Norwood:rastelli

1. Staged Biventricular Repair for Neonates With Left
Ventricular Outflow Tract Obstruction, Ventricular
Septal Defect, and Aortic Arch Obstruction
Mohammad Shihata, MD, Chawki El-Zein, MD, Katie Wittle, APN, Tarek Husayni, MD,
and Michel Ilbawi, MD
Madinah Cardiac Center, Taibah University, Madinah, Saudi Arabia; and Heart Institute for Children, Advocate Children’s Hospital,
Oak Lawn, Illinois
Background. The purpose of this study is to evaluate
clinical outcomes of neonates who underwent a Norwood
operation as a first step of a planned biventricular repair
and the impact of associated risk factors.
Methods. A retrospective cohort study was performed
on all neonates (n [ 44) undergoing the Norwood opera-tion
as the first stage of a biventricular (Norwood-Rastelli)
repair from January 2000 to December 2012 at a single
center. Multivariable analysis was performed to identify
predictors of survival.
Results. Stage one mortality was 9%. The interstage
survival for nonsyndromic and syndromic patients was
100% versus 46%, respectively (p < 0.001). Twenty-four
patients (55%) underwent biventricular completion repair
with no mortality. Freedom from reintervention after
biventricular completion was 53% at 6 years. The overall
survival for nonsyndromic patients versus syndromic pa-tients
was 86% versus 43%, respectively (p[0.01). Genetic
syndromes and prematurity were significant predictors of
interstage mortality on multivariable analysis.
Conclusions. Staged biventricular repair for patients
with complex left ventricular outflow tract obstruction,
ventricular septal defect, and aortic arch obstruction can be
achieved with excellent outcomes for neonates without
genetic syndromes. The staged approach is associated with
longer time to reintervention after the biventricular
completion.
(Ann Thorac Surg 2014;-:-–-)
2014 by The Society of Thoracic Surgeons
Neonates presenting with critical left ventricular
outflow tract obstruction (LVOTO), aortic arch
obstruction (AAO [interruption or coarctation]), and
ventricular septal defect (VSD) can vary in their presen-tation
[1]. These patients are invariably dependent on a
patent ductus arteriosus to maintain adequate systemic
circulation. Some present with hypoplastic left heart
syndrome or one of its variants and are triaged down a
single ventricle repair pathway utilizing the Norwood
procedure or one of its modifications. Others have a
normal left ventricle and mitral valve and are suitable
for biventricular repair. Some neonates have borderline
left cardiac structures and can present a true challenge
in deciding which surgical pathway to follow. If the
native left ventricular outflow tract cannot be used
as the sole systemic outflow, it needs to be replaced
by a Ross or a Ross-Konno operation or augmented
with a Damus-Kaye-Stansel procedure, as in the Yasui
operation if a VSD is present [2]. The neonatal Ross
operation is associated with high mortality, especially
when associated with arch repair [3, 4]. Furthermore,
the Yasui operation can be performed as a single stage or
as a staged repair (Norwood-Rastelli). In the staged
approach, a Norwood type repair is performed followed
at a later stage by complete septation, channeling the
VSD to the native pulmonary valve and establishing right
ventricle to pulmonary artery (RV-PA) continuity [5].
Both the anatomic substrate and institutional preference
influence the type of surgical repair.
The superiority of one approach over the other remains
an unanswered question. Complete repair has the
advantage of avoiding a shunt type physiology and hyp-oxemia
during early development stages. The staged
approach carries less surgical trauma initially and is
particularly helpful in borderline left-side heart structures
for it preserves the fallback option to a single ventricle
repair, should the left ventricle or mitral valve become
clearly inadequate on subsequent follow-up. Conversion
of biventricular repair to single ventricle repair has been
notoriously associated with highmortality [6]. The purpose
of this study is to evaluate outcomes of neonates who
underwent a Norwood procedure as a first step of a
planned biventricular repair and to identify factors that
affect these outcomes.
Patients and Methods
The study was approved by the Institutional Review
Board at Advocate Children’s Hospital, Oak Lawn,
Accepted for publication May 27, 2014.
Presented at the Poster Session of the Fiftieth Annual Meeting of The
Society of Thoracic Surgeons, Orlando, FL, Jan 25–29, 2014.
Address correspondence to Dr Shihata, Madinah Cardiac Center, Taibah
University, PO Box 6167, Madinah 41311, Saudi Arabia; e-mail: mshihata@
gmail.com.
2014 by The Society of Thoracic Surgeons 0003-4975/$36.00
Published by Elsevier Inc http://dx.doi.org/10.1016/j.athoracsur.2014.05.077

2. 2 SHIHATA ET AL Ann Thorac Surg
STAGED REPAIR FOR LVOTO, VSD, AND AAO 2014;-:-–-
Illinois. All neonates undergoing the Norwood operation
(or one of its modifications) as a first stage of a biven-tricular
repair from January 2000 to January 2013 at our
hospital were retrospectively analyzed. A combination
of complex LVOTO, VSD, and AAO was present in all of
the neonates in the study cohort (Fig 1). The mitral valve
and left ventricular function and dimensions were normal
or near normal (z scores 2 to 2). Genetic testing was
performed as part of the routine assessment for
neonates referred for cardiac surgery at our center. That
includes testing for chromosome 22q11.2 deletion.
