Indian J Thorac Cardiovasc Surg (April–June 2011) 27(2):83–87
DOI 10.1007/s12055-011-0096-2
ORIGINAL ARTICLE
Single stage versus two stage repair for univentricular
heart—our experience
Sadashiv Tamagond & Saket Agarwal & Shashi Prakash &
Sanjeev Singh & Deepak Kumar Satsangi
Received: 30 October 2010 / Revised: 22 February 2011 / Accepted: 13 March 2011 / Published online: 5 May 2011
# Indian Association of Cardiovascular-Thoracic Surgeons 2011
Abstract
Background Definitive palliation for complex cardiac
anomalies with a functional single ventricle usually
involves different modifications of Fontan operation/ Total
Cavopulmonary Connection (TCPC). However, whether it
should be done as one-stage procedure or two-stage
procedure with initial bidirectional Glenn shunt remains
an area of debate though many recent studies have shown
benefit of two stage procedure particularly in high risk
cases.
Objective This retrospective study has been undertaken to
compare the operative outcome and morbidity following
one stage TCPC with that of two stage TCPC with
preliminary BDG in our setup.
Material and methods We retrospectively reviewed the
clinical records of all the patients of single ventricle
physiology who underwent cavopulmonary connections
(TCPC) at our institution during the period of January 2001
to December 2009. 15 patients (9 male and 6 female)
underwent single stage TCPC. 31 patients underwent initial
Bidirectional Glenn (BDG) procedure of which 24 patients
(14 male and 10 female) underwent completion TCPC
6 months to 26 months later (two-stage TCPC group). For
all the patients in the two study groups various peri-operative
parameters were analysed including cardiopulmonary bypass
S. Tamagond : S. Agarwal : S. Prakash : S. Singh :
D. Kumar Satsangi
Department of Cardio Vascular & Thoracic Surgery,
G. B. Pant Hospital,
New Delhi, India
S. Singh (*)
Department of Cardiothoracic & Vascular Surgery,
G B Pant Hospital,
New Delhi 110002, India
e-mail: sadashiv18@yahoo.com
duration, ventilation time, Intensive Care Unit (ICU) stay,
hospital stay, postoperative pleural drainage and other compli-
cations rate, in-hospital mortality and TCPC takedown.
Results Overall mortality in two-stage TCPC is lower than
one-stage TCPC (4.2% in two-stage TP vs 13.3% in one-
stage TCPC). There is no mortality with initial BDG in
two-stage TCPC. TCPC has to be taken down in 2 patients
in one-stage TCPC. In two-stage TCPC completion TCPC
could not be carried out in 5 patients due to high pulmonary
artery pressure and small pulmonary arteries. Others
postoperative outcomes are comparable in both groups.
Conclusion Two-stage TCPC with intervening bidirectional
Glenn shunt can offer a higher probability to proceed
successfully to complete palliation in patients with single
ventricle.
Staging the TCPC also lowers the postoperative mortality
in the present study.
Keywords Glenn shunt . Cardiopulmonary bypass .
Sternotomy
Introduction
Nearly 10% of congenital heart defects belong to the group
of functionally univentricular hearts, a heterogeneous group
of cardiac malformations usually characterized by a
dominant ventricle of either left or right ventricular
morphology and usually a fatal course in the neonatal
period or in early infancy. However many of these patients
were benefited when Fontan and Baudet in 1971 [1]
introduced the principle of bypassing the right heart by
diverting the systemic venous blood directly to the
pulmonary arteries with the functional single ventricle
supporting the systemic circulation. Since then significant
84
modifications of this technique have been developed and in
1988 de Leval etal [2] and Jonas and Castaneda [3]
developed the technique of total cavopulmonary anasto-
mosis/connection (TCPC) which is mostly used nowadays
for managing these complex cardiac malformations. One of
the major improvements of the last decade or so resulting in
a significant decrease in morbidity and mortality following
modified Fontan/TCPC procedures has been the concept of
staging by a preceding Bidirectional Glenn procedure
(BDG). High-risk Fontan candidates who have undergone
BDG before TCPC have shown excellent results and in
many cases BDG preceding TCPC may extend the
indications for the Fontan procedure [4–7]. The exact
mechanism for the superiority of BDG, though, still poorly
understood may be the fact that volume reduction of BDG
preceding TCPC allows for any afterload mismatch to be
corrected, thereby improving ventricular energetics after
TCPC [8].
