surgical approach of cyanotic congenital heart disease
TRANSCRIPT
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Surgical Approach of Cyanotic CHD
Dr. Dibbendhu Khanra
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Disclaimer
• No cong Acyanotic Heart diseases• No Eisenmenger’s
• No clinical or Echo diagnosis• No medical management• No surgical details
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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
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Cyanotic CHD
PULMONARY STENOSISPulm ESM
NOPULMONARY
STENOSIS
NO VSD VSD PULMONARYHYPERTENSION
NOPULMONARY
HYPERTENSION
INCREASEDPBF
DECREASEDPBF
PULMONARYVENOUS
HYPERTENSION
ASD+PS(Triology)
1
Fallot’sPhysiology
2
Transposition physiology
3
Eisenmenger’s physiology
4Obstructive
TAPVC5
PAVFSV to LA
6
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So many surgeries!• ICR/ ASO
• Blalock-taussig• Glenn/ Fontan
• Banding/ TCV repair• Mustard/ senning• Norwood- sano
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Rome was not built in a day
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71945: BT shunt
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1958Glenn shunt 1971
Fontan surgery1973
Kreutzer
1983Kawashima
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1954Lillehei: TOF
1957Kirkin: DORV
1959Senning: TGA
1959Mustard: TGA
1966Rashkind: TGA
1975Jatene: ASO
1958Carpentier:
TC repair
1983Norwood
HLH
2003SanoHLH
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What we already knowDisease 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 repairRV – PA conduit
3-4 yrs
TAPVC Obstructive Total repair Urgently
Non obstructive Elective repair 1-2 yr
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What we already know (cont.)Disease Types Surgery Timing
PTA CHF Total RepairIf delayed
Urgently PA banding
NO CHF Total Repair 6-12 wks
Ebstein Deep cyanosisRV inadequate
Fontan pathwayASD enlargement
Good RV TCV repair>replacement
HLH Norwood Fontan pathway
3m1-2 yr
TOF like conditionsTwo ventr repair not possible
Mild cyanosis Direct fontanGlenn
3-4 yrs3-4 yrs
TA, SVTGA OR DORV With non-routable VSD
Significant cyanosis
Glenn Fontan
< 6m> 6m
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A gap in understandingGuidelines
• What?• When?
GAP
• Why?
Philosophy behind the surgeries
Surgeon’s perspective
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Necessity
Innovation
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The normal structure
• Two filling chambers• Two pumping chambers• Two septum• Two great vessels• Two coronary arteries
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The fetal circulation
% Cardiac output % saturation Pressure
RV is the main pump in Fetal life
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Fetal vs adult heartPoints Fetal heart Neonatal
heartImplications
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
RV is well trained in Fallot
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Normal relation
• 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)
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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
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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/OENERGY
Classic FontanBypasses RV
With Intact RA
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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 shuntSVC – PA = Glenn (3-6m)IVC – PA = Fontan (1-2yr)• Passive flow/ PVR low• Only when PA adequate
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Aortopulmonary shuntCentral 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 - +
Surgeon’s choice:Mod BT shunt
Side which PA is smallerAspirin for 3-6mSize mismatch
Thrombosis/ obstruction If IL Subclavian if <2.5mmCommon carotid can be used
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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)
Surgeon’s choice:BDG
If VSD not repairable
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Cavopulmonary shunt (IVC)
BDG To
Fontan
HemiFontan to Fontan
Passive (low PVR)
Fontan patient:Swollen face
Pulsations in head / neck veinsPAVF
IJV approach not possible
Surgeon’s choice:BDG to Fontan
Fenestration relieves RA pressureAt the cost of cyanosis
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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
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Complications
Prevalence Timing Reasons Prevention
Thromboembolism
(rarely PVOD)
20% 1st yrAfter 10 yrs
Dilated RAStasis in RA
Low COArryhtmia
Aspirin preferred
+ Warfarin (INR >2)
(high risk cases)
Arrythmia SVT
20-35%MC A flutter
As long as 20 yrs
surgical scarHigh RA pressure
RA distensionsinus node injury
Acute DC shockChronic
Amiodarone
Chronic FatigueExercise
Intolerance
Low COArrythmia/ CMPSyst congestion
Myo remodellingPLE
ACEIDigoxinAvoid
–ve ionotrops
LVF Pulm vein compression by dilated RA
More in classic Fontan
Fontan conversion
TCPC
Fontan complications
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Fontan complications Complications Prevalence Timing Reasons Prevention
Prolonged pl effPLE/ ascitis
Neutr deficinecyImmuodeficiencyThrombogenecity
3%
Bronchitis 1%
3 yrs High SVC pressureLymphatic drainage
impaired Interstitial LeakageL/o α1AT in stool
Loss of ATIII
High protein diet
AB/ vaccineMLCFA
SomatostatinOcteotride
Heparin
Hepatopathy Ascitis
ALICLD
DiureticsSpiranolactone
NO heart transplantation
Cyanosis Fenestration leakMicroemboli PVOD
PAVFPulm dis
Abnormal SVC