Variables are expressed as mean SD or proportions,
as appropriate. Kaplan-Meier survival curves were used
for survival rates at different stages and freedom from
reintervention. The Cox proportional hazards model was
used to identify significant predictors of survival, with the
purposeful model-building method. All statistical anal-ysis
was performed using Stata for Windows (Stata Corp,
College Station, TX).
Results
A total of 44 neonates were identified (Table 1). Aortic
arch obstruction (interrupted aortic arch, 70%; CoA,
30%; and VSD 5 mm) was present in all patients. The
mean aortic valve annulus size was 3.3 0.8 mm, with
a mean z score of 5.9 1.9. Aortic atresia was present
in 5 patients (11.4%). DiGeorge syndrome was present
in 22 patients (50%), and trisomy 22 in 1 patient. The
mean age at stage one was 11 9.6 days. Forty-four pa-tients
underwent the Norwood operation leaving a
restrictive atrial septal defect (4 mm). The type of shunt
was RV-PA in 21 neonates (48%) and a Blalock-Taussig
shunt in 20 neonates (45%); 3 patients (7%) underwent a
hybrid procedure. Our preference has been to use RV-PA
shunt with reversed beveling, as described in a previous
report [7]. Stage one survival to hospital discharge
was 91%. The overall survival for nonsyndromic
and syndromic patients was 86% and 43%, respectively
(p ¼ 0.01), and the interstage survival was 100% and
46%, respectively (p 0.001; Figs 2, 3). Twenty-one
patients (48%) required a catheter-based or surgical
interstage procedure (Table 2).
Fig 1. The spectrum of complex left ventric-ular
outflow tract obstruction (LVOTO) and
aortic arch obstruction (AAO) and the man-agement
algorithm at Advocate Children’s
Hospital. (AS ¼ aortic stenosis; AV ¼ aortic
valve; CoA ¼ coarctation of aorta; HLHS ¼
hypoplastic left heart syndrome; IAA ¼
interrupted aortic arch; LV ¼ left ventricle;
MV ¼ mitral valve; VSD ¼ ventricular septal
defect.)
Table 1. Stage One Characteristics
Stage One Characteristics % (n) Mean SD
Preterm 23 (10) .
Female 43 (19) .
Weight, kg . 2.9 0.63
Body surface area, m2 . 0.19 0.03
Interrupted aortic arch type B 70.5 (31) .
Coarctation of aorta 29.5 (13) .
Ventricular septal defect 100 (44) .
Aortic stenosis 88.7 (39) .
Aortic atresia 11.3 (5) .
Aortic valve annulus, mm . 3.3 0.8
Aortic valve, z score . 5.9 1.9
Borderline left ventricle 16 (7) .
Genetic syndrome 52.2 (23) .
Age at stage 1, days . 11.7 9.6
Norwood Blalock-Taussig 45 (20) .
Norwood RV-PA shunt 48 (21) .
Hybrid 7 (3) .
Extracorporeal membrane oxygenation 9.1 (4) .
Ventilation, days . 6 1.5
Intensive care unit stay, days . 11.9 4
Hospital stay, days . 36.3 56.7
RV-PA ¼ right ventricle-to-pulmonary artery.

3. Ann Thorac Surg SHIHATA ET AL 3
2014;-:-–- STAGED REPAIR FOR LVOTO, VSD, AND AAO
Fig 2. Comparison of interstage survival of the study cohort based on
presence (dotted line) or absence (solid line) of genetic syndromes.
Twenty-four patients underwent biventricular com-pletion
repair at a mean age of 19.9 9 months with no
deaths. Concomitant VSD enlargement was required in
38% (9 patients). Freedom from any reintervention after
biventricular completion was 53% at 6 years. The types
of reintervention procedures are listed in Table 3.
The presence of genetic syndromes (adjusted hazard
ratio for nonsyndromic, 0.06; p ¼ 0.02) and prematurity
(adjusted hazard ratio for term neonates, 0.1; p ¼ 0.04)
were significant predictors of interstage and overall
mortality.
Comment
This study was conducted to evaluate the outcomes of a
challenging cohort of neonates presenting with AAO,
VSD, and complex LVOTO. This combination is rather
uncommon in that it represents less than 4% of the
LVOTO cohort in the Congenital Heart Surgeons Society
studies [8]. In spite of that, a number of small series have
attempted to evaluate the best approach to managing this
complex problem. It became clear from a number of
reports that the Ross-Konno approach in combination
with arch augmentation in the neonatal period is associ-ated
with high mortality and failure rates. Our preference
is to reserve the Ross-Konno procedure for patients with
complex LVOTO who do not require arch augmentation.
