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PEDIATRIC CARDIAC
Impact of Postoperative Hemodynamics in Patients
With Functional Single Ventricle Undergoing
Fontan Completion Before Weighing 10 Kg
Noritaka Ota, MD, Yoshifumi Fujimoto, MD, Masaya Murata, MD,
Yuko Tosaka, MD, PhD, Yujiro Ide, MD, Maiko Tachi, MD, Hiroki Ito, MD,
Ai Sugimoto, MD, and Kisaburo Sakamoto, MD
Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children’s Hospital, Shizuoka, Japan
Background. Although the Fontan procedure is now
being performed in younger patients, the influence of the
early timing of Fontan on midterm to long-term results
remains unclear. We investigated whether the timing of
Fontan completion affects subsequent hemodynamics in
patients with functional single ventricle followed for
more than 3 years.
Methods. Between January 1997 and December 2008, 163
patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) at a single
institution. The survivors routinely underwent postoperative catheterization at 1 year and then every 5 years after
TCPC and were divided into group A (weight < 10 kg; n ⴝ
65) and group B (weight > 10 kg; n ⴝ 97), and retrospectively reviewed.
Results. Mean follow-up was 6.6 ⴞ 3.7 years. Mean
weight and conduit size were 8.5 ⴞ 0.8 kg and 17.1 ⴞ 1.2
mm for group A versus 20.2 ⴞ 13.1 kg and 18.8 ⴞ 1.9 mm
for group B, respectively (p < 0.001). There were 3
hospital deaths (group A, n ⴝ 1; group B, n ⴝ 2) and 5 late
deaths (group A, n ⴝ 3; group B, n ⴝ 2). No TCPC was
taken down. There were no thromboembolic events in
either group. The end-diastolic volume of ventricle
(eDV) (% of normal) was (232.7 ⴞ 91.4 before TCPC
versus 139.3 ⴞ 57.2 5 years after TCPC; p < 0.001) in
group A and (209.6 ⴞ 77.7 before TCPC versus 136.7 ⴞ
61.4 5 years after TCPC; p < 0.001) in group B. Ventricular
ejection fraction and cardiac index at 5 years were similar
in both groups. The end-diastolic pressure of ventricle
(eDP) (mm Hg) at 1 year (p ⴝ 0.0037) and at 5 years (p ⴝ
0.047) was significantly lower in group A compared with
group B.
Conclusions. TCPC can be performed earlier with good
intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous for
preservation of future ventricular function.
(Ann Thorac Surg 2012;94:1570 –7)
© 2012 by The Society of Thoracic Surgeons
S
gate whether or not the timing of Fontan completion
affects the postoperative hemodynamics more than 3
years after Fontan completion. We would like to determine whether or not Fontan operations can be done as
safely and well in babies weighing less than 10 kg (about
1 year old) as can be accomplished later.
ince the first report of a successful Fontan operation
in 1971, this maneuver has been performed with
various modifications in patients with single-ventricle
physiology [1, 2]. Among these modifications, total cavopulmonary connection (TCPC), introduced by de Leval
and colleagues [3] in 1988, is widely accepted because it
improves late mortality and morbidity, especially in atrial
arrhythmia. The Fontan procedure is now being safely
performed in younger patients [4], although how the
timing of Fontan completion affects long-term outcome
and hemodynamics remains unclear.
In a previous study examining the timing of the Fontan
operation, our group found that younger patients experienced longer peritoneal drainage and were in the
hospital longer [5]. The present study was undertaken to
extend the observation of the initial cohort and investi-
Accepted for publication June 4, 2012.
Presented at the Forty-eighth Annual Meeting of The Society of Thoracic
Surgeons, Fort Lauderdale, FL, Jan 28 –Feb 1, 2012.
Address correspondence to Dr Sakamoto, Department of Cardiovascular
Surgery, Mt. Fuji Shizuoka Children’s Hospital, 860 Urushiyama Aoi-ku
Shizuoka, Shizuoka, 420-8660 Japan; e-mail: [email protected].
shizuoka.jp.
© 2012 by The Society of Thoracic Surgeons
Published by Elsevier Inc
Material and Methods
Study Design
We conducted a single-center retrospective review of the
medical records of 163 consecutive patients (83 boys and
80 girls) with functional single ventricle who underwent
TCPC at Mt. Fuji Shizuoka Children’s Hospital in Shizuoka, Japan from January 1997 to December 2008. The
institutional review board of the hospital approved this
study, and individual consent for the study was waived.
One patient who came from a foreign country was not
included in the study.
