Ref. 172/05 Article No.

9
June - 2005

Symposium on Pediatric Cardiology-I

Univentricular Heart: Management Options

Usha Krishnan

Pediatric Cardiology, NY Medical College, Maria Fareri Children Hospital, Valhalla, NY.

Abstract. The term Univentricular Heart encompasses a wide variety of heart defects that functionally and physiologically
constitute a single ventricular chamber. The terminology univentricular repair is frequently used in the surgical literature to
include those biventricular hearts that are not amenable for a final two ventricle repair and need to go through the same surgical
stages as with a functionally univentricular heart, culminating finally in a total cavo-pulmonary connection. Broadly, treatment
is focused on controlling the pulmonary blood flow in early infancy, by means of aorto-pulmonary shunting in pulmonary atresia
or stenosis, and pulmonary artery banding or pulmonary artery disconnection with aorto-pulmonary shunt placement in high
pulmonary blood flow situations. Concomitant repair of other associated conditions is required. Babies with hypoplastic left heart
physiology undergo staged Norwood repair resulting in an eventual total cavo-pulmonary connection with the RV functioning
as the systemic ventricle. In this review, medical and surgical management of these patients will be discussed, after a brief
discussion of nomenclature, anatomic and physiologic considerations.
[Indian J Pediatr 2005; 72 (6) : 519-524] E-mail: usha_krishnan @nymc.edu

Key words: Univentricular heart; Superior and total cavo-pulmonary connections; Norwood repair; Bi-directional Glenn and
Fontan procedures.

Univentricular heart (UVH) is a term used to describe a
wide variety of structural cardiac abnormalities associated
with a functional single ventricular chamber.1 There has
been considerable controversy over the nomenclature and
classification of these complex conditions.1-4 Van Praagh et
al define UVH as one ventricular chamber that receives
both tricuspid and mitral valves or a common
atrioventricular (AV) valve. 2 Anderson et al suggest that
the entire atrioventricular junction is connected to one
ventricular mass, thus including hearts with tricuspid or
mitral atresia, which are excluded in Van Praaghs
definition.1,4 The consensus of the STS- Congenital Heart
Surgery and the European study group database
proposed that the nomenclature of UVH should include
double inlet left and right ventricles, absence of one AV
connection, common AV valves, and hearts with only one
well developed ventricle as unbalanced AV canal and
complex conditions with heterotaxy syndromes.3 This
incorporates both the segmental approach by Van Praagh
and the sequential approach by Anderson, and uses a
descriptive format to define the anatomy in such patients.
This nomenclature does not include hypoplastic left heart
syndrome (HLHS), pulmonary atresia with intact
ventricular septum and certain other biventricular hearts
which also undergo the stages of univentricular repair.
Medical management of these patients requires a
thorough understanding of the physiology of these
conditions which in turn is dependent on the anatomy.
The reader is referred to various excellent textbooks and
Correspondence and Reprint requests : Dr. Usha Krishnan, Asst
Professor, Pediatric Cardiology, NY Medical College, Maria Fareri
Childrens Hospital, 618 Munger Pavilion, Valhalla, NY 10595.
Fax : 1 914 594 4513
review articles for morphology and classification of UVH.
The physiology of UVH in a neonate depends upon
certain key anatomical factors.
These are:
1. Obstruction to systemic or pulmonary outflows.
2. Obstruction to ventricular inflow and atrial septum
and abnormal systemic or pulmonary venous return.
3. Amount of pulmonary blood flow and pulmonary
vascular resistance.
4. A-V valve regurgitation.
OBSTRUCTION TO SYSTEMIC OUTFLOW
When there is severe obstruction to systemic outflow
(HLHS and its variants, UVH with severe aortic valve or
arch obstruction), the neonate is dependent on the ductus
arteriosus for maintaining systemic output. If the
diagnosis is made antenatally, delivery should be
conducted at a center capable of neonatal surgery, and the
baby started on prostaglandin E2 (PGE2) immediately
after birth, in order to maintain ductal patency. If
undiagnosed previously, these infants often have a
catastrophic presentation, with severe acidosis and shock,
secondary to ductal closure and loss of cardiac output.
Therefore, a high index of suspicion is necessary to seek
out congenital heart disease as a cause of such
presentation, and to differentiate it from sepsis or
neonatal metabolic problems leading to acidosis and
shock. These babies are usually asymptomatic during the
first few days of life until the ductus closes down. A
careful 4 limb blood pressure evaluation done prior to
discharge from the newborn nursery may reveal a
discrepancy between arms and legs, and suggest further