Whenever possible, this is deferred by various valvulo-plasty
techniques.
Efforts in the more contemporary reports have been
directed toward utilizing a Norwood-type procedure
in combination with establishing RV-PA continuity in
one stage (Yasui operation) or in a staged approach
(Norwood-Rastelli). Each of these strategies has its ad-vantages
and drawbacks. The small number of patients in
each of these studies makes it difficult to draw any solid
conclusions. In our opinion, however, we believe that
the staged approach is associated with less myocardial
trauma and shorter ischemia times in the neonatal period.
Furthermore, a staged approach may be necessary in
cases with borderline left ventricle size because it allows
for following a single ventricle pathway should that be
indicated. An additional advantage would be the ability to
implant a larger RV-PA conduit, delaying the need for
subsequent interventions. The mean RV-PA conduit size
at the time of biventricular completion in this cohort was
16 1.2 mm.
The striking difference in the interstage mortality be-tween
the two groups is hard to attribute to a single cause
as most of the deaths occurred out of the hospital. Two
patients with 22q11.2 deletion required emergency read-mission
and extracorporeal support for shunt thrombosis.
Both these patient, however, underwent successful
biventricular completion and are alive and well to date.
In an attempt to provide a collective comparative
context, we summarized the approaches and commonly
reported outcomes in all published series dealing with
this subset of neonates from 1999 onward (Table 4).
The decision to use a primary or staged approach in the
summarized studies was largely based on the surgeon’s
Fig 3. Comparison of overall survival of the study cohort based on
presence (dotted line) or absence (solid line) of genetic syndromes.
Table 2. Interstage Procedures
Procedure % (n) Mean SD
Age, months . 7.6 4.8
Balloon pulmonary artery plasty 5 (2) .
Blalock-Taussig shunt 53 (21) .
Emergency Blalock-Taussig shunt 5 (2) .
Coarctation of aorta ballooning 7.5 (3) .
Table 3. Reintervention Procedures After Biventricular Repair
Reintervention Procedure % (n)
Right ventricle–PA conduit replacement 45.8 (11)
Patch PA plasty 8.3 (2)
PA stent 4 (1)
Ventricular septal defect enlargement 8.3 (2)
Pacemaker 4 (1)
PA ¼ pulmonary artery.

4. 4 SHIHATA ET AL Ann Thorac Surg
STAGED REPAIR FOR LVOTO, VSD, AND AAO 2014;-:-–-
Table 4. Summary of Recent Studies on Biventricular Repair of Left Ventricular Outflow Tract Obstruction, Ventricular Septal
Defect, and Aortic Arch Obstruction From 1999 to 2013
preference and to a lesser extent, the anatomic substrate.
The patients in these studies had very similar anatomic
substrates but varied on a number of characteristics,
including the prevalence of genetic syndromes and
prematurity. Both were found to be significant negative
predictors of survival in our analysis, consistent with a
recent report by Kanter and coworkers [9]. The lack of a
control group makes it difficult to reach conclusions
about the superiority of the staged approach in our
study. In comparison to previously published reports,
however, it seems that the staged approach allows for
performing the biventricular completion on larger babies.
In conclusion, staged biventricular repair for complex
LVOTO, VSD, and AAO is safe, reproducible, and
sometimes necessary. It allows for a larger RV-PA conduit
at the time of completion, delaying the need for subse-quent
interventions. Genetic syndromes and prematurity
are significant negative predictors of long-term survival.
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Series Review [Reference]
No. of
Cases AAO (%) SYN (%)
Yasui P
Versus S (n)
Early
Mortality (%) BiV (n)
Overall
Survival (%)
Reintervention
After BiV repair
(%)
Ann Arbor [10], 1999 20 90 NA P 11, S 9 5% 19 P (73%),
S (89%)
NA
Cincinnati [11], 2003 8 87 NA S 0% 6 100 33 (3 y)
Philadelphia [12], 2006 21 29 31 P 0% 21 95 67 (10 y)
Boston [13], 2006 17 80 18 P 18% 17 82 63 (3 y)
Birmingham, UK [14], 2007 16 75 31 P 19% 16 46 80 (5 y)
Riyadh, Saudi Arabia [3], 2010 14 79 NA P 13, S 1 21% 14 79 Surgery 43 (5 y)
Atlanta [9], 2012 21 81 48 P 6, S 15 0% 21 nSYN 100,
SYN 65
79% (3 y)
Current study 44 100 52 S 9% 24 nSYN 86,
SYN 43
46% (6 y)
AAO ¼ aortic arch obstruction; BiV ¼ biventricular repair; NA ¼ not applicable; nSYN ¼ nonsyndromic; P ¼ primary; S ¼ staged
repair (Norwood-Rastelli); SYN ¼ syndromic; y ¼ years.