surgical strategies for tof repair yong jin kim m.d. seoul national university childrens hospital
TRANSCRIPT
Surgical Strategies for TOF RepairSurgical Strategies for TOF Repair
Yong Jin Kim M.D.Yong Jin Kim M.D. Seoul National University Children’s Hospital Seoul National University Children’s Hospital
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Tetralogy of Fallot
DefinitionDefinition
Characterized by underdevelopment of right ventricular Characterized by underdevelopment of right ventricular
infundibulum with anterior & leftward displacement ofinfundibulum with anterior & leftward displacement of
infundibular ( conal, outlet ) septum & parietal extension. infundibular ( conal, outlet ) septum & parietal extension.
This displacement of infundibular septum is associatedThis displacement of infundibular septum is associated
with RV outflow stenosis & large VSD.with RV outflow stenosis & large VSD.
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Tetralogy of FallotTetralogy of Fallot
Definition Definition A congenital cardiac anomaly characterized by underdevelopment of the RV infundibuluA congenital cardiac anomaly characterized by underdevelopment of the RV infundibulu
m with anterior & leftward displacement of the infundibular septum & parietal extm with anterior & leftward displacement of the infundibular septum & parietal extension . This displacement of the infundibular septum is associated with RV outfloension . This displacement of the infundibular septum is associated with RV outflow stenosis & large VSD .w stenosis & large VSD .
ClassificationClassificationSimple TOFSimple TOFTOF with AV canalTOF with AV canalTOF with absent pulmonary valve syndromeTOF with absent pulmonary valve syndromeTOF and pulmonary atresia with well formed PDATOF and pulmonary atresia with well formed PDATOF and pulmonary atresia with MAPCAs TOF and pulmonary atresia with MAPCAs
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Backgrounds I
1. 1945 Blalock & Taussig
Subclavian - pulmonary artery anastomosis
2. 1948 Sellors & Brock
Closed pulmonary valvotomy & infundibulotomy
3. 1954 Lillehei & Varco
First successful repair using cross-circulation
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Backgrounds II
4. 1955 Kirklin
First successful repair using pump oxygenator
5. 1957 Warden and Lillehei Patch enlargement of the infundibulum
6. 1959 Kirklin
Transannular patching
7. 1963 Hudspeth
Transatrial approach
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Backgrounds III
8. 1965 Rastelli
Right ventricular-pulmonary artery conduit
9. 1966 Ross
Valved extracardiac conduit
10. 1969 Barratt-Boyes & Neutze
One-stage repair
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Surgical StrategiesSurgical Strategies
1. Around 3 months with symptoms 1. Around 3 months with symptoms
Early total correctionEarly total correction
2. 1 - 2 months with severe symptoms2. 1 - 2 months with severe symptoms
Palliative shunt or early total correctionPalliative shunt or early total correction
3. Asymptomatic and uncomplicated3. Asymptomatic and uncomplicated
Definitive repair at 6 - 24 monthsDefinitive repair at 6 - 24 months
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Surgical Indications I
1. Diagnosis is generally an indication for repair
2. Urgency : Symptpms at presentation
Associated lesions
3. Trend toward open correction in early infancy
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Surgical Indications II
1. Below 3 months with severe symptoms
Early total correction
2. 1 - 2 months with severe symptoms
Palliative shunt or early total correction
3. Asymptomatic & uncomplicated
Definitive repair at 6 - 24 months
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Early Total Correction
Advantages :
Avoid risk & complication of palliative shunt
Early correction of RVH
Prevention of LV volume overload
Early correction of chronic hypoxemia
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Palliation
DisadvantagesDisadvantages
: PA distortion - complicating & increasing risk: PA distortion - complicating & increasing risk
of subsequent complete repairof subsequent complete repair
AdvantagesAdvantages
: Lower mortality & RVOTO recurrence : Lower mortality & RVOTO recurrence
Rick factors of mortalityRick factors of mortality
: PA distortion from previous shunts : PA distortion from previous shunts
More than one palliationMore than one palliation
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Indications of Palliative Procedure
1. Anomalous coronary artery crossing RVOT
2. Extremely small pulmonary arteries
3. Unrelenting "tet" spells for several hours
4. Significant & severe associated lesions
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Mortality for Risk Factors
