This document describes a study of staged biventricular repair for neonates with left ventricular outflow obstruction, ventricular septal defect, and aortic arch obstruction. The study included 44 patients who underwent an initial Norwood procedure followed by a Rastelli operation. Overall survival after the staged repair was 46% at 6 years. Non-syndromic patients had significantly better long-term survival compared to those with genetic syndromes. Being term at birth also predicted improved overall survival compared to preterm infants. The staged approach allowed for placement of a larger RV-PA conduit, reducing need for reinterventions.

1. Staged Biventricular Repair for Neonates with Left
Ventricular Outflow Obstruction, Ventricular Septal
Defect, and Aortic Arch Obstruction
Mohammad Shihata, Chawki Elzein, Sujata Subramanian, and
Michel Ilbawi

2. Neonatal LVOTO
• The vast majority of neonates with LVOTO suitable for
biventricular repair are managed with balloon or to a
lesser extent surgical aortic valvuloplasty.
• The study population comprises a small subset of the
neonatal spectrum of LVOTO ( < 4% of the CHSS
LVOTO inception cohort from 29 centers, (n=1217,
1994 - 2008).

3. Complex Neonatal BiV Repair
• If the native LVOT cannot be used as
the sole systemic outflow, it needs to be
:
• Replaced: Ross ± Konno
• Augmented: Yasui; primary or staged
( Norwood/Rastelli )
• Bypassed: LV to DAo conduit

4. Neonatal Ross/Konno
• Neonatal Ross±Konno operation is associated with
high mortality especially if combined with an arch
repair; 33% - 67%. ( CHSS and STS-CHD )
• In the CHSS LVOTO cohort the Ross group had a
40% failure rate (conversion to SV or Transplant).

5. Staged vs. Primary Yasui
• For the last decade, no one approach
has been clearly superior.
• Primary Yasui is associated with the
need for early reintervention.
• A staged approach may be necessary
in borderline cases.

6. Study Population

7. Patient Characteristics
% (N) Mean ± SD Min. Max.
Gestational age (wk.) -- 37 ± 2.4 30 41
Preterm 23 (10) -- -- --
Gender (F) 43 (19) -- -- --
Weight (kg) -- 2.9 ± 0.63 1.6 3.9
BSA (m 2
) -- 0.19 ± 0.03 0.13 0.24
IAA-B 70.5 (31) -- -- --
CoA 29.5 (13) -- -- --
VSD 100 (44) -- -- --
Aortic Stenosis 88.7 (39) -- -- --
Aortic Atresia 11.3 (5) -- -- --
AV annulus (mm) -- 3.3 ± 0.8 1.5 5
AV ( z score ) -- - 5.9 ± 1.9 -10.3 -3.1
Borderline LV 16 (7) -- -- --
Genetic Syndrome 52.2 (23) -- -- --

8. Stage 1
% (N) Mean ± SD Median
Age (d) -- 11.7 ± 9.6 8
Norwood BT 45 (20) -- --
Norwood RV PA 48 (21) -- --
Hybrid 7 (3) -- --
CPB (min.) -- 135 ± 46 120
Cross Clamp (min.) -- 65.5 ± 26 59
Selective Perfusion
(min.) -- 38.2 ± 13.6 36
ECMO 9.1 (4) -- --
Ventilation (d) -- 6 ± 1.5 6
ICU stay -- 11.9 ± 4 11.5
Hospital stay (d) -- 36.3 ± 56.7 21

9. Post Norwood Survival

10. Interstage Survival

11. Interstage Procedures
% (N) Mean ± SD
Age (months) -- 7.6 ± 4.8
BPA plasty 5 (2) --
BT shunt 53 (21) --
Emergency BT
shunt 5 (2) --
CoA
Ballooning 7.5 (3) --
21/24 (88%) of completed BiV had interstage procedures

12. Stage 2
% (N) Mean ± SD
Age (months) -- 19.9 ± 9.6
Rastelli 96 (23) --
VSD closure/IAA
repair 4 (1) --
VSD enlargement 38 (9) --
Homograft size (mm) -- 15 ± 1.2
CPB (min.) -- 193.8 ± 37
Cross clamp (min.) -- 129.4 ± 32
Hospital stay -- 9.5 ± 7.5

13. Post Rastelli Survival

14. Reintervention Post BiV
Repair
Repair

15. Reintervention Procedures
% (N)
RV-PA conduit replacement 45.8 (11)
Patch PA plasty 8.3 (2)
PA Stent 4 (1)
VSD enlargement 8.3 (2)
Pacemaker 4 (1)

16. Overall Survival

17. COX PH - Predictors of
Overall Survival
Univariable HR Multivariable HR
HR p value HR p value
Term vs.
Prem. 0.3 0.22 0.1 0.04
Birth wt. 0.6 0.36 -- --
Gender (M) 0.6 0.4 -- --
nSYN vs. SYN 0.1 0.01 0.06 0.02
Norwood vs.
Hybrid 0.2 0.07 -- --
Sano vs. BT 1.02 0.9 -- --
ECMO 0.9 0.9 -- --

18. Total # AAI/CoA Genetic
Syndrome
Yasui
P vs S
Early
Mortality BiV # Overall
Survival Reintervention
1999 20 90% N/A P (11)
Ann Arbor
S (9) 5% 19 P (73%)
S (89%) N/A
Cincinnati
2003 8 87% N/A S 0% 6 100 33% (3y)
Philadelphia
2006 21 29% 31% P 0% 21 95% 67% (10y)
Boston
2006 17 80% 18% P 18% 17 82% 63% (3y)
Birmingham, UK
2007 16 75% 31% P 19% 16 46% 80% (5y)
2010 14 79% N/A P (13)
Riyadh, KSA
S (1) 21% 14 79% surg. 43%
(5y)
Atlanta
2012 21 81% 48% P (6)
S (15) 0% 21 nSYN (100%)
SYN (65%) 79% (3y)
Current
Study 44 100% 52% S 9% 24 nSYN (86%)
SYN (43%) 46% (6y)

19. Conclusion
• Staged BiV repair for complex
LVOTO,VSD & AAO is safe,
reproducible, and sometimes
necessary.
• It allows for a bigger size RV-PA
conduit at the time of completion,
delaying the need for subsequent
interventions.
• Genetic syndromes and prematurity are
significant negative predictors of long
term survival.

20. Thank You