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

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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

5

So many surgeries!• ICR/ ASO

• Blalock-taussig• Glenn/ Fontan

• Banding/ TCV repair• Mustard/ senning• Norwood- sano

6

Rome was not built in a day

71945: BT shunt

8

1958Glenn shunt 1971

Fontan surgery1973

Kreutzer

1983Kawashima

9

1954Lillehei: TOF

1957Kirkin: DORV

1959Senning: TGA

1959Mustard: TGA

1966Rashkind: TGA

1975Jatene: ASO

1958Carpentier:

TC repair

1983Norwood

HLH

2003SanoHLH

10

11

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

12

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

13

A gap in understandingGuidelines

• What?• When?

GAP

• Why?

Philosophy behind the surgeries

Surgeon’s perspective

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Necessity

Innovation

15

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

17

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

18

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

22

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

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)

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

40

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

45

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

48

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

50

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

55

Coronary abnormalities

56

So, What to do?

57

Cardiopulmonary Bypass (CPB)

• PUMP• Cross-clapms• Cardioplegia • Hypothermia • Ischaemia• ECMO for neonates

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

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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

60

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

61

Surgeon’s view

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

Surgeon’s choice: transRA+transpulm approach

Hegar passageSubannular 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 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

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/RPANot Discernable

RV – PA conduit

RV – PA conduit

Collateral arteriesanastomosis

Collateral arteriesanastomosis

Unifocalization

66

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

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

68

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

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

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

73

CMRI: one stop shop

• RV function• Coronary artery anomalies• Pulmonary artery & branches• Collaterals • VSD routability• Earlier shunts• Venous drainages

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

75

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

77

Surgeon’s approach for DORV

78

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

79

CCTGA

BT shunt

Surgeon’s choiceDouble switch

Surgeon’s choiceSenning

+ REV

80

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

81

TA

Surgeon’s choiceSV

FONTAN

82

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)

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

84

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

85

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

86

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

87

Truncus Arteriosus

TYPE IVSD

repairRV – PA conduit

TYPE A2

Dacron patch

Anastomosis

88

A long presentation..

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

90

Acknowledgement:Dr. Neeraj Prakash Dr. Sandip Chandra

Dr. Kaushik Chatterjee Thank you