This document summarizes the outcomes of an emerging pediatric cardiac surgery program's experience with surgical palliation of hypoplastic left heart syndrome (HLHS) and related anomalies between 2010-2014. The program achieved an overall hospital survival rate of 81% by utilizing different surgical strategies including the Norwood procedure, hybrid procedure, and salvage hybrid-bridge-to-Norwood procedure based on individual patient factors. Cardiac comorbidities such as obstructed pulmonary venous return influenced the choice to use a hybrid or salvage strategy. The program's flexible approach and matching of surgical strategy to patient characteristics helped achieve outcomes comparable to benchmark data, despite being a new program.

1. Original Article
Achieving Benchmark Results for Neonatal
Palliation of Hypoplastic Left Heart
Syndrome and Related Anomalies in an
Emerging Program
Ali Dodge-Khatami, MD, PhD1
, William Z. Chancellor, MS1
,
Bhawna Gupta, PhD1
, Samantha R. Seals, PhD2
,
Makram R. Ebeid, MD3
, Sarosh P. Batlivala, MD3
,
Mary B. Taylor, MD3,4
, and Jorge D. Salazar, MD1
Abstract
Background: Results of surgical management of hypoplastic left heart syndrome (HLHS) and related anomalies are often
compared to published benchmark data which reflect the use of a variety of surgical and hybrid protocols. We report encouraging
results achieved in an emerging program, despite a learning curve at all care levels. Rather than relying on a single preferred
protocol, surgical management was based on matching surgical strategy to individual patient factors. Methods: From 2010 to
2014, a total of 47 consecutive patients with HLHS or related anomalies with ductal-dependent systemic circulation underwent
initial surgical palliation, including 30 Norwood stage I, 8 hybrid stage I, and 9 salvage-to-Norwood procedures. True hybrid
procedures entailed bilateral pulmonary artery banding and ductal stenting. In the salvage-to-Norwood strategy, ductal stenting
was withheld in favor of continued prostaglandin infusion in anticipation of a deferred Norwood procedure. Cardiac comor-
bidities (obstructed pulmonary venous return, poor ventricular function, and atrioventricular valve regurgitation) and noncardiac
comorbidities influenced the choice of treatment strategies and were analyzed as potential risk factors for extracorporeal
membrane oxygenation (ECMO) support or in-hospital mortality. Results: Overall hospital survival was 81% (Norwood 83.3%,
hybrid 88%, ‘‘salvage’’ 67%; P ¼ .4942). Extracorporeal membrane oxygenation support was used for eight (17%) patients with two
survivors. For cases with obstructed pulmonary venous return (n ¼ 10, 21%), management choices favored a hybrid or salvage
strategy (P ¼ .0026). Aortic atresia (n ¼ 22, 47%) was treated by a Norwood or salvage-to-Norwood. No cardiac, noncardiac, or
genetic comorbidities were identified as independent risk factors for ECMO or discharge mortality in a multivariable analysis.
Conclusions: Our emerging program achieved outcomes that compare favorably to published benchmark data with respect to
hospital survival. These results reflect rigorous interdisciplinary teamwork and a flexible approach to surgical palliation based on
matching surgical strategy to patient factors. With major associated cardiac/noncardiac comorbidity and antegrade coronary flow, a
true hybrid with ductal stenting was our preferred strategy. For high-risk situations such as aortic atresia with obstructed pulmonary
venous return, the salvage hybrid-bridge-to-Norwood strategy may help achieve survival albeit with increased resource utilization.
Keywords
CHD, hypoplastic left heart syndrome, Norwood operation, outcomes
Submitted December 07, 2014; Accepted May 12, 2015.
Introduction
Since the early 1980s, patients with hypoplastic left heart syn-
drome (HLHS) and related anomalies with ductal-dependent
systemic circulation1–3
have been offered surgical palliation
with the Norwood stage I operation in the first few days of life
with a systemic-to-pulmonary artery (PA) shunt or a right ven-
tricle–pulmonary artery (RV-PA) conduit.2,3
A decade later,
the true hybrid I palliation, consisting of bilateral PA banding
1
Division of Pediatric and Congenital Heart Surgery, The Children’s Heart
Center, The University of Mississippi Medical Center, Jackson, MS, USA
2
Center of Biostatistics and Bioinformatics, The University of Mississippi
Medical Center, Jackson, MS, USA
3
Division of Pediatric Cardiology, The Children’s Heart Center, The
University of Mississippi Medical Center, Jackson, MS, USA
4
Division of Pediatric Critical Care, The Children’s Heart Center, The
University of Mississippi Medical Center, Jackson, MS, USA
Corresponding Author:
Ali Dodge-Khatami, Division of Pediatric and Congenital Heart Surgery,
Children’s Heart Center, University of Mississippi Medical Center, 2500
North State Street, Room S345, Jackson, MS 39216, USA.
