REVIEW ARTICLE

Management of the single ventricle and

potentially obstructive systemic ventricular
outflow tract
Bahaaldin Alsoufi a,
a

Division of Cardiothoracic Surgery, Department of Surgery, Childrens Healthcare of Atlanta, Emory University School
of Medicine, 1405 Clifton Road, NE, Atlanta, GA 30322
a

USA

Multi-stage palliation is the current management strategy for the treatment of children with various single ventricle
(SV) cardiac malformations. The success of this strategy depends on the presence of favorable anatomic and
hemodynamic criteria. Several SV anomalies have the potential of developing systemic ventricular outflow tract
obstruction (SVOTO) that might be evident early on or progress later after palliative surgeries. SVOTO could result in
ventricular hypertrophy, impaired diastolic function and subendocardial ischemia with subsequent deleterious effects
on the SV and disturbance of some of those criteria for a successful multi-stage palliation strategy. Careful
identification of SV patients at risk of developing SVOTO and proper planning of the optimal palliation sequence
beginning at the 1st stage procedure are vital factors that would affect long-term outcomes in those patients. In the
current review, we describe the morphology of SV patients with potential SVOTO risk, surgical procedures that
address potential or present SVOTO, and optimal timing of those procedures within the multi-stage palliation chain.
We attempt to provide a treatment algorithm for various patients taking into consideration their unique anatomic and
physiologic characteristics.
2013 Production and hosting by Elsevier B.V. on behalf of King Saud University.
Keywords: Single ventricle, Norwood, Pulmonary artery band, Hybrid
Contents
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Patients with potential risk of SVOTO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Treatment strategies for SV patients with potential SVOTO risk. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
The strategy of initial PAB followed by DKS or VSD/bulboventricular foramen enlargement at the time of BCPC . . . . . . . . . . . .
PAB . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
BCPC . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
DKS. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
VSD/bulboventricular foramen enlargement. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
The strategy of initial DKS plus shunt at time of 1st stage palliation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Other treatment strategies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Received 7 March 2013; revised 19 May 2013; accepted 21 May 2013.

Available online 31 May 2013

Tel.: +1 404 785 6330; fax: +1 404 785 6266.

E-mail address: balsoufi@hotmail.com

P.O. Box 2925 Riyadh 11461KSA

Tel: +966 1 2520088 ext 40151
Fax: +966 1 2520718
Email: sha@sha.org.sa
URL: www.sha.org.sa

10167315 2013 Production and hosting by Elsevier B.V. on behalf of King Saud University.
Peer review under responsibility of King Saud University.
URL: www.ksu.edu.sa
http://dx.doi.org/10.1016/j.jsha.2013.05.003

Production and hosting by Elsevier

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Palliative arterial switch operation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Hybrid strategy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Management of the old SV patients with SVOTO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Management algorithm of SV patients with SVOTO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Disclosure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Introduction

ulti-stage palliation is the current management strategy for the treatment of children
with various single ventricle (SV) cardiac malformations. The 1st stage neonatal palliative surgery
varies based on underlying anatomy. However,
the general aim is to achieve the following four
objectives: (1) unobstructed systemic cardiac output; (2) controlled source of pulmonary blood
flow; (3) reliable source of coronary blood flow;
and (4) unobstructed egress of pulmonary venous
effluent across the atrial septum. At a 2nd stage,
the bidirectional cavo-pulmonary connection
(BCPC) is performed by suturing the superior
vena cava to the pulmonary artery and is typically
done at four to six months of age. At a 3rd stage,
the Fontan procedure is performed to channel
the remaining systemic venous return from the
inferior vena cava to the pulmonary artery and is
typically done at two to four years of age.
Good systolic and diastolic ventricular function,
stable rhythm, competent atrioventricular valve,
and unobstructed systemic ventricle outflow tract
(SVOT) are among some of the well-identified
stringent hemodynamic and anatomic criteria for
successful completion of all palliation stages and
achievement of a stable and durable Fontan
circulation.
Several SV anomalies have the potential of
developing SVOT obstruction (SVOTO) that might
be evident early on or progress later after palliative surgeries [15]. SVOTO could result in ventricular hypertrophy, impaired diastolic function
and subendocardial ischemia with subsequent
deleterious effects on the SV and development of
unfavorable hemodynamic conditions that might
negatively influence the success of palliation strategy towards the Fontan circulation [15].
Therefore, careful identification of SV patients at
risk of developing SVOTO and proper planning of
optimal palliation sequence beginning at 1st stage
procedure are vital factors that would affect longterm outcomes in those patients.

