (TGA, ECD)
• Complex congenital CHD
e.g. criss cross heart, swiss cheese VSD
small fetal heart
• Biventricular repair not possible
Preparation for Glenn/Fontan
PVR needs to be low for passive forward flow
• Preparation for ASO
Late presenting TGA with CHF
• HLHS: stage I Hybdrid procedure
Bilateral PA banding 38
Surgeon’s choice
High risk of PVOD
And not in a state of repair

39. VA relation establishment: switch
• Atrial level
• Ventricular level
• Great arterial level
• Le Compte (PA anterior to Ao)
• Coronary artery manipulation
RV
systemic ventricle
LV
systemic ventricle
Physiological
repair
Anatomical
repair
39

40. Atrial switch
Mustard
Intracardiac Baffle
Senning
Pericardial patch
SVC/IVC - LA – LV – PA
PV – RA – RV - AO 40

41. Atrial switch
Arrythmia 50%
Baffle leak 20%
RV dysfunction / TR 10 %
SVC obstruction 5%
Pulm Venous occlusion 3%
Dense adhesion:
transition to ASO difficult
41

42. Switch at ventricular level
• VSD closure
• LV – AO tunnel
• RV – PA conduit
• Le Compte (PA brought anterior to Ao)
• No Coronary reimplantation
VSD routing SBF
PBF
42
Surgeon’s choice
VSD PS (non TOF)
TGA/DORV
Not correcting the
abnormal great
artrey relation

43. RV-PA conduit
Rastelli
VSD routing
Long tunnel
Subaortic AS
Aneurysm
Operative mortality
30%
20 year survival
50%
VSD closure
43
Extracardiac conduit
Not suitable for neonate
Occlusion high

44. REV (Réparation à l'Etage Ventriculaire)
)
VSD routing RV-PA conduit
Operative mortality
20%
Incision
above
coronaries
LeCompte
VSD closure
44
Short
VSD-AO tunnel
Intacardiac
conduit
Surgeon’s choice
For VSD PS
REV

45. Nikaidoh
VSD routing RV-PA conduit
Operative mortality
10%
Incision
below
coronaries
LeCompte
VSD closure
45
Not suitable for
anomalous coronaries
Limited
Experience

46. Arterial switch operation (ASO)
LeCompte
Coronary
reimplantation
LV function
Must be normal
Difficult
Post atrial baffle
Dense adhesion
LV dysfunction:
PA Band – ASO
not enough for
-TGA PS (fallot)
- TGA AS (PAB)
- Coronary anomalies
Complications
-Supravalvular PS (12%)
-Neoaortic regurgitation
-Coronary artery obstruction
46
Surgeon’s choice
ASO for TGA
Surgeon’s choice
for TGA+VSD+PS
ASO +REV

47. Coronary
anomalies in TGA
47

48. Damus Kaye Stensel
No Coronary
reimplantation
Subaortic stenosis
Often after PAB
AP shunt
MPA – Asc aorta
48
Surgeon’s choice
TGA VSD PS
subaortic AS
Abnormal coronaries
DKS+RV-PA
= YASUI procedure

49. CCTGA
Atrial switch Arterial switchDouble switch
49

50. HLH
HLH
50
Norwood
AP shunt
MPA – Asc aorta
Sano
RC-PA conduit

51. Raskind: Balloon atrial septostomy
51

52. The right ventricle
PA without VSD
Normal RV
-Inflow
- Trabecule
- Infandibulum
(outflow)
O
TI I I I
O O
T
Tripartite RV
(Z score >-2.5)
-Inflow
- Trabecule
- Infandibulum
(outflow)
Bipartite RV
(Z score -2.5 to -5)
-Inflow
-Infandibulum
(outflow)
Monopartite RV
(Z score <-5)
-Inflow
Biventricular repair Univentricular repair
52

53. Tricuspid annular Z score
• Z score = observed value – expected value/ SD
RV size and function: CMRI
53
Z score <-2.5
Small RV size
RV-coronary communications
RV dependent circulation

54. High RV pressure
PA without VSD
- RV myocardial fibrosis, ischaemia or infarction
- RV decompressed through RV – coronary connections
- If prox coronary art absent – RV dependent coronaries (Hhb)
- However, presence of TR or VSD or RV-PA conduit decompresses RV pressure
- RV decompression leads to coronary steal 54

55. Coronary abnormalities
55

56. So, What to do?
56

57. Cardiopulmonary Bypass (CPB)
• PUMP
• Cross-clapms
• Cardioplegia
• Hypothermia
• Ischaemia
• ECMO for neonates
57

58. Surgical approach
Total repair
• Definite / desired
• Anatomical repair
• CPB required
• VSD repair
• RVOTO relief
• ASO/ DKS
• Collateral closure
• unifocalization
Palliation
• Total repair not possible
• Anatomical reasons
• CPB not tolerable
• AP shunt/ RV PA conduit
• Glenn/ Fontan
• PAB
• BAS
• ASO/ DKS
58

