Surgical Approach of Cyanotic CHD

DR PARMINDER SINGH
 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
2
 Cyanotic
CHD
NO
PULMONAR
PULMONAR
Y STENOSIS
Y STENOSIS
Pulm ESM

NO VSD VSD PULMONARY NO PAVF

HYPERTENSION PULMONARY SVCto
ASD+PS Fallot’s HYPERTENSI
Physiolo LA 6
(Triolog ON
y) 1 gy 2
PULMONARY
INCREASED DECREASED
VENOUS
PBF PBF
HYPERTENSI
Transpositio Eisenmenger ON
n ’s physiology Obstructive
physiology 4 TAPV
4
3 C
Rome was not built in a
day 4
1958
Glenn 1971
shunt Fontan
surgery 1973
Kreutze
r

1983
 1954 1957 1959 1959
Lillehei: Kirkin: DORV Senning: Mustard:
TOF TGA TGA
1983
Norwoo
d HLH

2003
San
o
1966 1958 1975 HLH
Carpentie
Rashkind: r: TC Jatene: 9
Anita Saxena et al : Indian Paediatrics Journal 2008
10
 Consensus
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 3m

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 3-4 yrs

RV – PA conduit
TAPVC Obstructive Total repair Urgently

Non obstructive Elective repair 1-2 yr

11
 Disease Types Surgery Timing

PTA CHF Total Repair Urgently

If delayed PA
banding
NO CHF Total Repair 6-12 wks

Ebstein Deep cyanosis Fontan pathway

RV ASD
inadequate enlargement
Good RV TCV repair>
replaceme
nt
HLH Norwood 3m
Fontan 1-2 yr
pathway
TOF like conditions Mild cyanosis Direct 3-4 yrs
Two ventr repair not possible fontan 3-4 yrs
Glenn
9
TA, SV Significant Glenn < 6m
 The normal
structure

• Two filling chambers

• Two pumping
chambers
• Two septum
• Two great vessels
• Two coronary arteries

10
 The fetal
circulation

% Cardiac output % Pressur

saturation e
RV is the main pump in Fetal
life 11
 Fetal vs Adult
heart
Points Fetal heart Neonat Implications
al
heart
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
12
 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)

13
 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

14
 The right
heart
• SVC – RA (passive)
• IVC – RA (passive)
Classic Fontan
L/O • RA – RV (RA = flowing Bypasses RV
ENERG With Intact
Y reservoir) RA
• RV – RVOT (active pump)
• RVOT – MPA
• MPA – LPA – LT LUNG
• MPA – RPA –RT LUNG
15
 PB
F
PA growth Complete venous
• PA in-confluent drainage
• In Pulm atresia/ absent PA • RV not functional
• P annular hypoplashia • TA
• Collaterals • SV
• Aorto-pulmonary shunt (few • PA IVS small RV
wks)
• PDA stenting
• Ebstein with small RV
• RV – PA conduit • Cavo-pulmonary shunt
• Active flow SVC – PA = Glenn (3-6m)
• Lung maturation IVC – PA = Fontan (1-2yr)
• Makes PA adequate • Passive flow/ PVR low
• Only when PA adequate 16
 Aortopulmonary
shunt
Central shunt:
- CHF
- PAH
- Distorted PA
- Difficult to
closeClassical 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
Surgeon’s choice: Age >3m <3m
Mod BT shunt
Thrombosis High in <3m Common
Side which PA is
smaller Aspirin for Size mismatch - +
3-6m Size
mismatch If IL Subclavian if <2.5mm
22
Thrombosis/ obstruction Common carotid can be
 Cavopulmonary
shunt (SVC)
Passive
(low
PVR)

