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Analysis of Preoperative Condition and Interstage Mortality in Norwood and Hybrid Procedures for Hypoplastic Left Heart Syndrome Using the Aristotle Scoring System

Authors:
  • Sophia Kinderziekenhuis, Erasmus Medical Centre Rotterdam

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Background: 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 (CPB) and circulatory arrest. In some centers, it is reserved for low-birth-weight, premature, or unstable neonates. Its role in unselected cases of HLHS is 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 and 2011 was analyzed retrospectively, using a modified comprehensive Aristotle score (AS). We then compared the early (< 30 days) and interstage mortality for each cohort. Results: Of 138 patients with HLHS, 27 had hybrid and 111 had Norwood procedures. The hybrid group had significantly higher scores (mean AS: 4.1 vs. 1.8; p < 0.001); however, there was no significant difference in the early postoperative mortality (< 30 days, 33 vs. 28%; p= 0.64) or overall interstage mortality (44 vs. 37%, p= 0.51). Conclusions: The hybrid approach is a reasonable alternative for primary palliation of HLHS in higher risk patients, with comparable mortality to the Norwood procedure performed on a lower risk cohort. More prospective work is needed to establish whether the hybrid offers benefits over the Norwood procedure in unselected HLHS patients, and whether more complex outcomes linked to CPB, such as neurodevelopmental status, could be improved by adopting this approach.
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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 Childrens
Hospital, Guys & St Thomas
NHS Foundation Trust, London,
UK
Correspondence to
Dr Thomas Krasemann, Evelina
London Childrens Hospital,
Department of Paediatric
Cardiology, Guys & 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;100:775780.
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 dened.
Methods The preoperative condition of all patients
with HLHS who underwent either the hybrid or the
Norwood procedure for HLHS between 20052011 was
analysed retrospectively, using a modied 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
signicantly higher Aristotle scores (mean 4.1 vs 1.8;
p<0.001); however, there was no signicant 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 rst stage operation to
palliate hypoplastic left heart syndrome (HLHS) is
>70%, although there remains signicant 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.
24
Survival rates are independent of the type of pul-
monary shunt used.
5
The obligatory use of cardio-
pulmonary bypass may be a signicant contributing
factor to adverse outcomes.
In view of these concerns, an off-pump hybrid
procedurecombining 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 nally 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 modied 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
68
; however, objective comparison has been
difcult 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 modied comprehen-
sive Aristotle score.
METHODS
The study was approved as an audit by the institu-
tional board of the Evelina Childrens Hospital
Lloyd DFA, et al.Heart 2014;100:775780. doi:10.1136/heartjnl-2013-304759 775
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(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 identied 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 modied BT shunt (the Sanomodication 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 polytetrauoroethyl-
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 12 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
inow occlusion but no cardioplegic arrest.
6
A total of 139 patients fullled 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 modied 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 1.515) for
the procedure being undertaken, based on the anticipated
technical difculty 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 specic, procedure independentcharacteristics, 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 modied 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 0.54.0 points (theoretical
maximum score 39.0 in a single patient); 42 additional
comorbidities were also included counting between 0.54.0
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
Students t tests and Fishers 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 modied
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 fullling 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 056 days), and 5.3 days
for the hybrid group (median 4 days, range 019 days; p=0.68)
(gure 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
3483 min and a circulatory arrest time of 412 min. Of the
remaining 22 hybrid patients eight required transcatheter atrial
septostomy, either at the primary procedure (two patients) or
subsequently (six patients, range 121 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;100:775780. doi:10.1136/heartjnl-2013-304759
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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 modied BT shunt of diam-
eter 3.54.0 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 modied 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 012.5)
for the Norwood group, and 4.1 (median 4, range 011) 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 signicantly higher mean modied 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 ve 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
specic modied comprehensive
Aristotle scores. *Forty-three patients
in the Norwood group attracted a
score of zero (not charted).