In accordance with our institutional policy, Fontan
candidates underwent cardiac catheterization before operation (n ⫽ 162 patients) and this was repeated in
survivors at 1 (n ⫽ 156), 5 (n ⫽ 84), and 10 (n ⫽ 22) years
0003-4975/$36.00
http://dx.doi.org/10.1016/j.athoracsur.2012.06.022
Ann Thorac Surg
2012;94:1570 –7
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FONTAN OPERATION IN INFANTS LESS THAN 10 KG
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Variable
Sex (F/M)
Age at Fontan operation (y)
Median
Mean
Body weight at Fontan operation (kg)
Median
Mean
Size of conduit (mm)
Long follow-up (catheter study ⬎ 5 y)
Main diagnosis
Heterotaxy
HLHS
DORV
Pulmonary atresia
Tricuspid atresia
Single ventricle
Others
Type of ventricle (RV/LV)
Concomitant anomaly
CAVV
TAPVR
(extracardiac type)
MAPCA
Hernia
Group A (⬍ 10 kg, n ⫽ 65)
Group B (⬎ 10 kg, n ⫽ 97)
p Value
34/31
46/51
0.63
1.5 (0.8–3.2)
1.6 ⫾ 0.5
5.1 (1.2–25)
7.3 ⫾ 6.0
⬍ 0.0001
8.7 (5.7–10)
8.5 ⫾ 0.8
17.1 ⫾ 1.2
32 (49.2%)
14.9 (10.1–66)
20.18 ⫾ 13.13
18.79 ⫾ 1.9
52 (53.6%)
⬍ 0.0001
⬍ 0.0001
0.42
16 (24.6%)
20 (30.8%)
5
7
3
7
7
45/20
30 (30.9%)
3 (3.1%)
12
4
12
19
17
53/44
0.48
⬍ 0.0001
0.43
0.20
0.11
0.18
0.43
0.072
21
20
11
0
1
37
23
6
2
0
0.505
0.365
0.037
0.52
0.40
CAVV ⫽ common atrioventricular valve;
DORV ⫽ double-outlet right ventricle;
HLHS ⫽ hypoplastic left heart syndrome;
MAPCA ⫽ major
aortopulmonary collateral artery;
RV/LV ⫽ right ventricle/left ventricle;
TAPVR ⫽ total anomalous pulmonary venous return.
after TCPC to assess Fontan circulation. Patients also
underwent additional cardiac catheterization if clinically
indicated.
The central venous pressure and end-diastolic pressure of the systemic ventricle were measured at the time
of catheterization. The cardiac index was also measured
using the Fick principle from the oxygen consumption
and oxygen content in the systemic and pulmonary
blood. The ventricular volume was calculated using
Simpson’s rule. The ventricular ejection fraction was
calculated as the ratio of the stroke volume to the
end-diastolic volume (%). The end-diastolic volume was
divided by the body surface area for standardization, and
the values are presented as ratios (% of normal value).
Patients
From January 1997 to December 2009, 162 patients with
functional single ventricle underwent TCPC at Mt. Fuji
Shizuoka Children’s Hospital. The survivors routinely
underwent postoperative catheterization at 1 year and
then every 5 years after TCPC and were divided based on
body weight at TCPC into group A (⬍ 10 kg; n ⫽
65— heterotaxy, n ⫽ 16 [24.6%]; hypoplastic left heart
syndrome [HLHS], n ⫽ 20 [30.8%]; and others, n ⫽ 29
[44.6%]) and group B (⬎ 10 kg; n ⫽ 97— heterotaxy, n ⫽
31 [32.0%]; HLHS, n ⫽ 3 [3.1%]; and others, n ⫽ 63
[64.9%]). The timing of the TCPC procedure was at the
discretion of the attending cardiologist and at the convenience of the family of the patient.
Anomalies associated with these patients were a common atrioventricular valve in 59 of the 162 patients
(36.4%), total anomalous pulmonary venous drainage in
43 of the 162 (26.5%) patients, major atriopulmonary
collateral arteries in 2 of the 162 (1.2%) patients, and
hiatal hernia in 1 of the 162 patients (Table 1).
Operative Procedure
Our standard maneuver has been to establish TCPC
consisting of expanded polytetrafluoroethylene tubing
(Gore-Tex, WL Gore & Associates, Flagstaff, AZ) with
cardiopulmonary bypass. Aortobicaval cardiopulmonary
bypass with moderate hypothermia (32–34°C) was used
on the beating heart. Cardioplegic arrest was used for
short periods if intracardiac repair and unusual rerouting
for patients with separated hepatic vein [6] was needed.