Patients with potential risk of SVOTO

Several groups of children with SV are at potential risk of developing SVOTO. The larger group

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comprises patients in whom the aorta arises from

an outlet chamber that is connected to the dominant ventricle via a bulboventricular foramen or
a ventricular septal defect (VSD). This group includes patients with double inlet left ventricle
(DILV) or tricuspid atresia associated with ventriculo-arterial discordance. Also included in this
group are some infants with double outlet right
ventricle (DORV) and remote VSD that are unsuitable for biventricular repair; and those with dextro- or levo-transposition of the great arteries
associated with VSD and hypoplastic right ventricle. In the above listed anomalies, the systemic
blood flow traverses through the bulboventricular
foramen or VSD from the dominant ventricle to
the rudimentary ventricle that serves as an outlet
chamber from which the aorta arises. Those patients are at risk of developing SVOTO as a result
of reduction in VSD/bulboventricular foramen
size early after pulmonary artery banding (PAB)
or late after volume unloading with BCPC [16].
The second group of patients at potential risk of
developing SVOTO includes those with welldeveloped subaortic conus that could enlarge with
time to cause subaortic obstruction, as for example
with patients with DORV, often associated with
hypoplastic left ventricle and mitral stenosis/atresia [2,4,6]. Another group of patients at potential
risk of developing SVOTO includes those with
unbalanced atrioventricular septal defect of right
dominance in whom flow to the aorta might become compromised by a small inlet VSD or restricted by atrioventricular valve tissue, in
addition to the presence of elongated left ventricular outflow tract and accessory atrioventricular
valve tissue, all of which could contribute to the
obstruction of systemic cardiac output towards
the aorta [6].

Treatment strategies for SV patients with

potential SVOTO risk
Given the impending negative effects of SVOTO
on hemodynamic conditions and future outcome,
the treatment plan of patients at potential SVOTO
risk should be carefully deliberated beginning at
the time of 1st stage palliative surgery and individualized to allow the design of the most proper

surgical sequence that is suitable for patients

anatomy. Variations in management strategies
are related to the type of procedure utilized to address SVOTO and timing of that procedure during
the multi-stage palliation chain.
Several options are available at the surgeons
disposal to alleviate SVOTO. These include the
Damus-Kaye-Stansel operation (DKS), VSD/
bulboventricular foramen enlargement, resection
of subaortic conus and palliative arterial switch
operation [4,5,7]. Each procedure is associated
with distinctive advantages and disadvantages.
The decision to address SVOTO at the time of
1st palliation stage or to delay that until the 2nd
stage depends on many factors including the presence of arch obstruction, size of VSD/bulboventricular foramen, and presence of subaortic
obstruction.
In general, VSD/bulboventricular foramen
enlargement is not performed in neonates due
to difficulties with exposure and tissue fragility.
Therefore, strategies that address SVOTO in
neonates generally include DKS or palliative
arterial switch operation. On the other hand,
strategies that address SVOTO at the time of
BCPC involve neonatal palliation with a PAB
followed at 2nd stage BCPC combined with
either DKS or VSD/bulboventricular foramen
enlargement.

The strategy of initial PAB followed by DKS or

VSD/bulboventricular foramen enlargement at the
time of BCPC
PAB
Those SV anomalies are usually associated with
excessive pulmonary blood flow that requires
early control with a PAB to prevent development
of pulmonary vascular disease [46,814]. The