Email: adodgekhatami@umc.edu
World Journal for Pediatric and
Congenital Heart Surgery
2015, Vol. 6(3) 393-400
ª The Author(s) 2015
Reprints and permission:
sagepub.com/journalsPermissions.nav
DOI: 10.1177/2150135115589605
pch.sagepub.com

2. Abbreviations and Acronyms
BT Blalock-Taussig
CT computed tomography
ECMO extracorporeal membrane oxygenation
HLHS hypoplastic left heart syndrome
IAA interrupted aortic arch
ICU intensive care unit
RV right ventricle
RV-PA right ventricle–pulmonary artery
TAPVR total anomalous pulmonary venous return
TGA transposition of the great arteries
and interventional catheter stenting of the ductus arteriosus,
emerged as an alternative strategy to treat HLHS and other
anomalies with single-ventricle physiology with arch hypopla-
sia.4
However, the indications or ideal candidates to undergo
one or the other are still debated, and the outcomes are heavily
institution dependent with a fairly steep learning curve before
attaining expected benchmark results.5
Although performed
and described early on in the surgical experience to palliate
HLHS by William Norwood himself,6
initial bilateral PA band-
ing with maintenance of ductal patency by continuous prosta-
glandin infusion followed by a deferred Norwood operation
has somewhat been ignored, and only recently revived as an
alternative strategy in higher risk candidates.7
This approach
to buy time and gain patient stability is referred to in our prac-
tice as the ‘‘salvage’’ hybrid-bridge-to-Norwood, also coined
as the ‘‘rapid 2-stage Norwood I’’ by some.7
A new comprehensive Children’s Heart Center was launched
in April 2010 at the only children’s hospital in Mississippi.
The goal in serving the state’s population of over 3 million
was to establish a full-service program to meet the needs of
Mississippi’s families. Prior to 2010, congenital heart surgery
of low complexity was being performed in this hospital at a
rate of two to four cases monthly, without a resident congenital
heart surgeon, without dedicated pediatric cardiac anesthesia,
cardiac critical care, perfusion, intensive care nursing, or
respiratory therapy. Patients with congenital heart defects of
middle or high complexity were transferred to out-of-state
centers for surgical care. Given the advances in prenatal diag-
nosis and neonatal resuscitation, the number of potentially
treatable patients with HLHS and related anomalies with
ductal-dependent systemic circulation in need of some form
of palliation was increasing.
In April 2010, the congenital heart program was initiated by
J.D.S. A comprehensive program was formed quickly by estab-
lishing the essential elements and partnerships necessary, pat-
terned after successful centers worldwide. Key investments
included congenital heart–dedicated critical care and nursing,
operating rooms and surgical team, cardiac anesthesia, perfu-
sion, catheterization laboratory, in-house extracorporeal mem-
brane oxygenation (ECMO) program, respiratory therapy,
neonatal care and fetal center, imaging, transport, and biweekly
patient management conferences. Recruitment of a strong med-
ical codirector (M.B.T.) was of central importance, giving a
leadership structure based on equality and equal empowerment.
Investment in and reliance upon the expertise of nursing
quickly expanded the team and created a culture of excellence.
These systems took some time to put in place fully, requiring a
flexible strategy in terms of timing and surgical approach to
complex congenital heart defects, with a strong focus on not
exceeding the capacity of the team. Multidisciplinary rounds
are made on every patient, every day, with no exceptions.
With a newly assembled team finding its bearings in all the
related domains of neonatology, perinatal imaging, surgery,
pediatric cardiology, anesthesia, critical care, perfusion, and
nursing, an individualized approach to clinical decision making
for babies with HLHS and related anomalies was applied. This
included consideration of the Norwood stage I operation, a true
hybrid, or salvage hybrid-bridge-to-Norwood approach, in an
effort to optimize outcomes while the multidisciplinary team
approach evolved and while we mutually experienced our
program’s ‘‘learning curve.’’ We continually evaluated our
experience with these strategies which were tailored to each
patient’s anatomy and condition,8
rather than applying a ‘‘one
size fits all’’ approach. Herein we summarize our experience to
date, including results and resource utilization.