Presence of multiple VSDs
Down's syndrome
Large aortopulmonary collaterals
Complete AV canal defects
Early age at presentation
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Total Correction
The goals of operation
1. VSD closure
2. Relief of RVOT obstruction
3. Relief of pulmonary artery stenoses
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Transventricular Approach
Vertical extension across annulus to relieve PS
Division in parietal extension of infundibular
septum to expose VSD
Not to much resect muscle in infants
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Transventricular ApproachTransventricular Approach
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amputationamputation
TV ant. leaflet
TV post.leaflet
TV septal leafletTransection
a
Transventricular Approach
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AV
VSD a Condunction
bundle
Hypoplastic PV
A
TV a
Transventricular Approach
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Transventricular ApproachTransventricular Approach
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Transventricular ApproachTransventricular Approach
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Transatrial Approach
By retracting TV leaflet or incising TV
Relief of RVOT obstruction
Preserving long-term RV function
Limiting ventricular dysrhythmias
Access to atrial septum - ASD closure
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Transatrial ApproachTransatrial Approach
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Transatrial Approach
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Transatrial Approach
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Transatrial Approach
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Infundibular septum
Transatrial Approach
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Limited Ventriculotomy
Patch enlargement in the infundibulum
for hypoplasia of infundibular septum
Muscle resection is not always required
Leave a small ASD in infants
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Infundibularpatch
Infundibular Patch
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Methods of RVOT Reconstruction
Long ventriculotomy : Long-term outcome ↓
Transatrial approach : In some , small ventriculotomy
is necessary for the patch of hypoplastic
infundibulum
Limited ventriculotomy : Less than the half length
Preserve late right ventricular function
Adequate enlargement of hypoplastic RVOT
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PT
Ao
Pul. valve
Relief of RVOT Obstruction
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Pericardium Dacron
Relief of RVOT Obstruction
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Transannular Patch
Pulmonary annular Z-value
> - 2 : postrepair RV/LV pressure ratios (< 0.7)
< - 3 : transannular patch
Hegar dilator : assess annulus size
Patch : autopericardium, Dacron, Gore-Tex
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Transannular Patch
RV dysfunction requiring reoperation for PI
Not employed unless necessary for RVOT
Limit PI to preserve long-term RV dynamics
Monocusp valve for short-term
Homograft for the long-term
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Transannular patch
TiedTransannular patch
Transannular Patch
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Pulmonary Artery Stenoses
Obstruction in main PA branch
Previous shunt
Tissue from ductus arteriosus
Spectrum of anatomy of defect
Angioplastic technique
Patch to bifurcation & LPA
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Relief of Pulmonary Artery Stenoses
Distal aspect of transannular patch
Blunt and not tapered
Obstruction in MPA
Distal stenosis in PA
Stent at operation
Balloon angioplasty later
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LPAPericardium
Dacron patch
Relief of Pulmonary Artery Stenoses
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LPA
RPA
PT
Ao
Pericardial patch Dacron
patch
Relief of Pulmonary Artery Stenoses
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Anomalous Left Coronary Artery Crossing the RVOT (I)
Transverse incision in infundibulum & separate
incision in the MPA
- patching of pulmonary artery, valvotomy
Dissecting with patch beneath coronary artery
- RV distension causing coronary ischemia by
stretching
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Anomalous Left Coronary Artery Crossing the RVOT (II)
Systemic-pulmonary artery shunt followed
by RV-PA conduit
Complete repair with homograft in infancy
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TOF and Pulmonary Atresia
Surgical strategies
- Initial ductal stabilization with PG
- Shunt or total correction