Surgeon’s choice:
BDG
If VSD not repairable
Classic Modified Glenn (BDG) Hemi Fontan
Glenn
Classic Glenn BDG /BDCPA Cavopulmonary shunt
Connection End to end End to side -IVC blood bypasses lung
- No Hepatic vasoconstrictor
Flow unidirectional Bidirectional
PG
Left lung Deprived Normal growth -PAVF
- remain
cyanotic 18
 Cavopulmonary
shunt (IVC)
Passive
(low Fenestration relieves RA
PVR) pressure At the cost of
BDG cyanosis
To
Fonta Fontan patient:
n Swollen face
Pulsations in head / neck
veins PAVF
IJV approach not possible
HemiFonta
n to
Fontan

Surgeon’s choice:
BDG to Fontan

19
 Fontan
(TCPC)

• Total cavo-pulmonary
connection
• Physiologically flawed • Pulm vein compression
• PLE
• Cyanosis
• CLD
• RA overloaded • No Heart transplant
• Chronic low CO • Obstructed FONTAN
• Syst ven congestion
• Exercise intolerance
• Arrythmia
• Thromboembolism
20
 Ten commandments (Fontan and
Baudet)
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 28
 Fontan
Evolution

Classic Fontan Kreutzer Bjork

1. SVC – RPA (end to end) modificatio modifications
2. RAA – RPA (outlet n
Valve) 1. RAA – 1. RAA –
3. IVC-RA
4. ASD(inlet valve) 1. RAA – RVOT RPA
closure MPA 2. ASD 2. ASD
5. MPA closure closure
inlet/ligated
outlet valve No valve R No
2. ASD
R R
closure V RV 29
 Fontan
Evolution

Classic
Glenn

Modified
Fontan

IVC cont of
hemiazygous
vein BD
Glenn
Kiwoshima
modifications
Total venous return into
RPA NO RA
23
 Fontan
Evolution

Fenestration

Intracardiac tunnel Extracardiac conduit

24
 Font
an

Classic Lateral Tunnel PTFE

Fontan Intra-atrial Extra cardiac
RAA - RPA Baffle Intracardi conduit
Extracardi
ac ac
Surgeon’s choice: baffle conduit
1-3 yr: Pleural effusion ++ +++
intracardiac
Thromboembolism ++ +
>3 yr:
extracardiac SVT +++ +
Age I year > 3yr
Exercise intolerance ++ +++
 Fenestrati
on
 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
 Cyanosis
output
 decrease pleural
effusions
Surgeon’s choice:  Less hospital stay
Fenestrated
Fontan  Can be closed (if
required) 26
 complications
Complications Prevalence Timing Reasons Prevention
Thrombo 20% 1st yr Dilated RA Aspirin
embolism After Stasis in preferred
(rarely 10 RA Low CO + Warfarin
PVOD) yrs Arryhtmia (INR >2)
(high risk cases)
20-35% As surgical scar Acute
Arrythmi MC A flutter long High RA DC
a SVT as 20 pressure RA shock
yrs distension sinus Chronic
node injury Amiodarone
Low CO ACEI
Chronic Arrythmia/ CMP Digoxin
Fatigue Syst congestion Avoid
Exercise Myo –ve ionotrops
Intoleranc remodelling
e PLE
LVF Pulm vein Fontan
compression by conversio
 complications
Complications Prevalence Timing Reasons Prevention

Prolonged pl eff 3% 3 yrs High SVC pressure High protein

PLE/ ascitis Lymphatic drainage diet
Neutr deficinecy impaired AB/ vaccine
Interstitial
Immuodeficiency Bronchitis Leakage L/o α1AT MLCFA
Thrombogenecity 1% in stool Somatostatin
Loss of ATIII Octeotride
Heparin
Hepatopathy ALI Diuretics
Ascitis CLD Spiranolactone
NO heart
transplantation
Cyanosis Fenestration leak
Microemboli
PVOD PAVF
Pulm dis
Abnormal 27
SVC
Cyan CHD with
increased PBF

