Download Evaluation of a Venous Unifocalization of the Bilateral Superior

Original Articles
Juntendo Medical Journal
2016
Evaluation of a Venous Unifocalization of the Bilateral Superior Vena Cava and
Comparison with the Bilateral Bidirectional Glenn Procedure
SHIORI KAWASAKI＊1), KEISUKE NAKANISHI＊1), KEN TAKAHASHI＊2), ATSUSHI AMANO＊1)
＊1) Department
of Cardiovascular Surgery, Juntendo University Faculty of Medicine, Tokyo, Japan,
＊2) Department
of
Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
Objective: A bilateral-bidirectional Glenn procedure is generally performed in patients with a functional single ventricle and a
bilateral superior vena cava. In bilateral superior vena cava, unbalanced blood flow due to its unique anatomy and reduced
volume due to its small aperture can cause blood stasis, unbalanced pulmonary blood flow, and thrombosis formation.
Unifocalization of bilateral superior vena cava, a new surgical technique which makes pulmonary blood flow more equally
distributed, was performed and evaluated.
Methods: We retrospectively reviewed the clinical and surgical records of 65 patients who underwent Glenn procedure at the
Juntendo University Hospital, Tokyo, from January 1997 to March 2014. Sixteen patients had bilateral superior vena cava
anatomy. Unifocalization of superior vena cava was performed in 8 cases and conventional surgery in 8. Perioperative data were
evaluated to compare outcomes and clinical courses between the two groups.
Results: There were no significant differences between the 2 groups in age (group 1: 1.0 ± 0.5, group 2: 1.2 ± 1.1, years of
age), body weight (group 1: 7.7 ± 2.3, group 2: 6.5 ± 4.3, kg), change in central venous pressure before and after the operation
(group 1: 6.5 ± 3.1, group 2: 9.9 ± 6.2, mmHg), postoperative oxygen saturation (group 1: 82 ± 3.3, group 2: 83 ± 9.3, %),
duration of surgery (group 1: 371 ± 120, group 2: 439 ± 168, min), or cardiopulmonary bypass time (group 1: 143 ± 38, group 2:
131 ± 53, min). Unilateral blood flow, which is purportedly better than bilateral bidirectional Glenn procedure, was achieved
without any disadvantages that are reported of the conventional procedure.
Conclusion: There was no distinct advantage or disadvantage to using the new method.
Key words: Glenn procedure, bilateral superior vena cava, bilateral bidirectional Glenn procedure
Introduction
In the early 1970s, Fontan and Kreutzer independently developed strategies for palliation in
patients with tricuspid atresia that involved baffling
the caval veins directly to the pulmonary artery 1) 2).
Usually, patients with a functional single ventricle
are treated with a series of staged surgical procedures culminating in a Fontan operation. Bidirectional Glenn procedure is used as an intermediate
stage, which has been a good treatment option for
patients with a single ventricle resulting in lower
mortality than the single-stage Fontan proce-
dure 3)-6). In Glenn stage patients with a bilateral
superior vena cava (SVC), the bilateral bidirectional
Glenn procedure has generally been performed 7).
When the Fontan operation is performed in
patients who have previously had a bilateral
bidirectional Glenn procedure, the blood flow from
bilateral SVCs face the flow from the inferior vena
cava. Because blood flow is divided between two
SVCs, there is an imbalance in volume such that
blood flow to the lungs is not equally distributed,
and the risk of thrombosis formation increases 8).
To resolve this issue, we introduced a new
surgical method, superior vena cava unifocalization
Corresponding author: Keisuke Nakanishi
Department of Cardiovascular Surgery, Juntendo University Faculty of Medicine
2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
TEL: + 81-3-3813-3111
〔Received
FAX: + 81-3-3813-3210
Sep. 18, 2015〕〔Accepted
E-mail: [email protected]
Jan. 21, 2016〕
Copyright © 2016 The Juntendo Medical Society. This is an open access article distributed under the terms of Creative Commons Attribution License (CC BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original source is properly credited.
doi: 10.14789/jmj.62.●
1
Kawasaki, et al: Evaluation of a venous unifocalization
Figure-1
Unifocalization of bilateral superior vena cava
Smaller SVC was anastomosed to the lateral side of larger SVC,
then the unifocalized SVC was anastomosed to the pulmonary
artery. Unilateral Glenn-like flow was obtained.