Patients and Methods
Between April 2010 and June 2014, a total of 47 consecutive
babies with HLHS and/or related anomalies with ductal-
dependent systemic circulation, who were born in our center
or transferred from elsewhere in the region, were treated at the
Children’s Heart Center of the University of Mississippi Med-
ical Center in Jackson, Mississippi. Our Institutional Review
Board approved this retrospective observational study. Ini-
tially, all patients with suspected ductal dependency of the
systemic circulation were placed on a continuous intravenous
prostaglandin infusion, and the cardiac diagnosis confirmed/
made by transthoracic echocardiography and routine screening
for intracranial malformations/bleeding and renal/abdominal
malformations made by ultrasound. Depending on the presence
or absence of concomitant cardiac or noncardiac comorbidity
and patient stability, all patients were discussed in our multidis-
ciplinary case conference, and the decision to proceed with a
surgical and/or hybrid palliation was reached on a case-by-
case basis. In rare instances, new findings or events on the day
of planned surgery resulted in reconsideration of the initial
plan and dictated an alternative strategy. Options included a
Norwood I operation (n ¼ 30; 27 RV-PA conduit [Sano] and
3 modified Blalock-Taussig [BT] shunts), true hybrid with ini-
tial bilateral PA banding in the operating room followed by
elective patent ductus arteriosus (PDA) stenting in the interven-
tional catheter laboratory (n ¼ 8), or salvage hybrid-bridge-to-
Norwood consisting of bilateral PA banding and continuous
prostaglandin infusion, followed by a deferred Norwood I
operation a few days later after achieving relative patient
stability (n ¼ 9). All patients had aortic arch hypoplasia and/
or obstruction with ductal-dependent systemic perfusion. Ven-
tricular morphology varied considerably, but in no case was
394 World Journal for Pediatric and Congenital Heart Surgery 6(3)

3. initial biventricular repair considered to be a feasible alterna-
tive to initial functionally univentricular palliation. Diagnoses
included HLHS (n ¼ 33), truncus arteriosus with a diminutive
right ventricle (RV) and interrupted aortic arch (IAA; n ¼ 2);
unbalanced common atrioventricular canal defect with arch
hypoplasia (n ¼ 3); Shone’s syndrome with IAA and RV dys-
function/dilatation (n ¼ 2); Shone’s syndrome with aortic arch
hypoplasia (n ¼ 2); D-transposition of the great arteries (TGA)
with a hypoplastic RV and aortic arch hypoplasia (n ¼ 2); tricus-
pid atresia with D-TGA, left ventricular outflow tract obstruction,
and aortic arch hypoplasia (n ¼ 2); and double-inlet left ventricle
with TGA and aortic arch hypoplasia (n ¼ 1). Associated cardiac
and noncardiac comorbidities are listed in Table 1 and were
included as variables in analysis of potential associations
between patient factors and end points of requirement of ECMO
support or hospital mortality (Table 1).
Following an institutional/surgeon-based preference, most
patients whom we considered to be ‘‘standard-risk’’ HLHS,
meaning without cardiac or noncardiac comorbidity, were pri-
marily considered for a Norwood I operation with an RV-PA
conduit (Sano shunt). Early in the program’s experience, before
standardizing the operative strategy to include the Sano (RV to
PA) shunt, three patients received a modified BT shunt as the
source of pulmonary blood flow. Due to incomplete diagnosis,
two patients assessed as ‘‘standard risk’’ and assigned to
undergo a primary Norwood I operation required a concomitant
total anomalous pulmonary venous return (TAPVR) repair
(n ¼ 1) or atrial septectomy for intact interatrial septum (n ¼ 1).
In other cases, factors that were identified before surgery and
were considered to place the patient in a higher risk category
included associated significant noncardiac or cardiac comor-
bidities, and the latter was most often accounted for by either
TAPVR or an intact atrial septum or highly restrictive intera-
trial communication (hereafter referred to as ‘‘obstructed
pulmonary venous return,’’ n ¼ 10). The presence of such
factors tended to direct the strategy toward a hybrid proce-
dure. Noncardiac and significant cardiac comorbidities are
specified in Table 1. For patients with antegrade coronary
flow, a true hybrid procedure with deployment of a ductal
stent was judged an acceptably safe initial palliation as a
bridge to cavopulmonary anastomosis. Of these patients, one
underwent TAPVR repair at the time of bilateral PA banding
and one underwent an atrial septectomy. For patients aortic
atresia and ductal-dependent coronary blood flow, a salvage
hybrid-bridge-to-Norwood strategy was preferred, whereby
the babies initially underwent surgical bilateral PA banding
with TAPVR repair (n ¼ 5) or atrial septectomy (n ¼ 1) and
were maintained on a continuous prostaglandin infusion.
These patients were managed in the intensive care unit (ICU)
until general patient stability and/or end-organ recovery
was reached, at which time PA debanding and a deferred
Norwood stage I operation were performed. Specifically, a
planned salvage hybrid was done in seven patients, and an
improvised salvage was required in two patients, after unex-
pected findings in the operating room, including severe
abdominal distension and suspected sepsis (in a baby with
Jacobsen’s syndrome which was ultimately discovered after
the deferred Norwood) and TAPVR, and another baby with
standard risk HLHS with hemo-pericardium and tamponade
upon sternal entry, in whom only bilateral PA bandings were
done. Among the planned hybrid-bridge-to-Norwood patients,
two had severe seizure disorders with either cerebral infarction
and/or subdural hematoma precluding the immediate use of
cardiopulmonary bypass and one had single-ventricle anatomy
with arch hypoplasia and associated TGA in whom the great
vessel arrangement precluded PDA stenting.