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main advantage of the strategy of early PAB

followed by BCPC and DKS or VSD/bulboventricular foramen enlargement is avoidance of cardiopulmonary bypass and deep hypothermic
circulatory arrest in the neonatal period. PAB
can also be performed via a left thoracotomy
thereby facilitating 2nd stage procedure (Fig. 1).
Nonetheless, there are several drawbacks of
PAB related to the difficulty of obtaining adequate pulmonary vascular protection with PAB,
possibility of pulmonary artery distortion if the
PAB was placed too distally, risk of distortion of
pulmonary valve with subsequent regurgitation
if the PAB was placed too proximally and, most
importantly, occurrence of ventricular hypertrophy that might accelerate the rate of VSD/bulboventricular foramen
narrowing with the
subsequent early development of SVOTO
[1,3,15]. Another complicating issue in initial
PAB strategy is the frequent incidence of aortic
coarctation at original presentation. While there
are several series reporting aortic coarctation repair and PAB via a left thoracotomy as 1st stage
palliation, that approach might not be applicable
in patients with hypoplastic aortic arch necessitating a sternotomy and cardiopulmonary bypass
for adequate arch augmentation. In addition, it
might be challenging to carry out adequate banding when the PAB is performed via a left thoracotomy because of patients position and lung
collapse.
A landmark study by Freedom et al. from Toronto reviewed 43 patients with SV who did not have
evidence of SVOTO at time of PAB done at a mean
0.21 year of age. The study noted that 31/43 patients (72%) developed SVOTO at a mean age of
2.52 years, with an especially increased risk in
84% of patients with DILV or tricuspid atresia
associated with ventriculo-arterial discordance.

Figure 1. Left thoracotomy view of a single ventricle patient with normally related great vessels who has aortic coarctation, hypoplastic aortic
arch and patent ductus arteriosus (A). The patient underwent extended end-to-end repair of aortic coarctation, ligation and division of the ductus
arteriosus, and main pulmonary artery banding as a 1st stage palliation procedure (B).

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The study concluded that PAB produces myocardial hypertrophy with resultant decrease in
VSD/bulboventricular foramen size that accelerates potential SVOTO and they cautioned against
PAB in those patients with potential SVOTO risk
[1]. Another study by Matitiau et al. from Boston
examined the indexed bulboventricular foramen
area in patients who had undergone various initial
palliative procedures. The authors found that all
patients who did not have an initial DKS (the
majority had PAB) and subsequently developed
early SVOTO had an initial bulboventricular foramen area index less than 2 cm2/m2. They also
noted that patients with aortic coarctation had
smaller bulboventricular foramen area index at
time of initial presentation, and were likely to be
at higher risk of developing SVOTO after PAB
[3]. Accordingly, initial VSD/bulboventricular
foramen size and presence of aortic coarctation/
hypoplastic aortic arch should be taken into
consideration when deciding on the type of 1st
palliative surgery.

BCPC
The performance of BCPC results in volume
unloading that might further decrease VSD/
bulboventricular foramen size and accelerate the
rate of SVOTO development. In a study by Donofrio et al. from Philadelphia, the authors noted a
decrease in mean VSD/bulboventricular foramen
area index following PAB or BCPC with greater
diminution noted after BCPC (41 19%) than after
PAB (25 28%) [15]. While volume unloading occurs primarily at time of BCPC, the decrease in
systemic ventricle end diastolic volume continues
for years after the Fontan operation and subsequently the risk of SVOTO might appear late after
completion of the Fontan circulation [6].
Therefore, there is a general agreement that procedures addressing potential SVOTO should be
performed at the time of BCPC to avoid late
SVOTO which would be more difficult to manage,
would require an unnecessary re-operation, and
could be associated with inferior outcomes.

DKS
The DKS operation was originally described for
the treatment of selected patients with transposition of the great arteries. Subsequently, there have
been reports of successful management of patients with SVOTO utilizing DKS operation
[4,9,13]. Furthermore, DKS use in SV patients expanded into a planned procedure at the time of
BCPC in patients with anatomic substrate to develop SVOTO, regardless of the presence of the