of bilateral superior vena cava, in a previously
published report. In this method, the smaller SVC is
anastomosed to the lateral side of the larger SVC,
and the unifocalized SVC is anastomosed to the
pulmonary artery 9). In the current study, we
hypothesized that this new surgical method,“SVC
unifocalization”, would be more effective than the
Glenn stage procedure in patients with bilateral
SVC, because single-vessel flow from the dual SVCs
would distribute to both lungs in a manner similar
to that of the unilateral Glenn procedure (Figure-1). Our aims of this study were, in patients with
a functional single ventricle and bilateral SVC, to
compare the results of our new method with those
of conventional bilateral bidirectional Glenn procedure, and to examine the efficacy of the method.
Materials and methods
1. Retrospective data analysis
We retrospectively reviewed the clinical and
surgical records of 65 patients who had undergone
bidirectional Glenn procedure in a staged right
cardiac bypass at Juntendo university hospital,
Tokyo, between January 1997 and March 2014. We
performed the first venous unifocalization in 2002.
Since then, the novel method has been performed in
patients in whom both the inferior vena cava and
larger main SVC were on the same side; otherwise
conventional bilateral bidirectional Glenn procedure
is performed, since the outcome of the new method
2
had yet to be established.
Sixteen of the patients had a bilateral superior
vena cava. These patients were divided into 2
groups: group 1: patients who had received venous
unifocalization (n = 8), and group 2: patients who
had received the bilateral bidirectional Glenn
procedure (n = 8). We retrospectively reviewed the
clinical data including age, body weight, central
venous pressure, blood oxygen saturation, time on
cardiopulmonary bypass, total time of the procedure, and complications after surgery. Central
venous pressure and oxygen saturation were measured at the preoperative catheter examination and
postoperatively in the intensive care unit. Postoperative central venous pressure was measured at the
main larger superior vena cava in all cases.
Results
Patient characteristics are shown in Tables-1 and
2. Patient age ranged from 4 months to 2 years in
group 1 and 1 month to 3 years in group 2. Body
weight ranged from 6.1 kg to 10.6 kg in group 1
and 2.78 kg to 13.5 kg in group 2. Tricuspid atresia
was diagnosed in four patients in group 1; single
ventricle associated with right isomerism was
present in 1 patient in group 1 and 2 patients in
group 2; pulmonary atresia was present in 1 patient
in group 1 and 2 in group 2; double outlet right
ventricle was present in 1 patient in group 1 and 2
in group 2, and transposition of the great arteries
occurred in 2 patients in group 2. Shunt division,
pulmonary artery plasty, common atrioventricular
valve plasty, posterior descending artery ligation,
and the Fontan procedure were performed subsequently in both groups.
There were no significant differences in age
(group 1: 1.0 ± 0.5, group 2: 1.2 ± 1.1, years of age),
body weight (group 1: 7.7 ± 2.3, group 2: 6.5 ±
4.3, kg), change in central venous pressure before
and after operation (group 1: 6.5 ± 3.1, group 2:
9.9 ± 6.2, mmHg), postoperative oxygen saturation
(group 1: 82 ± 3.3, group 2: 83 ± 9.3, %), duration
of surgery (group 1: 371 ± 120, group 2: 439 ± 168,
min), or cardiopulmonary bypass time (group 1:
143 ± 38, group 2: 131 ± 53, min). Preoperative
pulmonary artery index was shorter in group 1
(group 1: 269 ± 120, group 2: 365 ± 124, p = 0.03)
(Table-3).