In patients who underwent cross-clamping, myocardial
protection was achieved with antegrade cold crystalloid
cardioplegia. Conduit diameter was based on patient
weight, morphologic relationships, and inferior vena
cava (IVC) diameter. The clamp was applied to the
atrium-IVC junction and the latter was transected with
the intention to preserve a sizable atrial cuff at the IVC
stump to allow a longer anastomosis and potential for
growth. Conduit-to-IVC anastomosis was completed
PEDIATRIC CARDIAC
Table 1. Subgroup Comparison
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Ann Thorac Surg
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Fig 1. Actual survival.
PEDIATRIC CARDIAC
with running sutures of 5-0 or 6-0 Prolene (Ethicon,
Somerville, NJ). The inferior pulmonary artery wall was
opened longitudinally and extended to the level of the
main pulmonary artery on 1 side and the takeoff of the
right lower lobe pulmonary artery on the others. In small
patients, the pulmonary artery incision was extended
into the anterior wall of the superior vena cava to obtain
enough space for anastomosis. The pulmonary artery end
of the conduit was beveled with the medial end longer
and the pulmonary conduit anastomosis was completed
with a 6-0 Prolene running sutures. All patients underwent modified ultrafiltration. In patients in whom Fontan
pressure greater than18 mm Hg developed after modified ultrafiltration, the fenestration was created in the
form of side-to-side anastomosis between the conduit
and the atrial wall using a 4-mm aortic puncher. In both
patient groups, we routinely administered 5 mg/kg ticlopidine as antiplatelet therapy. Warfarin was not routinely
used.
Statistical Analysis
Data are presented as means ⫾ standard deviation as
indicated. Differences between the 2 groups (as shown in
Table 1) were determined with Fisher’s exact test, the
Mann-Whitney rank-sum test, or the Student’s t test, as
appropriate. The rate of freedom from reoperation and
survival were estimated using the Kaplan-Meier method,
and differences in these rates between the groups were
assessed using the log-rank test. All analyses were conducted with Prism software, version 5.0.0 (GraphPad
Software Inc, LaJolla, CA). All values of p less than 0.05
were considered statistically significant.
of TCPC. The major cardiac defects were heterotaxy
syndrome in 46 patients (28.4% [46/162]) (n ⫽ 16 in group
A, n ⫽ 30 in group B), HLHS in 23 patients (14.2%
[23/162]) (n ⫽ 20 in group A, n ⫽ 3 in group B), and others
in 93 patients (Table 1). All 162 patients underwent
TCPC. One hundred forty of them had previously undergone bidirectional cavopulmonary Glenn anastomosis as
a staged approach (group A, n ⫽ 60; group B, n ⫽ 80)
before TCPC.
The median interval from initial palliation to the time
of the bidirectional Glenn operation (BDG) was 6.1
months (range, 2.0 –27.3 months) in group A and 16.9
months (range, 3.4 –165 months) in group B (p ⬍ 0.0001).
The median interval from BDG to Fontan operation was
11.7 months (range, 4.4 –30.5 months) in group A and 23.2
months (range, 5.4 –204 months) in group B (p ⬍ 0.0001).
There were 3 hospital deaths (group A, n ⫽ 1; group B,
n ⫽ 2) and 5 late deaths (group A, n ⫽ 3; group B, n ⫽ 2).
The causes of death in these patients were supraventricular tachyarrhythmia in 2 patients, noncardiac infection
in 4 patients, and indeterminate issues in 2 patients. The
Table 2. Surgical Procedure at the Initial Palliation
Procedure
Group A
Group B
(⬍ 10 kg, n ⫽ 65) (⬎ 10 kg, n ⫽ 97) p Value
Patients
Fontan
Modified Norwood
Shunt type
B-T shunt
Central shunt
RV-PA conduit
PAB
PVS repair
AVV repair
Groups A and B were aged 1.6 ⫾ 0.5 years and 7.3 ⫾ 6.0
years, respectively, (p ⫽ 0.0001) and weighed 8.5 ⫾ 0.8 kg
and 20.18 ⫾ 13.1 kg, respectively, (p ⫽ 0.0001) at the time
AVV ⫽ atrioventricular valve;
B-T shunt ⫽ Blalock-Taussig shunt;
PAB ⫽ pulmonary artery banding;
PVS ⫽ pulmonary valve stenosis;
RV-PA conduit ⫽ right ventricle-pulmonary artery conduit.