This retrospective study has been undertaken to compare
the operative outcome and morbidity following one stage
TCPC with that of two stage TCPC with preliminary BDG
in our setup.
Patients & methods
In the present study we retrospectively reviewed the clinical
records of all the patients of single ventricle physiology
who underwent cavopulmonary connections (TCPC) at our
institution during the period of January 2001 to December
2009. Of the total of 46 patients so identified, 15 patients (9
male and 6 female) underwent single stage TCPC. 31
patients underwent initial Biderectional Glenn procedure
(BDG) procedure of which 24 patients (14 male and 10
female) underwent completion TCPC 6 months to
26 months later (two-stage TCPC group). The patients in
the single stage TCPC group underwent operation at the
mean age of 3.8 yrs (range 1.8–12 yrs) while in the staged
group patients underwent the bidirectional Glenn shunt at
an average age of 3.1 years (age range, 1.0 to 10.2 years)
and completion TCPC procedure at the mean age of
4.3 years (age range 2.5 to 12.6 years). The main diagnoses
for patients undergoing total cavopulmonary connections in
the two groups are presented in (Table 1). In both groups
tricuspid atresia was the leading cause of single ventricle
physiology. Clinical records of all the patients in the present
study were evaluated to obtain important preoperative
variables which were considered to have an impact on
final postoperative outcome. These preoperative parameters
included age at operation, bodyweight, arterial oxygen
saturation, venrticular morphology of functional single
ventricle, functional status of single ventricle, pulmonary
artery hypoplasia (Nakata index), Pulmonary Artery (PA)
Indian J Thorac Cardiovasc Surg (April–June 2011) 27(2):83–87
Table 1 Main diagnoses in patients undergoing TCPC in the two
groups
Diagnoses One stage Two stage
TCPC (n=15) TCPC (n=24)
Tricuspid atresia 6 (40%) 11 (45.8%)
Unbalanced complete atrio- 3 (20%) 5 (20.8%)
ventricular septal defect
Ebstein anomaly with severe 1 (6.7%) 0
right ventricle hypoplasia
Double outlet right ventricle 1 (6.7%) 2 (8.3%)
with malposed great arteries
Mitral atresia 0 1 (4.2%)
Other complex functional 1 (6.6%) 3 (12.5%)
right single ventricle
Other complex functional 3 (20%) 2 (8.3%)
left single ventricle
mean pressure, pulmonary vascular resistance and atrio–
ventricular valve regurgitation.
Primary TCPC were routinely done with standard
midline sternotomy on beating heart normothermic cardio-
pulmonary bypass with high Superior Vena Cava (SVC)
canulation at the junction of SVC with innominate vein.
Both SVC and Inferior Vena Cava (IVC) and Right
Pulmonary Artery (RPA) and Left Pulmonary Artery
(LPA) were adequately mobilized. Superior vena cava was
divided at the level of RPA between vascular clamps and
the proximal right atrial end of superior vena cava closed
primarily with running polypropylene 6–0 sutures with care
being taken to avoid injury to sino-atrial node. Distal end of
transacted superior vena cava was then anastomosed end to
side on the superior aspect of the central portion of fully
mobilised RPA using two running polypropylene 6–0
sutures. The procedure was repeated on the left side when
there was a left SVC. Adequately mobilized IVC was
transacted between vascular clamps with distal right atrial
end oversewn with prolene 5–0 running suture. Proximal
part of IVC was then connected to inferior surface of RPA
using 18 mm Dacron vascular tube graft. Fenestration was
created in all cases as per institutional policy. Fenestration
in tube graft was done by 4 mm coronary punch and it was
connected to right atrium with a 4 mm separate tube graft.
Main pulmonary trunk was routinely suture ligated. In two
stage TCPC, the SVC to RPA anastomosis had already been
performed during initial BD glenn operation. Previous
anastomotic site was carefully mobilized and inferior
surface of RPA was cleared. After adequately mobilizing
IVC it was transected and connected to inferior surface of
right pulmonary artery using a extracardiac Dacron vascular
tube graft in the usual manner. Main Pulmonary Artery
(MPA) was ligated if it was not at the time of initial BDG.
For all the patients in the two study groups various peri-
operative parameters were analysed including cardiopulmonary
Indian J Thorac Cardiovasc Surg (April–June 2011) 27(2):83–87 85

bypass duration, ventilation time, Intensive Care Unit (ICU)
stay, hospital stay, postoperative pleural drainage and other
complications rate and operative failure. Operative failure was
defined as death or take down of the TCPC operation to the
other form of palliation (BDG). All the patients who were
discharged were followed up in outpatient department at
regular interval.
