surgical treatment for tetralogy of fallot · 2. karl t, stocker c. tetralogy of fallot and its...
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Surgical Treatment for
Tetralogy of Fallot Masakazu Nakao
Consultant, Paediatric Cardiothoracic Surgery
1
2
History
2015
Blalock-Taussig shunt
(Blalock & Taussig)
1944 1946 1948 1954 1955 1962
Potts shunt (Potts)
Brock procedure
(Brock)
Intracardiac repair of TOF with
cross-circulation (Lillehei)
Intracardiac repair of
TOF with CPB (Kirklin))
Waterston shunt
(Waterston)
Today
1980
Modified BT shunt
(de Leval)
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Indications
“Blue” Fallot: SaO2 < 75%
Hypoxaemic spell
Otherwise, operate electively around 12 months
“Pink” Fallot: Same as VSD
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Surgical Treatment - Overview
• Palliative –
• Corrective – VSD closure +
Systemic-to-pulmonary shunt
Right ventricular outflow tract patch
Right ventricle-to-pulmonary shunt
Right ventricle-to-pulmonary stent
Infundibulectomy only
Transannular patch with/without valve
Pulmonary valve preservation
When to choose palliative, when to choose corrective?
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Surgical Treatment - Palliative vs Corrective
TOF
Symptomatic
> 3 month-old
Branch PA
Z-score >2
Corrective repair
Palliation
Palliation
Asymptomatic
Corrective repair
Yes No
Yes No
Aim of palliation
1. Increase pulmonary blood flow
2. Let branch PAs grow
3. Let the patient grow
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PA Index Pre-branching RPA diameter + LPA diameter
Descending aorta diameter at the diaphragm McGoon ratio
Pre-branching RPA area + LPA area
Body surface area Nakata index
The risks of failure of the corrective repair is higher for less than 1.0 (normal range: 2.0-2.5)
Lower Nakata index (< 150~200) has been identified as a risk factor
(normal range: 330±30mm2/BSA)
The usefulness of these indices is controversial
the compliance of the pulmonary vascular bed
distortion of the pulmonary arteries
They don’t consider
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Neonatal Repair vs Late Repair
Benefits of neonatal repair
1. Promote normal growth and development of organs
2. Eliminate hypoxaemia
3. Operate before extensive right ventricular hypertrophy
4. Better late left ventricular function
5. Decreased incidence of late dysrhythmias
6. No multiple procedures
Benefits of late repair (>3~6 months)
1. Lower mortality
2. Better chance of avoiding transannular patch
3. Shorter ICU stay
4. Lower incidence of re-intervention
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Surgical Treatment – Palliative
• Palliative –
Systemic-to-pulmonary shunt
Right ventricular outflow tract patch
Right ventricle-to-pulmonary shunt
Right ventricle-to-pulmonary stent
Which technique to be used?
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Surgical Treatment - Systemic-to-pulmonary shunt
Modified BT shunt Potts shunt Waterston shunt
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Surgical Treatment - RVOT Patch & RV-PA Shunt
1. Adopted from Bove EL, Hirsch JC. Tetralogy of Fallot : Surgery for congenital heart defect, third
edition. Eds. Stark JF, et al. John Wiley & Sons, 2006:399-410
2. Adopted from Brizard CP, et al. Pulmonary atresia, VSD and mapcas: repair without unifocalization.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 12:139-144
1. 2.
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Surgical Treatment - RVOT Stent
David J. Barron DJ, et al. Surgery following primary right ventricular outflow tract stenting for Fallot’s
tetralogy and variants: rehabilitation of small pulmonary arteries Eur J Cardiothorac Surg 2013;44:656-662
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BT shunt RV-PA stent/patch/shunt
Mortality 7~8% in CCAD, STS
Some reported >15% Small cohort reports only (2~3%)
Diastolic control No Yes
Landmark for coronary artery
during the second stage No
No
Yes – if patch or shunt
Stress on RV No change Reduced
Shear stress Arterial pressure Pulsatile flow
Flow limiting factor (inflow and
outflow)
Inflow – bracheocephalic
Outflow - RPA
Inflow – size of stent / patch / shunt
Outflow – MPA / LPA
CPB No / sometimes Yes No / Yes
BT Shunt vs RA-PA Stent/Patch/Shunt
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Concerns in Postoperative TOF & TOF/PA
• Right ventricular hypertrophy
• RV fibrosis
• Restrictive RV physiology
• Severe third spacing
• Haemodynamic instability
• Prolonged chest tube
Is RV-PA patch / shunt better?
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Inconsistent Choices
Birmingham Children’s Hospital
• BT shunt for PA
• RV-PA stent for TOF
• RV-PA shunt for Norwood stage I
Great Ormond Street Hospital for Children
• RV-PA shunt for PA
• RV-PA patch for TOF
• BT shunt for Norwood stage I
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Birmingham Criteria for RVOT Stent
• Low birth-weight neonates
• < 4 kg
• Branch PAs Z-score < −2
• Complex anatomy or comorbidity
- AVSD / Fallot
- anomalous LAD,
- unroofed coronary sinus,
- chronic lung disease
David J. Barron DJ, et al. Surgery following primary right ventricular outflow tract stenting for Fallot’s
tetralogy and variants: rehabilitation of small pulmonary arteries Eur J Cardiothorac Surg 2013;44:656-662
16
PA growth (Z-scores) at the time of RVOT stenting and prior to surgery.
