Gen Thorac Cardiovasc Surg (2015) 63:496–501
DOI 10.1007/s11748-015-0550-y
ORIGINAL ARTICLE
The long-term outcomes of physiologic repair for ccTGA
(congenitally corrected transposition of the great arteries)
Keiichi Hirose1 • Takeshi Nishina1 • Naoki Kanemitsu1 • Akihiro Mizuno1
Daisuke Yasumizu1 • Masashi Yada1 • Yohei Onga1 • Kazuo Yamanaka1
Received: 8 December 2014 / Accepted: 13 April 2015 / Published online: 12 May 2015
Ó The Japanese Association for Thoracic Surgery 2015
Abstract
Purpose The short-term outcome of physiologic repair
for congenitally corrected transposition of the great arteries
(ccTGA) is generally considered favorable; however, the
long-term outcome is the greatest problem, especially with
regard to right ventricular (RV) function and tricuspid re-
gurgitation (TR). Although tricuspid valve replacement
(TVR) appears to be a realistic choice for treating severe
TR, determining the timing of TVR may be difficult.
Methods We carried out a retrospective analysis of the
long-term outcomes of physiologic repair for ccTGA fo-
cusing on patients with TVR. The study involved 23 pa-
tients after physiologic repair 10 or more years prior. There
were 9 TVR cases in 5 pediatric patients (before age 18)
and 4 adult patients.
Results There were two late deaths; however, there was
no case related with cardiac events. Overall survival at 10
and 20 years were 95.5 and 90.2 %, respectively, and 7 of
8 patients after TVR were NYHA class I or II. No patient
has presented postoperative complications in the form of
bleeding or embolism after TVR with mechanical valve.
Conclusions An analysis of the results of physiologic
repair for ccTGA showed that the long-term outcome was
overall favorable. To maintain RV function, early TVR
may be a reasonable option, even in the management of
patients during childhood.
Presented at the 67th Annual Scientific Meeting of the Japanese
Association for Thoracic Surgery.
& Keiichi Hirose
khirose@tenriyorozu.jp
1
Department of Cardiovascular Surgery, Tenri Hospital,
200 Mishima-cho, Tenri, Nara 632-8552, Japan
123
•
Keywords Congenitally corrected transposition of the
great arteries (ccTGA)
Conventional physiologic repair