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actual gradient between the systemic ventricle

and aorta at time of BCPC [4,6,1014].
Advantages of performing DKS concomitant
with BCPC include the ability to adequately address most SVOTO cases, ability to perform arch
reconstruction if needed, and low risk of
development of heart block. In addition, the modified-in-series circulation after BCPC and DKS is
more stable than the balanced parallel circulation
after DKS with aortopulmonary shunt with no
associated risk of steal from the coronary and systemic circulation. Potential disadvantages of DKS
include distortion of the semilunar valves with
subsequent regurgitation and increased SV volume load, in addition to the risk of compression
of left pulmonary artery or left main bronchus,
especially in patients with great vessels orientation that is not side by side [4]. While there is a
theoretical concern that prior PAB could result
in pulmonary valve distortion with consequent
regurgitation at time of DKS, results from
numerous studies of concomitant BCPC and
DKS after PAB showed that the incidence of
semilunar valve regurgitation is low despite prior
PAB [4,6,814].
While initial descriptions of DKS involved an
end-to-side anastomosis between the pulmonary
artery and aorta with or without patch augmentation, a modified side-by-side double barrel DKS
anastomosis has been suggested by Lamberti
and colleagues and shown to better preserve the
shape of pulmonary sinus and native aortic sinus
of Valsalva [16] (Fig. 2). Maintenance of original
semilunar valve and sinus geometry in their native positions might offer the advantage of avoiding potential distortion. In a study by Fujii et al.
from Okayama, the authors compared 13 patients
who had undergone end-to-side DKS with 34 who
had undergone double barrel DKS. The study
found that although both techniques were
associated with 100% freedom from SVOTO reinterventions, patients who had undergone endto-side DKS had more frequent deterioration in
pulmonary regurgitation than double barrel DKS
(4/11 vs. 1/34, P = 001) [11].
Results of concomitant BCPC and DKS have
been published in several series and showed low
mortality without much added operative risk,
excellent immediate SVOTO relief and freedom
from recurrent obstruction, good and durable
semilunar valve function, and good functional status following Fontan operation [4,6,814]. In a series from the King Faisal Specialist Hospital &
Research Center in Riyadh, Saudi Arabia, 36 children with SV underwent DKS at time of BCPC

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Figure 2. View from a single ventricle patient with normally related great vessels at time of 2nd stage procedure following initial pulmonary
artery banding. The band was removed, the main pulmonary artery was transected, the branch pulmonary arteries end was closed with a patch,
and an incision at the medial side of the aorta was created (A). The patient underwent an end to side Damus-Kaye-Stansel anastomosis,
supplemented with patch material, in addition to Glenn bidirectional cavopulmonary connection (B). View from a patient with single ventricle
and normally related great vessels at time of 2nd stage procedure following initial pulmonary artery banding. The band was removed, the main
pulmonary artery was transected at the site of the band, the aorta was transected at almost similar height above the sinotubular junction, and the
branch pulmonary arteries end was closed primarily (C). The patient underwent a side to side double barrel Damus-Kaye-Stansel anastomosis,
in addition to Glenn bidirectional cavopulmonary connection (D).

(n = 29) or Fontan (n = 7). Underlying anatomy was

DILV (n = 18), DORV (n = 8), unbalanced atrioventricular septal defect (n = 4) and other (n = 6). Prior
palliation included PAB (n = 35), aortic coarctation
repair (n = 11) and atrial septectomy (n = 8). At
time of DKS, 17 patients (47%) had no SVOT gradient and 19 patients (53%) had SVOT gradient
(median 19 mmHg, range 480 mmHg). Overall,
survival was 89% and 83% at one month and five
years, respectively. None of the deaths were related to SVOTO or DKS complications. When
present, SVOT gradient decreased from
23.4 18.7 mmHg pre-operatively to 0 after DKS
(p < 0.001). At last follow-up, none of the patients
developed any SVOT gradient; 78% of them had
zero or trivial aortic/neo-aortic valve regurgitation
while 22% had mild regurgitation. None of the patients had evidence of compression of left pulmonary artery or bronchus; 81% of patients had
reached or were suitable candidates awaiting final

palliative surgery and all survivors were in excellent functional status except for one patient with
protein-losing enteropathy [17].