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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 rst 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 KaplanMeier diagram depicting survival for both groups is
shown in gure 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
modied 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 specic 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 signicant 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 modied 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 signicantly 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 3235 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 KaplanMeier 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;100:775780. doi:10.1136/heartjnl-2013-304759
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improved outcome; hence the hybrid procedure must continue
to be compared with the contemporary outcomes of the
Norwood procedure and its modications.
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 20072010 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 outow, 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 recoveryin 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 decits 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 rst 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 modied comprehensive Aristotle score.
Alternative scoring systems such as RACHS were not felt to be
appropriate, as the procedure independent variables are
reected 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 rst step of staged surgery for high
risk patients. The comprehensive Aristotle score was designed
for risk stratication 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 Childrens Hospital (ECH),
London, on the 15th October 2009.
Provenance and peer review Not commissioned; externally peer reviewed.
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780 Lloyd DFA, et al.Heart 2014;100:775780. 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
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... Low BW is a risk factor for S1P, with technical and physiological challenges [222]. Comorbidities from other organ systems (central nervous system, renal, gastrointestinal) increase the risk in both the short and long term [223][224][225]. Multi-institutional studies have demonstrated increased mortality rates for low-BW infants with HLHS undergoing S1P, through IS-1 [54]. ...
... Multi-institutional studies have demonstrated increased mortality rates for low-BW infants with HLHS undergoing S1P, through IS-1 [54]. From a worldwide perspective, several strategies are currently utilized for premature infants with HLHS including compassionate care only, early Norwood procedure, h-S1P for bridging to Norwood, a heart transplant and a hybrid variant consisting of b-PAB with continuous PGE1 infusion [as a bridge to later Norwood or comprehensive S2P (c-S2P)] [199,200,[224][225][226][227]. In preterm newborns the lowest effective dosage of continuous PGE1 infusion should be used (<5 ng/kg/min); in most cases, 1-2 ng/kg/min is sufficient, with or without additional oral caffeine supplementation [211]. ...
... Nevertheless, there is class IIa (level C) evidence that use of the h-S1P (i.e. avoiding CPB) in premature infants may reduce the otherwise increased mortality and morbidity rates [225,358]. ...
Article
The IPCCC is a standardized international system of paediatric and congenital cardiac nomenclature that should be used in all registries, databases and research studies in the domain of paediatric and congenital cardiac care, including those related to HLHS. This recommendation is level 1 class C [15, 16, 700]. Multi-institutional databases and registries allow benchmarking of data concerning death, patterns of practice, morbidity and postoperative length of stay [540]. As a level 1 class C recommendation, all paediatric and congenital cardiac teams should routinely assess their own data against national and international benchmarks using multi-institutional databases and registries [540, 697–699, 701]. © 2020 European Association for Cardio-Thoracic Surgery. All rights reserved.
... and RV FAC < 35% had good sensitivity and specificity for poor outcome (AUCs of 0.71 and 0.73, respectively). These prior studies also did not find significant differences in conventional RV functional parameters when stratified [12][13][14]. ...
... (12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31) Content courtesy of Springer Nature, terms of use apply. Rights reserved. ...
Article
Full-text available
Cardiac dysfunction is associated with mortality in children with hypoplastic left heart syndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study, echocardiograms from 3 timepoints (pre-stage 1 palliation, 4–8 weeks post-stage 1 palliation, and pre-Glenn) were analyzed in infants with HLHS. Patients were stratified into two groups based on outcome of transplant-free survival post-Glenn (survivors) versus mortality or transplantation prior to Fontan (non-survivors). Images were retrospectively reviewed to obtain RV global longitudinal strain (RVGLS), RV-free wall strain (RVFWS), fractional area change (FAC), tricuspid annular systolic plane excursion (TAPSE), tissue motion annular displacement of the tricuspid valve (TMAD-TV) and qualitative systolic function assessment during the predetermined timepoints. An equal variance t-test and chi-square were used to determine significant differences and ROC curve analysis was performed to derive optimal cutoff values to predict mortality/transplant. A total of 47 patients met inclusion criteria, of which, 21 patients met composite endpoint. There were no significant differences in any RV functional parameter during the pre- or post-stage 1 palliation timepoints. The absolute values of RVFWS, RVGLS, and TMAD-TV were significantly greater in survivors than non-survivors during the pre-Glenn timepoint. A pre-Glenn RVGLS > −15.6 (AUC 0.79), RVFWS > −18.6 (AUC 0.75), and TMAD-TV < 12.6% (AUC 0.82) were sensitive and specific for predicting death or need for transplantation prior to Fontan completion. RVGLS, RVFWS, and TMAD-TV may help identify higher-risk HLHS patients during the interstage period.