Results
2
22
3
5
1.0
⬍ 0.0001
23
2
11
16
3
2
45
3
18
31
3
1
0.2
1.0
0.4
0.4
0.9
0.5
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FONTAN OPERATION IN INFANTS LESS THAN 10 KG
Variable
Cardiopulmonary bypass (min)
Aortic clamp (n)
Clamp time (min)
Concomitant procedure
Atrioventricular valve plasty
Atrioventricular replacement
Repair of pulmonary vein stenosis
Damus-Kaye-Stansel operation
Subaortic stenosis repair
Septostomy
Fenestration
Pulmonary artery plasty
Pacemaker related
Postoperative Course
Mechanical ventilation (h) (median)
Duration of chest tube (d) (mean)
Length of ICU stay (d) (median)
Length of hospital stay (d) (median)
Group A (⬍ 10 kg, n ⫽ 65)
Group B (⬎ 10 kg, n ⫽ 97)
p Value
141.1 ⫾ 45.1
26 (40%)
58.8 ⫾ 27.1
(24) 36.9%
3
2
2
0
0
3
3
10
1
165.1 ⫾ 48.7
62 (63.9%)
71.5 ⫾ 38.8
(41) 46.3%
8
0
2
4
2
5
5
11
4
0.01
0.004
0.1
0.5
12 (2–338.5)
7.9 ⫾ 3.3
4 (1–45)
28.5 (14–87)
7.3 (1.8–1385)
9.8 ⫾ 4.4
4 (1–35)
29.5 (5–143)
0.73
0.029
0.85
0.79
ICU ⫽ intensive care unit.
overall survival rates (%) in group A versus group B at 1,
5, and 10 years were 96.9 versus 96.9, 93.8 versus 96.9, and
93.8 versus 94.5, respectively (p ⫽ 0.41, log-rank test)
(Fig 1).
Surgical Treatment
Twenty-seven (16.7%) of 162 patients underwent a modified Norwood procedure (group A versus group B: 33.8%
[22/65] versus 5.2% [5/97]; p ⬍ 0.001) at the first palliative
operation. Additionally, 102 of the 162 patients (63.0%)
had a systemic-to-pulmonary shunt (Blalock-Taussig)
[n ⫽ 68], a central shunt [n ⫽ 5], a ventricle-to–
pulmonary artery conduit [n ⫽ 29]), 47 patients (29.0%)
had a pulmonary artery band, 3 patients had atrioventricular valve repair, and 6 patients had repair of total
anomalous pulmonary venous drainage at the initial
palliative operation (Table 2). Operative factors, including concomitant procedures (n ⫽ 45 [group A, n ⫽ 12;
group B, n ⫽ 33]) performed at the time of Fontan
operation are summarized in Table 3. The intrapulmonary artery septation procedures, which consist of unilateral cavopulmonary anastomosis, aortopulmonary
shunt, and septation between 2 blood sources [7], were
applied in both patient groups (group A, n ⫽ 8; group B,
n ⫽ 12) to promote growth in small pulmonary arteries
before the Fontan operation. The diameter of the extracardiac graft ranged from 14 to 24 mm. Fenestration was
reconstructed in 8 patients (group A, n ⫽ 3; group B, n ⫽
5) at the time of TCPC (Table 3). The older Fontan
patients had a longer bypass time. However the crossclamp time was similar in both groups (Table 3). Aortic
cross-clamping was used in 88 patients, including 26
(40%) in group A and 62 (63.9%) in group B. There were
a greater number of patients undergoing an intracardiac
concomitant procedure and patients with heterotaxy syn-
drome who had separated hepatic vein drainage in group
B compared with group A.
The duration of chest tube drainage was significantly
longer for group B patients than for group A patients.
However there were no differences in the duration of
mechanical ventilation and the length of stay in the
intensive care unit between the 2 groups (Table 3).
Twenty-seven patients required reoperation after Fontan completion: repair of the atrioventricular valve in 5
patients, repair of pulmonary vein stenosis in 1 patient,
creation of a fenestration in 1 patient, rerouting for
pulmonary arteriovenous malformation in 1 patient, ligation of collateral arteries in 2 patients, repair of pulmonary arteries in 2 patients, ligation of aortopulmonary
collateral arteries in 2 patients, permanent pacemaker–
Table 4. Reoperation
Variable
Atrioventricular valve
repair
Valve replacement
Pulmonary vein
stenosis repair
Pulmonary artery plasty
Ligation of systemic-topulmonary collateral
Pacemaker related
Fenestration
Rerouting
Others
Takedown
Total
Group A
(⬍ 10 kg, n ⫽ 65)
Group B
(⬎ 10 kg, n ⫽ 97)
3
2
2
1
0
0
2
2
0
0
1
1
0
0
0
12
9
0
1
3
0
15
PEDIATRIC CARDIAC
Table 3. Surgical Data at Fontan Procedure
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FONTAN OPERATION IN INFANTS LESS THAN 10 KG
Ann Thorac Surg
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Fig 2. Freedom from reoperation.