There were no significant differences in any
Juntendo Medical Journal 2016
Table-1
No
Clinical data in group 1 (unifocalization of the superior vena cava procedure)
Age,yrs
BW (kg)
Sex
Diagnosis
Procedure
complication
Status now
f/u span, yrs
1
1.3
10.3
M
TA (1-b)
A
(-)
p/o e-TCPC
8.3
2
0.9
9.6
M
PA, SV
A
(-)
p/o e-TCPC
4.6
3
0.3
6.1
M
TA (2-a)
A + PA plasty
Desaturation
Dead
2.6
4
0.7
4.2
F
DORV, hypo RV
A + CAVVP + PMI
5
1.9
10.6
M
TA (3)
A
(-)
p/o e-TCPC
0.67
6
0.9
6.4
M
SA, SV
A + PA plasty
Seizure
p/o e-TCPC
0.4
7
1.1
7.1
F
c AVSD
apicocaval
juxtaposition
A + PA banding
(-)
p/o e-TCPC
8
1.1
7
M
SA, SV
A; PDA ligation
(-)
p/o e-TCPC
1.0 ± 0.6
7.7 ± 2.3
Mean
Pleural effusion p/o e-TCPC
2.9
2.9 ± 2.8
A: unifocalization of superior vena cava, TA: tricuspid atresia, PA: pulmonary atresia, SV: single ventricle, DORV: double outlet right
ventricle, CAVVP: common atrio-ventricle valve plasty, PMI: pacemaker implantation, TCPC: total cavopulmonary connection,
e-TCPC: Extra-cardiac total cava-pulmonary connect, c AVSD: complete atrioventricular-septal-defect
Table-2
Patient Age, yrs
No.
Clinical data in group 2 ( bilateral bidirectional Glenn procedure)
BW, kg
Sex
Diagnosis
Procedure
Complication
Status now
f/u span, yrs
1
1.7
7.1
F
PA, SV,
TAPVD (2-a)
B
None
p/o e-TCPC
12.8
2
1
3.1
M
Asplenia, SA, SV
B
LOS
Early death
10
3
2
2.5
M
PA, VSD
B + PA plasty
None
p/o e-TCPC
8
4
4.1
13.5
F
DORV, PS,
Straddling TPM
B
None
p/o e-TCPC
7.1
5
1.8
10.2
M
TGA (3)
B (one staged
Fontan)
Pleural effusion
p/o Fontan
(L-T method)
13.2
6
2.2
8.3
M
DORV, hypo LV
B
Mediastinitis
Late death
2.4
7
1.2
6.5
M
TGA (3)
B
Mediastinitis
Late death
3.3
8
2
2.8
F
B
Pericardial
effusion
p/o e-TCPC
3.7
1.2 ± 1.1
6.5 ± 4.3
7.6 ± 4.2
BDG: bilateral bidirectional Glenn procedure, TAPVD: total anomalous pulmonary vein drainage, SA: single atrium, PS: pulmonary
stenosis, TGA: transposition of great arteries, TPM: tricuspid pupillary muscle, hypo LV: hypo plastic left ventricle, L-T: lateral tunnel
Table-3
Comparison of peri-operative variables in group 1 (unifocalization of the superior vena cava procedure) and group 2
(bilateral bidirectional Glenn procedure)
group 1
group 2
Operation time, min
371 ± 120
439 ± 168
n.s.
CPB time, min
143 ± 42
131 ± 53
n.s.
PA index (pre)
269 ± 120
365 ± 124
p = 0.03
71 ± 9.8
74 ± 14
n.s.
2
SpO % (pre)
p value
SpO % (post)
82 ± 3.3
83 ± 9.3
n.s.
CVP mmHg (pre)
6.8 ± 1.5
4.6 ± 2.4
n.s.
CVP mmHg (post)
13.8 ± 2.5
14.5 ± 0.7
n.s.
Duration of ICU stay, days
2.0 ± 1.5
5.7 ± 7.0
n.s.
2
CPB: cardio-pulmonary bypass, PA: pulmonary artery, SpO 2: oxygen saturation, CVP: central venous pressure, ICU: intensive care unit
3
Kawasaki, et al: Evaluation of a venous unifocalization
64 patients
Bidirectional Glenn
（BDG）procedure
48 patients
Unilateral BDG
8 patients
Venous
unifocalization
8 patients
Conventional
method
7 TCPC
1 early death
Figure-2
5 TCPC
1 early, 2 late deaths
Clinical course of two groups
other indices. Although the differences were not
significant, there were 4 mortalities (1 early in
group 1, and 1 early and 2 late in group 2), and 6
morbidities (2 in group 1, 4 in group 2). In group 1,
7 patients received the Fontan procedure. In the
patient that died early, the larger SVC was located
on the left side, and the inferior vena cava was
located on the right side. On the patientʼs right side,
the left larger SVC was anastomosed to the right
smaller SVC. Post-operatively, central venous
pressure was elevated, and severe edema of the
face was observed. Two patients had phrenic nerve
palsy in group 1. In group 2, 4 patients received the
Fontan procedure, and 1 is awaiting the Fontan
procedure. The patient with early death died due to
low output syndrome, and the 2 late deaths were
due to respiratory infections. 2 cases of mediastinitis and 2 cases of long-standing pleural effusion
were seen in Group 2 (Figure-2).
