Management of Older Single

Functioning Ventricles With Outlet Obstruction

due to a Restricted “VSD” in Double Inlet Left
Ventricle and in Complex Double Outlet Right Ventricle
Francois Lacour-Gayet

The occurrence of a restriction of the bulbo-ventricular foramen (BVF) in older patient

with double inlet left ventricle (DILV) or tricuspid atresia (TA) with ventriculo-arterial
discordance is a well-known condition. Today, the surgical management is to perform
a Damus-type operation at the time of the bi-directional Glenn or at the Fontan
completion. The ventricular septal defect (VSD) enlargement, associated with muscular
resection and a patch enlargement of the subaortic accessory ventricular chamber, is
rarely performed but remains indicated in cases with pulmonary valve atresia or
regurgitation. This condition is essentially prevented by doing an early Norwood-type
operation in the presence of DILV/TA with transposition of the great arteries associated
with an aortic arch obstruction. The palliative switch operation is an option that was
abandoned because of poor control of the pulmonary blood flow. It is only in cases of
large unobstructed BVF that pulmonary artery banding could be undertaken in neo-
nates, followed by close echocardiographic follow-up. The occurrence of a restriction
or a closure of the VSD in complex DORV following a Fontan operation is a dramatic
event and is quite “new business.” It has been recently recognized that the VSD
becomes restricted in a number of patients with DORV-nc-VSD treated with a Fontan
palliation. This new condition is not surprising knowing that 75% of the VSDs must be
enlarged preventively in DORV-nc-VSD repair. In the setting of a Fontan circulation, the
supra-systemic left ventricle has severe consequences the right ventricle performance.
Attempts at surgical VSD enlargement or catheter-based procedures have resulted in
almost constant recurrence. This recently reported complication is in favor of also
performing a VSD enlargement at the time of the Fontan completion in complex DORV.
It justifies the biventricular repair in complex DORV with two viable ventricles.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 12:130-132 © 2009 Elsevier Inc.
All rights reserved.

T he occurrence of a systemic obstruction due to a restric-

tion of the bulbo-ventricular foramen (BVF) or of the
VSD is seen in two different groups of patients: (1) In double
inlet left ventricle (DILV) or tricuspid atresia (TA) with ven-
triculo-arterial discordance and (2) in complex DORV
treated by univentricular palliation.
The Denver Children’s Hospital, Aurora, CO.
Address correspondence to Francois Lacour-Gayet, MD, The Denver Children’s
Hospital, 13123 East 16th Ave, Aurora, CO 80045; E-mail: lacour-gayet.
francois@tchden.org
Management of a Restrictive
BVF in DILV or TA With
Ventriculo-Arterial Discordance
This lesion is typically seen in older patients, when the neo-
natal palliation of the DILV or TA with transposition of the
great arteries has been a pulmonary artery (PA) banding more
or less associated with aortic arch repair.1-5 It is an urgent
condition because the single left ventricle is blocked on both
outlets: the VSD and the PA banding. The presence of a
restricted VSD is seen either at the time of the bidirectional
Glenn or at the completion of the Fontan. The anatomic
lesions include a restriction of the VSD/BVF and a hypertro-