PEDIATRIC CARDIAC
related complications in 10 patients, and noncardiacrelated issues in 3 patients (Table 4). The rate of freedom
from reoperation at 10 years after TCPC was 80.1% in
group A and 83.5% in group B (p ⫽ 0.39) (Fig 2).
Catheterization Data
The mean pulmonary artery pressure, pulmonary artery
resistance, and pulmonary artery index immediately before TCPC did not significantly differ between the 2
groups. The number of interventions for systemic-topulmonary collateral arteries was higher in group A
(group A versus group B: 66.2% [43/65] versus 30.9%
[30/97]; p ⬍ 0.001) (Table 5).
The eDV (% of normal) was 232.7 ⫾ 91.4 versus 139.3 ⫾
57.2 before TCPC versus 5 years after TCPC, respectively;
p ⬍ 0.001) in group A and (209.6 ⫾ 77.7 versus 136.7 ⫾
61.4, respectively; p ⬍ 0.001) in group B (Fig 3). Although
ejection fraction and cardiac index (before TCPC versus 5
years after TCPC) did not differ significantly (p ⫽ 0.116
and p ⫽ 0.266, respectively) in group A, ejection fraction
and cardiac index in group B at 5 years were significantly
lower than before TCPC (p ⫽ 0.0057 and p ⫽ 0.0358,
respectively) (Fig 4). The eDP (mm Hg) at 1 year (4.8 ⫾ 0.4
versus 6.8 ⫾ 0.4; p ⫽ 0.0037) and eDP at 5 years (6.5 ⫾ 0.4
versus 8.2 ⫾ 0.6; p ⫽ 0.047) were significantly lower in
group A (Fig 1). The Fontan pressure (group A versus
group B) at 1 year and 5 years after TCPC were (11.9 ⫾ 2.1
mm Hg versus 12.0 ⫾ 2.3 mm Hg, respectively; p ⫽ 0.8)
and (11.6 ⫾ 2.1 mm Hg versus 11.1 ⫾ 2.7 mm Hg,
respectively; p ⫽ 0.4).
Comment
Better understanding of the natural history and anatomic
morphologic features of functional single ventricle, advances in surgical techniques and intraoperative myocardial protection, and comprehensive postoperative management have contributed to a remarkable improvement
in early and late mortality before and after Fontan operations [4, 8 –10]. However elimination of cyanosis and
ventricle unloading from the systemic and pulmonary
circulation by Fontan operation at an earlier age remains
a controversial topic. However volume unloading can be
evident when there is collateral accessory flow between
the systemic circulation and the pulmonary circulation
[11]. More recent approaches to Fontan completion include volume-unloading operations at a younger age to
reduce the adverse effects of prolonged ventricular volume overload on ventricular function. Furthermore, recent reports have demonstrated safe Fontan completion
during the first year of life independent of anatomic
diagnosis without additional morbidity or mortality
Table 5. Catheter Study Before Fontan Operation
Variable
Pulmonary artery index
Resistance of pulmonary artery
PAP
Atrial Pressure
Transpulmonary pressure gradient
Ejection fraction
Cardiac index
Intervention for collateral arteries
PAP ⫽ pulmonary artery pressure.
Group A (⬍ 10 kg, n ⫽ 65)
Group B (⬎ 10 kg, n ⫽ 97)
p Value
219.6 ⫾ 108.1
1.8 ⫾ 0.9
11.8 ⫾ 2.7
6.3 ⫾ 2.4
5.3 ⫾ 2.1
58.4 ⫾ 9.7
3.2 ⫾ 0.6
43 (66.2%)
245.8 ⫾ 115.8
1.8 ⫾ 0.9
11.9 ⫾ 3.2
6.8 ⫾ 2.2
5.2 ⫾ 2.8
58.8 ⫾ 9.4
3.4 ⫾ 0.9
30 (30.9%)
0.1
0.8
0.8
0.2
0.8
0.8
0.1
⬍ 0.001
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FONTAN OPERATION IN INFANTS LESS THAN 10 KG
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Fig 3. Changes in ventricular end-diastolic
volume (eDP) (% of normal volume) and ventricular end-diastolic pressure (eDP) (mm Hg)
before and at 1, 5, and 10 years after Fontan
operation in group A (solid circle) and group
B (solid triangle). The eDP was significantly
lower in group A than in group B at 5 and 10
years after total cavopulmonary connection.
