Dr M S ADITYA

MANAGEMENT OF TETRALOGY OF
FALLOT
PREVALANCE

 3.36% -9.27% of all congenital heart diseases

 0.21-0.26/1000 live births(0.22)
 Most common cyanotic heart defect to be
seen in children beyond infancy
 Most common complex lesion to be
encountered in the adult population after
repair
INTRODUCTION

 Tetralogy of Fallot with absent pulmonary

valve syndrome
 Tetralogy of Fallot with common
atrioventricular canal
 Tetralogy of Fallot with pulmonary atresia
 Tetralogy of Fallot with pulmonary stenosis
 INTRODUCTION(CLINICAL)
 The degree of cyanosis is often related to the severity of
RVOTO.
 Patients who are already cyanotic become more
cyanotic than before as a result of worsening
infundibular stenosis
 Hypoxic spells may develop in infants.
 Growth retardation may be present if cyanosis is severe.
 Brain abscess and stroke can occur but are rare.
 Subacute bacterial endocarditis is occasionally a
complication.
 Aortic regurgitation may develop in some patients,
particularly those with severe tetralogy of Fallot.
ANATOMY

 VSD is a perimembranous malaligned VSD

 Additional muscular VSDs may be present
 RVOTO most frequently infundibular (45%),only
valvular(10%), combined( 30%)
 Pulm annulus and MPA is hypoplastic, PAs are small
with variable degrees of pulmonic stenosis, LPA stenosis
is common
 Abnormal coronaries are seen in 5%
 Associated defects include ASD, PDA, right aortic
arch,lSVC,unroofed coronary sinus,TV
abnormalities,PAPVC, TAPVC,AVCD
GENETICS

 Inheritance multi factorial, autosomal

recessive or dominant
 Mis-sense mutation in JAG1 expressed in
right heart
 Mutation in transcription factor NKX2.5
 Microdeletion of 22nd chrmosome, more
commonly associated with interrupted aortic
arch
INVETIGATIONS

 The number, size, and location of all VSDs

 The severity and location of RVOTO
 The size and distribution of the pulmonary
artery
 The origins and branching pattern of the
coronary arteries
 The origin and distribution of all sources of
pulmonary blood flow, including MAPCAs
Natural history

 Bertranou et al 66% 1  Blalock taussig(1945)

yr, 48% 3yr, 24%10yr  Potts shunt9(1946)
 Median age at death is  Waterston shunt(1954)
9yrs  Prostaglandin(1976)
 TOF natural history
 ICR(1973)
altered by palliative
shunts and ICR
PALLIATIVE SHUNTS
Palliative shunts

 Neonates with TOF and pulmonary atresia

 Infants with hypoplastic pulmonary annulus,
which requires a transannular patch for
complete repair
 Children with hypoplastic PAs
 Unfavorable coronary artery anatomy
 Infants younger than 3 to 4 months old who
have medically unmanageable hypoxicspells
 Infants weighing less than 2.5 kg
BLALOCK TAUSSIG shunt

 Classic shunt involves RSCA in LAA and visa versa

 Infants older than 3 mths
 Modified involves placement of gore tex tube in ipsilateral
PA( < 3mths of age)
 Earlier series had exceptionally high mortatlity
 High incidence of PA distortion, PAH 33%
 Al jubair et al reported a series of 546 patients , 78 <1 yr,270
1wk-1 yr, 198>1 yr
 Hospital mortality of 2.9%,6.4<1wk, 3.7% 1wk-1 yr, 0.5%>1yr
 Shunt failure common in less than 3 kg, not anticoagulated
prior
BLALOCK TAUSSIG SHUNT

 Taussig reported 20-28 yr follow up of patients

operated between 1945-51
 50% of patients were alive after 20 yrs
 432 patients alive at 15 th yr were followed
 At 20 th yr 24 had died , 34 lost to follow up
 169 did not undergo further surgery, 227
complete repair
 37% of shunt patients considered to be doing
well, 79% of ICR were well
OTHER SHUNTS

 Central shunts easier to perform esp when PA

was small
 More prone for PAH and PA distortion
 Aneurysms of Pas which ruptured
 Chronic volume overload of ventricles
 Difficult to take down from midline
sternotomy at time of complete repair
 Phrenic nerve injury, RLN injury and others
BROCK CLOSED TRANSVENTRICULAR
INFUNDIBULECTOMY AND OTHERS
 Pulmonary infundibulectomy and valvotomy,
leaving the VSD
 Procedure was suitable in older patients
 Uniform flow in both PAs
 Pleurectomy, pleurodesis, used to stimulate
growth of trans pleural collateral vessels and
improve crippling hypoxemia
 Great omentum brought in contact with left lung
 Formalin injection in PDA to maintain patency
TOF INDICATION FOR OPERATION

