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
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
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
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
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
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
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
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
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
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
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
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
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
William glenn
Francois Marie Fontan
Guillermo Kreutze
C. (Clarence) Walton Lillehe
John Webster Kirklin
Ake senning
William T mustard
William rashkind
Adib jatene
carpentier
William Norwood
Shunji sano
50 years of history is lost in translation
No antiarrythmic with negative ionotropism
So digitalis (not potent) or amiodarone (proling QT and VT, may need ICD)
RA RV INLET VALVE OUTLET VALVE
RA RV
RA RV
RA
Kiwoshima a forgotten hero
The way vivien thomas a forgotten hero
Supravalvular PS (12%)
Commonest complication
Commonest indication for reoperation
ANGIOGRAPHIC ASSESSMENT
MC GOON
LPA RPA IMMEDIATE PREBRANCHING PORTION
DA JUST ABOVE DIAPHRAGM
NAKATA
IMMEDIATELY PROX TO ORIGIN OF FIRST LOBAR BRANCHES
AT MAXIMAL AND MINIMAL DURING ONE CARDIAC CYCLE
IN AP VIEW OF PULM ARTIOGRAM
3,14XR2XMAGNIFICATION COEFFICIENT
Patrick mc goon IVR repair AO/ PA are ant post or l position
Kawashima IVR in TBA with large distance b/w TCV and PV
Oulet foramen or bulvoventricular foramen
Kuhne et al
Rule out ebstein
Great Ormond Street Echocardiography (GOSE) score
The ratio of the combined area of the RA and atrialized RV is compared to the functional RV and left heart