- 5mm RMBT in full-term baby last up to 18 months or 2 y
ears
- In LPA coarctation, early complete repair within a few
months or 4mm LMBT
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TOF & Pulmonary AtresiaTOF & Pulmonary Atresia
Assessment of repair quality Assessment of repair quality
pRV/LV pressure ratiospRV/LV pressure ratios Postrepair RV/LV ratio above 0.7 Postrepair RV/LV ratio above 0.7
Unfavorable outcomeUnfavorable outcome
Early repair is advantageous before spellsEarly repair is advantageous before spells
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TOF and Pulmonary AtresiaTOF and Pulmonary Atresia
Morphology Morphology
Differentiating features from TOF Differentiating features from TOF
1. No blood from RV to PA 1. No blood from RV to PA
2. Pulmonary artery anomalies2. Pulmonary artery anomalies
3. Aortopulmonary collaterals3. Aortopulmonary collaterals
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TOF and Pulmonary AtresiaTOF and Pulmonary Atresia
Definite repairDefinite repair
1. Closure of VSD1. Closure of VSD
2. Continuity between RV & PA2. Continuity between RV & PA
3. Occlusion of collaterals & shunts3. Occlusion of collaterals & shunts
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TOF and Pulmonary AtresiaTOF and Pulmonary Atresia
Preparation for definitive repairPreparation for definitive repair
1. Maximize the pulmonary artery1. Maximize the pulmonary artery
The size & distributionThe size & distribution
2. Maintain the adequate PBF2. Maintain the adequate PBF
3. Avoid the excessive PBF3. Avoid the excessive PBF
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TOF and Pulmonary AtresiaTOF and Pulmonary Atresia
Selection for final repairSelection for final repair1. Central combined Rt & Lt PA area at least 1. Central combined Rt & Lt PA area at least
50-75% of predicted normal50-75% of predicted normal
2. Distribution of unobstructed confluent PAs 2. Distribution of unobstructed confluent PAs
equivalent to at least one whole lungequivalent to at least one whole lung
3. Presence of a predominant Lt to Rt shunt 3. Presence of a predominant Lt to Rt shunt
without restrictive RV-PA connectionwithout restrictive RV-PA connection
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TOF with Complete AV Canal
General principle of complete repair
: At a time when heart is volume loaded
- hazard relate to operative length &
difficulty in dividing single AV valve
: Shunt when cyanosis & later complete repair: Shunt when cyanosis & later complete repair
until 12-24 monthsuntil 12-24 months
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TOF with Complete AV Canal
CHF due to AV regurgitation & not high PBF complete repair Heart failure with poor PBF
simply repair of AV valve combined with shunt CHF because of inadequate RVOTO
complete repair at 3 to 4 months Inadequate shunt & no longer volume loadedInadequate shunt & no longer volume loaded
not waitnot wait
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TOF with Complete AV Canal
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TOF with Absent PV Syndrome
Definition (I)
1. Ringlike and stenotic malformation rather than absenc
e of PV with failure of development
2. Hugely dilated or aneurysmal central PA
3. Tightly stenotic pulmonary annulus with free PI against
high PVR in utero
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TOF with Absent PV Syndrome
Definition (II)
1. Abnormal tufted segmental PA branching
2. Branching arteries : spread peripherally with little chan
ge in size entwing and compressing associated bronch
i
3. Bronchi : deficient or defective cartilage formation, abn
ormal broncho-alveolar multiplication
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TOF with Absent PV Syndrome
Aim : Alleviate bronchial compression
Prevent right-sided heart failure
Palliative procedures : not successful
Surgery : In a one stage procedure
VSD closure
Pulmonary artery plication
Insertion of RV-PA homograft
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TOF with Absent PV Syndrome
Timing - related to symptom presentation
Neonate : urgent repair
Infants : deferred selectively
RVOT reconstruction
Transannular patch - not wise ( PI, RV failure)
Insertion of a valved conduit - valved > monocusp
Aortic or pulmonary homograft - larger homograft
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TOF with Absent PV Syndrome
Operative techniques
VSD closure
Insertion of homograft
- in infants for increased PVR
- severe intrapulmonary stenoses
Reduction pulmonary angioplastyReduction pulmonary angioplasty
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TOF with Absent PV Syndrome
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TOF with Absent PV Syndrome