Content uploaded by David F A Lloyd
Author content
All content in this area was uploaded by David F A Lloyd on Aug 15, 2017
Content may be subject to copyright.
ORIGINAL RESEARCH
Analysis of preoperative condition and interstage
mortality in Norwood and hybrid procedures for
hypoplastic left heart syndrome using the Aristotle
scoring system
David F A Lloyd, Lindsay Cutler, Shane M Tibby, Sunitha Vimalesvaran,
Shakeel Ahmed Qureshi, Eric Rosenthal, David Anderson, Conal Austin,
Hannah Bellsham-Revell, Thomas Krasemann
Evelina London Children’s
Hospital, Guy’s & St Thomas
NHS Foundation Trust, London,
UK
Correspondence to
Dr Thomas Krasemann, Evelina
London Children’s Hospital,
Department of Paediatric
Cardiology, Guy’s & St Thomas
NHS Foundation Trust,
Westminster Bridge Road,
London SE1 7EH, UK;
thomas.krasemann@gstt.nhs.uk
Received 31 July 2013
Revised 3 December 2013
Accepted 11 December 2013
Published Online First
10 January 2014
▸http://dx.doi.org/10.1136/
heartjnl-2013-305404
To cite: Lloyd DFA,
Cutler L, Tibby SM, et al.
Heart 2014;100:775–780.
ABSTRACT
Objective The ‘hybrid procedure’, consisting of surgical
banding of the pulmonary arteries with intraoperative
stenting of the arterial duct, was developed as primary
palliation in hypoplastic left heart syndrome (HLHS),
avoiding the risks of cardiopulmonary bypass. In many
centres, it is reserved for low birth weight, premature or
unstable neonates; however, its role in such high risk
cases of HLHS has yet to be defined.
Methods The preoperative condition of all patients
with HLHS who underwent either the hybrid or the
Norwood procedure for HLHS between 2005–2011 was
analysed retrospectively, using a modified comprehensive
Aristotle score. We then compared operative, interstage
and 1 year mortalities between the groups after Aristotle
adjustment via Cox proportional hazards analyses.
Results Of 138 patients with HLHS, 27 had hybrid and
111 Norwood procedures. The hybrid group had
significantly higher Aristotle scores (mean 4.1 vs 1.8;
p<0.001); however, there was no significant difference
in mortality at any stage. At 1 year, the overall
unadjusted survival among Norwood and hybrid patients
was 58.6% and 51.9%, respectively, yielding an
Aristotle adjusted hazard ratio for mortality among
hybrid patients of 1.09 (95% CI 0.56 to 2.11, p=0.80).
Conclusions Applying a hybrid approach to high risk
patients with HLHS produces a comparable early and
interstage mortality risk to lower risk patients undergoing
the Norwood procedure. Prospective studies are needed to
establish whether the hybrid procedure is a viable
alternative to the Norwood procedure in all HLHS patients
in terms of both mortality and long term morbidity.
INTRODUCTION
In most centres, the 30 day survival following the
Norwood procedure as the first stage operation to
palliate hypoplastic left heart syndrome (HLHS) is
>70%, although there remains significant peri-
operative and interstage mortality and morbidity.
1
The risk increases with factors such as poor pre-
operative condition, prematurity, low birth weight,
preoperative obstruction to pulmonary venous
return, small ascending aorta diameter, and if the
procedure is performed beyond 2 weeks of age.
2–4
Survival rates are independent of the type of pul-
monary shunt used.
5
The obligatory use of cardio-
pulmonary bypass may be a significant contributing
factor to adverse outcomes.
In view of these concerns, an off-pump ‘hybrid
procedure’combining both surgical and catheter
techniques has been developed, consisting of surgi-
cal placement of bilateral pulmonary artery bands
through a midline sternotomy, followed by stenting
of the arterial duct (percutaneously or via direct
pulmonary artery access), and finally percutaneous
septostomy if needed.