PA
B

VSD
repair

-Anatomical repair
- overcomes RV
failure
- Qp:Qs = 1:1 29
 PA
banding

Too Too
loos tight
e

- PBF/ CHF - Pulm Dysfunction

- cyanosis
- PAH/ PVOD
- anatomic
- IPPR/ NO
distortion
CPB
-Asym LVH
30
 PA
banding
How tight? Where to band?
• Diamater 50% reduction • MPA (not annulus)
- TRUSLAR FORMULA • If too high
NRGA : - branch PA stenosed
20mm+1mm/KgBW TGA: • If too low
- coronary reimplntation
24mm+1mm/KgBW difficult
• mPAP 50% reduction
Not reliable in TGA
• Maintaining
Needs multiple banding
SPO2 to Surgeon’s choice
93% Often PBF Proper size hegar should pass
reduces At the
cost of
Subaortic LVH
Asymmetric 31
 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 Surgeon’s choice
• Biventricular repair not High risk of
possible PVOD
Preparation
PVR needs tofor
beGlenn/Fontan And not in a state of
low for passive forward
flow repair
• Preparation for ASO
Late presenting TGA with CHF
• HLHS: stage
Bilateral PAI Hybdrid procedure 38
 The left
heart
• PV – LA
(abnormality=TAPV
Late presenting TGA
C) LV is not trained
• LA – LV
• LV – LVOT Surgeon’s choice
PAB
• L(active
VOT – AOpump: high
pressure)
• AO – BRAIN/ ARMS/ LEGS
BT shunt
33
VA relation establishment:
switch
• Atrial level
• Ventricular level
RV
systemic
Physiological
repair
ventricle
• Great arterial level LV Anatomic
systemic
• Le Compte (PA anterior to ventricle
al
repair
Ao)
• Coronary artery
manipulation

39
ATRIAL SWITCH
Physiologic correction
Senning repair: atrial baffle is fashioned in situ
using tissue from RA wall and IAS
Mustard operation: after resection of most of
IAS, baffle is made from autologous pericardial
tissue or (rarely) synthetic material
SENNIN
G
MUSTARD
Senning vs Mustard
SENNING PROPONENTS MUSTARD PROPONENTS

1. Easier to perform 1. Reduced early mortality,

2. Used minimal (if any) prosthetic2. Reduced systemic and
material or nonviable tissue pulmonary venous
obstructions
3. Less compromised atrial
volumes & compliances 3. Less dysrhythmia
4. Lower frequency of systemic
venous obstruction and late
dysrhythmia
OUTCOMES OF ATRIAL
SWITCH
10 yr survival rates: 85 – 90%
TGA/VSD: 5 yr survival rates 60 – 70%
◦ Increased coexistence of adverse morphologic (associated anomalies) and
physiologic (PAH and vascular disease) factors
ISSUES WITH ATRIAL
SWITCH
Residual intra-atrial shunts
◦ MC at the superior RA baffle suture line
◦ In the absence of major coexistent systemic
venous obstruction, there is usually little or
no arterial oxygen desaturation, and a net
left-to-right shunt takes place
ISSUES WITH ATRIAL
SWITCH
SVC obstruction
1. Clinically in 5% to 10% of Mustard repair
2. Asymptomatic: 14% of Senning, 17% of Mustard
3. Usual location of obstruction is distal to SVC
entrance, within the systemic venous atrium at the
site of excision of the superior remnant of IAS

4. Balloon dilation, stenting, innominate vein to LAA

shunt
ISSUES WITH ATRIAL
SWITCH
IVC obstruction: 1%
PV obstruction: 2%, more lethal
◦ Pulmonary venous congestion or
unexplained arterial desaturation in the
postoperative period
◦ Reoperation
ISSUES WITH ATRIAL
SWITCH
TR:
◦ TGA/IVS: 1 – 2%
◦ TGA/VSD: 5 – 10%
◦ Manipulation & damage to TV apparatus, and
the occurrence of RBBB during repair of the
defect