Indian Journal of Pediatrics, Volume 72June, 2005 519

 Usha Krishnan
evaluation. Four limb pulse oximetry is an important
investigation and an oxygen saturation difference
between the upper and lower limbs suggests pulmonary
origin of the leg blood supply, even before ductal closure
and BP differences become apparent. This would facilitate
diagnosis of these patients at an appropriate time, even
before the PDA closes and clinical deterioration sets in.5
Physiologically, there is complete mixing of blood within
the heart at the atrial and ventricular levels, with all the
blood ejected through the pulmonary valve and
distributed to the pulmonary and systemic beds by the
pulmonary artery branches and PDA respectively. 6 The
balance of resistances in these 2 circulations determines
the blood flow.
OBSTRUCTION TO PULMONARY OUTFLOW:
UVH with critical pulmonary outflow obstruction
(usually in the form of pulmonary atresia) is an important
ductal dependent neonatal congenital heart disease. If
associated with heterotaxy syndromes, there may be other
critical associated abnormalities of systemic and
pulmonary venous return and systemic abnormalities.1,2
Presentation is usually not as catastrophic as with HLHS,
as the neonatal cyanosis is often apparent as the PDA
starts to close, and diagnosis may be made prior to
hospital discharge. Pulse oximetry done prior to hospital
discharge will detect cyanosis before it gets apparent to
the naked eye and suggest further evaluation before
cardiac decompensation occurs.5 In this situation, there is
complete mixing of blood within the heart and the degree
of cyanosis depends on the severity of pulmonary
stenosis. Once the ductus is opened using PGE2, the
pulmonary and systemic circulations become
interdependent on each other, and the resistances in the
two circulations determine the amount of flow.
Additionally, anatomic obstructions along the pulmonary
vascular tree would further influence pulmonary blood
flow and distribution.
OBSTRUCTION TO SYSTEMIC OR PULMONARY
VENOUS RETURN OR VENTRICULAR INFLOWS:
Systemic and pulmonary venous abnormalities are often
seen in association with other complex congenital cardiac
anomalies and especially with heterotaxy syndrome. 7,8
Pulmonary venous obstructions cause severe pulmonary
hypertension because of backpressure into the pulmonary
capillary bed and need to be detected early and corrected
during surgery.8 Severe mitral or tricuspid stenosis or
atresia may be a component of the UVH. In order to
ensure unobstructed communication between the two
venous inflows and the single ventricular chamber, an
unrestricted atrial septal defect is necessary for egress of
blood from the atrial chamber with the obstructed valve.
In the presence of mitral atresia, a restrictive ASD acts
physiologically similar to pulmonary venous obstruction
520
and leads to elevated pulmonary vascular resistance from
back pressure. Similarly, in tricuspid atresia, a restrictive
ASD causes signs of systemic venous obstruction. The
atrial septal defect needs to be widened either by balloon
septostomy prior to surgery or by surgical resection of the
atrial septum during the first stage of repair.
IDEAL PULMONARY BLOOD FLOW.
An ideal patient with UVH should have good ventricular
function, unobstructed venous return, unrestrictive ASD
and optimal pulmonary blood flow.9 This situation is
rarely found in patients with UVH variants and PS, where
the amount of pulmonary blood flow is just enough to
prevent severe cyanosis as well as avoid development of
pulmonary vascular disease. Patients with tricuspid
atresia and pulmonary stenosis occasionally have
optimal pulmonary flow which allows for the patient to
wait and undergo a superior cavo-pulmonary connection
or even a direct Fontan procedure during early
childhood, without going through staged repair. Such
fortuitous situations are not common. In patients with
aortic obstruction, often pulmonary blood flow is
unrestricted and since the pulmonary and systemic
outputs are interdependent, this situation leads to
excessive pulmonary blood flow and consequently, low
cardiac output and hemodynamic decompensation.
Similarly, when the ductus is kept open, to supply either
the systemic or pulmonary circulation, the patients
pulmonary blood flow has to be very carefully fine tuned
in the pre-operative period in order to maintain
appropriate cardiac output.6
THE GOAL OF CORRECTIVE PROCEDURES
The single most important principle in UV circulation is
that the systemic and pulmonary circulations balance
each other and this critical balance is maintained by the
ratios of the respective resistances. The goal of initial
surgical palliation is to provide unobstructed systemic
outflow, restricted pulmonary blood flow to maintain
normal pulmonary pressures and unobstructed systemic
and pulmonary venous return to the heart.
Patients with UVH and pulmonary atresia or critical
stenosis undergo aortopulmonary shunting. If the
pulmonary vascular tree is suspected to be inadequate or
abnormal, a pre-operative angiography to delineate the
pulmonary arterial tree is performed. The smallest size
shunt that will allow enough pulmonary circulation to
maintain saturations in the low 80s is placed.
Management in the ICU involves ventilator care and
manipulating the systemic Vs pulmonary resistances
especially if a larger size shunt is placed. In such
situations, the baby may need diuresis and anti failure
management in the initial few months until the baby
grows into the shunt. Very small shunts run the risk of
acute blockade and thrombosis and usually babies with
Indian Journal of Pediatrics, Volume 72June, 2005
 Univentricular Heart: Management Options
shunts are maintained on aspirin for its anti-platelet effect.
A baby with pulmonary atresia and pulmonary
circulation solely via aortopulmonary collaterals is a
potentially difficult management situation as surgery
would entail unifocalization of the collaterals along with