 ICR advisable before 3 yrs of age to avoid

polycythemia, brain abscess and death and
long term complications
 Primary Vs two stage surgery comparisons of
risks essential
 If shunt is performed prior ICR is carried out
at 2-3 yrs of age
PULMONARY ARTERY SIZE

 The McGoon ratio is determined by summing

the diameter of the immediately
prebranching portion of the right and left
pulmonary artery and dividing this sum by
the diameter of the descending aorta at the
level of the diaphragm, all the measurements
being taken in systole
PULMONARY ARTERY SIZE

 RPA AND LPA can be considered non

restrictive when combined diameter ratio is
more than 2,mildly restrictive when ratio is
1.6,1 moderately rectrictive
PULMONARY ARTERY SIZE

 The Nakata index or pulmonary artery index

is determined by measuring the diameters of
the right and left pulmonary arteries
immediately proximal to the origin of the first
lobar branches; the cross-sectional areas are
then calculated, summed and divided by the
body surface area
 Index of 330±30mm2 in normal situation
 low output symptoms at <150mm2
POST REPAIR RV/LV

 Peak pressure in RV/LV , to estimate residual obstruction

 As low as possible desired
 0.70 is acceptable if alternative is only transannular patch
 Corresponds to a gradient of 40mmhg
 Estimated in late postop
 Related to pulmonary arteriolar resistance, RPA and LPA
size, and segmental and peripheral stenosis
 Determines long term result f operartion
 Practice is to accept RV/LV upto .85 post repair 30 mins
after CPB
Z VALUE

 Z value is the number of standard deviations

that the patients annulus size is away from
mean normal value
 Annulus diameter is normalized to patients
BSA
 Determined according to cineangiograms or
hager dilator estimation
 Correlates with Post op RV/LV pressures
PRIMARY Vs TWO STAGE REPAIR

 Primary repair favoured when age more than 3

mths except in special situations
 Pre op RV/LV estimated <0.63 transannular
patch not needed , child more than 6 mths ICR,
younger patient shunt with later repair
 If RV/LV >0.63 chance of requiring transannular
patch is increased, if BSA is more than .38m2 ICR
 70 % CL of probability of hospital death after
primary and 2 stage repair
TOTAL REPAIR OF TOF

 Era of corrective surgery was ushered in by

Lillehei in 1955
 Casteneda et al at boston children hospital,1n
1970 advocated primary reapir of TOF at any
age
 Approaches
1. Right atrial
2. Vertical incision in outflow of RV
3. Transverse ventriculotomy
TOTAL REPAIR OF TOF

 Right atrial approach allows

1. Infundibular dissection
2. Repair of VSD
3. Assesment of Pulmonary valve
4. Avoids cutting of RCA and ventriculotomy
scar
5. Damage to TV is an issue
6. Useful when transannular patch is not needed
TOTAL REPAIR OF TOF

 RVOT
1. Infundibular dissection
2. Pulmonar y valvotomy
 VSD
1. Closed with a preclotted dacron velour patch
TOF REPAIR TRANSANNULAR
PATCH
 After closing VSD hegar dilators are placed to
determine the narrowest portion of RVOT
 If the post opRV/LV is predicted to be equal to or
greater than 0.85 , diffuse hypoplasia, infundibular
+ valvar+annular, transannular patch is placed
 Pulmonary arteriotomy extended to widest part of
the pulmonary trunk, which is opposite the RPA
 ¾ diameter of ascending aorta
 Patch can be constructed from pericardium or
dacron patch
TOTAL REPAIR OF TOF CORONARY
ANOMALIES
 Anomalous LAD from RCA crossing RVOT
close to pulmonary ring
 Usual course of first part of LAD inspected
 Dissection of the anomalous artery from its
bed done right ventriculotomy is done by
giving incision beneath it
 Valved conduit is used to bypass the area if
required
INCREMENTAL RISK FACTORS FOR
HOSPITAL DEATH
 Presence of pulmonary artery problems
1. Diffuse severe hypoplasia
2. Severe and multiple localized areas of hypoplasia
3. Iatrogenic stenoses
4. Incomplete distribution of cantral and hilar
portions of Pas
 Pot op outcomes related to high postRV/LV ratio,
surgical complexity, hypoperfusion of lung
INCREMENTAL RISK FACTORS FOR
HOSPITAL DEATH
 More than one previous palliative surgery
1. Highest with central shunts and b/l
plerectomy, brock procedure,BT+waterston
shunts
 Size of patient(BSA) in creased susceptibility
to CPB
 High hematocrit, reflects arterial hypoxia,
and widespread effects esp clotting systems.
(0.45-.55 double sthe risk of death)
INCREMENTAL RISK FACTORS FOR
HOSPITAL DEATH
 Use of transannular patch
1. Could indicate more sevre PS
2. Diastolic over load of RV
 Early date of operation
 Absent pulmonary valve
 Major associated cardiac anomalies
 In pulmonary atresia use of vlaved conduit
and high postop RV/LV
Incremental risk factors for
late functional status
 Pulmonar y valve incomptence
 Age at repair
 RV/LV ratio ( residual gradient)
 Multiple VSds
STRATEGIES TO PROMOTE GROWTH
OF Pas
 Systemic to PA shunts
 Brock procedure
 RVOT patch, conduit
 Trans catheter approach
LONG TERM FOLLOW UP