6
The patient then undergoes
a staged surgical pathway similar to the Norwood
approach, although the next stage is more complex,
involving debanding of both pulmonary arteries,
removal of the ductal stent, and reconstruction of
the aortic arch with proximal aortopulmonary
anastomosis. A source of pulmonary blood supply
must be established either by a superior cavopul-
monary anastomosis (‘combined stage I and II’)or
a modified Blalock-Taussig (BT) shunt as for a clas-
sical stage I operation. The subsequent stages
(upper cavopulmonary and then total cavopulmon-
ary anastomosis=Fontan circulation) are the same
whichever route is adopted.
Initial results suggested that outcomes following the
hybrid procedure were comparable to the Norwood
procedure
6–8
; however, objective comparison has been
difficult as the indications for hybrid versus Norwood
are not standardised. In some centres, including our
own, the hybrid procedure is reserved for higher risk,
more complex and/or unstable patients. Thus, to
provide a more practical comparison of the two
approaches, both the preoperative condition of the
patient and extracardiac risk factors need to be consid-
ered. To date there have been no reports published
that compare the outcomes of the Norwood and
hybrid procedures in this way.
The aim of this study was therefore to compare
the medium term outcomes following the hybrid
versus Norwood procedures in HLHS patients after
risk adjustment based upon a modified comprehen-
sive Aristotle score.
METHODS
The study was approved as an audit by the institu-
tional board of the Evelina Children’s Hospital
Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759 775
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
(ECH), London, on 15 October 2009. The need for parental
consent was waived.
Patients with HLHS who underwent either the Norwood or
hybrid procedure during the hybrid era (from December 2005
onwards) were identified from the institutional database of the
Department of Congenital Heart Disease (Heartsuite, Systeria,
Glasgow, UK) up to and including September 2011. Any
patients without a diagnosis of classical HLHS (eg, unbalanced
atrioventricular septal defects) or patients with an incomplete
dataset were excluded. The remaining patients were divided into
two groups according to the treatment type.
The Norwood procedure was carried out using cardiopul-
monary bypass employing short periods of hypothermic circula-
tory arrest combined with isolated cerebral perfusion and
insertion of modified BT shunt (the ‘Sano’modification with
right ventricle to pulmonary artery conduit was not used in any
patient). Inclusion criteria for the hybrid procedure during the
study period were preoperative asphyxia, low birth weight
(<2.5 kg), restrictive interatrial septum, and certain anatomical
variances (eg, hypoplastic left pulmonary artery), as these
patients had a poor outcome in the historical Norwood popula-
tion before the beginning of the hybrid programme. The pro-
cedure was performed as described previously by our group.
6
Bilateral branch pulmonary artery banding was performed via
midline sternotomy with two pieces of cut polytetrafluoroethyl-
ene sutured in place over the branch pulmonary arteries to an
external diameter of 3 mm to prevent band migration. A
balloon expandable premounted Genesis PSS stent (Cordis,
Corp, Miami, Florida, USA), sized at 1–2 mm greater than the
diameter of the arterial duct, was deployed via direct catheter-
isation of the pulmonary artery.
If indicated, transcatheter balloon atrial septostomy was also
performed either at the time of the procedure, or later if
required. In cases where an intact or severely restrictive intera-
trial septum had been diagnosed antenatally, elective caesarean
section was performed in an adjacent operating theatre, and
immediate surgical septectomy performed on cardiopulmonary
bypass at 28°C with very brief circulatory arrest and venous
inflow occlusion but no cardioplegic arrest.
6
A total of 139 patients fulfilled the selection criteria. None
had to be excluded for incomplete data. One hybrid patient
died at 1 week of age due to an autosomal recessive metabolic
disorder (Zellweger syndrome, undiagnosed at the time of
surgery) and was excluded from further analysis. Of the 138
remaining patients, 113 (82%) had been diagnosed antenatally
with HLHS. A preponderance of males was noted (n=94,
68%).
Risk adjustment via the modified Aristotle score
The Aristotle and Risk Adjustment for Congenital Heart
Surgery (RACHS) scoring systems were designed to risk adjust
outcomes in congenital heart disease surgery, taking into
account the procedure performed and/or patient complexity.