None of the cases presented pressure gradients
by echocardiography on postoperative day 3.
Discussion
The bidirectional Glenn (BDG) procedure is an
important intermediate stage preceding the Fontan
operation. Generally, the bilateral BDG is performed
in patients who have bilateral superior vena cava. In
the bilateral SVC anatomy, the imbalance of flow
4
through the pulmonary artery can cause stasis of
blood and thrombosis formation, because the small
apertures of the bilateral SVC reduces blood flow
volume 8). The clinical impact of thrombosis in the
cavopulmonary circulation is a higher risk of
mortality after BDG and/or increased hospitalization time 10) 11). In addition, there is a concern that
blood flow from the inferior vena cava might not
distribute equally after the Fontan procedure,
because there is a dimensional mismatch between
the baffle and the connecting vessels. It was not
until 1994, when Srivastava and colleagues 12) conclusively demonstrated that pulmonary arterialvenous fistulae occurred due to the absence of
“hepatic factor”or“mesenteric factor”,and that an
unidentified factor in the hepatic venous drainage
inhibited the recruitment and dilation of preexisting
pulmonary arteriovenous connections 12)-14). Unequally distributed inferior vena cava blood flow to
the lungs can lead to pulmonary arteriovenous
malformations. Currently, with the widespread use
of the bidirectional Glenn procedure as an intermediate stage in the palliation of children with
single-ventricle physiology, pulmonary arteriovenous malformations continue to be a cause of
considerable morbidity. Although the bidirectional
Glenn procedure provides excellent hemodynamic
palliation for these children, its durability is often
limited by the development of progressive cyanosis
due to pulmonary arteriovenous malformations 15) 16). These facts suggest that undisturbed
pulmonary blood flow from SVCs and the distribution of blood flow from the inferior vena cava are
important factors for the prognosis of patients with
a single ventricle. Iyer GK et al has reported there
was a lower operative mortality and a higher
conversion rate to the Fontan circulation in children
undergoing a unilateral bidirectional Glenn than in
children undergoing bilateral bidirectional Glenn 17).
In patients with bilateral SVCs, our new method
would generate undisturbed blood flow similar to
that of unilateral BDG but without anatomical or
surgical limitations. We believe our new method
would resolve some of theissues that occur in
bilateral-SVCs patients.
Although we speculated that blood flow from the
inferior vena cava, which includes the hepatic
factor, might be distributed better than with the
unilateral Glenn procedure, proof that the new
Juntendo Medical Journal 2016
method creates more equally distributed blood
flows could not be demonstrated. In a future study,
we will measure the equality of blood flow distribution in the two methods by using hydrodynamic
simulation. Although evaluation of a new method
requires patient follow-up early and long-term, we
predict a positive outcome based on literature 18).
Study limitations
One limitation of this study was the lack of some
useful information, which includes quantitative data
such as echocardiography or cardiac angiography,
as well as data on flow volume ratio between the
SVCs and the IVC. Another major limitation of this
study was the small sample size, which was not
sufficient to establish the validity of this surgical
method. However, evaluation of a novel method on a
rare disease such as bilateral superior vena cava is
valuable in itself. Further study with a larger
sample size, long-term follow-up, and additional
data including flow volume ratio and quantitative
analysis is essential to show the applicability of this
method in various anatomical subsets.
Conclusion
Although we had hypothesized that the new
surgical method,“SVC unifocalization”, would be
more effective than the Glenn stage procedure in
patients with bilateral SVC, the results of this study
demonstrated that there was no distinct advantage
or disadvantage to using our new method. Therefore, further clinical study was warranted.
Conflict of interest
The authors have declared that no conflict of
interest exists.
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