... [1][2][3][4][5] Several reports have analyzed different management strategies, including Norwood 6 and hybrid stage 1 palliation comprising pulmonary artery (PA) banding and continuous prostaglandin infusion or ductus arteriosus stenting. [7][8][9] Nevertheless, considerable debate exists regarding which approach offers better outcomes. 10 We sought to explore treatment approaches in the care of high-risk HLHS infants across the United States as well as short-and long-term outcomes for different interventions and the role of comfort care. ...
Article
Background: Hypoplastic left heart syndrome with low birth weight or prematurity comprises a high-risk population with no optimal treatment pathway. Using the Pediatric Health Information System, we compared management approaches across the United States. Methods: We analyzed neonates (≤30 days) with birth weight <2500 grams or gestational age <36 weeks between 2012 and 2021. Four strategies were identified: Norwood procedure, ductus arteriosus stent + pulmonary artery banding, pulmonary artery banding + prostaglandin infusion, or comfort care. Outcomes included hospital survival, discharge disposition, staged palliation completion, and one-year transplant-free survival. Results: Of 383 infants identified, 36.4% (134/383) received comfort care, 43.9% (165/383) Norwood, 12.4% (49/383) ductal stent + pulmonary artery bands, and 8.8% (34/383) pulmonary artery bands + prostaglandins. Neonates receiving comfort care had the lowest gestational age [35 weeks (31.5-37)] and birth weight [2.0kg (1.5-2.3)]; 24.6% (33/134) had chromosomal anomalies. Infants undergoing primary Norwood had the highest birth weight [2.4kg (2.2-2.5)] and gestational age [37 weeks (35-38)]. Two-thirds [66.1% (109/165)] underwent Glenn palliation versus 18.4% (9/49) receiving ductal stent + pulmonary artery band and 35.3% (12/34) with pulmonary artery band + prostaglandins. Only 11.3% (6/53) born <2kg survived to 1 year, all following Norwood. Primary Norwood yielded higher hospital and one-year transplant-free survival than hybrid strategies. Conclusions: Comfort care is routinely provided, particularly for infants with low birth weight, gestational age, or chromosomal anomalies. Primary Norwood offered the lowest hospital and one-year mortality and highest palliation completion rates; birth weight was the most important factor determining one-year survival.
... It was considered to be less intensive, thereby, being a better approach for neonates at high risk of developing ischemic sequelae. The data from some single-center studies has suggested the hybrid procedure as an alternative to the standard Norwood [41,42]. This has led to the adoption of the hybrid procedure as the primary approach in several centers irrespective of risk stratification, based on the reasoning that it postpones major surgery to an older age and avoids CPB insults in early life. ...
Article
Full-text available
Hypoplastic left heart syndrome is a constellation of malformations which result from the severe underdevelopment of any left-sided cardiac structures. Once considered to be universally fatal, the prognosis for this condition has tremendously improved over the past four decades since the work of William Norwood in the early 1980s. Today, a staged surgical approach is applied for palliating this distinctive cohort of patients, in which they undergo three operative procedures in the first 10 years of their life. Advancements in medical technologies, surgical techniques, and our growing experience in the management of HLHS have made survival into adulthood a possibility. Through this review, we present the different phases of the staged approach with primary focus on stage 1—its modifications, current technique, alternatives, and latest outcomes.