staged procedures leading to a total cavo-pulmonary
connection (TCPC). Such procedures are exceedingly high
risk and may not be feasible or advisable in our setting.
Such babies may be referred for a heart- lung transplant in
the Western world and there is very high mortality
awaiting transplant as well as following it.
The patient then undergoes the next stage of
procedure, the Bi-directional Glenn or the superior cavo-
pulmonary shunting at around 6 months of age. Cardiac
catheterization is performed in many centers to evaluate
the pulmonary pressures and anatomy of the pulmonary
tree and to occlude aortopulmonary collaterals that may
complicate the post- Glenn course. Careful attention to the
systemic venous anatomy is necessary, especially
evaluation for the presence of a left sided SVC or a levo-
atrio-cardinal vein and its communication with the right
SVC is necessary (using a balloon occlusive injection in the
innominate vein). If such a vein is found, with adequate
communication with the SVC, it is closed at the time of
surgery. If there is no communicating vein, then a bilateral
bi-directional Glenn is performed, connecting the LSVC to
the LPA and RSVC to RPA. Any obstructions or
distortions to the pulmonary tree because of the previous
shunt or native anatomy are corrected during this repair.
Opinions vary on the advisability of leaving some
forward flow through the pulmonary valve creating a
pulsatile Glenn.10 Proponents argue that the pulsatility
allows continues normal growth of the pulmonary tree,
whereas opponents contend that the additional blood
flow hampers the smooth flow from the SVC to the
pulmonary arteries by increasing the pulmonary artery
pressures and creating competing flow. The final stage of
conversion from a parallel circulation to one in series
is the total cavo-pulmonary connection (TCPC) or Fontan
procedure. This is ideally performed between 18 months
to 4 years of age. Most anatomical details are obtained on
echocardiography, including a-v valve competence and
adequacy; inter-atrial communication adequacy, systemic
and pulmonary venous return and presence of collaterals.
Cardiac catheterization is performed in most centers to
evaluate the pulmonary pressures and resistances, to
occlude aorto-pulmonary or systemic venous collaterals
and evaluate ventricular function and end diastolic
pressures. TCPC can be performed using either extra
cardiac conduit between the IVC to the PA or an intra-
cardiac baffle. 11 The extra cardiac TCPC is now the
procedure of choice in most centers as studies have shown
a reduced incidence of new onset arrhythmias as
compared to the lateral tunnel procedure.11 A fenestration
is usually placed in between the conduit and the atrium to
allow for a pop off especially during the early post-
operative period, when the PA pressures may be higher
Indian Journal of Pediatrics, Volume 72June, 2005
and thus pressures in the venous circuit. This has been
shown to reduce the incidence and length of effusions
which are related to high venous pressures and early
Fontan failures. 12 Patients may run lower oxygen
saturations due to right to left shunting across the
fenestration. These fenestrations can be closed with
devices, using transcatheter techniques, months to years
later, once systemic venous pressures are demonstrated to
be low by cardiac catheterization. 13 Patients without
fenestrated TCPC may need a subsequent fenestration
performed because of long standing effusions. Some
centers perform a transcatheter completion of Fontan,
where a covered stent from the SVC to the IVC is used to
complete the Fontan baffle. 14,15 Such patients are
prepared during the Glenn or hemi Fontan stage, by
leaving the SVC to RA connection intact and patch closing
the atrial opening, for a future transcatheter completion.
Long term complications following TCPC include atrial
and ventricular arrhythmias, heart blocks needing dual
chamber pacing, protein losing enteropathy, development
of venovenous and arteriovenous communications,
worsening a v valve incompetence and ventricular failure
leading to death or needing cardiac transplantation.
Patients with unrestricted pulmonary as well as
systemic blood flow need initial palliation by means of
either PA banding or PA disconnection with creation of
an aortopulmonary shunt to limit pulmonary blood flow
and achieve normal pulmonary artery pressures. 16,18
Placing a band may lead to development of sub aortic
stenosis or narrowing of a bulboventricular foramen, and
may need to be tackled in the second stage surgery by
either sub aortic resection or a DamusKayeStansel
procedure.16 It has been shown that long standing PA
bands are associated with worse outcomes after the
Fontan procedure.18 It may be difficult to accurately judge
the effectiveness of a band in restricting pulmonary
circulation, especially in very young infants with high
pulmonary resistances. Even though such infants are
allowed to grow into their bands, the residual
pulmonary pressures and resistances may be high and
lead to a much worse outcome after completion of
Fontan.19 It has been suggested by Chowdhury et al that
patients with mean PA pressures more than 25mm and
resistance more than 4 wood units are poor candidates for
TCPC, with high morbiditiy and mortality, and the PA
banding should be considered as final palliation in such
patients.19
Systemic Outflow obstructions associated with UVH
usually necessitate Norwood Procedure or its variations
for repair. (Fig 1) The Norwood repair involves
conversion of the pulmonary artery into the systemic
artery or neo-aorta. The small native aorta is
anastomosed to the neo aorta and serves to carry blood
retrograde to the coronary arteries. The aortic arch is
augmented and then connected to the neoaorta. The
branch pulmonary arteries are transected and connected
to the systemic circulation by means of a Gore-Tex shunt.
521
 Usha Krishnan