Statistical analysis was performed with the SAS soft-
ware, version 8.2 (SAS Inc). Continuous variables were
expressed as means ± standard deviation and were analyzed
using the two tailed student t test. Categorical variables
were presented as frequencies and percentage and were
analyzed with the x2 test or Fisher exact test when
appropriate. A p value of 0.05 or less was considered
statistically significant.
Results
The preoperative characteristics of the patients in both
groups are presented in (Table 2). Patients in both groups
were similar with respect to age at the time of TCPC (mean
3.8 yrs in group I vs 4.3 yrs in group II), bodyweight (mean
16.8 kg in group I vs 18.2 kg in group II), arterial oxygen
saturation ( mean 78% in group I vs 82% in group II) and
pulmonary artery size (mean Nakata index 337 in group I
vs 301 in group II pre BDG ).In one stage TCPC 26.7%
patients (n=4) had right ventricle as Functional Single
Ventricle (FSV) while in 73.3% patients (n=11) had left
ventricle as functional single ventricle. In two stage TCPC
33.3% patients (n=8) had right ventricle as FSV while in
66.7% patients (n=16) had left ventricle as functional
single ventricle. Two stage TCPC group had more risk
factors before initial BDG procedure compared to one stage
TCPC - ventricular dysfunction was present in 6 case
(19.3%) in two stage TCPC compared to 1 case(6.7%) in
one stage TCPC, PA mean pressure ≥17 mm Hg in 11cases
(35.5%) in two stage TCPC vs 2 cases (13.3%) in one stage
TCPC, Pulmonary vascular resistance ≥3 Woods unit in 9
cases (29%) in two stage TCPC vs 2 cases (13.3%) in one
stage TCPC, mean Ventricular end-diastolic pressure
7.9 mm Hg in two stage TCPC vs 5.3 mm Hg in one stage
TCPC and more than mild atrio-ventricular valve regurgi-
tation in 9 cases (29%) in two stage TCPC vs 1 case (6.7%)
in one stage TCPC. In two stage TCPC after initial BDG,
systemic arterial oxygen saturation increased from 76.2±
4.2% to 82.1±3.6% while there were decrease in pulmo-
nary arterial size (mean Nakata index 267 vs 301), mean
pulmonary arterial pressure (11.3 vs 13.2 mm Hg),
pulmonary vascular resistance (mean 1.86 vs 1.91 Woods
unit), Ventricular end-diastolic pressure (mean 4.8 vs
7.9 mmHg and decrease in more than mild atrio-ventricular
valve regurgitation (12.5% vs 29%). There was no mortality
after BDG in two stage TCPC. Completion TCPC could not
be carried out in 3 patients due to high pulmonary artery
pressure and in 2 patients for small pulmonary arteries.
However, these patients’ symptoms have been relieved by the
bidirectional Glenn operation and were maintaining systemic
arterial oxygen saturation of more than 80%. 2 patients with
initial BDG were lost to follow up.