Statistical Methods
Values are expressed as means and standard deviations. Cate-
gorical predictors were compared using the chi-square or
Fisher’s exact tests, where appropriate. Continuous predictors
were compared using the one-way analysis of variance. Prob-
ability of ECMO support and in-hospital mortality was
modeled using logistic regression, and survival rates were com-
pared using the log-rank test. Hypotheses were tested at the .05
level of significance. Data were analyzed with SAS software,
version 9.3 (SAS Institute, Cary, North Carolina), and Kaplan-
Meier curves were produced using Stata statistical software,
release 13 (StataCorp, College Station, Texas).
Results
Mean age at surgery was 6.6 + 3.5 days, at a gestational age of
37.7 + 2.4 weeks, and was similar across groups based on pal-
liative strategy. Similarly, mean birth weight (3.0 + 0.6 kg)
and mean weight at surgery (3.1 + 0.7 kg) were comparable
among groups. Prematurity, defined as a gestational age at birth
of less than 37 weeks, was present in 23.4% (11 of 47) of the
patients, with prevalence of 20% (6 of 30) in those undergoing
a Norwood I operation, 37.5% (3 of 8) of those undergoing a
Table 1. Variables Analyzed With Respect to End Points of ECMO
Support and In-Hospital Mortality.
Noncardiac comorbidities (n ¼ 8)
Intestinal malrotation (4), congenital diaphragmatic hernia,
sepsis, anal atresia, hydronephrosis/renal dysfunction, vascular
ring, prematurity, and low birth weight (2.2 kg)
Genetic syndromes (n ¼ 5)
DiGeorge (3), Trisomy 21, and Jacobsen
Cardiac comorbidities
Preoperative mechanical circulatory support (n ¼ 1)
Moderate systemic ventricular dysfunction (n ¼ 2)
Total anomalous pulmonary venous return (n ¼ 7)
Restrictive interatrial septum (n ¼ 3)
Moderate semilunar/systemic atrioventricular valve
regurgitation (n ¼ 3)
Cardiac arrest (n ¼ 1)
Cardiac tamponade (n ¼ 1)
Aortic arch hypoplasia (2 mm)
Heterotaxia syndrome (n ¼ 1)
Cardiomyopathy (n ¼ 1)
Dodge-Khatami et al 395

4. true hybrid, and 22.2% (2 of 9) in the salvage group. Prenatal
diagnosis was available in 34% of cases. Patient characteristics
by treatment strategy are described in Table 2.
Extracorporeal membrane oxygenation support was
required postoperatively in 8 (17%) of 47 patients, varying
between 5 (19%) of 27 after Norwood/Sano, 1 (33%) of 3 after
Norwood/BT shunt, none after true hybrid, and 2 (22%) of 7
after salvage to Norwood (P ¼ .4638). Six (75%) of the eight
patients who required ECMO died before discharge, and six
(67%) of the nine total hospital deaths had been on ECMO sup-
port. Associated obstructed pulmonary venous return was asso-
ciated with choice of initial palliative strategy being either a
true hybrid (2 of 8; 25%) or a salvage hybrid-to-Norwood strat-
egy (6 of 9, 67%). That choice was also influenced by the occur-
rence of other noncardiac or cardiac comorbidities and by the
presence or absence of antegrade coronary blood flow (aortic
atresia). There was sufficient evidence to suggest an imbalance
of obstructed pulmonary veins between the surgical groups
(P ¼ .0026). Similarly, the presence of aortic atresia was imbal-
anced between the surgical groups (P ¼ .0494) and influenced
palliative strategy, being present in 48% (13 of 27) of patients
undergoing a Norwood/Sano, 33% (1 of 3) undergoing a
Norwood/BT shunt, only 13% in true hybrids (1 of 8), and 78%
(7 of 9) of those for whom the salvage pathway was selected.
Mean length of ICU stay was 27 + 34 days, and mean
length of hospital stay was 41 + 42 days for the entire cohort.
Length of ICU and hospital stays were significantly shorter for
those directly undergoing a Norwood I operation (mean 19 +
19 and 30 + 22 days, respectively), compared to that after a
true hybrid (mean 24 + 18 and 45 + 43 days, respectively)
or a salvage hybrid-bridge-to-Norwood (mean 59 + 61 and
77 + 69 days, respectively; P ¼ .005 and P ¼ .009, respec-
tively). In the salvage group, the interval between bilateral
PA banding and deferred Norwood was a mean of 14.3 +
10.5 days (range 1-31), which contributed to the comparatively
prolonged length of stay for this group.