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1. Age above 4 years2. Adequate size of right atrium3. Normal systemic venous return 4. mean pulmonary artery pressure (below 15 mmHg)5. Low PVR6. No atrio-ventricular valve regurgitation7. Normal ventricular function8. No distortion of pulm art from prior shunt/ band9. Normal sinus rhythm10. Adequate pulmonary artery size
Ten commandments (Fontan and Baudet)
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Fontan Evolution
Classic Fontan1. SVC – RPA (end to end)
2. RAA – RPA (outlet Valve)3. IVC-RA (inlet valve)
4. ASD closure5. 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
No RV
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Fontan Evolution
Kiwoshima modifications
IVC cont of hemiazygous vein
Total venous return into RPANO RA
Classic Glenn
BD Glenn
Modified Fontan
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Fontan Evolution
Intracardiac tunnel Extracardiac conduit
Fenestration
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Fontan
Classic FontanRAA - RPA
Lateral TunnelIntra-atrial Baffle
PTFEExtra cardiac conduit
Intracardiac baffle
Extracardiac conduit
Pleural effusion ++ +++
Thromboembolism ++ +
SVT +++ +
Age I year > 3yr
Exercise intolerance ++ +++
Surgeon’s choice:1-3 yr: intracardiac>3 yr: extracardiac
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Fenestration right-to-left shuntpop-off valve◦ prevent rapid volume overload to
the lungs◦ Limit caval pressure◦ Increase preload to the systemic
ventricle◦ Increase cardiac output
Cyanosisdecrease pleural effusionsLess hospital stayCan be closed (if required)
Surgeon’s choice:Fenestrated Fontan
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The left heart
• PV – LA (abnormality=TAPVC)• LA – LV• LV – LVOT• LVOT – AO (active pump: high pressure)• AO – BRAIN/ ARMS/ LEGS
Late presenting TGALV is not trained
BT shuntUpper limb is deprived
Surgeon’s choicePAB
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Cyan CHD with increased PBF
PAB
VSD repair
- Anatomical repair- overcomes RV failure
- Qp:Qs = 1:1
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PA banding
Too loose
Too tight
- PBF/ CHF- PAH/ PVOD
- IPPR/ NO CPB
- Pulm Dysfunction- cyanosis
- anatomic distortion- Asym LVH
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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 TGANeeds multiple banding
Surgeon’s choiceProper size hegar should pass
Often PBF reducesAt the cost of
Asymmetric LVHSubaortic AS
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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
Surgeon’s choiceHigh risk of PVOD
And not in a state of repair
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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
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Atrial switch
Mustard Intracardiac Baffle
Senning Pericardial patch
SVC/IVC - LA – LV – PAPV – RA – RV - AO
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Atrial switch
Arrythmia 50%Baffle leak 20%
RV dysfunction / TR 10 %SVC obstruction 5%
Pulm Venous occlusion 3%
Dense adhesion: transition to ASO difficult
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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
Surgeon’s choiceVSD PS (non TOF)
TGA/DORV
Not correcting the abnormal great artrey relation
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RV-PA conduit
Rastelli
VSD routing Long tunnelSubaortic AS
Aneurysm
Operative mortality30%
20 year survival50%
VSD closure Extracardiac conduit
Not suitable for neonateOcclusion high
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REV (Réparation à l'Etage Ventriculaire))
VSD routing RV-PA conduit
Operative mortality20%
Incision above
coronaries
LeCompte
VSD closure
ShortVSD-AO tunnel
Intacardiacconduit
Surgeon’s choiceFor VSD PS
REV
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Nikaidoh
VSD routing RV-PA conduit
Operative mortality10%
Incision below
coronaries
LeCompte
VSD closure
Not suitable for anomalous coronaries
Limited Experience
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Arterial switch operation (ASO)
LeCompte
Coronaryreimplantation
LV functionMust be normal
Difficult Post atrial baffleDense 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
Surgeon’s choiceASO for TGA
Surgeon’s choicefor TGA+VSD+PS
ASO +REV
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Coronary anomalies in TGA
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Damus Kaye Stensel
No Coronaryreimplantation
Subaortic stenosisOften after PAB
AP shuntMPA – Asc aorta
Surgeon’s choiceTGA VSD PSsubaortic AS
Abnormal coronaries
DKS+RV-PA= YASUI procedure
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CCTGA
Atrial switch Arterial switchDouble switch
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HLH
HLH
NorwoodAP shunt
MPA – Asc aorta
SanoRC-PA conduit
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Raskind: Balloon atrial septostomy
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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
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Tricuspid annular Z score
• Z score = observed value – expected value/ SD
RV size and function: CMRI
Z score <-2.5 Small RV size
RV-coronary communicationsRV dependent circulation
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High RV pressurePA 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
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Coronary abnormalities
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So, What to do?
