Surgical Strategies for TOF Repair Yong Jin Kim M.D. Seoul National University Children

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

3
Tetralogy of Fallot

• Definition
• 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

4
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

5
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

6
Backgrounds III

• 8. 1965 Rastelli
• Right ventricular-pulmonary artery conduit
• 9. 1966 Ross
• Valved extracardiac conduit
• 10. 1969 Barratt-Boyes Neutze
• One-stage repair

7
Surgical Strategies

• 1. Around 3 months with symptoms
• Early total correction
• 2. 1 - 2 months with severe symptoms
• Palliative shunt or early total correction
• 3. Asymptomatic and uncomplicated
• Definitive repair at 6 - 24 months

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
  

9
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

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

11
Palliation

• Disadvantages
• 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

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

13
Mortality for Risk Factors

• Presence of multiple VSDs
• Down's syndrome
• Large aortopulmonary collaterals
• Complete AV canal defects
• Early age at presentation

14
Total Correction

• The goals of operation
• 1. VSD closure
• 2. Relief of RVOT obstruction
• 3. Relief of pulmonary artery stenoses

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

16
Transventricular Approach
17
Transventricular Approach
amputation
amputation
a
TV ant. leaflet
TV septal leaflet
Transection
TV post.
leaflet
18
Transventricular Approach
A
Hypoplastic PV


AV


VSD
a
a
TV
Condunction bundle
19
Transventricular Approach
20
Transventricular Approach
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

22
Transatrial Approach
23
Transatrial Approach
24
Transatrial Approach
25
Transatrial Approach
26
Transatrial Approach
Infundibular septum
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

28
Infundibular Patch
Infundibular patch
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

30
Relief of RVOT Obstruction
PT
Pul. valve
Ao
31
Relief of RVOT Obstruction
Dacron
Pericardium
32
Transannular Patch

• Pulmonary annular Z-value
• gt - 2 postrepair RV/LV pressure ratios (lt
  0.7)
• lt - 3 transannular patch
• Hegar dilator assess annulus size
• Patch autopericardium, Dacron, Gore-Tex

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

34
Transannular Patch
Transannular patch
Tied
Transannular patch
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

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

37
Relief of Pulmonary Artery Stenoses
LPA
Pericardium
Dacron patch
38
Relief of Pulmonary Artery Stenoses
LPA
Pericardial patch
Dacron patch
PT
RPA
Ao
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

40
Anomalous Left Coronary Artery Crossing the RVOT
(II)

• Systemic-pulmonary artery shunt followed
• by RV-PA conduit
• Complete repair with homograft in infancy

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

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

43
TOF and Pulmonary Atresia

• Morphology
• Differentiating features from TOF
• 1. No blood from RV to PA
• 2. Pulmonary artery anomalies
• 3. Aortopulmonary collaterals

44
TOF and Pulmonary Atresia

• Definite repair
• 1. Closure of VSD
• 2. Continuity between RV PA
• 3. Occlusion of collaterals shunts

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

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

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 12-24 months

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

49
TOF with Complete AV Canal

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

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

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

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 gt
  monocusp
• Aortic or pulmonary homograft - larger homograft

54
TOF with Absent PV Syndrome

• Operative techniques
• VSD closure
• Insertion of homograft
• - in infants for increased PVR
• - severe intrapulmonary stenoses
• Reduction pulmonary angioplasty

55
TOF with Absent PV Syndrome
56
TOF with Absent PV Syndrome