The RACHS score has been shown to be a more powerful pre-
dictor of overall mortality
9
; however, the comprehensive
Aristotle score, which also takes into account key variables such
as birth weight, prematurity, and major extra-cardiac abnormal-
ities, has been shown to be a superior predictor of 30 day post-
operative mortality.
10
Originally designed by consensus opinion from a group of
experienced cardiothoracic surgeons to compare surgical out-
comes across centres,
11
the Aristotle score has two forms: the
basic score simply assigns a numerical value (from 1.5–15) for
the procedure being undertaken, based on the anticipated
technical difficulty and the potential for morbidity and mortal-
ity. For the Norwood procedure, this value is 14.5; there is cur-
rently no value assigned for the hybrid procedure. The
comprehensive Aristotle score further adjusts for the complexity
of the individual patient by taking into account numerous
patient specific, ‘procedure independent’characteristics, and has
been shown to be a good predictor of surgical mortality even
when used without the basic score values.
12
Thus, to compare
the preoperative status of each patient, a modified procedure
independent Aristotle score was calculated retrospectively from
the medical records. A number of scoring criteria in the compre-
hensive score were excluded: ‘left ventricular function’, as this is
not applicable in HLHS; ‘elevated lung resistance’, as this is not
routinely measured in patients before the initial palliation, and
‘surgical factors’, as these were not directly relevant or compar-
able.In total, 26 clinical perioperative factors were considered
for scoring counting between 0.5–4.0 points (theoretical
maximum score 39.0 in a single patient); 42 additional
comorbidities were also included counting between 0.5–4.0
points (theoretical maximum 43.0 points).
Follow-up
All patients were followed up until 1 year of age, with deaths
reported as occurring within four periods:
1. Early post stage I death: death at primary procedure or
within 30 days
2. Interstage death: all deaths between the initial and the
second procedure
3. Early post stage II death: death at second stage procedure or
within 30 days
4. Late post stage II death: death occurring from after 30 days
post second stage up to 1 year of age.
Statistics
Unadjusted bivariate comparisons were performed using
Student’s t tests and Fisher’s exact tests as appropriate. Thirty
day outcomes between the Norwood and hybrid groups were
compared after adjustment for Aristotle scores using multivari-
able logistic regression. Survival up to 1 year of age was com-
pared in two ways: (1) in unadjusted form via the log rank test
using Kaplan-Meier plots; (2) after adjustment for modified
Aristotle score via Cox proportional hazards regression. Data
were analysed using the Stata statistical software package (Stata
Statistical Software: Release 12. College Station, StataCorp LP,
Texas, USA).
RESULTS
Of the 138 patients with HLHS fulfilling the inclusion criteria,
111 underwent the Norwood procedure and 27 the hybrid pro-
cedure. The average age at operation was 6.0 days for the
Norwood group (median 4 days, range 0–56 days), and 5.3 days
for the hybrid group (median 4 days, range 0–19 days; p=0.68)
(figure 1A). Five of 27 hybrid procedures were performed on
day 1 of life. In each case a restrictive or intact interatrial
septum had been diagnosed antenatally; the hybrid procedure
was performed immediately after birth by caesarean section in
theatres and included surgical septectomy with a bypass time of
34–83 min and a circulatory arrest time of 4–12 min. Of the
remaining 22 hybrid patients eight required transcatheter atrial
septostomy, either at the primary procedure (two patients) or
subsequently (six patients, range 1–21 days postoperatively).
One of these had an atrial stent placed 53 days after the
hybrid procedure. Fourteen hybrid patients did not require
any intervention to enlarge the atrial communication. In
776 Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
contrast, one of 111 Norwood procedures (19% vs 1%;
p<0.001) was performed on day 1 of life (again due to
restrictive atrial physiology). A modified BT shunt of diam-
eter 3.5–4.0 mm was used in all Norwood cases (3.5 mm:
n=87; 4mm: n=34). One of them underwent atrial septal
stenting 89 days after the Norwood operation.
Extracorporeal membrane oxygenation (ECMO) was not uti-
lised on any patient postoperatively.
The modified comprehensive Aristotle score was calculated
for each patient retrospectively. All categories in which more
than one patient from either group attained a score are docu-
mented in table 1.