... A number of small single-center studies have reported early and midterm outcomes of HP in high-risk patients with variable results. [7][8][9][10][11][12][13] These studies have been limited by small sample size and lack of comparison of HP patients with similar high-risk NP patients. Two studies have made such a comparison and have demonstrated conflicting results, with the initial study showing no difference in early or midterm survival 7 and the more recent study suggesting an early survival benefit in HP patients that did not persist beyond the first few months of life. ...
Article
Background: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients. Methods: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups. Results: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02). Conclusions: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.
... Preoperative hemodynamic instability, moderate-to-severe dysfunction of the dominant ventricle and atrioventricular valve regurgitation, restrictive atrial septum and/or obstructed total anomalous pulmonary venous return classifies HLHS-newborns as high risk (3); further birth weight less of 2.6 kg as well as prematurity (<37 weeks). Considering a Norwood surgery in high-risk HLHS patients, the Aristotle score increases from an already high baseline value of 15 to 19 or even above 20 calculated points; latest is associated with a dramatic decrease of the post-surgical survival rate (3,4). Exact 20 years ago, Hakan Akintuerk performed in Giessen, Germany the first successful comprehensive stage II in a 5-month-old HLHS patient (5). ...
Article
Full-text available
Fetal hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease with a lethal prognosis without postnatal therapeutic intervention or surgery. The aim of this article is to give a brief overview of new findings in the field of prenatal diagnosis and the therapy of HLHS. As cardiac output in HLHS children depends on the right ventricle (RV), prenatal assessment of fetal RV function is of interest to predict poor functional RV status before the RV becomes the systemic ventricle. Prenatal cardiac interventions such as fetal aortic valvuloplasty and non-invasive procedures such as maternal hyperoxygenation seem to be promising treatment options but will need to be evaluated with regard to long-term outcomes. Novel approaches such as stem cell therapy or neuroprotection provide important clues about the complexity of the disease. New aspects in diagnostics and therapy of HLHS show the potential of a targeted prenatal treatment planning. This could be used to optimize parental counseling as well as pre- and postnatal management of affected children.
Article
Objective Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period. Design Retrospective analysis. Setting The Heart Center at Nationwide Children's Hospital, Columbus, Ohio. Patients Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution. Interventions All patients underwent hybrid stage 1 palliation. Outcome Measures Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality. Results Study group comprised 57 patients. Five patients only admitted once during the interstage period for scheduled cardiac catheterization were included in the no readmission group. Therefore, 43 patients (75%) had a total of 87 interstage readmissions. Fourteen patients had 15 major interstage adverse events accounting for 17% of total readmissions. Stroke (n = 1); sepsis (n = 1); pericardial effusion requiring drainage (n = 1); mesenteric ischemia (n = 1); shock (n = 1); and cardiac catheterization requiring intervention (n = 11)—ductal stent balloon angioplasty (n = 3), enlargement of atrial septal defect/stent placement (n = 3), retrograde aortic arch stenosis (n = 4). Thirty‐three readmissions were secondary to gastrointestinal/feeding issues; 15 cyanosis; 15 work of breathing; and 9 asymptomatic patients. Four patients suffered interstage deaths (7%). Five patients (9%) spent >30 days in the hospital during the interstage period. Of the 47 newborns (82%) discharged exclusively orally feeding, 74% remained all orally feeding throughout interstage period. No patient discharged with tube feedings learned to eat during the interstage period. Conclusion Interstage readmissions are common in the hybrid patient population. Seventeen percent were secondary to major adverse events. Interstage mortality was 7%. Future studies to identify interventions aimed at decreasing feeding issues and viral bronchiolitis in this tenuous patient population will hopefully improve quality outcomes, reduce readmissions, and lessen health care costs.
