Rastelli, REV, Nikaidoh : How I

Manage Conotruncal Problems with

Pulmonary Stenosis or Atresia

V. Mohan Reddy

Lucile Packard Childrens Hospital

Stanford University

Conotruncal Anomalies
l
l
l
l
l
l

Tetralogy of Fallot
Most DORVs with
subaortic VSD
Truncus Aeteriosus
IAA with VSD
TGA
Corrected TGA

l
l
l

TGA.VSD. PS / PA
DORV. VSD. PS / PA
Corrected TGA. PS / PA

Conotruncal Anomalies
l TOF

with or without Pulmonary Atresia

l DORV

with subaortic VSD with Pulmonary

stenosis or Pulmonary Atresia (TOF like)

Standard TOF repair wth RV-PA valved

conduit

DORV. VSD. PS

LV to Ao baffle
RVOT enlargement

TGA or DORV
l

Pulmonary Stenosis
Arterial Switch
Rastelli
REV
Aortic Root Translocation

(Nikaidoh and
modifications)
Double root translocation

Pulmonary Atresia

Rastelli
Palliation Shunt

TGA. VSD PS

Arterial Switch
LVOT resection

TGA VSD Pulmonary Atresia

Rastelli
Alt : BT Sunt

Rastelli, REV or Nikaidoh ???

l TGA

with VSD and Pulmonary Stenosis

l DORV

with VSD and Pulmonary Stenosis

Straddling AV Valves

DORV with AV Septal Defect

DORV. Ao remote, AV valve

Abnormality

Remote VSD

Restrictive VSD

Coronary Anomalies
l

l Rastelli
l REV
l Nikaidoh
l Double

root

Alternatively palliate
with a systemic to
Pulmonary artery
shunt

Fetal Diagnosis

Case # 1
TGA, VSD, PS
3 wk old neonate
Weight 3.0 Kg

TGA.VSD.PS

Surgical Technic
Bicaval venous cannulatio
Deep Hypothermia : 200 C
Aorta -RA inspection
Proceed with repair

Surgical Technic

Surgical Technic

Surgical Technic
Rastelli
PV implantation
Into RV
ASD closure

Follow up At 2.0 yrs

Follow up At 2.0 yrs

Follow up
l Transannular

patch repair of RVOT

obstruction
l At

3.1 yrs No RVOT0 or LVOT0

Case #2
1 wk old neonate
DORV pulmonary atresia
TV straddle, remote aorta

BT shunt
PDA ligation

Case #2

Case #2

Bidirectional
Glenn

Case #2
l

Rastelli procedure
VSD enlargement
LV to Ao pericardial baffle
TV chordal relocation
RV-PA valved homograft conduit

Case #2 : Fu at 6 yrs

Final Outcome :
One and Half
Ventricle repair

Case #3
l1

week old neonate

l TGA. VSD, PS,, TV straddle
l On PGE, Duct dependant
l On Ventilator

Case #3
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Case #3: Surgical Technic

Bicaval venous
cannulation
Mild Hypothermia : 320 C
Aorta -RA inspection
Proceed with repair

Case #3: Surgical Technic

Moderate Hypothermia : 250 C
RV: ventriculotomy parallel to LAD
Resect Conal septum, Enlarge VSD, Explant
TV pm-chordae
LV-AO glutaraldehyde fixed autologous
pericardium

Case #3: Surgical Technic

Reimplant the TV pm-chordae
ASD patch closure : leave PFO like opening
Release cross clamp
RV-PA conduit with 10mm valved homograft
Conduit
Off CPB : Dopamine 3mcg, Milrinone 0.5mcg

Case #3: Post OP Echo

Corrected TGA, VSD, PS

l Almost

always staged
l BT shunt - Glenn - Rastelli + HemiMustard
l BT shunt to Rastelli + Senning
l?

Double Root Translocation

Rastelli, REV or Nikaidoh

l
l
l
l
l

Rastelli
Staged Rastelli with or wthout Glenn
Rastelli with reimplantation of native PV into RV
Nikaidoh : selective pts and pts with TGA, IVS
and PS, and TGA. VSD. PS
REV : ??? may be in older pts with PS and low
PVR