Table 2 Preoperative patient characteristics

Variables One stage TCPC (n = 15) Two stage TCPC

Pre BDG (n=31) Pre TCPC (n=24)

Age (yrs) 3.8 (1.8–12) 3.1 (1–10.2) 4.3 (2.5–12.6)

Females 6 (40%) 12 (38.7%) 10 (41.7%)
Bodyweight(kg) 16.8 (9.6–36.4) 15.9 (8.7–31.3) 18.2 ( 10.1–38.1)
Arterial oxygen saturation (%) 78±3.8 76.2±4.2 82.1±3.6
venrticular morphology of functional single ventricle (FSV)
Right ventricle 4 (26.7%) 11 (35.5%) 8 (33.3%)
Left ventricle 11 (73.3%) 20 (64.5%) 16 (66.7%)
ventricular dysfunction of functional single ventricle (FSV) 1 (6.7%) 6 (19.3%) 2 (8.3%)
pulmonary artery size (Nakata index) 337±154 301±186 267±128
Nakata index<200 mm2/m2 3 (20%) 10 (32.3%) 11 (45.8%)
Pulmonary artery mean pressure (mm Hg) 11.8±3.6 13.2±5.1 11.3±3.4
Pulmonary artery mean pressure≥17 mm Hg 2 (13.3%) 11 (35.5%) 2 (8.3%)
pulmonary vascular resistance (Woods unit) 1.7±0.8 1.91±1.2 1.86±1.0
Pulmonary vascular resistance≥3 Wood unit 2 (13.3%) 9 (29.0%) 3 (12.5%)
Ventricular end-diastolic pressure (mm Hg) 5.3±2.4 7.9±3.1 4.8±2.6
Atrio-ventricular valve regurgitation (more than mild) 1 (6.7%) 9 (29.0%) 3 (12.5%)
86
The operative outcomes in both groups of TCPC are
presented in (Table 3). The cardiopulmonary bypass time
was significantly shorter in the staged group (mean 162
minutes) than in the primary group (mean 138 minutes)
because the bidirectional Glenn shunt had already been
made in this groups. After completing the TCPC, almost
half of the patients required inotropic support in both
groups and there was no significant difference in the
duration of inotropic support (1.6±3.1 days in two stage
TCPC vs 1.8±2.3 days in one stage TCPC). The two
groups were also similar with respect to duration of
mechanical ventilation (18.1±9.3 hrs in two stage TCPC
vs 19.2±11.6 hrs in one stage TCPC), incidence of
perioperative arrhythmias (20.8% in two stage TCPC vs
26.7% in one stage TCPC), duration of pleural drainage
(4.9±9.2 days in two stage TCPC vs 5.3±5.4 days in one
stage TCPC), postoperative arterial oxygen saturation
(90.3±6.1% in two stage TCPC vs 91.6±5.3% in one
stage TCPC), duration of intensive care stay (4.3±5.1 days in
two stage TCPC vs 4.8±3.1 days in one stage TCPC)
and total hospital stay (12.7±3.9 days in two stage TCPC
vs 12.1±4.5 days in one stage TCPC). In one stage
TCPC group in two patients (13.3%) TCPC had to be
taken down because of systemic desaturation and they
were converted to BDG 6 hours and 12 hours later. Overall in
hospital mortality in two stage TCPC was 4.2% (n=1)
compared to 13.3% (n=2) in one stage TCPC. 1 patient with
one stage TCPC died due to postoperative bleeding following
re-exploration, 2nd died on 9th postoperative day due to
sepsis and multi-organ dysfunction. 1 patient in two stage
TCPC died due to acute lung injury with endotracheal bleed.
Discussion
In the current era, definitive palliation for complex cardiac
anomalies with a functional single ventricle usually involves
different modifications of Fontan operation and of these Total
Cavopulmonary Connection (TCPC) with extracardiac con-
Table 3 Operative outcomes in both TCPC groups
Variables One stage TCPC (n=15)
Mechanical ventilation (hrs) 19.2±11.6
Inotropic support (days) 1.8±2.3
Postoperative arrhythmias 4 (26.7%)
Pleural drainage duration (days) 5.3±5.4
Arterial oxygen saturation (%) 91.6±5.3
Intensive care stay duration (days) 4.8±3.1
Hospital stay duration (days) 12.1±4.5
TCPC takedown 2 (13.3%)
In hospital mortality 2(13.3%)
Indian J Thorac Cardiovasc Surg (April–June 2011) 27(2):83–87
duit is an attractive option. One of the major improvements of
the last decade or so resulting in a significant decrease in
morbidity and mortality following modified Fontan/TCPC
procedures has been the concept of staging the TCPC by a
preceding Bidirectional Glenn procedure (BDG). Norwood et
al in 1993 stated that the total cavopulmonary connection
could be better managed in two steps to allow better diastolic
function of the single ventricle [9]. This first step in
separating systemic venous return from the heart is aimed
at reducing volume loading of the dominant ventricle which
is destined to support systemic circulation and also to
decreases operative risk in a subsequent Fontan procedure
and ensures better long-term ventricular function.
In the present study we have found good results with two-
stage TCPC compared with one-stage TCPC despite two-
stage TCPC having more of risk factors. Overall mortality in
two-stage TCPC is lower than one-stage TCPC (4.2% in two-
stage TP vs 13.3% in one-stage TCPC). There is no mortality
with initial BDG in two-stage TCPC. TCPC has to be taken
down in 2 patients in one-stage TCPC. In two-stage TCPC
completion TCPC could not be carried out in 3 patients due to
high pulmonary artery pressure and in 2 patients with small
pulmonary arteries. Others postoperative outcomes are com-
parable in both groups. Our findings in the present study are
consistent with many other previous studies.