VSD/bulboventricular foramen enlargement

Several groups have advocated VSD/bulboventricular foramen enlargement at time of BCPC or
later as an alternative strategy for the management of SV patients with SVOTO [2,4,5,7,1820]
(Fig. 3). VSD/bulboventricular foramen enlargement is associated with drawbacks related to the
necessity of performing a ventriculotomy in many
cases with subsequent risk of forming ventricular
aneurysm. Moreover, the intimate relationship of
the VSD/bulboventricular foramen with the semilunar valves and atrioventricular valves might disallow adequate enlargement thus leading to
insufficient SVOTO relief or recurrence of
obstruction. Finally, VSD/bulboventricular foramen enlargement could result in a high incidence

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Figure 3. View from a single ventricle patient with ventriculo-arterial discordance who underwent ventriculotomy into the rudimentary ventricle
and enlargement of the bulboventricular foramen. The dashed line indicates the safe area for enlargement while the circles demonstrate the likely
site for conduction system (A). Following enlargement of the bulboventricular foramen, the ventriculotomy is closed with a patch (B).

of complete heart block necessitating permanent

pacemaker implantation [2,4,1820]. Approach to
VSD/bulboventricular foramen enlargement varies based on anatomy and location of VSD, and
can be performed through the aortic valve, atrioventricular valve or frequently via a ventriculotomy. Similarly, site of enlargement depends on
ventricular looping and dominant ventricle morphology and requires knowledge of potential
course of conduction system in various morphologies. When the accessory chamber of DILV is located on the right with a D loop (SDD),
bulboventricular foramen could be enlarged superiorly as the conduction bundle is located inferiorly. When the accessory chamber is located to
the left side, with an L loop (SLL), the conduction
bundle is supposed to run superiorly and the incision should therefore be done inferiorly. VSD
enlargement might be particularly problematic in
patients with unbalanced atrioventricular septal
defect as it might interfere with atrioventricular
valve function following enlargement. In a report
by Pass et al. from New York, nine patients underwent bulboventricular foramen enlargement following SV palliation; there was one operative
and one late death, while one patient required
permanent pacemaker insertion and three patients (33%) required re-operation for recurrent
SVOTO [18]. Therefore, due to the clear advantage
of DKS over VSD/bulboventricular foramen
enlargement, many surgeons reserve VSD/bulboventricular foramen enlargement to children with
pulmonary regurgitation, pulmonary stenosis or
atresia, DORV with restricted VSD, or patients
who develop late SVOTO after BCPC with inability to perform DKS due to sutured pulmonary
valve cusps [2,4,5,7].

The strategy of initial DKS plus shunt at time of

1st stage palliation
The strategy of performing PAB +/
aortic
coarctation repair as initial palliative surgery for
SV with excessive pulmonary blood flow is associated with numerous disadvantages, listed earlier.
Results following PAB in those patients have not
been optimal with significant operative and late
mortality risk and a high incidence of poor candidacy to progress through palliation stages towards
Fontan circulation [4,6,814,2124].
In a study by Franklin et al. from London, the
authors found that survival following PAB was
77% at one year and 45% at five years for those
who had PAB alone and was 44% at one year
and 22% at five years for those who had PAB
and aortic coarctation repair.[23] In another study
from the same authors, suitability for Fontan operation was 42% for those who had PAB alone and
8% for those who had PAB and aortic coarctation
repair.[24] In a study by Miura et al. from Osaka
examining 21 SV patients who had undergone initial PAB, the authors noted that the bulboventricular foramen area index decreased from a median
of 10375% necessitating DKS at 2nd stage in the
majority of patients; however 6/21 (29%) patients
died before reaching 2nd palliation stage [14]. In
another study from Fiore et al. from St. Louis
and Indianapolis examining 27 SV patients who
had undergone initial PAB with concomitant aortic coarctation in 18/27 (66%) patients, the authors
noted that all those patients required DKS at 2nd
stage. However, an aortopulmonary shunt was
necessary as a sole or supplemental source of pulmonary blood flow in 11/27 children with high
mortality in 6/11 of those patients (55%) [12].

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Figure 4. View from a single ventricle patient with ventriculo-arterial discordance who underwent a Norwood-type 1st stage palliation. The
main pulmonary artery and ascending aorta were transected above the sinotubular junction, the hypoplastic aortic arch was opened and the
coarctation area was excised (A). The reconstruction was completed by performing a Damus-Kaye-Stansel anastomosis, arch reconstruction and
augmentation with patch. The source of pulmonary blood flow is provided with an aortopulmonary shunt, or a right ventricle to pulmonary
artery shunt (Not shown) (B).