◦ Annuloplasty, TV replacement
 Atrial
switch

Arrythmia 50%
Baffle leak 20% Dense adhesion:
RV dysfunction / TR 10 transition to ASO
% SVC obstruction 5% difficult
Pulm Venous occlusion
3% 44
 Switch at
ventricular level
• VSD closure
VSD SBF
• LV – AO tunnel routing
• RV – PA PBF
• conduit
Le Compte (PA brought anterior to
Ao)
• No Coronary reimplantation
Surgeon’s choice Not correcting
VSD PS (non the abnormal
TOF) TGA/DORV great artrey
relation
45
RASTELLI
PROCEDURE
Initially developed for TGA/ VSD/ extensive
LVOTO (PS)
Also used for PA/ VSD, DORV/ VSD/ PS-PA

Major steps:
◦ Proximal MPA division
◦ LV to aorta intraventricular patch tunnelling
◦ RV to MPA valved extracardiac conduit
 Raste
lli

Operative
mortality 30%

20 year survival
50%

VSD VSD RV-PA

closure routing
Long tunnel conduit
Extracardiac conduit
Subaortic Not suitable for
AS neonate Occlusion high 43
Aneurysm
REV PROCEDURE
For TGA/ VSD/ LVOTO
Principles of REV operation:
◦ Resection of a posteriorly deviated conal septum to
enlarge the VSD and facilitate the construction of a
wide and straight LV to aorta tunnel, bulging very
little in RV cavity. The native pulmonary artery is
sutured.
◦ Transection of the great arteries and French
manoeuvre to permit direct MPA implantation on the
right ventriculotomy.
REV (Réparation à l'Etage Ventriculaire
)
) LeCompte

Incision
above
coronari
es

Operative mortality
Short 20%
VSD-AO tunnel
Intacardiac
conduit
Surgeon’s choice
For VSD PS REV
RV-PA conduit
VSD VSD
closure routing 49
REV vs Rastelli
•Application earlier in infancy
•Avoidance of prosthetic extracardiac conduit,
•Avoidance of intracardiac tunnel obstruction
•Feasible with anatomic C/I to Rastelli
•May reduce the need for reoperation and the
prevalence of residual pulmonary OTO
•But lifelong risk of PR and related issues
TGA/ VSD/ LVOTO
Infant Rastelli better than BTS, but need for
conduit revision
But normal series circulation, avoidance of
prolonged hypoxemia, and, presumably, a
better long-term hemodynamic and neurologic
outcome
Conduit replacement at low risk
Hence infant Rastelli considered best option
NIKAIDOH PROCEDURE
•Aortic root, including AV is excised from RVOT
•Coronaries mobilised and reimplanted
•Pulmonary root divided at level of PV
•Ao. root translocated posteriorly to LVOT
•VSD closed with a patch
•PA to RV via pulmonary homograft
 Nikaido
h
Incision
below
coronari
es Limited
Experienc
e
Not suitable for Operative mortality
anomalous coronaries LeCompt 10%
e

VSD VSD RV-PA

closure routing 53
NIKAIDOH vs RASTELLI
Nikaidoh: homograft more posteriorly than in Rastelli
procedure
Therefore less prone to sternal compression.
However some risk of late deterioration of aortic valve
function and coronary patency
Nikaidoh can be done in difficult anatomy
Small IVC and located away from LVOT, multiple
IVC, small RV, mitral-tricuspid straddling, cone-
shaped implants of the TV, or the anterior implant of
MV
ARTERIAL SWITCH
OPERATION
Anatomic correction