(A) eDV. (B) eDP.
[4, 12]. We found no significant difference in long-term
mortality and morbidity rates between the 2 groups,
indicating that the Fontan procedure can be safely completed without increasing these rates over the long
term.
Initially, we used a 16-mm graft in 52 patients and a
14-mm graft in 2 patients during the extracardiac Fontan
procedure. After gaining experience, we recently increased the use of 18-mm conduits. None of these grafts
has required revision. Therefore we do not consider that
the Fontan operation in younger patients is disadvantageous from this perspective.
To eliminate cyanosis earlier in life in our patients, in
1997 we started to perform Fontan operations when
patients weighed about 10 kg. Similar to other series
[4, 8, 9, 13–16], there was extremely low late mortality.
In our series, age at TCPC had no effect on mortality.
Earlier Fontan completion has several theoretical advantages in terms of minimizing the effect of persistent
cyanosis, the potential for paradoxical embolization,
and the duration of chronic volume overload of the
single ventricle [17].
Long-standing cyanosis and volume overload to the
ventricle are believed to cause progressive ventricular
fibrosis by inducing histopathologic changes, which must
result in both systolic and diastolic dysfunction. Therefore earlier elimination of cyanosis and volume overload
can enhance cardiac performance in patients undergoing
Fontan operations. We found that the ventricular enddiastolic pressure was significantly lower 5 years after the
Fontan procedure had been completed in the younger
patient group. Although the cardiac index and ventricular ejection fraction were significantly lower at 5 years
after the Fontan procedure compared with those measurements before the Fontan operation in the older
patient group, in the younger group we found there were
no significant differences between cardiac index and
ejection fraction before the Fontan procedure and at 5
years after the Fontan procedure. These results suggest
that earlier Fontan completion helps to maintain ventricular contractility, prevent the progression of ventricular
dysfunction, and maintain a good ejection fraction and
cardiac index.
Earlier unloading of a univentricular heart by means of
Fontan completion in patients with collateral accessory
flow between the systemic circulation and the pulmonary
circulation might be advantageous for future ventricular
function preservation [18, 19]. Other authors have recognized that patients with high systemic-to-pulmonary
collateral flow were more likely to be older at the time of
the Fontan operation [19]. It is possible that an increasing
systemic oxygen saturation after the Fontan operation
removes the stimulus for further growth of systemic-topulmonary collateral flow. In particular, it might be
postulated that the systolic and diastolic function of the
Fig 4. (A) Changes in ventricular ejection
fraction (%) and (B) changes in cardiac index
(L/min/m2) before total cavopulmonary connection (TCPC), at 1, 5, and 10 years after
TCPC. There are no significant differences in
group A; however, cardiac index and ejection
fraction at 5 and 10 years after TCPC were
lower than before TCPC in group B.
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single ventricle would be preserved by early reduction of
volume loading.
In addition, relief from early cyanosis might alleviate
deleterious effects from a prolonged cyanotic state.
Furthermore, the hemodynamics in Fontan circulation
depend not only on cardiac contractility but also on
pulmonary circulation and function, and the growth of
respiratory and skeletal muscles is considered to be an
important factor affecting Fontan circulation. For these
reasons, we postulate that earlier elimination of cyanosis allows better growth of respiratory and skeletal
muscles, which would subsequently preserve the cardiac index, especially at 5 and 10 years after the Fontan
procedure.
Our experience shows that in our current series, the
TCPC achieves excellent survival. In our series of 162
patients, the hospital survival was 98.1% (159/162). There
was little mortality during the follow-up period, with a
10-year actual survival of 93.5%. These outcomes were
achieved in a broad spectrum of single-ventricle anatomies, including HLHS and heterotaxy syndrome, in a
total of 43% of the patients.
Study Limitations
The study described here covered a long period, and its
retrospective nature and inadequate number of patients
for meaningful subgroup analysis should be recognized
as limitations of the study, although the data in our
database are collected prospectively for all operations.
The effect of general improvement in surgical technique
and perioperative care with increasing experience could
not be analyzed in this study.
In conclusion, earlier TCPC can be performed with
good intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous
for future preservation of ventricular function.