59. TOF
Palliative
• AP shunt
• RVOT stenting
• MAPCA embolzation
Definitive ICR
- VSD closure
- RVOTO relief
- TAP for hypoplastic annulus
- Intact PV/ FU for PR/RV dysfunction
- Confluence of PA
- Unifocalization
- Avoid injury to coronaries
- Any other defect - repair
Lowest morbidity
3-12 months of age
59

60. Cath study before ICR
• Pulmonary artery assessments (CT, MRI)
• Mascular VSD (Echo)
• Abnormal coronaries
• Collaterals and embolisation
• Previous shunt patency
60
Surgeon’s choice:
To see
Collaterals
Coronaries
Shunts

61. Surgeon’s view
61

62. Pulmonary infandibulum assessment
• RA incision routinely
• VSD repair with Dacron patch
• A Hegar dilator (as per Z table) pass through TCV
• If passes freely thru RVOTO, no resection needed
• If does not passes, resection of RVOT done
• Sewed back with Dacron or PTFE patch
• Patch is always kept subannular to avoid PV injury
62
Surgeon’s choice:
transRA+transpulm approach
Hegar passage
Subannular patch

63. Pulmonary annulus assessment
MC GOON RATIO
• Diameter
• RPA+LPA/DA
• N = 2-2.5
• <1.5 : BT shunt
• >1.8: Fontan
• <1.5 : TAP
NAKATA INDEX (mm2/m2)
• Area
• RPA+LPA/BSA
• N = 330 +/- 30
• <200 : BT shunt
• >250: Fontan
• <200 : TAP
63
Z score<-3: TAP
Z score
Surgeon’s choice:
Z score <-3
Transannular patch

64. Pulmonary valve assessment
• In subannular patch Pulm valve not injured
• In transannular patch Pulm valve Is injured
• Mild to moderate PR develops
• But RV is trained so no RV dysfunction
• FU for more than severe PR or RV dysfunction
• PVR(bovine jugular, monocusp, porcine valve)
• PVR must be done in absent or dysplastic PV
64
Surgeon’s choice:
Mild to mod PR is normal
PVR only if PV dysplastic or absent

65. Pulmonary artery assessment
3-6m 1-3yr
MPA/ LPA/RPA
MPA/ LPA/RPA
Not Discernable
RV – PA conduit
RV – PA conduit
Collateral arteries
anastomosis
Collateral arteries
anastomosis
65
Uni
focalization

66. Pulmonary artery confluence
TAP
• MPA stenosis
• LPA/ RPA stenosis near
branch
RV-PA conduit
• MPA atresia
• Distal branch PS
66
BT shunt
in sick babies
Absent PA
unifocalize
the collaterals

67. Embolization of collaterals
• TOF Pulm atresia – more than 3yrs
• Routine CAG for collaterals
• Embolize if >2.5mm pre-operatively
• More chance of bleeding
• Pulmonary edema
• Intraoperative embolization also done
67

68. Embolization vs unifocalization
Embolization
• Only the large collaterals
Unifocalization
• In nonconfluent/ absent PA
68
Surgeon’s choice:
Cath backup:
Preoperaitve embolization
No cath backup:
Intraoperative embilization
Surgeon’s choice:
Unifocalization
Multiple sitting

69. Coronary
anomalies in TOF
69

70. Coronary anomaly assessment
• Long conus artery crossing RVOT
• RVOT resection is risky in infandibular stenosis
• Try RVOT stenting by total atrial approach
• RV to PA conduit
• Sometimes BT shunt is the only palliation
70
Surgeon’s choice:
RV PA conduit

71. BTT shunts
Only to buy time for ICR
• Wt <2 kg or very sick newborn
• MPA atresia
(RV –PA conduit)
• Hypoplastic Pulm Annulus
(Transannular patch)
• Unfavourable Coronaries
• Uncontrollable cyanosis
• Distal branch PA stenosis
• Too small for surgery
• Too sick for CPB
AP shunts: pitfalls
• Cyanosis
• I/L Radial pulse absent
• Less growth of upper limb
• High PBF
• Chronic LVF
• PVOD
• Focal PA stenosis
• Rib notching
71
Surgeon’s choice:
Take down the BT shunt
When CPB is established
To have blood-free surgical field/ pulm edema

72. Outcome of ICR
Long-term Sequale of ICR
• PR
• Residual RVOTO
• Residual VSD/ ASD
• Arrythmia (QRS>160 ms)
• TR
• LV dysfunction
• PA stenosis
• RVOT aneurysm
Results of severe PR
• RV dilation
• RV failure
• TR
• Arrythmia
• Sudden death
72

73. CMRI: one stop shop
• RV function
• Coronary artery anomalies
• Pulmonary artery & branches
• Collaterals
• VSD routability
• Earlier shunts
• Venous drainages
73
Surgeon’s choice:
RV failure

