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

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


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AV

VSD a Condunction

bundle

Hypoplastic PV

A

TV a


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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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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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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


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LPA

RPA

PT

Ao

Pericardial patch Dacron

patch


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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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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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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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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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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 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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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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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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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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Operative techniques

VSD closure

Insertion of homograft

- in infants for increased PVR

- severe intrapulmonary stenoses

Reduction pulmonary angioplastyReduction pulmonary angioplasty

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Documents

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