Tricuspid valve regurgitation (TR)
Tricuspid valve
replacement (TVR)
Introduction
Congenitally corrected transposition of the great arteries
(ccTGA) is a congenital anomaly that is characterized by
both atrioventricular and ventriculoatrial discordance [1].
Anatomical repair and physiologic repair are available as
surgical procedures for the treatment of ccTGA. Double-
switch operation, an anatomical surgery procedure, has
been reported to yield favorable outcomes [2, 3, 4] and its
use is on the increase, although factors such as the com-
plexity of the surgical manipulations have prevented it
from becoming a common procedure. Physiologic repair,
on the other hand, has a long history and has been called
‘‘conventional repair’’. Its short-term outcome is generally
considered to be favorable by normal standards [4]. Pa-
tients who have ccTGA without any accompanying cardiac
anomaly, such as a large ventricular septal defect (VSD) or
pulmonary stenosis, are sometimes considered eligible for
physiologic repair during follow-up observation with no
treatment. If such cases are taken into account, there seems
to be a large population for whom physiologic repair is
applicable. The greatest problem with physiologic repair is
the long-term outcome because the anatomical right ven-
tricle is used as the systemic ventricle [5].
In patients undergoing physiologic repair, it is important
to control the function of the tricuspid valve (systemic
atrioventricular valve) to preserve anatomical right ven-
tricular function, i.e., to control tricuspid valve regurgita-
tion [6]. If the form of the tricuspid valve in patients with
Gen Thorac Cardiovasc Surg (2015) 63:496–501
ccTGA is considered, tricuspid valve replacement (TVR)
with a mechanical valve appears to be a realistic choice as
a method for treating severe tricuspid regurgitation [7, 8,
9], although there has been a report of valvuloplasty for
this purpose [10]. However, considering the size of the
valve to be implanted and subsequent need for antico-
agulant therapy, determining the timing of TVR is a
challenging issue for cardiac physicians.
In this study, we carried out a retrospective analysis of
the long-term outcomes of physiologic repair for ccTGA at
our facility, focusing on patients with TVR.
Materials and methods
The study involved 23 patients who had undergone
physiologic repair for treatment of ccTGA at our hospital
10 or more years previously (We had three early deaths,
but these were the first three cases of physiologic repair for
ccTGA patients performed in the 1960s–1970s in our
hospital, and we excluded these because their hospitaliza-
tion medical records were lost and we had inadequate in-
formation). There were 13 males and 10 females. The
Rastelli procedure was employed for physiologic repair
(including VSD closure and left ventricle–pulmonary
artery conduit reconstruction) in 12 cases, and VSD closure
and/or TVR in other cases (Table 1). Mean age at surgery
was 9.8 ± 5.9 years (range 1 year and 2 months to
19 years and 6 months). These 23 cases were divided into
three groups: Group C, patients who had undergone TVR
during childhood (before age 18) (n = 5); Group A, who
had undergone TVR during adulthood (at age 18 or over)
(n = 4); and Group N, who had not undergone TVR
(n = 14). A retrospective comparison was made among
these three groups, using medical records and other test
data.
Table 2 shows the data from Group C before TVR. Case
4 had previously undergone a Waterston procedure. Cases
1 and 2 underwent TVR as the first physiologic repair. In
Table 1 Conventional physiologic surgery
Rastelli (VSD PC ? LV-PA conduit): 12
TVR: 3
VSD PC ? PA plasty: 2
VSD PC: 2
VSD PC ? TVR: 1
Rastelli ? TVR: 1
VSD PC ? MVP: 1
ASD PC: 1
VSD ventricular septal defect, PC patch closure, TVR tricuspid valve
replacement, MVP mitral valve plasty, ASD atricular septal defect, PA
pulmonary artery
497
Cases 3 and 5, TVR was performed after physiologic re-
pair. In Case 4, TVR was performed as physiologic repair
simultaneously with the Rastelli procedure. The age at
TVR ranged from 5 years and 0 months to 13 years and
1 month; the size of the prosthetic valve implanted was
25 mm or 27 mm. TVR was considered to be indicated in
cases of severe tricuspid regurgitation, or moderate pro-
gressive tricuspid regurgitation accompanied by right
ventricular dysfunction. In Case 1, where the patient had a
poorly formed tricuspid valve resembling Ebstein’s
anomaly and moderate to severe tricuspid regurgitation,
tricuspid valvuloplasty was initially attempted, but the re-
gurgitation could not be sufficiently controlled; so the op-
eration was switched to TVR in mid-procedure. Tricuspid
valvuloplasty was not attempted in any subsequent case.
Table 3 shows the data from Group A before TVR.
Cases 1 and 2 had undergone physiologic repair during
childhood and underwent TVR during adulthood. In Cases
3 and 4, TVR was performed as the first physiologic repair
during adulthood, accompanied by VSD closure in Case 3.
The age at TVR ranged from 19 years and 10 months to
31 years and 5 months, and the size of the prosthetic valve
implanted was 29–31 mm. The indications for TVR in
Group A were similar to those in Group C. In both Group C
and Group A, mechanical valves were used as prosthetic
valves, and postoperative anticoagulant therapy used war-
farin as standard medication, occasionally combined with
aspirin.
In Group N (n = 14), there were 10 males and 4 fe-
males. Physiologic repair was performed at a mean age of
8.6 ± 4.5 years, employing the Rastelli procedure in 10
cases and VSD closure in the other cases (Table 4).
The follow-up period after physiologic repair averaged
16.9 years (maximum 32.4 years).
Statistical analyses
Numeric variables are expressed as the mean ± standard
deviation. In multiple comparisons among independent
groups in which analysis of variance indicated significant
differences, the statistical value was determined according
to the Bonferroni/Dunn method. Differences between
groups were determined with Student’s t test. Statview
software (Abacus Concepts, Berkeley, CA, USA) was used
for all statistical analyses. P values of less than 0.05 were
considered significant.
Results
Of the 23 patients studied, 2 died during long-term follow-
up. One committed suicide (Group C, 20 years and
7 months) and the other died in a traffic accident (Group N,
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498 Gen Thorac Cardiovasc Surg (2015) 63:496–501