The mean Aristotle score was 1.8 (median 1, range 0–12.5)
for the Norwood group, and 4.1 (median 4, range 0–11) for
the hybrid group. Figure 1B demonstrates the range of scores
obtained in each group and the number of patients achieving
each score. There was a significantly higher mean modified com-
prehensive Aristotle score in the patients in the hybrid group
compared with the Norwood group (p<0.001).
Early and interstage mortality
Neither unadjusted nor Aristotle adjusted mortality differed
between the groups for early (<30 days) or interstage mortality.
Of the 111 patients in the Norwood group, 31 (27.9%) died
within 30 days of surgery compared to 9/27 (33.3%) of the
patients in the hybrid group. The absolute early mortality risk
difference was 5.4% (95% CI −14.2% to +25.0%, p=0.64).
After adjustment for Aristotle score, the odds ratio (OR) for
early death among the hybrid patients was 1.08 (95% CI 0.41
to 2.84, p=0.87).
Similarly, the interstage death rate among Norwood and
hybrid patients was 10/80 (12.5%) and 3/18 (16.7%), respect-
ively, providing an absolute interstage mortality risk difference
of 4.2% (95% CI −14.5% to +22.8%, p=0.70). After adjust-
ment for Aristotle score, the OR for interstage death among
hybrid patients was 1.06 (95% CI 0.23 to 4.87, p=0.94).
One intraoperative death occurred in each group; both are
included in the above numbers. In the hybrid case this was one
of the five patients who underwent surgical septectomy
Figure 1 (A) Age (in days) of
patients in each group at the time of
their primary procedure. Three patients
in the Norwood group had operations
performed at >20 days of age: two
patients were diagnosed following
postnatal collapse at 34 and 56 days
respectively; one patient born at
32 weeks with a birth weight of
1.67 kg had primary palliation delayed
until day 52 of life. (B) The number of
patients in each group attracting
specific modified comprehensive
Aristotle scores. *Forty-three patients
in the Norwood group attracted a
score of zero (not charted).
Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759 777
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
immediately after birth. Two of the remaining four hybrid
patients who underwent this approach died before 3 days of
age, with two surviving to Fontan completion.
Interstage interventions
Additional interventions were performed in six patients in the
hybrid group (22%) and six patients in the Norwood group
(5%). One Norwood patient required transcatheter balloon dila-
tion of the left pulmonary artery, 14 days postoperatively before
stage II, and one hybrid patient underwent stenting of the left
and right pulmonary artery at 180 days postoperatively, before
combined stage I and II. Three Norwood patients had stents
placed in stenosed BT shunts at 17, 20, and 58 days after
surgery, and one patient who had an additional classical BT
shunt on the first postoperative day needed stenting of the
innominate artery on day 114 after Norwood. Four hybrid
patients required transcatheter re-stenting of the arterial duct
due to restenosis either at the proximal or distal end, at 21 days
after the hybrid procedure (two patients), 91 days after the pro-
cedure, or due to stent migration on day 3 after the hybrid pro-
cedure. One patient of each group received an interatrial stent
as described above.
Early stage 2 survival
Three of 70 (4.3%) Norwood patients who underwent stage II
died within 30 days of the procedure compared to two of the
15 (13.3%) hybrid patients who underwent the combined stage
I and II procedure, yielding a risk difference of 9.0% (95%
CI −8.8% to +26.9%, p=0.22).
A Kaplan–Meier diagram depicting survival for both groups is
shown in figure 2. The overall 1 year survival for the Norwood
and hybrid groups was 58.6% and 51.9%, respectively, giving a
risk difference of 6.7% (95% CI −14.3% to +27.7%, p=0.47,
log rank test). This lack of difference persisted in the Cox pro-
portional hazards analysis after adjustment for Aristotle score,
yielding a hybrid HR for mortality of 1.09 (95% CI 0.56 to
2.11, p=0.80).
Survival to Fontan procedure
Fifty-three Norwood and eight hybrid patients underwent
Fontan completion. There was no mortality after this operation.
Eight Norwood and six hybrid patients are awaiting Fontan
completion.