Hospital survival for the entire patient group was 81%. In
patients who underwent a Norwood/Sano, hospital survival was
85% (23 of 27). Hospital survival was 67% (2 of 3) after a
Norwood/BT shunt. For the combined group of all primary
Norwood I patients, hospital survival was 83.3%. After a true
hybrid, hospital survival was 88% and 67% after salvage
hybrid-bridge-to-Norwood. There was no statistically signifi-
cant difference in hospital survival across treatment groups
(P ¼ .9478 and P ¼ .6335 for 30- and 60-day survival, respec-
tively; Figure 1). Mortality in the primary Norwood group
included 1 patient with HLHS, early in the program’s experi-
ence, with a BT shunt who initially had an uneventful course
and delayed sternal closure on postoperative day 2. The patient
subsequently arrested from acute shunt-related hypoxia required
ECMO support, underwent shunt revision, and was successfully
weaned from ECMO two days later with chest closure but finally
Table 2. Patient Characteristics Compared by Treatment Strategies.
All Patients,
N ¼ 47
Norwood With
Sano, n ¼ 27 (57%)
Norwood with BT,
n ¼ 3 (6%)
Hybrid,
n ¼ 8 (17%)
Salvage,
n ¼ 9 (19%) P Value
Age at surgery (days) 6.6 (3.5) 7.6 (3.1) 5.7 (1.5) 6 (4.9) 4.3 (2.7) .0823
Gestational age 37.7 (2.4) 38 (1.2) 38.7 (0.6) 38 (1.1) 36.3 (4.8) .2718
Birth weight 3 (0.6) 3.2 (0.5) 3.3 (0.4) 2.6 (0.4) 3 (0.8) .0861
Weight at surgery 3.1 (0.7) 3.1 (0.6) 3.2 (0.6) 2.6 (0.5) 3.3 (1) .0991
6-month survival Yes 35 (74%) 21 (78%) 2 (67%) 6 (75%) 6 (67%) .9058
No 12 (26%) 6 (22%) 1 (33%) 2 (25%) 3 (33%)
Hospital mortality Yes 9 (19%) 4 (15%) 1 (33%) 1 (13%) 3 (33%) .4942
No 38 (81%) 23 (85%) 2 (67%) 7 (88%) 6 (67%)
ECMO Yes 8 (17%) 5 (19%) 1 (33%) 0 (0%) 2 (22%) .4638
No 39 (83%) 22 (81%) 2 (67%) 8 (100%) 7 (78%)
Obstructed pulmonary venous return Yes 10 (21%) 2 (7%) 0 (0%) 2 (25%) 6 (67%) .0026
No 37 (79%) 25 (93%) 3 (100%) 6 (75%) 3 (33%)
AVVR Yes 6 (13%) 4 (15%) 0 (0%) 2 (25%) 0 (0%) .4897
No 41 (87%) 23 (85%) 3 (100%) 6 (75%) 9 (100%)
Aortic atresia Yes 22 (47%) 13 (48%) 1 (33%) 1 (13%) 7 (78%) .0494
No 25 (53%) 14 (52%) 2 (67%) 7 (88%) 2 (22%)
Noncardiac comorbidities Yes 8 (17%) 3 (11%) 0 (0%) 3 (38%) 2 (22%) .2487
No 39 (83%) 24 (89%) 3 (100%) 5 (63%) 7 (78%)
Genetic comorbidities Yes 7 (15%) 3 (11%) 0 (0%) 2 (25%) 2 (22%) .6966
No 40 (85%) 24 (89%) 3 (100%) 6 (75%) 7 (78%)
HLHS subtype AA/MA 12 (26%) 6 (22%) 0 (0%) 1 (13%) 5 (56%) .3206
AS/MS 7 (15%) 3 (11%) 0 (0%) 3 (38%) 1 (11%)
AA/MS 7 (15%) 6 (22%) 0 (0%) 0 (0%) 1 (11%)
AS/MA 1 (2%) 1 (4%) 0 (0%) 0 (0%) 0 (0%)
N/A 20 (43%) 11 (41%) 3 (100%) 4 (50%) 2 (22%)
Abbreviations: BT, Blalock-Taussig; ECMO, extracorporeal membrane oxygenation; AVVR, atrioventricular valve regurgitation; HLHS, hypoplastic left heart
syndrome; AA, aortic atresia; AS, aortic stenosis; MA, mitral atresia; MS, mitral stenosis; N/A, not applicable.
396 World Journal for Pediatric and Congenital Heart Surgery 6(3)

5. died from an acute airway obstruction on postoperative day 55.
