1. Palliative RVOT procedures
양 지 혁
성균관의대 삼성서울병원
흉부외과학교실
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2. Palliative RVOT procedures
Pulmonary Valvotomy/valvectomy
Transannular patch enlargement
RV-PA conduit interposition
Right heart obstructive lesion
Pulmonary atresia (PA)
Pulmonary stenosis (PS)
intact ventricular septum
Intact ventricular septum
With VSD
TOF
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3. Pulmonary valvotomy / valvectomy
Closed pulmonary valvotomy (Brock procedure)
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4. Pulmonary valvotomy / valvectomy
Pulmonary valvotomy w/wo bypass
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5. Why these procedures can be found only in textbook?
Interventional valvotomy
Need for additional procedure
Excessive pulmonary blood flow
Early total correction
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6. Transannular patch enlargement
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7. Transannular patch enlargement
PA with ivs
Tricuspid z-value
Definitive repair
Initial procedure
> -2
2 ventricle
RV decompression
-5 ~ -2
1½ ventricle
RV decompression + shunt
-5 >
1 ventricle
Shunt only
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8. SMC experience of PAIVS (1997~2006)
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9. Morphologic spectrum of TOF/PA
Major Aorto-Pulmonary Colllateral Arteries ; MAPCA
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10. Outcome of systemic-to-PA shunt
(Circulation 2007;116: )
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11. Initial RVOT reconstruction for palliation in TOF/PA, MAPCAs
RV-PA connection
Patch widening
Piehler et al. (Mayo) 1980
Freedom et al. (Toronto) 1983
Rome et al. (Boston) 1993
Pagani et al. (Michigan) 1995
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12. Rationale for initial RV-PA connection
↑ PA size and angiogenesis of distal microvessels, especially early in life
Nonconfluence in the presence of PA is low (3 ~ 16%)
Significant arborization abnormality in the setting of centrally confluent arteries is low
80% of patients with confluent central PA have pulmonary blood flow to 15 or more pulmonary segments
11.8 segments received blood flow from central PA 5.1 segments from aortopulmonary collateral arteries only segments from a dual source.
Allows access to pulmonary arteries
balloon dilation of peripheral stenotic vessels
precise identification of native pulmonary artery segmental distribution
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13. Advantage of RV-PA connection
 LV volume overload
Pulsatile blood flow to enhance PA growth
 Branch PA distortion, pleural adhesion
→ easier staged procedures (unifocalization)
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14. RV-PA connection for hypoplastic PA
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15. RV-PA connection for hypoplastic PA
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16. RV-PA connection for hypoplastic PA
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17. Unifocalization and RV-PA for hypoplastic PA
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18. Unifocalization and RV-PA for non-confluence
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19. PA growth after RVOT reconstruction
Piehler et al. (JTCS 1980)
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20. PA growth after RVOT reconstruction
Piehler et al. (JTCS 1980)
Less PA distortion 18% (vs. 46% of shunt)
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21. How small is too small for true PA
PAI < 90 mm2/m2 (Rome et al.)
PA diameter < 3mm (Pagani et al.)
PA diameter < 1.5mm
Melbourne shunt
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22. Selection of Conduit - material
Valved homograft
Advantage
↓ regurgitation → more growth ?
↓ bleeding
↓ pseudointimal formation
Disadvantage
Aneurysmal change
Shortage of supply
Valveless conduit (Goretex)
Less energy loss
Unable to maintain distal diastolic PA pressure
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23. Selection of Conduit - size
Sano shunt for HLHS
Non-valved Gore-Tex shunt from RV to PA
5mm : neonate < 3~3.5 kg
6mm : neonate > 3~3.5 kg
RV-PA for biventricular repair
Bradley et al. (ATS 2008)
5mm : < 3 kg
6mm : 3 ~ 4.5 kg
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24. Selection of Conduit - size
Trusler’s formula (band circumference) for PAB
noncyanotic, nonmixing lesion 20mm + 1mm/kg Bwt
Mixing lesion 24mm + 1mm/kg Bwt
SV for Fontan 22mm + 1mm/kg Bwt
Z-value > 2 not recommended
5kg, BSA 0.3
25mm ~ 29mm / π = diameter
8.0 ~ 9.2 mm – thickness of vessel wall (1mm?)
Internal diameter : 7 ~ 8mm
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25. Predictors of successful definitive repair
PAI > 150 mm2/m2
Total neopulmonary artery index (TNPAI) > 200mm2/m2
Qp/Qs > 1.5
pRV/LV < 0.8
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26. RV-PA shunt for Norwood op. (Sano)
Advantage
↑ diastolic pressure
↓ coronary steal
Pulsatile flow
Disadvantage
RV incision
RV volumeload → TR
↓ PA growth
Better in low birth weight babies
Better at smaller center
Lower risk of graft thrombosis
Greater resistance to physiologic insults such as cardiopulmonary arrest
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27. RV-PA shunt ; technical considerations
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28. RV-PA vs. BT shunt - mathematic model
(JTCS 2008)
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29. RV-PA vs. BT shunt - mathematic model
3mm BTS vs. 4mm RV-PA
3.5mm BTS vs. 5mm RV-PA
4mm BTS vs. 6mm RV-PA
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30. RV-PA vs. BT shunt - mathematic model
RV-PA model exhibited, at similar SaO2
lower pulse pressure
lower Qp/Qs ratio
lower PAP
lower RV systolic and diastolic pressure
higher coronary perfusion pressure
higher O2 delivery
higher ventricular performance
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31. RV-PA vs. BT shunt - mathematic model
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32. RV-PA shunt in biventricular repair
Bradley et al. (Ann Thorac Surg 2008;86:183– 8)
10 infants excluding patients with PA VSD, MAPCA
Median age : 9 (4~86) days
Median Wt. : 3.0 (1.7 ~ 4.5) kg
Non-valved Gore-Tex shunt from RV to PA
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33. RV-PA shunt in biventricular repair
No hospital deaths
1 reoperation
Revision of shunt d/t distal anastomosis stenosis
SpO2 at the discharge : 94 ± 4 %
2 patients with a clipped shunt underwent successful balloon dilation (6 Mo after RV-PA)
Biventricular repair
8 patients
interval : median 10 (6~17) months
SpO2 : 86 ± 1
Conduit diameter : 14 ~ 16 mm
No early or late deaths
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34. Case 1, M/3Mo PA, VSD, MAPCAs, hypoplastic central PA (+)
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35. Case 1, M/3Mo RV-PA connection with homograft femoral vein 7mm
LPA angioplasty with GA-fixed autologous pericardium
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36. Case 2, F / 3.7yrs PA, VSD, MAPCAs, s/p staged unifocalization
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37. Case 2, F / 3.7yrs RV-PA connection with T-shaped homograft
6 Mo later, VSD closure (PRV/LV = 0.65)
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38. Case 3, F / 6Mo TGA, VSD, PA, Rt aortic arch
s/p LMBT shunt, PA angioplasty
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39. Case 3, F / 6Mo
RV-PA connection with femoral vein homograft (composite graft with Goretex 8mm)
RPA & LPA angioplasty
Shunt takedown
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