Subgroup analysis: high Aristotle score
As a series of sensitivity analyses, we recalculated the early and
late risk differences on the subgroup of patients with the highest
modified comprehensive Aristotle scores only (those in the top
quartile, with a score ≥3.5), encompassing the Norwood
(n=22) and hybrid (n=15) groups. This did not yield any sys-
tematic differences in mortality. The 30 day mortality rate for
the Norwood and hybrid subgroups was 36.4% and 40%,
respectively, yielding an absolute early mortality risk difference
of 3.6% (95% CI −28.2% to +35.5%, p=1.0). Similarly, the
1 year mortality rate for the Norwood and hybrid groups was
50.0% and 53%, respectively, yielding an absolute early mortal-
ity risk difference of 3.3% (95% CI −29.4% to +36.1, p=1.0).
DISCUSSION
The specific risks associated with circulatory arrest, cardiopul-
monary bypass and cardiotomy are obviated by the use of the
hybrid procedure instead of the traditional Norwood proced-
ure.
78
In unstable patients with significant comorbidities, the
hybrid procedure potentially offers a lower risk alternative to
the Norwood procedure, and some units, including our own,
utilise the procedure exclusively for such patients. This
approach appears to have merit. By using a modified compre-
hensive Aristotle score we have demonstrated that the outcomes
of the Norwood and hybrid procedures in patients with HLHS
were comparable, despite the hybrid cohort having more pre-
operative risk factors, such as lower birth weight, and undergo-
ing earlier intervention.
Our data add to that growing body of encouraging results
from the small number of centres offering the hybrid proced-
ure.
713
However, there continues to be wide variation between
centres both in terms of patient selection and the precise tech-
nique used. While the patients in the hybrid group in our series
were of significantly lower birth weight and higher risk overall,
our data show no clear ‘cut-off ’Aristotle score or other pre-
operative risk factor which could be directly associated with
Table 1 Frequency of patient characteristics attracting Aristotle
scores
Norwood Hybrid
Preoperative variable Score n % n % p Value
Birth weight <2.5 kg 2 11 10 7 27 <0.001
Gestation 32–35 weeks 2 3314 1.000
Renal dysfunction 1 23 20 11 41 0.045
Respiratory failure 2 28 25 7 26 1.000
Liver dysfunction 1 15 14 6 22 0.248
Shock (resolved) 1 12 11 5 19 0.326
Shock ( persistent) 3 6 5 4 15 0.105
NEC (medical treatment) 1 9 8 4 15 0.087
Platelets <100×10
9
/L 1 00270.047
Seizures (48 h) 1 2227 0.172
Sepsis 3 4427 0.411
CPR 2 1114 0.354
Genetic abnormality 0.5 4414 1.000
SVT/VT 1 6514 1.000
RSV 3 1100 1.000
In case of statistical significant differences, the p-values are printed in bold.
CPR, cardiopulmonary resuscitation; NEC, necrotising enterocolitis; RSV, respiratory
syncytial virus; SVT/VT, supraventricular tachycardia/ventricular tachycardia.
Figure 2 Kaplan–Meier diagram showing the survival until 1 year. All
patients who survived until 1 year had received conversion to a partial
cavopulmonary anastomosis.
778 Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
improved outcome; hence the hybrid procedure must continue
to be compared with the contemporary outcomes of the
Norwood procedure and its modifications.
In our series, total mortality before stage II in both the
Norwood and hybrid cohort was high (37% and 44%, respect-
ively). We are a quaternary referral centre covering a large geo-
graphical area, and results for the Norwood procedure in our
centre from 2007–2010 are comparable to those of other UK
centres
14
(data for the hybrid procedure are not routinely col-
lected). Other international reports have comparable outcomes
for the Norwood procedure, although there are differences in
the 30 day mortality and the further interstage mortality.
15
A
series from Kiel, Germany showed low perioperative mortality,
but overall interstage mortality comparable to our series.
16
Results reported by the US Paediatric Heart Network showed a
similar mortality rate to that presented in our study.
