Congenital

The Modified Norwood Procedure at Necker

Hospital: Stage I and II for Hypoplastic Left
Heart Syndrome

gis Gaudin and Olivier Raisky
Margaux Pontailler, Re

We describe a modified Stage I neonatal surgical palliation for hypoplastic left heart syn-
drome inspired by both the Norwood and hybrid procedures. This new technique consists
in: (1) replacement of the patent ductus arteriosus and aortic arch plasty with a pulmonary
homograft, (2) banding of both the right and left pulmonary arteries (PA), (3) atrial septec-
tomy, and (4) reimplantation of the Aorta (when needed). This surgery is performed under
cardio-pulmonary bypass without aortic cross clamping, except when the ascending Aorta
is atretic and needs to be reimplanted. Stage II consists in the division of the pulmonary
bifurcation, PA branch plasty (with debanding), bidirectional cavopulmonary shunt and
Damus-Kaye-Stansel anastomosis. This new surgical procedure allowed us to obtain hemo-
dynamically stable postoperative patients and decrease by twice our mortality rate after
Stage I palliation in hypoplastic left heart syndrome but with frequent left PA stenosis or
hypoplasia.
Operative Techniques in Thoracic and Cardiovasculary Surgery 27:313 326 Ó 2022 Elsevier
Inc. All rights reserved.

KEYWORDS Hypoplastic left heart syndrome, Norwood procedure, Hybrid Norwood proce-
dure, Pulmonary artery banding, Pulmonary homograft

Central Message which implicates a right ventriculotomy). Although interest-

Neonatal surgical palliation for HLHS consist-
ing in replacement of the patent ductus arterio-
sus and aortic arch plasty with a pulmonary
homograft, banding of both pulmonary arteries,
atrial septectomy
Introductory Section
HLHS can be currently palliated either surgically (Norwood
or Sano procedure) or with a hybrid procedure initially
developed by the Giessen Team.1,2 Surgical palliation
involves, under cardioplegic arrest and deep hypothermia, a
proximal anastomosis of the atretic Aorta with the main pul-
monary trunk (Damus-Kaye-Stansel anastomosis) combined
with an aortic arch repair using a homograft, an atrial septec-
tomy and the transection of the distal main pulmonary artery
(PA), whose blood flow will be either provided by a modified
Blalock-Taussig shunt (conduit from the right subclavian
artery) or a Sano shunt (conduit from the right ventricle
ing results are reported in high-volume centers with low
interstage mortality rates (less than 10%), mortality rate
varies up to 75% in small-volume centers3-9 with high mor-
bidity and extremely physician and nurse time-consuming.
Hybrid palliation is an alternative to total under cardio-pul-
monary bypass (CPB) surgery, and consists in surgically
banding the PAs concomitantly to endovascular patent duc-
tus arteriosus (PDA) stenting and atrial septoplasty when
required. Although less invasive for a neonate with an initial
simpler management, it still carries an important interstage
mortality with ongoing morbidity (recurrent angioplasty, ret-
rograde stenosis or anterograde blood flow obstruction) and
requires the involvement and synchronization of both sur-
geons and interventional cardiologists.10,11
This hybrid approach for HLHS inspired us for a new sur-
gical palliation: instead of PDA stenting, a pulmonary homo-
graft is inserted to both replace the PDA and allow the aortic
arch plasty.12 This homograft will then become the neo
ascending aorta in Stage II palliation.

Authors declare no conflicts of interest.