Article
Full-text available
Objective To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales. Methods The UK’s national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code. Results There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%). 44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2. The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive. Conclusion Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.
Article
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Information is limited regarding the effect of race, ethnicity, and gender on the outcomes of the three palliative procedures for hypoplastic left heart syndrome (HLHS). This study examined the effects of race, ethnicity, gender, type of admission, and surgical volume on in-hospital mortality associated with palliative procedures for HLHS between 1998 and 2007 using data from the University HealthSystem Consortium. According to the data, 1,949 patients underwent stage 1 palliation (S1P) with a mortality rate of 29 %, 1,279 patients underwent stage 2 palliations (S2P) with a mortality rate of 5.4 %, and 1,084 patients underwent stage 3 palliation (S3P) with a mortality rate of 4.1 %. The risk factors for increased mortality with S1P were black and "other" race, smaller surgical volume, and early surgical era. The only risk factors for increased mortality with S2P were black race (11 % mortality; odds ratio [OR], 3.19; 95 % confidence interval [CI] 1.69-6.02) and Hispanic ethnicity (11 % mortality; OR 3.30; 95 % CI 1.64-6.64). For S2P, no racial differences were seen in the top five surgical volume institutions, but racial differences were seen in the non-top-five surgical volume institutions. Mortality with S1P was significantly higher for patients discharged after birth (37 vs 24 %; p = 0.004), and blacks were more likely to be discharged after birth (12 vs 5 % for all other races; p < 0.001). No racial differences with S3P were observed. The risk factors for increased mortality at S1P were black and "other" race, smaller surgical volume, and early surgical era. The risk factors for increased in-hospital mortality with S2P were black race and Hispanic ethnicity.
Article
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Objective: Retrograde aortic arch malperfusion after ductal stenting can be life-threatening after univentricular hybrid palliation. Arch perfusion can be maintained with a main pulmonary artery to innominate artery shunt placed during the stage I procedure: a "reverse Blalock-Taussig shunt." Methods: A retrospective review of 37 infants who underwent hybrid palliation from January 2004 to March 2010 was performed. The infants were divided into 2 groups, those with (group I, n = 16) and those without (group II, n = 21) a reverse Blalock-Taussig shunt. Results: At the initial palliation, no differences were found in the demographics, systolic or diastolic pressures, or ventricular or atrioventricular valve function between the 2 groups. Group I had more infants with aortic atresia (P < .01) and smaller ascending aortas (P < .01). Before stage II, the retrograde aortic Doppler flow velocity increased in group I (P < .01) and was unchanged in group II. The reintervention rates before stage II were similar between the 2 groups. Before stage II, the ventricular end-diastolic pressure, left and right pulmonary artery pressures and diameters, and mixed venous and arterial saturations were similar between the 2 groups. The complication rates between the 2 groups were not significantly different, although a nonsignificant trend toward more neurologic complications was noted in group I. The Kaplan-Meier survival estimate at 1 year was similar between the 2 groups (63% for group I vs 71% for group II). Conclusions: The presence of a reverse Blalock-Taussig shunt was not associated with more adverse events than those without. Gradual retrograde arch obstruction occurs commonly in palliated infants with aortic atresia. A reverse Blalock-Taussig shunt might play an important role to address the potential of retrograde obstruction, augmenting arch blood flow.