References
1. Fontan F, Baudet E. Surgical repair of tricuspid atresia.
Thorax 1971;26:240 – 8.
2. Kreutzer G, Galindez E, Bono H, De Palma C, Laura JP. An
operation for the correction of tricuspid atresia. J Thorac
Cardiovasc Surg 1973;66:613–21.
3. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary
connection for complex Fontan operations. Experimental
studies and early clinical experience. J Thorac Cardiovasc
Surg 1988; 96:682–95.
Ann Thorac Surg
2012;94:1570 –7
4. Pizarro C, Mroczek T, Gidding SS, Murphy JD, Norwood WI.
Fontan completion in infants. Ann Thorac Surg 2006;81:
2243– 8; discussion 2248 –9.
5. Ikai A, Fujimoto Y, Hirose K, et al. Feasibility of the extracardiac
conduit Fontan procedure in patients weighing less than 10
kilograms. J Thorac Cardiovasc Surg 2008;135:1145–52.
6. Nakata, T, Fujimoto Y, Hirose L, et al. Fontan completion in
patients with atrial isomerism and separate hepatic venous
drainage. Eur J Cardiothorac Surg 2010;37:1264 –70.
7. Sakamoto K, Ikai A, Fujimoto Y, Ota N. Novel surgical
approach ’intrapulmonary-artery septation’ for Fontan candidates with unilateral pulmonary arterial hypoplasia or
pulmonary venous obstruction. Interact Cardiovasc Thorac
Surg 2007;6:150 – 4.
8. Tweddell JS, Nersesian M, Mussatto KA, et al. Fontan
palliation in the modern era: factors impacting mortality and
morbidity. Ann Thorac Surg 2009;88:1291–9.
9. Jaquiss RD, Siehr SL, Ghanayem NS, et al. Early cavopulmonary anastomosis after Norwood procedure results in
excellent Fontan outcome. Ann Thorac Surg 2006;82:1260 –5;
discussion 1265– 6.
10. Hosein RB, Clarke AJ, McGuirk SP, et al. Factors influencing
early and late outcome following the Fontan procedure in
the current era. The ’Two Commandments’? Eur J Cardiothorac Surg 2007;31:344 –52; discussion 353.
11. Sathanandam S, Polimenakos AC, Blair C, El Zein C, Ilbawi
MN. Hypoplastic left heart syndrome: feasibility study for
patients undergoing completion fontan at or prior to two years
of age. Ann Thorac Surg 2010;90:821– 8; discussion 828 –9.
12. Weber HS, Gleason MN, Myers JL, et al. The Fontan operation in infants less than 2 years of age. J Am Coll Cardiol
1992;19:828 –33.
13. Petrossian E, Reddy VM, Collins KK, et al. The extracardiac
conduit Fontan operation using minimal approach extracorporeal circulation: early and midterm outcomes. J Thorac
Cardiovasc Surg 2006;132:1054 – 63.
14. Fiore AC, Turrentine M, Rodefeld M, et al. Fontan operation:
a comparison of lateral tunnel with extracardiac conduit.
Ann Thorac Surg 2007;83:622–9; discussion 629 –30.
15. Anderson PA, Sleeper LA, Mahony L, et al. Contemporary
outcomes after the Fontan procedure: a Pediatric Heart
Network multicenter study. J Am Coll Cardiol, 2008;52:
85–98.
16. Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of
outcome after the Fontan operation: is hypoplastic left heart
syndrome still a risk factor? J Thorac Cardiovasc Surg
2002;123:237– 45.
17. Camposilvan S, Milanesi O, Stellin G, et al. Liver and cardiac
function in the long term after Fontan operation. Ann Thorac
Surg 2008;86:177– 82.
18. Banka P, Sleeper LA, Atz AM, et al. Practice variability and
outcomes of coil embolization of aortopulmonary collaterals
before Fontan completion: a report from the Pediatric Heart
Network Fontan Cross-Sectional Study. Am Heart J 2011;162:
125–30.
19. Prakash A, Rathod RH, Powell AJ, et al. Relation of systemicto-pulmonary artery collateral flow in single ventricle physiology to palliative stage and clinical status. Am J Cardiol
2012;109:1038 – 45. Epub 2012 Jan 3.
DISCUSSION
DR JAMES JAGGERS (Aurora, CO): Let me ask you just 1
quick thing about your conduit size, that 16 or 17 mm I believe
was your standard conduit size in either group. When you did
your postoperative catheterizations at 5 years, was there any
evidence that that was an inadequate size? Was there any
level of obstruction in the smaller conduit?