Adopted from David J. Barron DJ, et al. Surgery following primary right ventricular outflow tract stenting for
Fallot’s tetralogy and variants: rehabilitation of small pulmonary arteries Eur J Cardiothorac Surg 2013;44:656-
662
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Choice of Size
• Restrictive patch
– Small enough to restrict flow from VSD
• Should have L R shunt > R L shunt
• PA pressure < ½ systemic
• Some suggested 5, others 6mm for shunt
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Surgical Treatment – Corrective
• Corrective – VSD closure +
Infundibulectomy only
Transannular patch with/without valve
Pulmonary valve sparing surgery
Surgery consists of
1. Infundibulectomy
2. VSD closure
3. ±RVOT reconstruction
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Surgical Treatment - Infundibulectomy
1. Identify trabecular septomarginalis
2. Resect large septoprietal band
3. No more band before pulmonary valve
4. Divide smaller ones
- important to understand septum shape 1
2 2
3
4
Adopted from Bove EL, Hirsch JC. Tetralogy of Fallot : Surgery for congenital heart defect, third
edition. Eds. Stark JF, et al. John Wiley & Sons, 2006:399-410
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1
1’
1’’
Surgical Treatment VSD Closure
1. Pay attention to valleys
1: between tricuspid and aortic valve
1’: behind aortic valve
1’’: between superior and inferior rim of TSM
2. Conduction
1
1’’ 1’
2
Adopted from Bove EL, Hirsch JC. Tetralogy of Fallot : Surgery for congenital heart defect, third
edition. Eds. Stark JF, et al. John Wiley & Sons, 2006:399-410
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Surgical Treatment
- RVOT Reconstruction
1. Determine the size you aim for
2. Estimate the patch size
3. Ensure the size is enough in every dimension
4. ± monocusp
Adopted from Bove EL, Hirsch JC. Tetralogy of Fallot : Surgery for congenital heart defect, third
edition. Eds. Stark JF, et al. John Wiley & Sons, 2006:399-410
22
Monocusp 1.
2.
1. Adopted from Sasson L, et al. Right ventricular outflow
tract strategies for repair of tetralogy of Fallot: effect of
monocusp valve reconstruction. Eur J Cardiothorac
Surg 2013;43:743–751
2. Adopted from Park CS, et al. The long-term result of
total repair for tetralogy of Fallot. Eur J Cardiothorac
Surg 2010;39:311-317
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Surgical Treatment - Pulmonary Valve Sparing
1. Only PV thinning and commissurotomy
2. Add intraoperative balloon dilation
3. Incision of anterior leaflet and patch augmentation
1
2
3
Bacha E, et al. Valve-sparing options in tetralogy of Fallot surgery. 2013;18(4):316-327
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Short-Term Mortality
• 1950s – 20%
• 1960 ~ 1980 – 5%
• Contemporary data – 0~3%
EACTS congenital database
Adotepd from Karl T, Stocker C. Tetralogy of Fallot and its variants. Pediatr Crit Care Med 2016;17:S330–S336
25
Long -Term Mortality
Adopted from Park CS, et al. The long-term result of total repair for tetralogy of
Fallot. Eur J Cardiothorac Surg 2010;39:311-317
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Short-Term Morbidities
• Residual shunt
• Complete heart block
• Tricuspid valve regurgitation
• Aortic regurgitation
• Pulmonary regurgitation
• Restrictive right ventricular physiology
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Long-Term Morbidities
• Arrhythmias
• Right ventricular ouflow tract obstruction
• Pulmonary regurgitation
• RV dilatation PV replacement
• Reduced right ventricular function
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Adopted from Park CS, et al. The long-term result of total repair for tetralogy of
Fallot. Eur J Cardiothorac Surg 2010;39:311-317
29
Other Variations
• Absent pulmonary valve syndrome
• TOF + AVSD
• Strategy for small branch PAs
• Pulmonary atresia
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Conclusion
• Surgical repair has excellent outcome
• While there is general consensus that repair should be
performed between 3 and 12 months, controversy
persists regarding neonatal repair and staged repair
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Further Reading
1. Park CS, et al. The long-term result of total repair for tetralogy of
Fallot. Eur J Cardiothorac Surg 2010;39:311-317
2. Karl T, Stocker C. Tetralogy of Fallot and its variants. Pediatr Crit
Care Med 2016;17:S330–S336
3. Apitz C, et al. Tetralogy of Fallot. Lancet 2009; 374: 1462–71
4. Sasson L, et al. Right ventricular outflow tract strategies for repair
of tetralogy of Fallot: effect of monocusp valve reconstruction. Eur J
Cardiothorac Surg 2013;43:743–751
32
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