Article
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Hybrid and Norwood strategies differ substantially in terms of stage II palliative procedures. We sought to compare these strategies with an emphasis on survival and reintervention after stage II and subsequent Fontan completion. Of 110 neonates with functionally single-ventricle physiology who underwent stage I palliation between 2004 and 2010, 75 (69%) infants (Norwood, n=43; hybrid, n=32) who subsequently underwent stage II palliation were studied. Survival and reintervention rates after stage II palliation, anatomic and physiologic variables at pre-Fontan assessment, and Fontan outcomes were compared between the groups. Predictors for reintervention were analyzed. Freedom from death/transplant after stage II palliation was equivalent between the groups (Norwood, 80.4% versus hybrid, 85.6% at 3 years, P=0.66). Hybrid patients had a higher pulmonary artery (PA) reintervention rate (P=0.003) and lower Nakata index at pre-Fontan evaluation (P=0.015). Aortic arch and atrioventricular valve reinterventions were not different between the groups. Ventricular end-diastolic pressure, mean PA pressure, and ventricular function were equivalent at pre-Fontan assessment. There were no deaths after Fontan completion in either group (Norwood, n=25, hybrid, n=14). Survival after stage II palliation and subsequent Fontan completion is equivalent between the groups. The hybrid group had a higher PA reintervention rate and smaller PA size. Both strategies achieved adequate physiology for Fontan completion. Evolution of the hybrid strategy requires refinement to provide optimal PA growth.
Article
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The Single Ventricle Reconstruction trial randomized 555 subjects with a single right ventricle undergoing the Norwood procedure at 15 North American centers to receive either a modified Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt. Results demonstrated a rate of death or cardiac transplantation by 12 months postrandomization of 36% for the modified Blalock-Taussig shunt and 26% for the right ventricle-to-pulmonary artery shunt, consistent with other publications. Despite this high mortality rate, little is known about the circumstances surrounding these deaths. There were 164 deaths within 12 months postrandomization. A committee adjudicated all deaths for cause and recorded the timing, location, and other factors for each event. The most common cause of death was cardiovascular (42%), followed by unknown cause (24%) and multisystem organ failure (7%). The median age at death for subjects dying during the 12 months was 1.6 months (interquartile range, 0.6 to 3.7 months), with the highest number of deaths occurring during hospitalization related to the Norwood procedure. The most common location of death was at a Single Ventricle Reconstruction trial hospital (74%), followed by home (13%). There were 29 sudden, unexpected deaths (18%), although in retrospect, 12 were preceded by a prodrome. In infants with a single right ventricle undergoing staged repair, the majority of deaths within 12 months of the procedure are due to cardiovascular causes, occur in a hospital, and within the first few months of life. Increased understanding of the circumstances surrounding the deaths of these single ventricle patients may reduce the high mortality rate.
Article
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Objectives: Previous studies have indicated that results for orthotopic heart transplantation (OHT) in patients with surgically palliated hypoplastic left heart (HLHS) are worse compared with patients with other forms of congenital heart disease (CHD) or acquired cardiomyopathy (CM) as well as those undergoing primary OHT for HLHS. In light of the decreasing donor pool for transplantation and increasing numbers of palliated HLHS patients with improving survival, we sought to review our results for OHT in surgically palliated HLHS patients and failing Glenn or Fontan circulations. Methods: We conducted a single centre, retrospective study of patients undergoing OHT from 2000 to 2011. Patients who were transplanted following any of the three stages of palliation were included. Indications for OHT were severe impairment of systemic right ventricular (RV) function with/without significant atrioventricular (AV) valve regurgitation or failure of Fontan physiology. The primary outcome of interest was survival; the secondary outcomes examined were the incidence of post-transplant RV failure and the need for extracorporeal membrane oxygenation (ECMO) support. Results: A total of 209 patients were transplanted during the study period. Of these, 16 were surgically palliated HLHS patients, 1 following Norwood I, 4 post-Fontan and 11 post-Glenn. Thirty-one patients had non-HLHS CHD and 154 patients had forms of acquired CM. Preoperative patient characteristics including age, weight and donor/recipient weight ratio were similar across groups, though the incidence of pulmonary hypertension (PHT) was higher in the CM group. Thirty-day survival was 100% in the palliated HLHS patients (vs 98.1% for the CM group), with 1- and 5-year Kaplan-Meier survivals of 100 and 87.5% (P = 0.393 vs CM; log-rank test). Intensive care unit stay was comparable with transplanted CM patients as was the incidence of RV failure and ECMO post-OHT. Conclusions: Our results suggest that good early and mid-term outcomes following OHT in surgically palliated HLHS are achievable. These findings have implications for the optimal strategy and timing for managing palliated patients with HLHS as well as for counselling parents and affected children.