5
Our
hybrid mortality rate is comparable to this unselected cohort of
patients undergoing the Norwood procedure in these reports;
however, it should be noted that the hybrid may be performed
differently in other centres. For example, the stenting of the
arterial duct can be performed percutaneously
17
or via direct
pulmonary artery access as in our centre. The use of a ‘reversed’
BT shunt to address the issue of retrograde aortic arch malperfu-
sion has also been described.
18
While it is clear that patients undergoing surgery for HLHS
have an important interstage mortality risk, the precise reasons
for this are unclear,
1
and perioperative management varies con-
siderably between centres. The recent introduction of interstage
home surveillance programmes for infants with HLHS has
shown that regular monitoring of somatic growth, transcutane-
ous oxygen saturations, and earlier timing of intervention can
reduce the total interstage mortality.
19
During the study period,
we did not have such a monitoring programme in place. This
practice is currently under review.
An alternative approach to primary palliation of HLHS, con-
sisting of pulmonary arterial banding with medical support of the
arterial duct with intravenous prostaglandin and later conversion
to Norwood, was not yet published during our study period and
has not been adopted in our institution.
20
However, owing to the
complexity of the combined stage I and II, and to reduce the pro-
blems associated with branch pulmonary artery stenosis, we have
recently changed our management strategy to an intermediate
stage I operation beyond the neonatal period following the
hybrid procedure. Analysis of the implications of this approach is
ongoing and does not apply to the cohort in this paper.
Following the hybrid procedure without subsequent conver-
sion into stage I, a more complex second stage is required, in
which the reconstruction of ventricular outflow, usually per-
formed during the Norwood procedure, is performed simultan-
eously with superior cavopulmonary anastomosis (combined
stage I and II). While the numbers in our series were small, we
could show no appreciable difference in early mortality beyond
the stage II procedure, in keeping with other published data.
21
The prophylactic use of mechanical ventricular assistance
devices (VADs) after stage I palliation is controversial
22
and is
not offered in our centre. The use of postoperative ECMO has
been associated with an increased mortality and morbidity.
23
This may be due in part to ECMO being generally reserved for
use as a ‘bridge to recovery’in unstable patients following
surgery, as is the policy in our centre. In our series there was
one intraoperative death in each group; however, all other
patients were successfully weaned off bypass (if used) in the
operating theatre, and no patients required postoperative
ECMO.
The treatment of HLHS with primary orthotopic heart trans-
plantation is not routinely offered in the UK, not least due to
the extreme scarcity of suitable donor organs in this age group.
It remains an option for patients in whom surgical palliation has
failed.
24
At the time of publication, only one of our 111
Norwood patients has undergone cardiac transplantation
8 months after stage II.
Finally, in long term survivors of HLHS the burden of long
term neurodevelopmental deficits is high. The association
between congenital heart disease and abnormal structural
25
and
functional
26
brain development is well described and multifac-
torial; however, cardiopulmonary bypass with deep hypother-
mic circulatory arrest during surgery has been strongly
implicated.
27
There is evidence to suggest that children with
congenital heart disease may have a unique vulnerability in the
neonatal period.
28
To date, no studies have examined the poten-
tial advantages of postponing the first bypass-dependent stage
from the early neonatal period to later in infancy.
STUDY LIMITATIONS
This is a retrospective single institution study. Nevertheless, the
number of patients allowed an appropriate statistical comparison
of patients undergoing either hybrid or conventional Norwood
procedures using the modified comprehensive Aristotle score.
Alternative scoring systems such as RACHS were not felt to be
appropriate, as the procedure independent variables are
reflected in greater detail in the comprehensive Aristotle score.
CONCLUSION
A hybrid approach to high risk patients with HLHS produces
comparable early and 1 year mortality risks to lower risk
patients undergoing the Norwood procedure. Prospective
studies are needed to establish whether the hybrid procedure is
a viable alternative to the Norwood in all HLHS patients, in
terms of both mortality and long term morbidity.
Key messages
What is already known on this subject?
Hybrid treatment for neonates with hypoplastic left heart (HLHS)
is offered as an alternative first step of staged surgery for high
risk patients. The comprehensive Aristotle score was designed
for risk stratification for children undergoing surgery for
congenital heart disease.