Two deaths occurred in patients having HLHS with associ-
ated TAPVR (one with moderate to severe pulmonary valve
insufficiency requiring a Norwood/Sano with pulmonic valve
replacement with a 9 mm aortic homograft) and concomitant
TAPVR repair. Both of these required postoperative ECMO,
with inability to wean from severely diseased lungs. The two
other mortalities after a primary Norwood/Sano occurred
after uneventful surgeries, acute profound desaturations from
a plugged endotracheal tube in one and shunt insufficiency in
another, requiring chest compressions but leading to a down-
ward spiral which could not be salvaged, despite ECMO sup-
port and/or shunt revision, respectively. After a true hybrid
procedure, one death occurred in a patient with Shone’s syn-
drome and IAA with RV dysfunction, in whom intrahepatic
bleeding and hypovolemic shock developed after accidental
PDA stent deployment in the hepatic veins. The two post-
Norwood deaths after salvage hybrid-bridge-to-Norwood
sequences occurred in babies with obstructed pulmonary
veins. In one case, the initial TAPVR repair and bilateral
PA bandings required postoperative ECMO support that was
continued up to the point of conversion from ECMO to con-
ventional cardiopulmonary bypass support at the time of the
Norwood operation. In the other case, initial salvage strategy
allowed stabilization and sternal closure, followed by a suc-
cessful Norwood/Sano three weeks later. The baby developed
intractable chylothorax, requiring thoracic duct ligation on
postoperative day 40. Multiple intracranial infarcts were dis-
covered on computed tomography (CT) scan, and care was
withdrawn at the request of the parents on postoperative
day 55.
Analysis of potential risk factors for end points of need for
ECMO or in-hospital mortality included consideration of
patient-related anatomic and demographic data, documented
noncardiac and cardiac comorbidities, and the treatment
strategy performed (Table 1). By univariate analysis, only
obstructed pulmonary venous return was associated with the
need for ECMO support (odds ratio 5.5, P ¼ .0412), while
obstructed pulmonary venous return and younger age at sur-
gery were associated with hospital mortality (odds ratio
8.25, P ¼ .0105 and odds ratio 0.67, P ¼ .0149, respectively;
Table 3). However, by multivariable analysis, neither proce-
dure type nor any cardiac or noncardiac comorbidity signifi-
cantly impacted the need for ECMO support or hospital
mortality (Table 4).
Outcomes at One Year and Relationship With Palliative
Strategy
After the Norwood stage I operation, there were five hospital
deaths and one interstage mortality. Twenty-two patients have
undergone a cavopulmonary anastomosis of which two died
after the Glenn and one is awaiting transplantation because
of poor ventricular function after right coronary artery throm-
bosis. One patient was converted to biventricular repair with
a Rastelli operation.
After the true hybrid procedure, there have been one
hospital mortality; two interstage deaths; one death after
repair of truncus arteriosus with concomitant PDA stent
removal, bilateral PA debanding, and IAA correction; and
four patients with a successful comprehensive second stage
reconstruction.
After the salvage hybrid approach, one mortality occurred
prior to the planned deferred Norwood from complete heart
block and asystole. Eight patients went on to a deferred Nor-
wood stage I at a mean of 14.3 + 10.5 days (range 1-31 days)
after bilateral PA banding of which six survived to hospital dis-
charge. As mentioned earlier, one patient experienced intract-
able chylothorax and eventual death following withdrawal of
care after multiple intracranial infarcts were discovered on
CT scan. Five patients have undergone a cavopulmonary
anastomosis, with one post-Glenn death in the previously men-
tioned patient with Jacobsen’s syndrome. There are four survi-
vors at one year following the initial salvage hybrid approach.
A program of interstage home monitoring was implemented in
March 2014, which we hope will have a positive impact on our
one-year outcomes.
Comment
Significant progress in the treatment of HLHS and related
anomalies with Norwood-type operations or hybrid strategies
has made a disease that was previously considered to be univer-
sally fatal in the newborn period, not only a manageable entity
but for which increasingly encouraging survival rates are
achieved at the highest performing centers.5
Any strategy to
palliate HLHS or related anomalies with ductal-dependent sys-
temic circulation is demanding in terms of human and material
resources. Indeed, the intensive care and hospital stay lengths
are among the longest in the care of any congenital heart defect,
including the occasional need for perioperative ECMO support,
a huge cost in itself.9,10
Accordingly, any aspiring congenital
heart program requires meticulous planning and support from
Figure 1. Probability of survival per treatment group at 60 days. BT
indicates Blalock-Taussig.
Dodge-Khatami et al 397

6. its hospital administration, when deciding to embark upon the
management of these most challenging CHD lesions.
Decisions concerning the choice of palliative strategy in the
newborn period depend on institutional preference, experience,
and/or expertise, with options including the Norwood stage I
procedure with either of two methods of providing and regulat-
ing pulmonary blood flow, a true hybrid procedure, or a salvage
hybrid-bridge-to-Norwood. The purpose of the current retro-
spective study was not to compare the three different strategies
but to describe our emerging program’s experience in surgical
palliation of HLHS and related anomalies, based on a policy of
trying to optimally match the choice of palliative strategy to the
individual patient. We recognized the importance of imposing
structure, discipline, repetition, and streamlining in the man-
agement process so that each member of the care team could
gain confidence and expertise, as exists in established centers
treating HLHS and related anomalies with Norwood operations
and hybrid palliations, with the goal of achieving benchmark
in-hospital survival outcomes. We feel that flexibility in indivi-
dualizing palliative strategies, rather than reliance on a single
approach for all, or nearly all patients, has enabled us to
achieve favorable results in a program that is relatively
‘‘young,’’ chronologically speaking.