Article
Outcomes after surgical ligation of patent ductus arteriosus (PDA) in preterm infants are often complicated by prematurity associated comorbidities. The Aristotle comprehensive complexity score (ACCS) has been proposed as a useful tool for complexity adjustment in the analysis of outcome after congenital heart surgery. The aims of this study were to define preoperative risk factors for mortality and to demonstrate the usefulness of ACCS to predict mortality after surgical ligation of PDA in the preterm. Included were 49 preterm babies (≤35 weeks of gestation) who had surgical ligation of PDA between May 2009 and July 2012. Median gestational age was 27.6 weeks (range, 23 to 35 weeks) and median birth weight was 1,040 g (range, 520 to 2,280 g). Median age at operation was 15 days (range, 4 to 44 days) and median weight was 1,120 g (range, 400 to 2,880 g). Initial oral ibuprofen was ineffective in 24 patients and contraindicated in 25. All surgical ligations were done at bedside in the neonatal intensive care unit. Preoperative clinical and laboratory profiles were reviewed and ACCS was derived. Eight of 49 patients (16.3%) died at a median of 14 days (range, 2 to 73 days) after PDA ligation. Patients who had contraindications for oral ibuprofen (odds ratio [OR] 8.94; p = 0.049), coagulopathy (OR 12.13; p = 0.025), renal dysfunction (OR 28.88; p = 0.003), intraventricular hemorrhage greater than grade II or seizure (OR 34.00; p = 0.002), and ACCS points (OR 29.594; p < 0.05) were significantly associated with an increased risk for mortality. Among the risk factors, ACCS showed the largest area under curve (0.991) by receiver-operating characteristic curve analysis. Optimal cutoff value of ACCS for mortality were 15 or greater, with sensitivity of 87.5%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 97.6%. The ACCS, especially for procedure-independent complexity factors, is a useful tool to predict mortality after ligation of PDA in preterm infants.
Article
Background. The purpose of this study was to evaluate the surgical outcomes and pulmonary artery (PA) development associated with a new strategy wherein the modified Norwood (N) procedure is performed at 1-2 months after bilateral pulmonary artery banding (PAB). Methods. Between January 2008 and February 2010, 16 patients underwent Norwood-type operation after previous bilateral PAB. For analysis, patients were divided into two groups. Group I (n = 11) underwent modified Norwood procedure with either right modified Blalock Taussig (RMBT) shunt (n = 4) or right ventricle to pulmonary artery (RV-PA) conduit (n = 7). Group II (n = 5) underwent Norwood procedure plus bidirectional Glenn anastomosis. Diagnoses were hypoplastic left heart syndrome in 6 and its variants in 10. Results. There was no surgical death and no late death. Pulmonary artery interventions were performed at the time of the Norwood procedure in 27% in Group I and in 100% in Group II (p < 0.05). Additional PA interventions were performed during the period of follow-up in 4 cases in Group I (36.4%), and in 4 cases in Group II (80.0%). Additional Blalock Taussig shunts were performed in 7 patients, resulting in significant increase in PA index. In all, four patients have reached total cavopulmonary connection, and one has undergone biventricular repair. Eight patients in Group I and one patient in II Group reached bidirectional Glenn anastomosis. In Gp II, two patients showed LPA narrowing or obstruction with PA index of 80 ± 12 mm(2)/m(2). Conclusions. Regarding the second-stage palliation after bilateral PAB, modified Norwood procedure with either RMBT or RV-PA conduit has some advantages compared with Norwood plus BDG with respect to subsequent pulmonary artery development. Additional BT shunt may contribute to PA development, even in the patients with Norwood procedure with RV-PA conduit.
Article
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