Pediatric Cardiac Surgery, Necker Sick Children Hospital and Paris
University, Paris, France.
Address reprint requests to Olivier Raisky, MD, PhD, Department of
Pediatric Cardiac Surgery, Necker Sick Children Hospital and Paris
University, 149 rue de Sevres, 75015, Paris, France. E-mail: olivier.
raisky@aphp.fr
1522-2942/$ see front matter © 2022 Elsevier Inc. All rights reserved.
https://doi.org/10.1053/j.optechstcvs.2022.04.009
Stage I Palliation
Before skin incision, a cryopreserved pulmonary branch
homograft is tube-shaped on a 9 mm Hegar dilator (8 mm if
weight under 2.5 kg) using interrupted non-absorbable 6/0
stitches and cut obliquely at its distal end.
313
314
Surgery is performed via a median sternotomy on a beat-
ing heart. After thymectomy, CPB is initiated between an
arterial cannula in a 3.5 mm PTFE graft tube anastomosed to
the innominate artery and 2 venous cannulas (Fig. 1). When
CPB is initiated, the PDA is snagged. Meanwhile the patient
is cooled, the atrial septectomy is performed through a lim-
ited right atriotomy, with a snagged superior vena cava,
while the inferior vena cava is left opened. Aortic arch and
descending Aorta are extensively mobilized and the duct is
divided. At 26°C degrees, a neurosurgical clamp is applied
after the innominate artery, keeping both the brain and the
coronary arteries perfused. Snares are secured around both
the left common carotid and subclavian arteries, and the
descending Aorta is clamped. The ductal tissue is resected
and the Aorta is opened from the left carotid artery to the
descending Aorta (Fig. 2). The oblique extremity of the
tubulised homograft is anastomosed to the opened Aorta
(Fig. 3). The proximal end of the homograft is clamped,
snares and aortic clamps are removed after de-airing to
ensure full body perfusion. Proximal anastomosis of the
homograft is initiated on the upper part of the pulmonary
bifurcation where the PDA was extensively resected (Fig. 4).
The PAs are banded with PTFE 2-0 stitches which are tied
on a Hegar dilator inserted in the PAs. PA bands can be alter-
natively realized using a segment of a 3 mm or 3.5 mm PTFE
conduit (depending on the patient’s weight and the achieve-
ment of a balanced circulation) and has now our preference
for a better PA growth (Fig. 5). Proximal anastomosis of the
homograft is then resumed (Fig. 6).
If the ascending Aorta has a diameter of at least 3 mm, the
clamp on the homograft is removed and CPB is gradually
weaned under adrenaline 0.05 mg/kg/min and milrinone 0.5
mg/kg/min, adjusted depending on arterial saturation. PA
bandings can be tightened depending on the successive
measurements.
In the case of a small ascending Aorta (<3 mm) and aortic
atresia, with a risk of inadequate retrograde coronary perfu-
sion, the “coronary” Aorta is reimplanted on the pulmonary
artery, below the pulmonary bifurcation and the homograft,
just before the right PA takeoff, using interrupted 9/0 stitches
(one shot of blood cardioplegia is delivered with a venous
catheter after dividing the ascending Aorta) (Fig. 7). The
clamp is then released and CPB gradually weaned as previ-
ously described.
Stage II Palliation
CPB is initiated between one venous cannula and the Aorta
while the PAs are clamped. Aorta is cross clamped and cardi-
oplegia delivered. The pulmonary bifurcation is separated
from the homograft and the pulmonary root (Fig. 8). The PA
bands are removed and PA bifurcation plasty is realized with
fresh autologous pericardium (Fig. 9). If not reimplanted
at first procedure, the ascending Aorta is transsected and
sutured to the pulmonary root (Damus). Aortic continuity is
re-established and clamp is released. A bidirectional superior
cavopulmonary shunt is performed on a beating heart
(Fig. 10).
The Norwood procedure is at risk in many hands and
encounters high morbidity, leading to staff time-consuming
M. Pontailler et al.
implication. Few HLHS expert centers worldwide have
achieved high survival despite the tremendous efforts
engaged elsewhere. The hybrid procedure development was
partly due to these well-shared difficult surgical experiences.
Since its description,1,2,10,11 the hybrid procedure has defini-
tively gained popularity in small volume centers where the
“classic” Norwood procedure was associated with a high
morbidity and mortality.
Despite its real success, the hybrid strategy still carries a
significant morbidity, high interstage reinterventions rate,
requires full Heart-Team availability/training and a hybrid
suite. Although facilitating Stage I, it complicates compre-
hensive Stage II palliation and only delays aortic arch recon-
struction and pulmonary bifurcation individualization,
realized at redo surgery. Several authors proposed technical
variations to improve and facilitate comprehensive Stage II
palliation.13 Sakurai et al. performed bilateral PA banding
with continuous prostaglandin administration instead of
PDA stenting, in order to decrease interstage stent-related
morbidity and allow easier aortic arch reconstruction during
stage II. Although improving early and intermediate mortal-
ity, it required interstage full hospitalization and earlier stage
II palliation in comparison with classic Norwood proce-
dure.14 DeCampli et al. proposed a new comprehensive stage
II procedure to avoid redo distal aortic arch dissection and
the Damus-Kaye-Stansel anastomosis. This procedure con-
sists in dilatation/stenting of the residual PDA with creation
of a pulmonary stented baffle between both PAs instead of
aortic arch reconstruction.15
The Necker modified Norwood palliative strategy aimed
to reduce the inconveniences and complications related to
the hybrid procedure: full Heart-Team availability, recurrent
coarctation at the distal end of the PDA stenting, retrograde
malperfusion with coronary ischemia and recurrent restric-
tive ASD.
This technique tried to reproduce some of the hybrid pro-
cedure’s advantages (no aortic cross clamping, no right ven-
triculotomy) and to reduce the adverse events of the hybrid
palliation. This strategy enabled to maintain an unobstructed
systemic circulation by the means of aortic arch plasty with
the distal anastomosis of the homograft. In comparison with
the hybrid procedure, it seemed to reduce stent-related mor-
bidity and clearly simplified comprehensive stage II aortic
arch repair as in the classic Norwood operation.13 This tech-
nical aspect of the aortic reconstruction excluded retrograde
malperfusion of the aortic arch. If we had any doubt about
coronary retrograde perfusion in the presence of tiny ascend-
ing Aorta and aortic atresia, it allowed us to perform a proxi-
mal aortic reimplantation and avoid any potential future
ischemia. With bilateral PA banding, it allowed us to control
the PA pressure and the pulmonary overflow. Technically,
this is probably the most critical part of the operation: to get
a stable patient with an adequate balanced pulmonary flow,
but at the same time, to allow the future growth of the PAs
before the bidirectional Glenn anastomosis. We faced diffi-
cult recurrent situations with underdevelopment of the left
PA, either with a very tight left PA branch banding or com-
pression from the aorta after the Glenn anastomosis. This is
probably the most delicate and disappointing part of this
strategy but shouldn’t be different from the hybrid approach.
For Operative Techniques in Thoracic and Cardiovascular Surgery
Significant improvements might be obtained by a different
approach, for example, by a pre calibrated banding or an
adjustable one. An endo banding with a fenestrated PTFE
patch could also be a solution for the future.
This new surgical procedure allowed us to drastically
reduce our postoperative in-hospital morbidity (chest primary
closure in half of the patients, limited bleeding, low dose of
inotropes, stable hemodynamic, low peak lactate level), to
decrease the staff consuming-time for these patients and to
diminish by twice our mortality rate after Stage I palliation in
HLHS. During the learning curve period, the interstage mor-
tality remained high (around 25%) due to systemic ventricular
dysfunction. This led us to modify our initial management:
315
reimplantation of the ascending “coronary” Aorta during stage
I when aorta was atretic or tiny (<3 mm) and tightening of
the PA bandings when pulmonary outflow remained high
(sometimes paid by the price of inappropriate development of
the left PA). We also increased our interstage supervision with
a weekly cardiologist appointment and/or hospitalization
when onset of ventricular dysfunction, to adapt medication
and balance systemic and pulmonary circulations.12
Finally, this strategy offers to any surgical team with limited
experience and low volume HLHS patients, to significantly
reduce the surgical complexity and the postoperative morbid-
ity. The long-term development of the banded left PA remains
a concern as for hybrid procedure and needs to be reevaluated.
316 M. Pontailler et al.