Table 2 The data from Group C before TVR

No. Male/female Operation Timing of TVR Indication for TVR

1 F TVR (#27) 11 years 6 months Moderate TR TAP ? TVR

2 F TVR (#25) 6 years 11 months Severe TR
3 M VSD PC (3 years 1 month) ? TVR (#25) 5 years 0 month Severe TR
4 F Waterston (4 months) ? Rastelli/TVR (#27) 13 years 1 month Moderate TR RV dysfunction
5 M Rastelli (5 years) ? TVR (#27) 6 years 1 month Severe TR

Table 3 The data from Group A before TVR

No. Male/female Operation Timing of TVR Indication for TVR

1 F VSD PC/PA plasty (11 years 5 months) ? TVR (#29) 31 years 5 months Severe TR
2 F Rastelli (7 years 7 months) ? TVR (#31)/MAP 19 years l0 months Severe TR RV dysfunction
3 M VSD PC/TVR (#33) 21 years 1 month Severe TR
4 F TVR (#31) 25 years 0 month Severe TR

Table 4 Conventional physiologic surgery in Group N
Rastelli (VSD PC ? LV-PA conduit): 10
VSD PC ? PA plasty: 1
VSD PC: 1
VSD PC ? MVP: 1
ASD PC: 1
VSD ventricular septal defect, PC patch closure, MVP mitral valve
plasty, ASD atricular septal defect, PA pulmonary artery
18 years and 9 months). Thus, neither of the deaths was
related to the heart. The survival rates at 10 and 20 years
were 95.5 and 90.2 %, respectively, as calculated from the
Kaplan–Meier curve.
Reoperation, excluding TVR, was performed in 1 case
during the acute postoperative period and in 5 cases during
the chronic postoperative period. The former case was Case
1, where valvuloplasty was switched to TVR during the
first operation. However, perivalvular leakage developed
soon afterwards, making the control of congestive heart
failure impossible and necessitating perivalvular leakage
repair on the 5th postoperative day. This patient followed
an uneventful course after repair. The reoperation during
the chronic postoperative period was pacemaker implan-
tation in 1 case (Case 2 from Group A, undergoing pace-
maker implantation 5 years and 6 months after a Rastelli
procedure, before TVR), left ventricle–pulmonary artery
conduit replacement with a Rastelli procedure in 2 cases,
and pulmonary arterioplasty in 2 cases (each from Group
N). No patient underwent reoperation related to the tri-
cuspid valve.
Table 5 shows the current data for the three groups. The
NYHA class was II or lower in all cases, except for one
patient from Group A who was rated as class III. Brain
natriuretic peptide levels tended to be lower in Group N, but
the difference was not significant because of the small
sample size. Complete atrioventricular block was seen in 1
case, requiring pacemaker implantation. There was no other
case with second- or higher-degree atrioventricular block
and no significant difference was noted in heart rate. The
function of the right ventricle (systemic ventricle) was
evaluated by echocardiography, but there was no significant
inter-group difference in right ventricular ejection fraction
calculated by Simpson’s method. This parameter also did
not differ significantly between TVR patients [Group
C ? Group A combined (n = 8)] and non-TVR patients
(Group N) (P [ 0.05). Moderate or more severe tricuspid
insufficiency was noted in one case from Group N.
Tables 6 and 7 show the postoperative to current status
of each case in Groups C and A. The follow-up period after
TVR ranged from 13 years and 3 months to 21 years and
9 months in Group C, and from 13 years 6 months to
21 years 6 months in Group A. Two patients (Cases 1 and
3) who underwent TVR alone had a history of pregnancy
and delivery. In Case 1, the first baby was lost to hy-
poplastic left heart syndrome, but two other babies were
free of complications. Case 1 from Group C is currently
free of symptoms requiring valve size-up, more than
10 years after TVR using a 25 mm prosthetic valve. Case 2
from Group A, rated as NYHA class III, underwent a
Rastelli procedure at age 7 years and 7 months and TVR at
age 19 years and 10 months. This patient, 14 years and
2 months after the last operation, now has persistent right
ventricular dysfunction, accompanied by an elevation in
brain natriuretic peptide levels. This case is under follow-
up with medical treatment of heart failure.