How might this impact on clinical practice?
Applying a hybrid approach to high risk patients (as estimated
with the comprehensive Aristotle score) with HLHS produces a
comparable early and interstage mortality risk to lower risk
patients undergoing the Norwood procedure.
How might this impact on clinical practice?
The hybrid procedure might be an alternative treatment option
not only for high risk patients, but for unselected neonates with
HLHS.
Competing interests None.
Ethics approval Institutional board of the Evelina Children’s Hospital (ECH),
London, on the 15th October 2009.
Provenance and peer review Not commissioned; externally peer reviewed.
Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759 779
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
REFERENCES
1 Ohye RG, Schonbeck JV, Eghtesady P, et al. Cause, timing, and location of death in
the Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg
2012;144:907–14.
2 Krasemann T, Fenge H, Kehl H-G, et al. A decade of staged Norwood palliation in
hypoplastic left heart syndrome in a midsized cardiosurgical center. Pediatr Cardiol
2005;26:751–5.
3 Gelehrter S, Fifer CG, Armstrong A, et al. Outcomes of hypoplastic left heart
syndrome in low-birth-weight patients. Pediatr Cardiol 2011;32:1175–81.
4 AlsoufiB, Manlhiot C, Al-Ahmadi M, et al. Older children at the time of the
Norwood operation have ongoing mortality vulnerability that continues after
cavopulmonary connection. J Thorac Cardiovasc Surg 2011;142:142–7.
5 Ohye RG, Sleeper LA, Mahony L, et al. Comparison of shunt types in the Norwood
procedure for single-ventricle lesions. N Engl J Med 2010;362:1980–92.
6 Venugopal PS, Luna KP, Anderson DR, et al. Hybrid procedure as an alternative to
surgical palliation of high-risk infants with hypoplastic left heart syndrome and its
variants. J Thorac Cardiovasc Surg 2010;139:1211–15.
7 Galantowicz M, Cheatham JP, Phillips A, et al. Hybrid approach for hypoplastic left
heart syndrome: intermediate results after the learning curve. Ann Thorac Surg
2008;85:2063–70; discussion 2070–1.
8 Knirsch W, Liamlahi R, Hug MI, et al. Mortality and neurodevelopmental outcome
at 1 year of age comparing hybrid and Norwood procedures. Eur J Cardiothorac
Surg 2012;0:33–9.
9 Al-Radi OO, Harrell FE, Caldarone CA, et al. Case complexity scores in congenital
heart surgery: a comparative study of the Aristotle Basic Complexity score and the
Risk Adjustment in Congenital Heart Surgery (RACHS-1) system. J Thorac Cardiovasc
Surg 2007;133:865–75.
10 Bojan M, Gerelli S, Gioanni S, et al. Comparative study of the Aristotle
Comprehensive Complexity and the Risk Adjustment in Congenital Heart Surgery
scores. Ann Thorac Surg 2011;92:949–56.
11 Lacour-Gayet F, Clarke D, Jacobs J, et al. The Aristotle score: a complexity-adjusted
method to evaluate surgical results. Eur J Cardiothorac Surg 2004;25:911–24.
12 Chang YH, Lee JY, Kim JE, et al. The Aristotle score predicts mortality after surgery
of patent ductus arteriosus in preterm infants. Ann Thorac Surg 2013;96:879–84.
13 Mahle WT, Visconti KJ, Freier MC, et al. Relationship of surgical approach to
neurodevelopmental outcomes in hypoplastic left heart syndrome. Pediatrics
2006;117:e90–7.
14 NICOR Web Portal [Internet]. National Institute for Cardiovascular Outcomes
Research; Congenital Heart Disease. [cited 2012 Oct 19]. https://nicor4.nicor.org.uk
15 Dean PN, McHugh KE, Conaway MR, et al. Effects of race, ethnicity, and gender on
surgical mortality in hypoplastic left heart syndrome. Pediatr Cardiol
2013;34:1829–36.
16 Furck AK, Uebing A, Hansen JH, et al. Outcome of the Norwood operation in
patients with hypoplastic left heart syndrome: a 12-year single-center survey.
