MANAGEMENT OF THE SINGLE VENTRICLE AND POTENTIALLY OBSTRUCTIVE SYSTEMIC VENTRICULAR OUTFLOW TRACT Joined 24th SHA & 10th GHA Conference 13-16 February 2013, Riyadh, Saudi Arabia Bahaaldin Alsoufi, MD King Faisal Specialist Hospital and Research Center Riyadh, Saudi Arabia
BACKGROUND • Multi-stage palliation: current strategy for SV anomalies • Success depends on favourable hemodynamic conditions • SVOTO: hypertrophy and unfavourable hemodynamics • Inability to progress or late failure
PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA RV BVF
Freedom et al, Circulation 86
PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA
RA
LA
RV BVF
LV
LV
AO
BVF
RV
PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD
PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD – DORV with uncommitted VSD – D- or L-TGA with VSD and small RV
PATIENTS AT RISK • Aorta from outlet chamber connected to dominant ventricle via VSD/BVF: – TA with TGA – DILV with TGA – Unbalanced AVSD – DORV with uncommitted VSD – D- or L-TGA with VSD and small RV
• Hypertrophy of sub-aortic conus: – DORV +/- small LV +/- MS/MA
PATIENTS AT RISK
MANAGEMENT • Determinant factors: – Semilunar valves – BVF / VSD – Arch obstruction
Neonate
Neonatal SVOT relief DKS
+/- Arch
SVOT relief with BCPC Neonatal PAB +/- COA
VSD enlargement
BCPC DKS
Palliative switch
VSD enlargement
Neonate
Neonatal SVOT relief DKS
+/- Arch
X
VSD enlargement
Palliative switch
SVOT relief with BCPC Neonatal PAB +/- COA
BCPC DKS VSD enlargement
• Address SVOTO at neonatal age: – DKS + shunt (+ arch = Norwood)
• Address SVOTO at BCPC: – 1st stage: PAB +/- COA – 2nd stage: • BCPC + DKS • BCPC + VSD enlargement
DKS + SHUNT • Advantages: – Address SVOTO early – Arch augmentation
• Disadvantages: – CPB and DHCA in neonates – Complex post-operative course – Early and interim mortality
Modified Norwood Operation for Single Left Ventricle and Ventriculoarterial Discordance: An Improved Surgical Technique Ralph S. Mosca, MD, Hani A. Hennein, MD, Thomas J. Kulik, MD, Dennis C. Crowley, MD, Erik C. Michelfelder, MD, Achi Ludomirsky, MD, Edward L. Bove, MD
N=20 Absolute BVF size: increase with time in 50% of patients Not predictable Indexed BVF size: 20% overall decrease
Ann Thorac Surg 1997
Modified Norwood Operation for Single Left Ventricle and Ventriculoarterial Discordance: An Improved Surgical Technique Ralph S. Mosca, MD, Hani A. Hennein, MD, Thomas J. Kulik, MD, Dennis C. Crowley, MD, Erik C. Michelfelder, MD, Achi Ludomirsky, MD, Edward L. Bove, MD
Ann Thorac Surg 1997
Ann Thorac Surg 1997
Norwood / ASO
COA + PAB
Ann Thorac Surg 1995
PAB + COA • Advantages: – No CPB or DHCA – Can be done via thoracotomy
• Disadvantages: – Residual arch gradient (HAA) – Distortion of PAs – Distortion of PV? – Development of SVOTO
Sub-As in 31/43 (72%) of PAB SV Pts Mean PAB age 0.21 Y Mean Sub-AS diagnosis age 2.52 Y
PAB produces myocardial hypertrophy and accelerates potential stenosis
Circulation 1986
BVFAI (cm2/m2)
Smaller BVF = higher chance of sub AS BVFAI < 2 cm2/m2
DKS
Early Obst.
Late Obst.
JACC 1992
BVFAI (cm2/m2)
COA = higher chance of sub AS
COA
No COA
JACC 1992
Effect of volume unloading with CPC
JTCVS 2012
Decrease in VSD size with CPC and PAB More difference after CPC
JACC 1995
BCPC + DKS • Advantages: – Stable modified in series physiology – Address SVOTO – Concomitant arch – No heart block
• Disadvantages: – Seminlunar valve function – Lt main bronchus and LPA
DKS
Originally described for D-TGA
Deleon et al, Ann Thorac Surg 1994
DOUBLE BARREL DKS
Carter et al, Ann Thorac Surg 1994
Preserved semilunar valve function with double barrel technique
JTCVS 2011
KFSHRC EXPERIENCE 1997-2012 n=36 Males 69% Age Weight
8.9 M 6.7 kg
(3.6 M – 9.1 Y) (5 – 27 Kg)
• All had prior palliation (n=36): – PAB – COA/Arch repair – Atrial septectomy – PPI
n=35 n=11 n=8 n=1
Survival
83%
All deaths: first 6 months None SVOT / DKS related Years after DKS
86%
TCPC + DKS
69%
Survival
BCPC + DKS
P=0.30
Years after DKS
SVOT gradient (mm Hg)
P<0.001
P=1.0
23.4 Âą 18.7
0
0
Semilunar Valves
VSD ENLARGEMENT • Advantages: • Independent of semilunar valves • Can manage late SVOTO • Disadvantages: • Heart block • Inadequate SVOTO relief • Recurrent SVOTO • Ventriculotomy
VSD ENLARGEMENT
1/9 operative death 3/9: recurrent SVOTO 1/9 PPI
Ann Thorac Surg 2001
VSD ENLARGEMENT
Ann Thorac Surg 2008
L AVV BVD to AO Sub PS Dominant LV
R AVV
Neonate
Obstructive VSD
Non-Obstructive VSD
DKS + shunt Norwood
COA + HAA
No HAA
Norwood
COA PAB + COA Norwood
No COA PAB
PAB
BCPC + DKS
BCPC + VSD enlargement PS PI DORV remote VSD
Late presentation
PV sutured
PV not sutured
VSD enlargement
? DKS VSD enlargement
3 patients with previously transected MPA No SVOTO No Semilunar valve insufficiency
Ann Thorac Surg 1999
PALLIATIVE SWITCH • Advantages: • Can be done in neonates alternative to Norwood • No LPA entrapment • Unobstructed SVOT
• Disadvantages: • Coronaries • PAs
• Root • Concomitant PAB or shunt ?
0/14 early or late death 14/14 BCPC 11/14 TCPC + 3/14 awaiting TCPC 64% PA augmentation at TCPC
Ann Thorac Surg 2013
VSD ENLARGEMENT
Kirkland/BarrattBoyes Cardiac Surgery 3rd Edition
DKS
PALLIATIVE SWITCH