1. Surgical Strategies for TOF Repair Yong Jin Kim M. D
Surgical Strategies for TOF Repair Yong Jin Kim M.D. Seoul National University Children’s Hospital 1

2. Tetralogy of Fallot Definition
Characterized by underdevelopment of right ventricular
infundibulum with anterior & leftward displacement of
infundibular ( conal, outlet ) septum & parietal extension.
This displacement of infundibular septum is associated
with RV outflow stenosis & large VSD.
99’ SNUCH TS

3. Tetralogy of Fallot Definition Classification 99’ SNUCH TS
A congenital cardiac anomaly characterized by underdevelopment of the RV infundibulum with anterior & leftward displacement of the infundibular septum & parietal extension . This displacement of the infundibular septum is associated with RV outflow stenosis & large VSD .
Classification
Simple TOF
TOF with AV canal
TOF with absent pulmonary valve syndrome
TOF and pulmonary atresia with well formed PDA
TOF and pulmonary atresia with MAPCAs
99’ SNUCH TS

4. Backgrounds I 1. 1945 Blalock & Taussig 2. 1948 Sellors & Brock
Subclavian - pulmonary artery anastomosis
Sellors & Brock
Closed pulmonary valvotomy & infundibulotomy
Lillehei & Varco
First successful repair using cross-circulation
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5. Backgrounds II 4. 1955 Kirklin 5. 1957 Warden and Lillehei
First successful repair using pump oxygenator
Warden and Lillehei
Patch enlargement of the infundibulum
Kirklin
Transannular patching
Hudspeth
Transatrial approach
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6. Backgrounds III 8. 1965 Rastelli 9. 1966 Ross
Right ventricular-pulmonary artery conduit
Ross
Valved extracardiac conduit
Barratt-Boyes & Neutze
One-stage repair
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7. Surgical Strategies 1. Around 3 months with symptoms
Early total correction
months with severe symptoms
Palliative shunt or early total correction
3. Asymptomatic and uncomplicated
Definitive repair at months
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8. 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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9. Surgical Indications II
1. Below 3 months with severe symptoms
Early total correction
months with severe symptoms
Palliative shunt or early total correction
3. Asymptomatic & uncomplicated
Definitive repair at months
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10. 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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11. Palliation Disadvantages Advantages
: PA distortion - complicating & increasing risk
of subsequent complete repair
Advantages
: Lower mortality & RVOTO recurrence
Rick factors of mortality
: PA distortion from previous shunts
More than one palliation
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12. 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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13. 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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14. Total Correction The goals of operation 1. VSD closure
2. Relief of RVOT obstruction
3. Relief of pulmonary artery stenoses
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15. 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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16. Transventricular Approach
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17. Transventricular Approach
amputation
amputation a
TV ant.
leaflet
TV septal leaflet
Transection
TV post.
leaflet
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18. Transventricular Approach
Hypoplastic PV AV
VSD a a TV
Condunction
bundle
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19. Transventricular Approach
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20. Transventricular Approach
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21. 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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22. Transatrial Approach
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23. Transatrial Approach
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24. Transatrial Approach
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25. Transatrial Approach
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26. Transatrial Approach
Infundibular
septum
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27. 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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28. Infundibular Patch
Infundibular
patch
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29. 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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30. Relief of RVOT ObstructionPT
Pul. valve Ao
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31. Relief of RVOT Obstruction
Dacron
Pericardium
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32. 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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33. 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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34. Transannular Patch Transannular patch Tied Transannular patch
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35. 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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36. 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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37. Relief of Pulmonary Artery Stenoses
LPA
Pericardium
Dacron patch
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38. Relief of Pulmonary Artery Stenoses
LPA
Pericardial
patch
Dacron
patch PT
RPA Ao
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39. 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
99’ SNUCH TS

40. Anomalous Left Coronary Artery Crossing the RVOT (II)
Systemic-pulmonary artery shunt followed
by RV-PA conduit
Complete repair with homograft in infancy
99’ SNUCH TS

41. 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 years
- In LPA coarctation, early complete repair within a few months or 4mm LMBT
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42. TOF & Pulmonary Atresia
Assessment of repair quality
pRV/LV pressure ratios
Postrepair RV/LV ratio above 0.7
Unfavorable outcome
Early repair is advantageous before spells
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43. TOF and Pulmonary Atresia
Morphology
Differentiating features from TOF
1. No blood from RV to PA
2. Pulmonary artery anomalies
3. Aortopulmonary collaterals
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44. TOF and Pulmonary Atresia
Definite repair
1. Closure of VSD
2. Continuity between RV & PA
3. Occlusion of collaterals & shunts
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45. TOF and Pulmonary Atresia
Preparation for definitive repair
1. Maximize the pulmonary artery
The size & distribution
2. Maintain the adequate PBF
3. Avoid the excessive PBF
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46. TOF and Pulmonary Atresia
Selection for final repair
1. Central combined Rt & Lt PA area at least
50-75% of predicted normal
2. Distribution of unobstructed confluent PAs
equivalent to at least one whole lung
3. Presence of a predominant Lt to Rt shunt
without restrictive RV-PA connection
99’ SNUCH TS

47. 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
until months
99’ SNUCH TS

48. 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 loaded
not wait
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49. TOF with Complete AV Canal
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50. TOF with Absent PV Syndrome
Definition (I)
1. Ringlike and stenotic malformation rather than absence 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
99’ SNUCH TS

51. TOF with Absent PV Syndrome
Definition (II)
1. Abnormal tufted segmental PA branching
2. Branching arteries : spread peripherally with little change in size entwing and compressing associated bronchi
3. Bronchi : deficient or defective cartilage formation, abnormal broncho-alveolar multiplication
99’ SNUCH TS

52. 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
99’ SNUCH TS

53. 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
99’ SNUCH TS

54. TOF with Absent PV Syndrome
Operative techniques
VSD closure
Insertion of homograft
- in infants for increased PVR
- severe intrapulmonary stenoses
Reduction pulmonary angioplasty
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55. TOF with Absent PV Syndrome
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56. TOF with Absent PV Syndrome
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