130 1092-9126/09/$-see front matter © 2009 Elsevier Inc. All rights reserved.
doi:10.1053/j.pcsu.2009.01.014
Management of older single functioning ventricles
phy of the accessory right ventricular chamber that creates a
second obstruction. Diagnosis relies on the echocardiogram.
Magnetic resonance imaging also provides excellent images.
Most frequently, these patients have a catheterization that
will show the gradient across the VSD/BVF. Elevation of the
left atrial pressure is responsible for some degree of the pul-
monary hypertension, which has the effect of decreasing the
gradient through the PA banding (the clinical presentation
often being a PA banding that is too loose).
A similar hemodynamic condition is found in patients
with moderately unbalanced atrio-VSD following neonatal
PA banding. When the left ventricle fails to grow, which is
almost constant, the univentricular palliation is at risk for
systemic obstruction at the level of the VSD. A Damus-
Kaye procedure needs to be performed at the time of the
bi-directional Glenn.
Enlargement of the VSD
The enlargement of the VSD was advocated by several au-
thors in the 1990s,1-3,5 but is rarely used today.1 The VSD is
approached through a vertical ventriculotomy performed on
the ventricular accessory chamber below and distant from
the aortic annulus. When the accessory chamber of the DILV
is located on the right with a D loop (SDD), the BVF/VSD
could be enlarged superiorly as the conduction bundle is
located inferiorly.1 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 theoret-
ically be done inferiorly.5 An additional muscular resection of
the walls of the accessory chambers is needed and the ven-
triculotomy is enlarged using a patch of bovine pericardium.
The indication of a BVF enlargement remains valid in the
presence of an associated pulmonary valve atresia or regur-
gitation following a long-lasting PA banding.
The Damus-Kay-Operation
Today, the Damus-Kay-Operation is the operation of choice
to relieve a subaortic obstruction due to a restrictive VSD in
DILV and TA with transposition. The double-barrel Lamberti
modification6 provides a safe solution associated with a patch
enlargement of the distal ascending aorta.
Prevention of the Restriction of the BVF
in DILV/TA With Transposed Great Vessels
The occurrence of a subaortic obstruction in relation with a
restriction of the BVF should be considered as a complication
and should essentially be prevented in the management of
DILV/TA with ventriculo-arterial discordance. Today, most
centers will choose a neonatal Norwood-type operation on
DILV with aortic arch obstruction.7 When there is no arch
obstruction associated and when the BVF is larger than the
aortic annulus, it is justified to perform only a PA banding
followed by careful echocardiographic follow-up.5
Another solution in neonates is to perform a palliative
arterial switch.8 The caveat of this technique is to have poor
control on the pulmonary blood flow that is passively con-
trolled by the restrictive BVF.
131
Management of a Restrictive
or Obstructed VSD Following
a Fontan Operation Performed
on Complex DORV
The surgical management of complex DORV remains contro-
versial, comparing biventricular repair9-12 with Fontan pallia-
tion. The restriction of the VSD is a major issue in DORV13
because it induces a left ventricular obstruction with potential
lethal evolution. We recently reported12 a series of 23 bi-ventric-
ular repair of DORV where the VSD required enlargement pre-
ventively in half of the cases at the time of repair and in 75% of
DORV non-committed VSD, 40% of DORV-VSD, and 30% of
DORV-Fallot, respectively. Enlarging the VSD in DORV remains
a matter of concern in many centers and was suspected of in-
creasing mortality and morbidity and to impair long-term myo-
cardial function. These concerns on the long term have led many
centers to favor a univentricular palliation in patients with com-
plex DORV, even in the presence of two viable ventricles.
VSD also Becomes Restrictive in
Complex DORV Undergoing Fontan Operation
A recent report from Boston Children’s Hospital14 describes
eight patients presenting with severe LV obstruction follow-
ing Fontan done in complex DORV. All had hypertrophied,
hypertensive, supra-systemic “isolated LV chambers,” with
three patients having a LV false aneurysm. Three of these
eight patients had undergone four prior surgical attempts at
left ventricular decompression. All patients had a catheter-
based VSD enlargement or creation, and in five patients the
VSD was totally closed requiring a VSD creation. At last fol-