Due to those disappointing results, several

authors recommended that SVOTO is addressed
at time of neonatal 1st stage procedure with DKS
plus aortopulmonary shunt, or a Norwood-type
1st stage palliation if concomitant arch reconstruction was needed [25,26] (Fig. 4). Advantages of this
approach include the ability to address SVOTO
early on and concomitantly address all types of
arch pathology. This approach has the potential
of avoiding development of ventricular hypertrophy, thereby improving candidacy for a Fontan
procedure. Moreover, detrimental effects of pulmonary regurgitation as a result of proximal displacement of the PAB or pulmonary artery
stenosis as a result of distal displacement of the
PAB are avoided by the strategy of neonatal 1st
stage DKS plus aortopulmonary shunt. Finally, a
shunt offers an advantage of having a uniform
diameter with more predictable pulmonary blood
flow as compared to PAB that is harder to adjust
as that is carried out under non-physiologic conditions that can be affected by anesthesia, positive
ventilation and open chest, and can also change
later on with a decrease in pulmonary vascular
resistance and pulmonary artery remodeling.
The obvious disadvantages of this strategy, however, include cardiopulmonary bypass and deep
hypothermic circulatory arrest needs during the
neonatal period, complex post-operative course,
shunt related complications, and early and interim
mortality risk [27].
In a study by Mosca et al. from Ann Arbor, the
authors reported outcomes of 38 patients with TA

or DILV and ventriculo-arterial discordance who

had undergone Norwood 1st stage palliation. They
found on echocardiographic follow-up that
although absolute bulboventricular foramen size
increased in approximately 50% of the patients,
when indexed to body surface area there was an
overall decrease with time. Their operative
mortality with the modified Norwood operation
was 8% and five year survival was 71% with no
incidence of recurrent SVOTO or significant semilunar valve insufficiency. They concluded that
their outcomes were superior to those reported
with other treatment strategies in that group of
patients [25]. Outcomes of the Norwood operation
in the current era have appreciably improved
through refinements in surgical technique, perfusion strategy, pre-operative stabilization, postoperative care and home monitoring; and expected
survival has increased, especially in the subset of
patients with tricuspid atresia or DILV [27]. A more
recent study from Bradley et al. from Charleston
reported outcomes of 21 SV patients with excessive
pulmonary blood flow who were treated with
aortopulmonary shunt rather than PAB along with
prophylactic DKS; they had one operative mortality and 90% five year survival supporting the
advantage of this treatment strategy over the
alternative PAB +/ aortic coarctation repair [26].
Furthermore, a study by Serraf et al. from Paris
compared outcomes in SV patients who had
undergone various initial palliations and found
that four year survival was better in those who
had undergone DKS or palliative arterial switch

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Figure 5. View from a single ventricle patient with ventriculo-arterial discordance who underwent a palliative arterial switch operation as 1st
stage palliation. Neo-aorta was reconstructed after coronary transfer, and a Lecompte maneuver was accomplished by placing the branch
pulmonary arteries anterior to the reconstructed neo-aorta (A). The neo-pulmonary artery was reconstructed with a patch. Very often, the
addition of either an aortopulmonary shunt or a pulmonary artery band is necessary. In this example, an aortopulmonary shunt is shown (B).

operation than those who had undergone PAB and

aortic coarctation repair (67% vs. 40%, P < 0.05)
[22].

Other treatment strategies

Palliative arterial switch operation
Several groups reported experience with palliative arterial switch operation for treatment of SV
anomalies such as tricuspid atresia or DILV and
ventriculo-arterial discordance as an alternative
to neonatal DKS or Norwood operations [4,28
31] (Fig. 5). Palliative arterial switch operation results in alignment of the systemic ventricle with
the systemic outflow tract and therefore eliminates SVOTO early on and decreases the chance
of development of ventricular hypertrophy and
diastolic dysfunction. Advantages of the palliative
arterial switch operation include the ability to address SVOTO in the neonate, establishment of
laminar flow to the aorta potentially decreasing
recurrent arch obstruction, and finally low risk of
left pulmonary artery or main bronchus compression.[4] Nonetheless, potential disadvantages are
related to the usual long-term concerns with the
arterial switch operation such as late coronary
problems, neo-aortic root dilatation, stretch and
stenosis of the pulmonary arteries after the
Lecompte maneuver. Moreover, it was originally
thought that the restricted bulboventricular
foramen would help prevent excessive pulmonary
blood flow after the palliative arterial switch
operation. However, this effect is unpredictable,
and often a PAB on the newly reconstructed
pulmonary artery is needed to limit pulmonary
blood flow while an aortopulmonary shunt might