As expertise with Norwood operations and hybrid pallia-
tions has increased so have expectations. A 2011 report on
variation in outcomes for benchmark operations in the Society
of Thoracic Surgeons Congenital Heart Surgery Database
revealed a multicenter aggregate discharge mortality rate of
19.3% among patients who underwent a Norwood operation
in 2005 to 2009.5
In the initial four years as an emerging con-
genital heart program selectively utilizing three treatment
strategies, we achieved a mortality rate of 19% in 47 consec-
utive patients, including some with multiple risk factors such
as associated cardiac and noncardiac comorbidities, genetic
Table 3. Univariate (Unadjusted) Associations.
Predictor
Hospital Mortality ECMO
Odds Ratio (95% CI) P Valuea
Odds Ratio (95% CI) P Valuea
Procedure (reference: ‘‘Salvage’’) Norwood: BT 1.00 (0.06-15.99) .5720 1.80 (0.11-29.07) .6738
Norwood: Sano 0.35 (0.06-1.99) 0.73 (0.12-4.36)
Hybrid 0.29 (0.02-3.52) 0.18 (0.01-5.19)
Obstructed pulmonary venous return 8.25 (1.64-41.55) .0105 5.5 (1.07-28.25) .0412
AVVR 2.43 (0.37-15.95) .3555 0.97 (0.1-9.65) .9804
Aortic atresia 2.75 (0.60-12.68) .1945 2.16 (0.45-10.32) .3357
Noncardiac comorbidities 0.55 (0.06-5.17) .6040 0.65 (0.07-6.19) .7104
Genetic comorbidities 0.67 (0.07-6.35) .7247 0.79 (0.08-7.6) .8350
Age at surgery 0.67 (0.48-0.92) .0149 0.75 (0.56-1.01) .0625
Weight at surgery 0.82 (0.27-2.45) .7153 1.03 (0.33-3.21) .9577
Birth weight 1.10 (0.32-3.81) .8834 1.65 (0.43-6.28) .4644
Gestational age 1.02 (0.73-1.42) .9067 1.01 (0.72-1.42) .9424
Abbreviations: ECMO, extracorporeal membrane oxygenation; AVVR, atrioventricular valve regurgitation; BT, Blalock-Taussig; CI, confidence interval; OR, odds
ratio.
a
P values are from the omnibus Wald chi-square test.
Table 4. Multivariable Associations.
Predictor
Hospital Mortality ECMO
Odds Ratio (95% CI) P Valuea
Odds Ratio (95% CI) P Valuea
Procedure (reference: ‘‘Salvage’’) Norwood: BT 0.14 (0.003-6.87) .6160 24.24 (0.18-3261.80) .3818
Norwood: Sano 3.80 (0.04-369.32) 11.43 (0.21-622.00)
Hybrid 1.35 (0.04-47.08) 0.16 (0.002-10.57)
Obstructed pulmonary veins 2.26 (0.12-41.08) .5814 7.98 (0.30-212.56) .2147
AVVR 43.60 (0.81-2251.90) .0638 3.73 (0.06-247.17) .5383
Aortic atresia 1.96 (0.25-15.36) .5215 0.97 (0.13-7.03) .9716
Noncardiac comorbidities 0.31 (0.01-7.60) .473 0.75 (0.04-15.90) .8557
Genetic comorbidities 2.28 (0.12-44.88) .5875 4.44 (0.28-70.86) .2922
Age at surgery 0.58 (0.32-1.06) .077 0.67 (0.38-1.17) .1576
Weight at surgery 0.40 (0.03-4.57) .4571 0.68 (0.06-8.34) .7599
Birth weight 2.20 (0.12-41.78) .5986 1.81 (0.09-38.21) .7030
Gestational age 0.98 (0.65-1.47) .9126 0.90 (0.60-1.35) .6127
Abbreviations: ECMO, extracorporeal membrane oxygenation; AVVR, atrioventricular valve regurgitation; BT, Blalock-Taussig.
a
P values are from the omnibus Wald chi-square test.
398 World Journal for Pediatric and Congenital Heart Surgery 6(3)

7. abnormalities, malnutrition, endemic infections, and prematur-
ity. For those undergoing a primary Norwood I operation,
including those with obstructed pulmonary venous return, hos-
pital survival was 83.3%. This compares favorably with many
centers with a long-standing tradition of treating babies with
HLHS and stage I palliation reporting surgical survival rates
ranging between 76% and 93%.11
Achieving in-hospital bench-
mark survival required flexibility in the Norwood/Sano proto-
col we set ourselves to follow for standard risk patients, that is,
those without associated obstructed pulmonary venous return
or major noncardiac comorbidity. Accordingly, palliation strat-
egy was tailored to patient anatomy and general condition,
offering a true hybrid for certain anatomical subtypes and
finally the salvage hybrid-bridge-to-Norwood in more desper-
ate situations. In such cases, we felt that breaking down the
treatment into two surgical stages would be safer than one
larger operation (with obstructed pulmonary venous return or
associated noncardiac comorbidity).