74. Severe PR
ECHO MRI
• Moderate or more PR
• PLUS:2 or more of
- RVEDV ≥ 160 ml/m2 (Z-score >5)
- RVESV ≥ 70 ml/m2
- LVEDV ≤ 65 ml/m2
- RV EF ≤ 45%
- RVOT aneurysm
• PR PHT>100ms
Severe PR plus
- New onset VT
- Severe exercise intolerance
- Right heart failure
-Late repair
PVR
74

75. Surgeon’s thoughts
1. Is VSD repairable?
2. How is the RV?
3. Is VSD routable?
4. Are the great arteries normally related?
5. Is there PS? need of patch?
6. How are the pulmonary arteries? (unifocalization? MAPCA embolization)
7. How is the pulmonary valve?
8. Are coronaries crossing over RVOT?
9. Any other repairable defects/ or lesions?
10. Previous shunt or conduit or bands?
75

76. DORV
76

77. Surgeon’s approach for DORV
77

78. TGA
Condition Surgery
TGA IVS Atrial switch 2WKS
Artreial switch 1YR
PA banding – switch
TGA IVS
If LV func poor
PA banding - switch
Two stage/ high mortality
TGA VSD Switch + VSD repair
If unfavourable coronary
anatomy
DKS
Instead of ASO
TGA+VSD+PS BT shunt initially
ASO+Rastelli
ASO+REV
ASO+Nikaidoh
TGA+VSD
+subaortic stenosis
DKS
TGA+VSD
Straddled TCV (RV small)
BT+ASO
BDG – Fontan
TGA+PVOD No repair
Sx not possible early BAS
78

79. CCTGA
BT shunt
79
Surgeon’s choice
Double switch
Surgeon’s choice
Senning
+ REV

80. Single Ventricle
VA
Concordant
VA
Discordant
(Aorta anterior)
Holmes Heart
(PS)
LV type
RV type
(DORV)
Non
Inverted
(D- TGA)
Inverted
(L- TGA)
% 15 25 35 5
Aorta Right Left Side/ ant
Outlet
chamber
+ + -
80
Surgeon’s choice
SV
FONTAN

81. TA
81
Surgeon’s choice
SV
FONTAN

82. PA IVS
Dilated RV
Small RV
Vulvotomy
(Ballon/ open)
PV atretic
BT
RV –P A
connection
Infandibulum atretic
Residual
RVOTO
Vulvotomy
(Ballon/ open)
PGEI
RVOTR ASD closure
BT
BDG
Fontan ASD closure
RV coronary
connections
Left alone
TV closure
(starnes Op)
82

83. Ebstein’s
Adult
- severe progressive cyanosis
- RVOTO
- NYHA 3-4 poor activity
- paradoxical embolus
- arrythmia
- RV dysfunction
Neonate: CHF/ cyanosis
-Biventricular repair
(Knott Craig approach)
-Single ventricular repair
(strane’s TC closure –Fontan)
83Ebstein
Danielson
Carpentier
De silva’s
Cone repair
Surgeon’s choice
Cone repair

84. HLH
MBT Sano
Connection SCA – IL PA RV - MPA
Supply One lung Both lung
DBP Lesser Higher
Coronary steal + -
SBF PBF
84
Surgeon’s choice
Sano shunt
Within 2 weeks of life
High surgical risk

85. HLH
Surgeon’s choice
Hybrid Process
B/L PAB
PDA stent
(1st week: NO CPB)
Norwood sano
Removal of PAB, PDA stents
(3-6m: CPB)
Fontan
1-2 yr
+ BDG
BAS
may be required
85

86. TAPVR
LT Innominate
LT
vertical
Supracardiac
50%
RA Coronary sinus
Intracardiac
20%
Infracardiac
20%
IVC
Esophageal
hiatus
Mixed
10%
ASD
PV
obstruction
Results in
PAH
End to end
Com PV - LA
Patch in ASD
All PV to LA
Unroofing End to end
Com PV - LA
Ligation
Ligation
86

87. Truncus Arteriosus
TYPE I
VSD
repair
RV – PA
conduit
TYPE A2
Dacron
patch
Anastomosis 87

88. A long presentation..
88

89. Take home messages
• AP shunts are only time buying
• Always Modified BT
• Repair when repairable
• Subannular patch. TAP causes PR. Long term RV dysfunction
• Collaterals – embolize or unifocalize
• Fontan is only when repair not possible
• Fontan complicated!
• PAB/ BAS has fallen out of grace except special indication
• ASO is the choice for TGA/ REV in PS/ DKS in AS
• RV plays a big role. CMRI is gold starndard
• PA IVS: ventriculo-coronary connections
• Ebstein: Cone Reconstruction
• CT angio: coronary abnormalities
89

90. Acknowledgement:
Dr. Neeraj Prakash
Dr. Sandip Chandra
Dr. Kaushik Chatterjee
90
Thank you