Fig 1. Diagrammatic Representation of the Norwood Stage 1

surgery for Hypoplastic left heart syndrome, involving Fig 2. Diagrammatic Representation of the Hybrid Procedure,
connection between the PA and the dimunitive ascending Ductal stenting to create the arch, ASD stenting to keep the
aorta, arch repair and aorto-pulmonary shunting, with PA ASD open, bilateral branch PA banding.
distally disconnected.

Fig 4. Stage 3: Extracardiac total cavo-pulmonary connection

Fig 3. Norwood stage 2- Bilateral SVC-PA Connection (Glenn) (Fontan)

522 Indian Journal of Pediatrics, Volume 72June, 2005

 Univentricular Heart: Management Options
Fig. 5. Pulmonary angiogram revealing the stented ductal arch,
and banded branch Pas, constituting the hybrid procedure.
The smallest shunt size to supply enough pulmonary
blood flow to maintain normal PA pressures and systemic
circulation in the high 70s to low 80s is placed. The baby
is ventilated in the post operative period with a strategy
to maintain the PCO2 in the 50s and PO2 in the 40s range.
This ensures that the pulmonary arterial resistance is
maintained on the higher side, to prevent pulmonary over
circulation, which would be at the expense of systemic
output. A recent modification of this procedure,
popularized by Sano et al involves using a RV to PA
Gore-Tex shunt to supply the pulmonary arteries rather
than an aorto-pulmonary shunt.20 This modification has
been shown by Norwood himself, to improve the
outcome and make postoperative ventilation easier to
handle.21 The RV to PA shunt avoids the diastolic run-off
that happens in aorto-pulmonary shunts, which lead to
compromised coronary flow and poor ventricular
myocardial perfusion and ultimately death. 20,21 A
combined catheterization and surgical approach to
achieve the same goals has been performed in some
centers.22 In this hybrid approach, the neo-aortic arch is
created by stenting the ductus (Figure 2). This ensures
continued presence of a ductal arch to function as a
new systemic arch. The arch vessels and coronaries are
perfused by reterograde flow. The atrial septal defect is
widened by septostomy or stenting the ASD if required.
The branch pulmonary arteries are initially individually
banded in a closed heart procedure, to limit pulmonary
blood flow. This hybrid procedure offers the
physiological results of a Norwood surgical procedure,
without subjecting these ventricles to cardio-pulmonary
bypass, and the neonate to deep hypothermic circulatory
Indian Journal of Pediatrics, Volume 72June, 2005
Fig. 6. Same patient, after an ASD stent placement to keep the
closing foramen open.
arrest. To date, this procedure has been used in higher
surgical risk babies- namely, those with ventricular
dysfunction, late presentation in shock or prolonged
acidosis and those with very diminutive ascending aortas
where repair may be associated with very high mortality.
In the future, as further data is obtained, the risks and
benefits of this procedure will become more apparent.
The second stage of Norwood procedure, namely the
superior cavo-pulmonary connection is guided by the
same principles as previously mentioned. When the
transcatheter procedure is used for the first stage, the
second stage repair becomes more complex, involving
extensive aortic reconstruction, as well as pulmonary
artery reconstruction, and removal of the ductal (and
atrial) stents. The final stage of Norwood procedure is the
TCPC with resultant separation of the systemic and
pulmonary circulations. It must be noted that the long
term outlook for a single left ventricle is better than a
single ventricle of RV morphology.
In conclusion, the clinical presentation in patients with
a single ventricle depends on the physiology of the
systemic and pulmonary circulations. A thorough
knowledge of the anatomic variables involved,
anticipatory management of the problems involved is
required for improvement in outcome. Balance of the
systemic and pulmonary circulations to maintain cardiac
output and oxygenation is necessary both in the pre and
post-operative periods in the neonate with UVH.
Management of subsequent stages of repair has also
undergone multiple modifications, with the goal of
achieving a perfect palliation and prevention of long
term complications associated with the Fontan procedure.
523
 Usha Krishnan
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Indian Journal of Pediatrics, Volume 72June, 2005