Surgical Approach of
Cyanotic CHD
Dr. Dibbendhu Khanra
Disclaimer
• No cong Acyanotic Heart diseases
• No Eisenmenger’s
• No clinical or Echo diagnosis
• No medical management
• No surgical details
2
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
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
So many surgeries!
• ICR/ ASO
• Blalock-taussig
• Glenn/ Fontan
• Banding/ TCV repair
• Mustard/ senning
• Norwood- sano
5
Rome was not built in a day
6
1945: BT shunt 7
1958
Glenn shunt 1971
Fontan surgery
1973
Kreutzer
1983
Kawashima 8
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
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
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
A gap in understanding
Guidelines
• What?
• When?
GAP
• Why?
Textbooks
• How ?
Philosophy behind the surgeries
13
Surgeon’s perspective
Necessity
Innovation
14
The normal structure
• Two filling chambers
• Two pumping chambers
• Two septum
• Two great vessels
• Two coronary arteries
15
The fetal circulation
% Cardiac output % saturation Pressure
16
RV is the main pump in Fetal life
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
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)
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
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
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
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
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
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
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
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
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
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
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
Fontan Evolution
Kiwoshima modifications
IVC cont of
hemiazygous vein
Total venous return into RPA
NO RA
Classic Glenn
BD Glenn
Modified
Fontan
30
Fontan Evolution
Intracardiac tunnel Extracardiac conduit
Fenestration
31
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
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
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
Cyan CHD with increased PBF
PAB
VSD
repair
-Anatomical repair
- overcomes RV failure
- Qp:Qs = 1:1
35
PA banding
Too
loose
Too
tight
- PBF/ CHF
- PAH/ PVOD
- IPPR/ NO CPB
- Pulm Dysfunction
- cyanosis
- anatomic distortion
-Asym LVH
36
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
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
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
Atrial switch
Mustard
Intracardiac Baffle
Senning
Pericardial patch
SVC/IVC - LA – LV – PA
PV – RA – RV - AO 40
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
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
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
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
Nikaidoh
VSD routing RV-PA conduit
Operative mortality
10%
Incision
below
coronaries
LeCompte
VSD closure
45
Not suitable for
anomalous coronaries
Limited
Experience
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
Coronary
anomalies in TGA
47
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
CCTGA
Atrial switch Arterial switchDouble switch
49
HLH
HLH
50
Norwood
AP shunt
MPA – Asc aorta
Sano
RC-PA conduit
Raskind: Balloon atrial septostomy
51
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
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
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
Coronary abnormalities
55
So, What to do?
56
Cardiopulmonary Bypass (CPB)
• PUMP
• Cross-clapms
• Cardioplegia
• Hypothermia
• Ischaemia
• ECMO for neonates
57
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
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
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
Surgeon’s view
61
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
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
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
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
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
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
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
Coronary
anomalies in TOF
69
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
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
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
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
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
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
DORV
76
Surgeon’s approach for DORV
77
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
CCTGA
BT shunt
79
Surgeon’s choice
Double switch
Surgeon’s choice
Senning
+ REV
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
TA
81
Surgeon’s choice
SV
FONTAN
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
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
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
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
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
Truncus Arteriosus
TYPE I
VSD
repair
RV – PA
conduit
TYPE A2
Dacron
patch
Anastomosis 87
A long presentation..
88
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
Acknowledgement:
Dr. Neeraj Prakash
Dr. Sandip Chandra
Dr. Kaushik Chatterjee
90
Thank you

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surgical approach of cyanotic congenital heart disease

  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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

Editor's Notes

  • #9: William glenn Francois Marie Fontan Guillermo Kreutze
  • #10: C. (Clarence) Walton Lillehe John Webster Kirklin Ake senning William T mustard William rashkind Adib jatene carpentier William Norwood Shunji sano
  • #11: 50 years of history is lost in translation
  • #27: No antiarrythmic with negative ionotropism So digitalis (not potent) or amiodarone (proling QT and VT, may need ICD)
  • #30: RA RV INLET VALVE OUTLET VALVE RA RV RA RV RA
  • #31: Kiwoshima a forgotten hero The way vivien thomas a forgotten hero
  • #47: Supravalvular PS (12%) Commonest complication Commonest indication for reoperation
  • #64: 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
  • #78: 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
  • #81: Oulet foramen or bulvoventricular foramen
  • #82: Kuhne et al
  • #83: Rule out ebstein
  • #84: 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