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Cardiopulmonary Bypass (CPB)
• PUMP• Cross-clapms• Cardioplegia • Hypothermia • Ischaemia• ECMO for neonates
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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
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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 morbidity3-12 months of age
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Cath study before ICR
• Pulmonary artery assessments (CT, MRI)• Mascular VSD (Echo)• Abnormal coronaries• Collaterals and embolisation• Previous shunt patency
Surgeon’s choice:To see
Collaterals Coronaries
Shunts
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Surgeon’s view
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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
Surgeon’s choice: transRA+transpulm approach
Hegar passageSubannular patch
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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 Z score<-3: TAP
Z score
Surgeon’s choice:Z score <-3
Transannular patch
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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
Surgeon’s choice:Mild to mod PR is normal
PVR only if PV dysplastic or absent
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Pulmonary artery assessment 3-6m 1-3yr
MPA/ LPA/RPA
MPA/ LPA/RPANot Discernable
RV – PA conduit
RV – PA conduit
Collateral arteriesanastomosis
Collateral arteriesanastomosis
Unifocalization
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Pulmonary artery confluence
TAP• MPA stenosis• LPA/ RPA stenosis near
branch
RV-PA conduit• MPA atresia• Distal branch PS
BT shunt in sick babies
Absent PA unifocalize
the collaterals
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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
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Embolization vs unifocalization
Embolization• Only the large collaterals
Unifocalization • In nonconfluent/ absent PA
Surgeon’s choice:Cath backup:
Preoperaitve embolizationNo cath backup:
Intraoperative embilization
Surgeon’s choice:UnifocalizationMultiple sitting
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Coronary anomalies in TOF
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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
Surgeon’s choice:RV PA conduit
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BTT shuntsOnly 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
Surgeon’s choice:Take down the BT shuntWhen CPB is established
To have blood-free surgical field/ pulm edema
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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
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CMRI: one stop shop
• RV function• Coronary artery anomalies• Pulmonary artery & branches• Collaterals • VSD routability• Earlier shunts• Venous drainages
Surgeon’s choice:RV failure
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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
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Surgeon’s thoughts1. 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?
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DORV
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Surgeon’s approach for DORV
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TGA
Condition Surgery
TGA IVS Atrial switch 2WKSArtreial switch 1YR
PA banding – switch
TGA IVSIf LV func poor
PA banding - switchTwo stage/ high
mortality TGA VSD Switch + VSD repair
If unfavourable coronary anatomy
DKSInstead of ASO
TGA+VSD+PS BT shunt initiallyASO+Rastelli
ASO+REVASO+Nikaidoh
TGA+VSD+subaortic stenosis
DKS
TGA+VSDStraddled TCV (RV small)
BT+ASOBDG – Fontan
TGA+PVOD No repair
Sx not possible early BAS
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CCTGA
BT shunt
Surgeon’s choiceDouble switch
Surgeon’s choiceSenning
+ REV
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Single VentricleVA
Concordant VA
Discordant (Aorta anterior)
Holmes Heart
(PS)
LV typeRV type
(DORV)Non
Inverted(D- TGA)
Inverted (L- TGA)
% 15 25 35 5
Aorta Right Left Side/ ant
Outlet chamber
+ + -
Surgeon’s choiceSV
FONTAN
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TA
Surgeon’s choiceSV
FONTAN
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PA IVSDilated RV
Small RV
Vulvotomy (Ballon/ open)
PV atretic
BT
RV –P A connection
Infandibulum atretic
ResidualRVOTO
Vulvotomy (Ballon/ open)
PGEI
RVOTR ASD closure
BT
BDG
Fontan ASD closure
RV coronary connections
Left alone
TV closure (starnes Op)
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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 choiceCone repair
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HLH
MBT Sano
Connection SCA – IL PA RV - MPA
Supply One lung Both lung
DBP Lesser Higher
Coronary steal + -
SBF PBF
Surgeon’s choiceSano shunt
Within 2 weeks of lifeHigh surgical risk
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HLH
Surgeon’s choiceHybrid Process
B/L PABPDA stent
(1st week: NO CPB)
Norwood sanoRemoval of PAB, PDA stents
(3-6m: CPB)
Fontan1-2 yr+ BDG
BAS may be required
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TAPVRLT Innominate
LT vertical
Supracardiac 50%
RA Coronary sinus
Intracardiac 20%
Infracardiac20%
IVC
Esophageal hiatus
Mixed10%
ASD
PV obstructionResults in
PAH
End to endCom PV - LA
Patch in ASDAll PV to LA
Unroofing End to endCom PV - LA
Ligation
Ligation
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Truncus Arteriosus
TYPE IVSD
repairRV – PA conduit
TYPE A2
Dacron patch
Anastomosis
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A long presentation..
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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
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Acknowledgement:Dr. Neeraj Prakash Dr. Sandip Chandra
Dr. Kaushik Chatterjee Thank you