Transcript Surgical strategy for TOF

Surgical Strategies for TOF Repair
Yong Jin Kim M.D.
Seoul National University Children’s Hospital
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.
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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
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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 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
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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
 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
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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 Approach
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Transventricular Approach
amputation
amputation
a
TV ant.
leaflet
TV septal leaflet
Transection
TV post.
leaflet
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Transventricular Approach
Hypoplastic
PV
A
AV
VSD
a
Condunction
bundle
TV
a
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Transventricular Approach
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Transventricular 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 Approach
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Transatrial Approach
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Transatrial Approach
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Transatrial Approach
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Transatrial Approach
Infundibular
septum
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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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Infundibular Patch
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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Relief of RVOT Obstruction
PT
Pul. valve
Ao
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Relief of RVOT Obstruction
Pericardium
Dacron
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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
Transannular
patch
Transannular
Tied
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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Relief of Pulmonary Artery Stenoses
LPA
Pericardium
Dacron patch
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Relief of Pulmonary Artery Stenoses
LPA
PT
Pericardial
patch Dacron
patch
RPA
Ao
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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
years
- In LPA coarctation, early complete repair
within a few
months or 4mm LMBT
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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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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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TOF and Pulmonary Atresia
Definite repair
1. Closure of VSD
2. Continuity between RV & PA
3. Occlusion of collaterals & shunts
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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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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
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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
until 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 loaded
not 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
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
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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
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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 angioplasty
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TOF with Absent PV Syndrome
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TOF with Absent PV Syndrome
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