Norwood et al [9] reported an excellent early mortality
rate of 7.6% in a staged completion of the Fontan operation,
but a primary Fontan operation had quite a high early
mortality rate of 16%. Similar series at both the University
of Michigan [5] and the University of Utah [10] have also
reported good results in performing the staged Fontan
operation with an operative mortality rate of less than 8%.
Lee and colleague in their study have found a lower
actuarial mortality (16.3 vs. 41.3%, p, 0:001) for the Fontan
operation in the group in which the Fontan procedure was
finished via Bidirectional Cavopulmonary Shunt (BCPS)
than in the group finished without BCPS [11]. Masuda et al
[7] reported a lower mortality and the higher probability to
complete the Fontan procedure when using a preliminary
Two stage TCPC (n=24) p value
18.1±9.3 0.745
1.6±3.1 0.830
5 (20.8%) 0.711
4.9±9.2 0.879
90.3±6.1 0.500
4.3±5.1 0.734
12.7±3.9 0.662
0 -
1 (4.2%) 0.547
Indian J Thorac Cardiovasc Surg (April–June 2011) 27(2):83–87
BDG. On the contrary, Kostelka et al [12] in their study of
75 patients have found higher mortality of 14.2% vs. 7.4%
in the group of two stage TCPC than in the primary TCPC
group. The mortality differed in three preoperative risk
groups: in low risk group 4% vs. 0%, in moderate risk
group 6% vs. 0%, and in high risk group 15% vs. 75% in
primary correction group and two stage group, respectively.
Particularly, BDG as initial palliative procedure towards
staging the TCPC has been considered for those patients at
high risk for primary TCPC because of low age, significant
pulmonary artery hypoplasia, elevated mean pulmonary
artery pressure and pulmonary resistance, significant ven-
tricular hypertrophy and impaired ventricular function.
Many previous studies have shown excellent results in
high-risk Fontan candidates who have undergone BDG
before TCPC and in many cases BDG preceding TCPC
may extend the indications for the Fontan procedure [4–7].
Staging the TCPC offers effective pulmonary blood flow
with stepwise adaptation of ventricular geometry to the
reduction in volume load [8] Recent studies have focused
on the impaired diastolic ventricular function early after the
Fontan/TCPC procedure [13, 14]. After the completion of
Fontan circulation, the ventricular end-diastolic volume
decreases suddenly and markedly with a constant ventricular
muscle mass resulting in relative hypertrophy of the ventricle.
This inappropriate ventricular hypertrophy is supposed to
impair the diastolic ventricular filling that causes an increase
in the pulmonary venous pressure and systemic venous
pressure associated with a low cardiac output. The staged
Fontan procedure may avoid the deleterious effect of a sudden
decrease in the diastolic ventricular volume, which might be
fatal, particularly in some high risk patients [7]. Although it
had been shown that pulmonary artery significantly
diminishes in size after a bidirectional Glenn shunt, the
changes in the pulmonary artery size in the staged group
seemed to have no significant impact on the operative results
of the Fontan procedure [7]. Masuda and colleagues [5] claim
that small Pulmonary Artery Index; Nakata index (PAI) is
not such an important a risk factor of Fontan completion,
since the staged approach became the main strategy.
However, many of us are reluctant to perform TCPC for a
patient whose PAI is 150 or less, as it is shown that the
smaller the PAI before TCPC, the higher the CVP after
TCPC procedure [15]. Mendelsohn et al [16] reported a
reduction in the pulmonary artery size more than 15 months
after a bidirectional shunt. We concur with their findings and
intend to complete the Fontan procedure within 18 months
after a bidirectional Glenn shunt, whenever possible.
In terms of long-term survival according to the various
staged approaches, little data is available even though a
number of series have reported superior or comparable results
for the staged Fontan operation with an early mortality of less
than 8% compared with that of the primary Fontan operation
87
[5, 7, 9, 15]. Present study is limited in this respect as it has
no long term survival statistics to demonstrate long term
benefit of staged TCPC. However it can be said that two-
stage TCPC with intervening bidirectional Glenn shunt can
offer a higher probability to proceed successfully to
complete palliation in patients with single ventricle. Staging
theTCPC also lowers the postoperative mortality in the
present study, particularly in high risk cases.
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