Standard median sternotomy

Thymus subtotally resected
TWO STAGE ASO
•Yacoub (1976): PA band to increase LV
pressure, ASO months later
• Band caused scarring, complicated PA
reconstruction
• Distorted neoaortic valve – regurgitation
• Difficult coronary transfer
RAPID TWO STAGE ASO
•MRBTS from the distal innominate artery and
proximal SCA to RPA
•3mm silastic Dacron impregnated band in MPA
•Pressure monitoring catheter in proximal MPA
•Band tightened so that proximal MPA pressure
66% systemic pressure
RAPID TWO STAGE ASO
•MRBTS from the distal innominate artery and
proximal SCA to RPA
•3mm silastic Dacron impregnated band in MPA
•Pressure monitoring catheter in proximal MPA
•Band tightened so that proximal MPA pressure
66% systemic pressure
RAPID TWO STAGE ASO
•By 5 – 7 days, LV expected to recover normal
function after an initial period of depressed
function
•2nd stage: removal of band, clipping and
division of shunt, ASO
Indications:
◦ dTGA btn 4 – 8 weeks when LVSp <66% systemic
◦ Failing Senning / Mustard
With VSD/ PDA, LV pressure may be preserved
◦ Still may need ECMO / VAD post op (4 – 8 wks)

Definitive early one stage ASO preferable to 2 stage

ASO
RESULTS OF ASO
Early, risk-unadjusted mortality rates are <10%
Anatomic variants with higher operative
mortality include:
◦ Intramural coronary
◦ Retropulmonary LCA
◦ Multiple VSDs
◦ Coexisting arch abnormalities
◦ Straddling AV valves
◦ Prior PA banding
ASO
•Causes of early mortality:
• Coronary obstruction during transfer to neoaorta
• Unprepared LV
• Hemorrhage from the multiple suture lines
•Late mortality 1 – 2%
• Myocardial ischemia or pulmonary vascular
obstructive disease or during reoperation for
supravalvular obstruction
Arterial switch operation
(ASO)
not enough for
Surgeon’s choice Surgeon’s choice
for -TGA PS (fallot)
ASO for TGA - TGA AS (PAB)
TGA+VSD+PS
ASO +REV - Coronary anomalies

LeCompte

LV function Coronary Complications

Must be reimplantatio
n -Supravalvular PS (12%)
normal
-Neoaortic
LV dysfunction: Difficult
Post atrial baffle -Coronary artery obstruction
regurgitation
PA Band – Dense adhesion
63
ASO
 ASO COMPLICATIONS
Postoperative sequelae Incidence (%)
Supravalvular PS 10
Supravalvular AS <5
Neoaortic root dilation 100
Neoaortic regurgitation 50
Significant arrhythmia <10
Asymptomatic coronary occlusion 2–3

PAH Rare
Hypertrophied bronchial collaterals <5

Abnormal coronary reserve Unknown

Reduced exercise capacity >75
SCD Rare
 Coronary
anomalies in
TGA

65
DAMUS KAYE STANSEL
PROCEDURE
Arterial level repair without coronary
translocation
Generally reserved for children with TGA and
coronary anatomy not suitable for transfer and
for DORV SPVSD severe subaortic stenosis
DKS
MPA transected, anastomosed end-to-side to the
ascending aorta.
A VSD (if present) closed to direct LV blood to the
native pulmonary valve.
RV – PA conduit

Coronaries perfused retrogradely. The native aortic

valve may be left intact.
 Damus Kaye
Stensel
AP shunt
MPA – Asc
aorta

Subaortic DKS+RV-PA
Surgeon’s choice = YASUI
TGA VSD PS stenosis
subaortic AS Often after PAB procedure
Abnormal
coronaries No Coronary
reimplantatio
n 68
Raskind: Balloon atrial
septostomy

69
 Cardiopulmonary Bypass
(CPB)

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

70
 Surgical
approach
Total repair Palliatio
• Definite / desired nrepair not possible
• Total
• Anatomical • Anatomical reasons
repair • CPB not tolerable
• CPB required
• AP shunt/ RV PA conduit