occasionally be necessary in patients with a very

restricted bulboventricular foramen and diminished pulmonary blood flow [2831]. Furthermore,
adjustments of PAB or a latter addition of aortopulmonary shunt can occasionally become necessary as bulboventricular foramen diminishes in
size with subsequent decrease in pulmonary
blood flow after discharge [2831]. Only a few
patient series that underwent palliative arterial
switch operation exist in the literature. In a series
from Ceresnak et al. from New York, nine patients
underwent palliative arterial switch operation as
initial palliation with one operative and one late
death while the seven remaining patients reached
or qualified for Fontan operation [30]. In a more
recent series by Heinle et al. from Houston, 14 patients underwent palliative arterial switch operation with 13/14 (93%) requiring concomitant arch
reconstruction. In their series, 6/14 (43%) patients
required concomitant PAB for excessive pulmonary blood flow while 1/14 (7%) required aortopulmonary shunt. There were no deaths and all
patients proceeded to 2nd stage BCPC. However,
four patients required additional procedures
before BCPC that included addition of aortopulmonary shunt in three patients and PAB adjustment in one patient. Overall, 11/14 reached
Fontan while the remaining 3/14 qualified for
Fontan surgery. While there was no incidence of
SVOTO, 64% of patients required pulmonary
artery augmentation at time of Fontan.[31]

Hybrid strategy
In the past decade, there has been an increased
experience in hybrid management of neonates

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Figure 6. View from a single ventricle patient who underwent hybrid-type 1st stage palliation with branch pulmonary artery banding and ductal
stenting (A). At time of 2nd stage surgery, the reconstruction was completed by performing a Damus-Kaye-Stansel anastomosis, arch
reconstruction and augmentation with patch, pulmonary artery de-banding and augmentation, in addition to Glenn bidirectional cavopulmonary
connection (B).

with hypoplastic left heart syndrome and other

related SV anomalies [27,3235]. Hybrid strategy
consists of a 1st stage procedure that is done in
the neonatal period and involves bilateral PA
branch banding, ductal stenting with atrial septostomy or stenting as needed. The 2nd stage
procedure is usually done at four to six months
of age and consists of stent removal, aortic arch
reconstruction, DKS and BCPC (Fig. 6). Advantages of this hybrid strategy are related to avoidance
of cardiopulmonary bypass and cardioplegic
arrest in the neonate with potential improved
long-term myocardial function, deferment of

reconstruction of the aortic arch and deep hypothermic circulatory arrest to an older age with potential
improved
long-term
neurological
outcomes; and finally exit of the operation at 2nd
stage with BCPC and a modified-in-series circulation that is more stable than a balanced circulation
after a Norwood operation with a shunt [27,3235].
Nonetheless, potential disadvantages of hybrid
strategy include ductal and atrial stent migration,
band migration, stent stenosis and thrombosis,
all requiring close observation for potential need
for catheter-based re-interventions or early 2nd
stage surgery [27,3235]. Reported outcomes of

Figure 7. A proposed management algorithm for the treatment of single ventricle patients at potential risk of developing systemic ventricle
outflow tract obstruction.

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this hybrid strategy from experienced centers are

encouraging, and its expected that additional
knowledge with this strategy and refinements of
technique and devices would further improve outcomes and support the role of hybrid strategy in
the palliation of various SV anomalies. All theoretical long-term advantages of hybrid strategy are
yet to be proven in order to replace other treatment strategies especially as Norwood outcomes
in patients with SV, other than hypoplastic left
heart syndrome, are superior to those with hypoplastic left heart syndrome [27].