Indications to offer the true hybrid palliative pathway vary
tremendously from center to center. Initially conceived as an
alternative to the Norwood I operation in higher risk surgical
candidates,4,12
some centers have adopted bilateral PA banding
and ductal stenting as their standard approach for all patients
with HLHS physiology,13
with hospital survival ranging from
78.5% to as high as 96.6%.12-14
Our hospital survival with the
true hybrid approach reached 88%, comparing favorably with
published results. As our team’s comfort with the Norwood/
Sano protocol in standard risk patients materialized, the true
hybrid strategy was reserved for higher risk patients with sig-
nificant cardiac and noncardiac comorbidity, having ante-
grade flow to the coronary arteries. For patients with aortic
atresia, the theoretical risk of compromised retrograde flow
into the diminuitive aortic arch and resultant coronary ische-
mia steered our decision making away from the true hybrid,
which was nonetheless performed in one patient (who did
survive).
Initially described by Dr Norwood as early as 1981,6
the
initial bilateral PA banding and continuous prostaglandin
infusion without ductal stenting, followed by a deferred
Norwood operation, never really attracted attention. What
we refer to as the salvage hybrid-bridge-to-Norwood, also
called the rapid 2-stage Norwood,7
is slowly gaining interest
as another possible strategy for very-high-risk patients.15,16
Literature on this approach is scant, reporting survival after
bilateral PA banding from 70.6% to 100%, and 87.5% to
100% discharge survival after the deferred Norwood I,7,15,16
most often performed a week after initial palliation (cumula-
tive procedure survival of 64.7%-100%). This includes a
report from Kitahori et al who included eight patients under-
going the Norwood-BT shunt, as well as eight undergoing a
combined Norwood and cavopulmonary anastomosis, at a
mean time of 130 + 88 days after bilateral PA banding.15
In our experience, including patients with obstructed pulmon-
ary venous return or cerebral insults precluding the use of
cardiopulmonary bypass, cumulative survival of the two pro-
cedures reached 67%, comparable to the London experience.7
In our experience, this was achieved at a high cost in human
and material resources as reflected by a threefold increase in
ICU stay and more than twofold increase in hospital length
of stay, when compared to those undergoing a primary
Norwood I operation alone. We acknowledge what could
be viewed by some as prohibitive in-hospital morbidity and
mortality, given the baseline cardiac and noncardiac lesions,
which each center will put into its own perspective before
embarking on such a treatment algorithm. Accordingly, whether
four of nine patients alive with a Glenn circulation at one year
is considered to be appropriate justification to continue to pur-
sue the salvage strategy is an ethical question beyond the scope
of this discussion.
Among our 47 patients, ECMO support was required by
8 (17%) patients of which 6 (75%) died. In a recent study on
current outcomes of the Norwood operation in patients with
malformations other than HLHS, Alsoufi et al17
resorted to
ECMO support in 9 (13.8%) of 65 patients, of which 5 died
(mortality on ECMO ¼ 56%). Given the implications with
regard to human and material resources, it may be argued that
ECMO should not be offered after Norwood-type operations
unless outcomes improve, which each program needs to con-
sider for itself.
Study Limitations
The limitations of the article are inherent to those of any retro-
spective database study. The analysis focuses on outcomes of
the immediate initial hospital stay during which the first pallia-
tion was performed and does not address the ongoing issues
pertaining to interstage mortality, suitability for stage II, the
results of either cavopulmonary anastomosis or comprehensive
stage II palliation, or further down the single-ventricle path-
way, which are beyond the scope of the article. It is explicit that
the purpose of the study was not to compare the three palliative
strategies but to describe our outcomes based on a protocol
implemented in a learning curve environment, relying on use
of the Norwood/Sano procedure for straightforward patients,
but further tailored to each patient’s condition with the selec-
tive use of alternative strategies, with the goal of achieving
benchmark survival levels.
In summary, in-hospital results comparable to those
achieved at some experienced centers can be obtained for stan-
dard risk patients having HLHS and related anomalies with
ductal-dependent systemic circulation undergoing a Norwood
I operation, even in the course of an institutional learning curve
phase at an emerging program. In patients with associated
significant noncardiac and cardiac comorbidity including
obstructed pulmonary venous return, the true hybrid with duc-
tal stenting can achieve comparable survival for the initial hos-
pital stay. In very-high-risk patients/situations, the salvage
hybrid-bridge-to-Norwood may still allow acceptable survival
rates by breaking down the palliation into two surgical stages,
with a perceived lower cumulative risk for the initial hospital
stay, albeit at a high cost in human and material resources and
continuous potential for attrition.
Dodge-Khatami et al 399

8. Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, author-
ship, and/or publication of this article.
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