It is my personal bias that that is actually a very reasonable
size, but that is contrary to others’ opinion.
DR OTA: Actually, we did not find conduit obstruction from
the catheter study which was done at 5 years after the Fontan
operation, and some paper said that mean diameters of the
IVC were changed 17 mm in the left lateral position to 23 mm
in the right lateral position in patients with right atrial
pressures greater than 8 mm Hg. Those findings might
suggest that a conduit of 18 or 20 mm diameter might be
acceptable I think.
DR CARL LEWIS BACKER (Chicago, IL): This was a really good
analysis with a considerable amount of data on a substantial
number of patients.
My question relates to the finding of the decreased ejection
fraction and increased end-diastolic pressure at that final measured point 5 years after the Fontan operation.
The group that had the “early” Fontan was about 1.5 years of
age at the time of their Fontan procedure. The group that had
the “delayed” Fontan was about 5 years of age at the time of
their Fontan procedure. The question I have is whether the
change in ejection fraction and end-diastolic pressure could be
related more to the age of the patient rather than to the time of
follow-up since the Fontan because the “delayed Fontan” group
was almost 4 years older at the time of the Fontan operation.
Did your analysis take the age of the patient into account, not
just the time period since the Fontan operation?
DR OTA: Thank you for your good question. Regarding of
ejection fraction, we can compare the number of group A at the
5 years after the Fontan with the number of group B at 1 year
after the Fontan. It is likely to be age-adjusted comparison, and
those age-adjusted comparison shows still the same trend as I
showed in the previous slide.
OTA ET AL
FONTAN OPERATION IN INFANTS LESS THAN 10 KG
1577
Regarding of ventricular end-diastolic pressure, the timing at
the Fontan operation is an important factor I think. There are
slight differences between 2 groups before Fontan operation as I
showed the previous slide.
Some other paper said that Fontan patients in the highest
amount of systemic to pulmonary collateral flow underwent the
Fontan operation at an older age. It is possible that an increase
in systemic oxygen saturation after the Fontan operation removes the stimulus for further growth of systemic-to-pulmonary
collaterals. These findings support this hypothesis.
Longstanding cyanosis and volume overload to the ventricle
are believed to cause progressive ventricular fibrosis by inducing histopathologic changes, which must result in both systolic
and diastolic dysfunction.
In theory, systemic-to-pulmonary collateral flow causes volume overload of the systemic ventricle, which could contribute
to adverse outcomes, and again I think the timing of Fontan
operation is important factor for end-diastolic pressure.
Thank you again for your great comments and questions.
DR BACKER: I will ask you more about this after the session.
Thank you again for your excellent paper.
The Society of Thoracic Surgeons:
Forty-Ninth Annual Meeting
Mark your calendars for the Forty-Ninth Annual Meeting of
The Society of Thoracic Surgeons (STS) to be held at the Los
Angeles Convention Center, Los Angeles, California, from
January 26–30, 2013. Visit Los Angeles to learn from the
experts, network with colleagues from around the world, and
prepare for whatever your future may hold.
This preeminent educational event in cardiothoracic
surgery is open to all physicians, residents, fellows,
engineers, perfusionists, physician assistants, nurses,
or other interested individuals who work with cardiothoracic surgeons. Meeting attendees will be provided
with the latest scientific information for practicing
cardiothoracic surgeons. Attendees will benefit from
traditional Abstract Presentations and Invited Lectures, as well as Surgical Forums, Early Morning Sessions, Surgical Motion Pictures, and Procedural
Hands-On Courses. Parallel sessions on Monday and
Tuesday will focus on specific subspecialty interests.
© 2012 by The Society of Thoracic Surgeons
Published by Elsevier Inc
An advance program with a registration form, hotel
reservation information, and details regarding spouse/
guest activities will be mailed to STS members this
Fall.
Nonmembers may contact the Society’s Secretary, David
A. Fullerton, MD, to receive a copy of the advanced program; however, detailed meeting information will be available on the STS website at www.sts.org.
David A. Fullerton, MD
Secretary
The Society of Thoracic Surgeons
633 N Saint Clair St, Ste 2320
Chicago, IL 60611-3658
Telephone: (312) 202-5800
Fax: (312) 202-5801
email: [email protected]
website: www.sts.org
Ann Thorac Surg 2012;94:1577
•
0003-4975/$36.00
PEDIATRIC CARDIAC
Ann Thorac Surg
2012;94:1570 –7