Management of the old SV patients with SVOTO

Management of older SV patients with SVOTO
who have already completed palliation stages is
challenging [2]. It is very doubtful that the pulmonary valve in the transected pulmonary artery
could be utilized for a DKS operation especially
as most surgeons over sew the pulmonary valve
cusps at the time of BCPC or Fontan to prevent
thrombus formation [36]. Nonetheless, there are
reports in the literature of late performance of
DKS in patients who previously had transection
of the main pulmonary artery at the time of BCPC
[37]. At the King Faisal Specialist Hospital in
Riyadh, one patient who had undergone transection of the main pulmonary artery at time of BCPC
followed by Fontan developed SVOTO nine years
after Fontan with 45 mm HG gradient across the
restricted bulboventricular foramen. That patient
underwent re-operation with DKS that successfully alleviated SVOTO with trivial neo-aortic
regurgitation on late follow-up [17].
In most cases, the only available option is VSD/
bulboventricular foramen enlargement that usually requires a ventriculotomy through the rudimentary chamber underneath the aortic valve
[2]. Late VSD/bulboventricular foramen enlargement is challenging and long-term results are disturbing due to risk of heart block, inadequate
SVOTO relief, recurrent obstruction, atrioventricular valve dysfunction, and hemodynamic effects
of hypertrophy on Fontan circulation. One interesting report from Meadows et al. from Boston
described eight patients who presented with
severe SVOTO following Fontan done in complex
DORV. All had hypertrophied, hypertensive, supra-systemic isolated LV chambers, and three
patients had a false aneurysm of the left ventricle.
Five patients underwent VSD creation and three
underwent enlargement of existing VSD. Initial
intervention resulted in a decreased gradient from
76.9 mmHg to 20.3 mmHg (P = 0.004). There was
no procedural mortality or sustained heart block.

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Two patients had moderate-to-severe atrioventricular valve regurgitation, and one required surgical repair. At last follow-up, all VSDs remained
patent but with recurrent obstruction in the
majority of cases caused by muscular hypertrophy
beyond the stent margins [38].

Management algorithm of SV patients with

SVOTO
Management strategy of patients at potential
SVOTO risk should be carefully planned beginning at the time of 1st palliative procedure and
individualized to patients anatomy (Fig. 7). Decision-making in neonates depends on the presence
of aortic coarctation/hypoplastic aortic arch, size
of VSD/bulboventricular foramen and presence
of subaortic obstruction. In neonates with small
bulboventricular foramen diameter less than aortic annulus or bulboventricular foramen area index less than 2 cm2/m2, or with evidence of aortic
or subaortic obstruction, neonatal DKS + aortopulmonary shunt is indicated. In those patients
with associated aortic coarctation/hypoplastic aortic arch, the Norwood operation is performed. In
patients in whom bulboventricular foramen is
large and who have no evidence of subaortic
obstruction or aortic coarctation/hypoplastic aortic
arch, it is justified to perform a PAB followed by
close echocardiographic follow-up and a planned
DKS at time of BCPC. In patients in whom bulboventricular foramen is large and who have no evidence of aortic or subaortic obstruction but with
aortic coarctation/hypoplastic aortic arch, initial
Norwood palliation is indicated, although some
surgeons report acceptable results of PAB and
aortic coarctation repair with close echocardiographic follow-up and a planned DKS at the time
of BCPC. In our experience, aortic coarctation in
this subset of patients is often associated with severe hypoplastic aortic arch, and adequate arch
repair via a thoracotomy is often not possible thus
necessitating a Norwood operation in the majority
of patients. VSD/bulboventricular foramen
enlargement should be reserved for patients with
severe pulmonary regurgitation following PAB,
DORV with non-committed VSD, those with pulmonary stenosis or atresia, and, of course, older
SV patients with SVOTO. Finally, the role of hybrid 1st stage palliation with ductal stenting and
branch pulmonary artery banding is evolving
but the advantage in those favorable nonhypoplastic left heart syndrome patients is yet to
be proven considering their improved outcomes
in the current era with the Norwood operation.

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Disclosure
Author has nothing to disclose with regard to
commercial support.

[16]

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