low-up, recurrent obstruction was observed in the majority
of cases caused by muscular hypertrophy beyond the stent
margins, requiring repeated intervention. As stated by the
authors14 “In the setting of double-outlet right ventricle re-
quiring single-ventricle palliation, left ventricular outflow
tract obstruction caused by progressive restriction at the VSD
poses an uncommon but recognized dilemma.”
Does the VSD also
need to be Enlarged at the
Time of the Fontan Procedure in
Patients With DORV and Non-committed VSD?
This report from Boston14 is quite alarming and further investi-
gations are needed to know the prevalence of later VSD restric-
tion or obstruction in the Fontan performed in DORV non-
committed VSD. Knowing the 75% need for a preventive VSD
enlargement in DORV-nc-VSD repair,12,13 it seems reasonable to
also enlarge the VSD at the time of the Fontan completion, when
the VSD diameter is smaller than the aortic annulus. The VSD
must be enlarged superiorly,12 as the VSD in DORV-nc-VSD is
not an inlet VSD but a peri-membranous VSD with the conduc-
tion tissue running inferiorly (Fig. 1). This anatomy finds con-
firmation in the 11 patients with DORV-nc-VSD, who had an
anterior VSD enlargement with no atrioventricular block.12
132
Figure 1 VSD enlargement should also be performed, when needed,
at the time of the Fontan completion in complex DORV. The inci-
sion is carried out superiorly; the conduction tissue being located
inferiorly in the DORV peri-membranous type VSD.
The restriction or closure of the VSD following Fontan
done in complex DORV is a tragic complication that is in
favor of a biventricular with VSD enlargement, when needed,
in DORV-nc-VSD with two viable ventricles.9,10
Acknowledgments
The author thanks Dr Steven Goldberg for the realization of
the drawing.
F. Lacour-Gayet
References
1. Cheung HC, Lincoln C, Anderson RH, et al: Options for surgical repair
in hearts with univentricular atrioventricular connection and subaortic
stenosis. J Thorac Cardiovasc Surg 100:672-681, 1990
2. Karl TR, Watterson KG, Sano S, et al: Operations for subaortic stenosis
in univentricular hearts. Ann Thorac Surg 52:415-416, 1991
3. Serraf A, Conte S, Lacour-Gayet F, et al: Systemic obstruction in uni-
ventricular hearts: Surgical options. Ann Thorac Surg 60:970-976,
1995
4. Franklin RC, Sullivan ID, Anderson RH, et al: Is banding of the pulmo-
nary trunk obsolete for infants with tricuspid atresia and double inlet
ventricle with a discordant ventriculoarterial connection? Role of aortic
arch obstruction and subaortic stenosis. J Am Coll Cardiol 16:1455-
1464, 1990
5. Hiramatsu T, Imai Y, Kurosawa H, et al: Midterm results of surgical
treatment of systemic ventricular outflow obstruction in Fontan pa-
tients. Ann Thorac Surg 73:855-860, 2002
6. Waldman JD, Lamberti JJ, George L, et al: Experience with the Damus
procedure. Circulation 78(suppl 3):32-329, 1988
7. Mosca RS, Hennein HA, Kulik, TJ, et al: Modified Norwood operation
for single left ventricle and ventriculoarterial discordance: An improved
surgical technique. Ann Thorac Surg 64:1126-1132, 1997
8. Lacour-Gayet F, Serraf A, Fermont L, et al: Early palliation of univen-
tricular hearts with subaortic stenosis and ventriculoarterial discor-
dance. The arterial switch option. J Thorac Cardiovasc Surg 104:1238-
1245, 1992
9. Lacour-Gayet F, Haun C, Ntalakoura K, et al: Biventricular repair of
double outlet right ventricle with non-committed ventricular septal by
VSD rerouting to the pulmonary artery and arterial switch. Eur J Car-
diothorac Surg 21:1042-1048, 2002
10. Lacour-Gayet F: Intracardiac repair of double outlet right ventricle.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 11:39-43, 2008
11. Artrip JH, Sauer H, Campbell DN, et al: Biventricular repair of double
outlet right ventricle: Surgical results based on the STS-EACTS Inter-
national Nomenclature classification. Eur J Cardiothorac Surg 29:545-
550, 2006
12. Goldberg SP, McCanta AC, Campbell DN, et al: Implications of incising
the ventricular septum in double outlet right ventricle and in the Ross-
Konno operation. Presented at the European Association of Cardiotho-
racic Surgery, 22nd Annual Meeting. Lisbon, Portugal, September,
2008 (in press)
13. Belli E, Serraf A, Lacour-Gayet F, et al: Surgical treatment of subaortic
stenosis after biventricular repair of double-outlet right ventricle.
J Thorac Cardiovasc Surg 112:1570-1580, 1996
14. Meadows J, Pigula F, Lock J, et al: Transcatheter creation and enlarge-
ment of ventricular septal defects for relief of ventricular hypertension.
J Thorac Cardiovasc Surg 133:912-918, 2007