• VSD repair • Glenn/ Fontan

• PAB
• RVOTO relief
• BAS
• ASO/ DKS
• ASO/ DKS
• Collateral closure
• unifocalization
71
 TO
F
Definitive
Palliativ ICR
e
• AP shunt - VSD closure
• RVOT stenting - RVOTO relief
• MAPCA - TAP for hypoplastic annulus
embolzation - 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 72
 Cath study
before ICR
• Pulmonary artery assessments (CT,
MRI)
• Muscular VSD (Echo)
• Abnormal coronaries
• Collaterals and embolisation
• Previous shunt patency
Surgeon’s choice:
To see
Collaterals
Coronarie
s Shunts

73
EARLY VS LATE ICR
•Issue: neurologic effects of neonatal CPB and
hypothermic circulatory arrest, and the
possible increased incidence of TAN patching
in very small babies
•Hence others – staged approach for the very
young symptomatic infants, with later ICR
SPECIFIC INDICATIONS
FOR SURGERY
•PGE1 dependent neonate
• Exclude MAPCAs, because baby more likely
to have TOF/ PA (more correctly PA/VSD)
•Worsening cyanosis
• Resting O2 sats <75 – 80%
•Cyanotic spells
TECHNICAL
CONSIDERATIONS
•TAP: if pulmonary annulus and MPA <2 SD
below normal (z score < -2)
•Ventriculotomy and division of infundibular
muscles with preservation of moderator band
•VSD closure, pericardial patch closure of
RVOT
 Pulmonary infundibulum
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
Surgeon’s choice:
injury transRA+transpulm
approach Hegar passage
Subannular patch
77
 Pulmonary annulus
assessment
MC GOON NAKATA INDEX (mm2/m2) Z
RATIO
• Diameter score
• Area
• RPA+LPA/DA • RPA+LPA/BSA
• N = 2-2.5 • N = 330 +/-
30
• <1.5 : BT • <200 : BT
shunt shunt
• >1.8: Fontan • >250: Fontan
• <1.5 : • <200 : Z score<-3:
TAP TAP Surgeon’s choice: TAP
Z score <-3
Transannular patch 63
 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) Key : Mild to mod
• PVR must be done in absent or dysplastic PV
PR is normal
PVR only if PV dysplastic
or absent 79
Pulmonary artery
assessment
3-6m 1-
3yr

Collateral
MPA/ LPA/RPA RV – PA
arteries Un
conduit
anastomosisfocalizatio
i
n

MPA/ LPA/RPA RV – PA Collateral arteries

Not conduit anastomosis
Discernable

80
 Pulmonary artery
confluence
TA RV-PA
P
• MPA stenosis conduit
• MPA atresia
• LPA/ RPA stenosis near • Distal branch
branch PS

Absent PA
BT shunt
unifocalize
in sick babies
the
collaterals

81
 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

82
 Embolization vs
unifocalization
Embolization Unifocalization
• Only the large • In nonconfluent/ absent
collaterals PA

Surgeon’s choice:
Cath backup: Surgeon’s choice:
Preoperaitve Unifocalization
embolization No cath Multiple sitting
backup:
Intraoperative embilization

83
 Coronary anomaly
assessment
• Long conus artery crossing RVOT
• RVOT resection is risky in infundibular
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

84
 BT
shunts
Only to buy time for
ICR
• Wt <2 kg or very sick AP shunts:
newborn pitfalls
• MPA atresia • Cyanosis
(RV –PA conduit) • I/L Radial pulse absent
• Hypoplastic Pulm • Less growth of upper
Annulus limb
(Transannular patch) • High PBF
• Unfavourable Coronaries • Chronic LVF
• Uncontrollable cyanosis • PVOD
• Focal PA stenosis
• Distal branch PA • Rib Surgeon’s
notchingchoice:
stenosis Take down the BT shunt
• Too small for When CPB is
established
surgery To have blood-free surgical
• Too sick for CPB field/ pulm edema
85
SURGICAL
OUTCOMES
•>96% survival to hospital discharge
•90% expected to be alive 30 yrs after repair
•5% reoperation, 6% catheter intervention in
childhood
•0.8%/yr risk of requirement of PVR
•0.5% annual risk of death (at 30 yrs and
beyond)
 Severe
PR
ECHO MRI
• PR • Moderate or more PR
PHT>100ms • PLUS:2 or more of
Severe PR plus - RVEDV ≥ 160 ml/m2 (Z-score
- New onset VT
>5)
- Severe exercise intolerance
- RVESV ≥ 70 ml/m2
- Right heart failure
- LVEDV ≤ 65 ml/m2
-Late repair - RV EF ≤ 45%
- RVOT aneurysm
PVR
87
 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?