J Thorac Cardiovasc Surg 2010;139:359–65.
17 Akintürk H, Michel-Behnke I, Valeske K, et al. Hybrid transcatheter-surgical
palliation: basis for univentricular or biventricular repair: the Giessen experience.
Pediatr Cardiol 2007;28:79–87.
18 Baba K, Honjo O, Chaturvedi R, et al.“Reverse Blalock-Taussig shunt”: application
in single ventricle hybrid palliation. J Thorac Cardiovasc Surg 2013;146:352–7.
19 Hansen JH, Furck AK, Petko C, et al. Use of surveillance criteria reduces interstage
mortality after the Norwood operation for hypoplastic left heart syndrome. Eur J
Cardiothorac Surg 2012;41:1013–18.
20 Sakamoto T, Harada Y, Kosaka Y, et al. Second-stage palliation after bilateral
pulmonary artery bands for HLHS and its variants--which is better, modified
Norwood or Norwood plus bidirectional Glenn? World J Pediatr Congenit Heart
Surg 2011;2:558–65.
21 Baba K, Kotani Y, Chetan D, et al. Hybrid versus Norwood strategies for
single-ventricle palliation. Circulation 2012;126(11 Suppl 1):S123–31.
22 Rellensmann G, Krasemann T, Kehl H-G. Should all stage-one Norwood patients
receive a prolonged period of postoperative mechanical circulatory support? Ann
Thorac Surg 2005;79:1098–9.
23 Sherwin ED, Gauvreau K, Scheurer MA, et al. Extracorporeal membrane oxygenation
after stage 1 palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg
2012;144:1337–43.
24 Murtuza B, Dedieu N, Vazquez A, et al. Results of orthotopic heart transplantation
for failed palliation of hypoplastic left heart. Eur J Cardiothorac Surg
2013;43:597–603.
25 McQuillen PS, Goff DA, Licht DJ. Effects of congenital heart disease on brain
development. Prog Pediatr Cardiol 2010;29:79–85.
26 Herberg U, Hövels-Gürich H. Neurological and psychomotor development of
foetuses and children with congenital heart disease--causes and prevalence of
disorders and long-term prognosis. Z Geburtshilfe Neonatol 2012;216:132–40.
27 Hirsch JC, Jacobs ML, Andropoulos D, et al. Protecting the infant brain during
cardiac surgery: a systematic review. Ann Thorac Surg 2012;94:1365–73.
28 Goff DA, Luan X, Gerdes M, et al. Younger gestational age is associated with
worse neurodevelopmental outcomes after cardiac surgery in infancy. J Thorac
Cardiovasc Surg 2012;143:535–42.
780 Lloyd DFA, et al.Heart 2014;100:775–780. doi:10.1136/heartjnl-2013-304759
Congenital heart disease
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from
syndrome using the Aristotle scoring system
procedures for hypoplastic left heart
interstage mortality in Norwood and hybrid
Analysis of preoperative condition and
Hannah Bellsham-Revell and Thomas Krasemann
Shakeel Ahmed Qureshi, Eric Rosenthal, David Anderson, Conal Austin,
David F A Lloyd, Lindsay Cutler, Shane M Tibby, Sunitha Vimalesvaran,
doi: 10.1136/heartjnl-2013-304759
2014 100: 775-780 originally published online January 10, 2014Heart
http://heart.bmj.com/content/100/10/775
Updated information and services can be found at:
These include:
References #BIBLhttp://heart.bmj.com/content/100/10/775
This article cites 26 articles, 2 of which you can access for free at:
service
Email alerting box at the top right corner of the online article.
Receive free email alerts when new articles cite this article. Sign up in the
Collections
Topic Articles on similar topics can be found in the following collections
(762)Congenital heart disease
(3752)Epidemiology (100)Congenital heart disease in adult patients
Notes
http://group.bmj.com/group/rights-licensing/permissions
To request permissions go to:
http://journals.bmj.com/cgi/reprintform
To order reprints go to:
http://group.bmj.com/subscribe/
To subscribe to BMJ go to:
group.bmj.com on August 15, 2017 - Published by http://heart.bmj.com/Downloaded from