 Survival
 Freedom from re intervention
 PR and requirement for PV replacement
 Ventricular arrhythmias and sudden death
 Atrial arrhythmias
 Complete heart block
 Ventricular function
 Quality of life
 Bacterial endocarditis
SURVIVAL

 Series form alabama at 1 mth, 1 yr,5 yr,20 yr

at 93%,93%,92%,87%(814 patients)
 Rat e of post op CHB % in earlier series, now
around 1%
REINTERVENTION

 VSD
 RVOUTFLOW AND PA obstruction
 PR
 TR
 AR
 RV dysfunction
 LV dysfunction
 RVOt aneurysm
 Pulmonary hypertension
 Most residual VSDs tend to be small and less
seen in modern era
 Dilatation of aortic root, sub pulmonary VSD,
patients present with AR in long term
 Surgical injury to AV
 PA stenosis at site o f arterioplasty, MPA, just
distal to transannular patch known to require
reintervention
 Aneurysmal bulging of patch along with PR
PULMONARY REGURGITATION

 Inevitable consequence of trans annular patching and

pulmonary valvatomy
 Post op PR is seen in 60-90%
 Long term PR can have adverse impact esp in children
operated at older age
 Requirement for pulmonary valve replacement
1. Exercise intolerance
2. Progressive cardiac enlargement
3. Aneurysmal dilatation of outflow tract
4. Onset of TR, atrial and ventricular arrhythmias
PULMONARY REGURGITATION

 Mayo clinic has has reviewd 42 patients who underwent

PV replacement
 Mean interval between TOF repair and PV replacement
was 10 yrs
 Exercise in tolerance 58%, right heart failur 21%,
arrhythmia14%, and syncope 10%, RV dilatation 7%
 Functional class improvement was seen
 Some patients required reimplantation
 Arrhythmia control was superior
 Age at time of PV replacement increased when operated
younger
PULMONARY REGURGITATION

 Some degree of regurgitation inevitable

 Trivial to mild PR may be tolerated in long
term
 Tendancy to delay PV repalcement
 Asymptomatic patients?
 Restrictive physiology in post repair patients
reduces PR
VENTRICULAR ARRHYTHMIAS AND
SUDDEN CARDIAC DEATH
 Risk of sudden cardiac death 25 to 100 times more
 Risk of sudden death late after surgery is 5%
 Older age of repair
 Increased fibrosis in RV with age
 Extent of surgery and ventriculotomy scar
 QRS duration >180msec,RV enlargement
 Severe PR, outflow aneurysms
 Inducible monomorphic VT
 Moderate, severe LV dysfunction more
 Abnormal adrenergic activity
ATRIAL FIBRILLATION AND
FLUTTER
 Older age at repair, hemodynamic
abnormalities and increased morbidity, older
age
 Atrial arrhythmias associated with CHF,
reoperation,,VT, stroke and death
 Higher mean right atrial volume
 Significant PR
VENTRICULAR FUNCTION

 Age of patient
 Degree and duration of hypoxemia exposure
 Duration and size of shunt
 Size of ventriculotomy scar and muscle
resected
 Postop PR
INFECTIVE ENDOCARDITIS

 Endocarditis may affect aortic valve,

TV,VSD,PA
 TOF accounts for 16% of IE in CHD
 1.3% f repaired TOF have IE over 30 yrs
 ENDOCARDITIS PROPHYLAXIS
RECOMMENDED
UNUSUAL COMPLICATIONS

 Coronary-RV fistulae or PA fistulae reported

 DCRV situation seen
 Subaortic stenosis
 Aneurysmal dilatation of subclavian aretery
at the systemic end