89
DOR
V

90
 Approach for
DORV

91
 Condition Surgery
TGA IVS Atrial switch 2WKS
Artreial switch 1YR
PA banding –
switch
TGA IVS PA banding - switch
If LV func poor Two stage/ high
mortality
TG TGA VSD Switch + VSD repair

A If unfavourable
coronary
DKS
Instead of ASO
anatomy
TGA+VSD+PS BT shunt
initially
ASO+Rastelli
ASO+REV
ASO+Nikaidoh
TGA+VSD DKS
+subaortic stenosis
TGA+VSD BT+ASO
92
Straddled TCV (RV small) BDG – Fontan
A

Surgeon’s choice
Double switch

BT
Surgeon’s choice
shunt
Senning
+ REV

95
 Single
Ventricle
VA VA
Concordant Discordant
(Aorta
anterior)
Holmes Heart LV type
RV type
Non Inverte
(PS)
Inverte d (L-
d (D- TGA)
TGA) (DORV)
% 15 25 35 5

Aorta Right Left Side/ ant

Outlet + + -
chambe
r

Surgeon’s choice
SV
FONTAN

96
 T
A

Surgeon’s choice
SV
FONTAN

98
 PA
IVS
Dilated RV RV coronary
PGE Small RV
I connection
sLeft alone
BT
BT TV closure
Vulvotomy RV –P A (starnes
(Ballon/ open) connection Op)
PV atretic Infandibulum atretic BDG

Residu
Fontan ASD closure
al
RVOTO
Vulvotomy
(Ballon/
open)
RVOTR ASD closure 82
 Ebstein
Surgeon’s choice
’s Cone repair

Neonate: CHF/ cyanosis

-Biventricular repair
(Knott Craig
approach)
-Single ventricular repair
(strane’s TC closure –
Fontan) Adult
- severe progressive cyanosis
- RVOTO
- NYHA 3-4 poor activity
- paradoxical embolus
- arrythmia
- RV dysfunction

Danielson De silva’s
Ebstei Carpentie Cone 83
n r repair
 HL
H

Norwood
AP shunt
HL MPA – Asc
H aorta
Sano
RC-PA
conduit 101
 HL
H

SB PB
F F
MBT Sano
Surgeon’s choice
Connection SCA – IL PA RV - MPA
Sano shunt
Supply One lung Both lung
Within 2 weeks of DBP Lesser Higher
life High surgical risk Coronary steal + -
102
 HL
H
BAS
may be required

Surgeon’s choice
Hybrid
Process

B/L PAB Norwood sano

Fonta
PDA stent Removal of PAB, PDA +
n 1-2
(1st week: NO stents BDG
yr 103
CPB) (3-6m: CPB)
 TAPV
LT
R PV
obstructio
n Results
Innominate R Coronary in PAH
LT A sinus
AS vertica
D l
Esophage
al
hiatus
IVC
Supracardi Intracardia Infracardia Mixe
ac 50% c 20% c 20% d
Ligation 10%

Ligation

End to end Patch in Unroofing End to end

Com PV - ASD All PV Com PV -
LA to LA LA
Truncus
Arteriosus
VSD RV – PA
TYPE
repair conduit
I

TYPE
A2

Anastomosi Dacro
87
s n
A long
presentation..

106
 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
107
HAPPY HOLI