Figure 1 Preparation and installation before hybrid surgical palliation: cardiopulmonary bypass initiated between the 2 vena cavas and the
innominate artery through an interposed PTFE graft. While cooling, atrial septectomy is performed through a right atriotomy on beating heart
(in case of aortic atresia) or on ventricular fibrillation (no aortic atresia).
For Operative Techniques in Thoracic and Cardiovascular Surgery 317

Figure 2 At 26°C degrees, on a beating heart, a neurosurgical clamp is applied after the innominate artery, snares are secured around both the
left common carotid and subclavian arteries, and the descending Aorta is clamped. The ductal tissue is resected and the Aorta is opened from
the left carotid artery to the descending Aorta.
318 M. Pontailler et al.

Figure 3 Homograft distal anastomosis to replace the PDA and repair the aortic arch in order to ensure systemic output via an unobstructed sys-
temic circulation.
For Operative Techniques in Thoracic and Cardiovascular Surgery 319

Figure 4 Suture of the proximal end of the homograft (after division at the appropriate level) is initiated on the upper part of the pulmonary
bifurcation where the PDA was extensively resected.
320 M. Pontailler et al.

Figure 5 Bilateral PA banding on Hegar dilators before resuming the Homograft proximal anastomosis on the pulmonary root.
For Operative Techniques in Thoracic and Cardiovascular Surgery 321

Figure 6 Surgical anatomy after Necker stage I HLHS palliation when ascending aorta did not require direct reimplantation: tubulized pulmo-
nary homograft replacing the PDA and enlarging the aortic arch combined with bilateral PA banding.
322 M. Pontailler et al.

Figure 7 Surgical anatomy after Necker stage I HLHS palliation and ascending « coronary » Aorta direct reimplantation in the pulmonary trunk,
below the right PA and the implanted homograft.
For Operative Techniques in Thoracic and Cardiovascular Surgery 323

Figure 8 Stage II Necker palliation of HLHS with reimplantation of the atretic ascending Aorta, debanding of the PAs and bidirectional superior
cavopulmonary shunt.
324 M. Pontailler et al.

Figure 9 PA bifurcation plasty realized with fresh autologous pericardium.

For Operative Techniques in Thoracic and Cardiovascular Surgery 325

Figure 10 Bidirectional superior cavopulmonary shunt realized on a beating heart.

326
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