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Table 5 The current data for the three groups

Group C (n = 4) Group A (n = 4) Group A ? C (TVR) (n = 8) Group N (non TVR) (n = 14)

NYHA I;3 II;1 I;3 II;1 I;6 II;2 I;12 II;2

Mean BNP (pg/ml) 53.1 (n = 3) 66.0 (n = 3) 59.1 (n = 6) 40.1 (n = 8)
Mean HR 69.6 67.5 68.4 76.6
Mean RVEF (%) 54.7 (n = 3) 53.7 (n = 4) 54.1 (n = 7) 57.6 (n = 12)
[TR3 0 0 0 1

Table 6 Postoperative to current status of Group C

No. Male/female Operation Timing of TVR Follow-up period after TVR Remarks

1 F TVR 11 years 6 months 19 years 4 months

2 F TVR 6 years 11 months 21 years 9 months 1 Birth
3 M VSDPC TVR 5 years 0 month 15 years 7 months Late death (suicide)
4 F Rastelli TVR 13 years 1 month 13 years 3 months
5 M Rastelli ? TVR 6 years 1 month 11 years 6 months RVEF <50 %

Table 7 Postoperative to current status of Group A

No. Male/ Operation Timing of TVR Follow-up period after TVR Remarks
female

1 F VSDPC ? TVR 31 years 5 months 13 years 6 months Valvular PS, b blocker

2 F Rastelli ? TVF 19 years l0 months 14 years 2 months Complete AV block ? pacemaker,
RV dysfunction, BNP161, NYHAIII
3 M VSDPC TVR 21 years 1 month 21 years 6 months
4 F TVR 25 years 0 month 16 years 0 month

Discussion In the present study, right ventricle function (systemic

In the present study, we showed that the long-term outcome
was overall favorable after physiologic repair for ccTGA
patients. In the field of cardiovascular surgery, the era in
which the focus was simply on saving life has given way to
an era when more emphasis is placed on strategies for sur-
gical treatment that take into account the patient’s postop-
erative quality of life and long-term outcome. It is clear that
strategies for the treatment of ccTGA should tend to favor
anatomical repair, i.e., double-switch surgery, if surgeons
are concerned with the long-term outcome. However, be-
cause of its complexity, this surgery has not yet become a
common procedure. In patients who have no intracardiac
anomaly that requires early repair (e.g., VSD), a treatment
strategy involving anatomical repair may not be valid, unless
another surgical intervention (e.g., pulmonary artery band-
ing [11]) is actively promoted as a preparatory step. Con-
sequently, physiologic repair may play a significant role as
first choice in the treatment of ccTGA for some time to come.
ventricle function) has been maintained fairly well with/
without TVR. The long-term outcome is an issue of great
concern when physiologic repair is applied. One factor that
may possibly determine the long-term outcome of this
surgery is maintenance of the function of the right ventri-
cle, which is used as systemic ventricle. Furthermore,
maintenance of tricuspid valve function (particularly con-
trol of tricuspid insufficiency) is an important issue in
maintaining right ventricular function. In resolving this
issue, not only medical treatment but also surgical treat-
ment strategy plays a key role.
We encountered a case (Case 1 from Group C) where
tricuspid valvuloplasty was attempted, but regurgitation
could not be controlled because the valve assumed an
Ebstein-like form; this necessitated a switch to valve re-
placement during the operation. For treatment of the atri-
oventricular valve (mitral valve) of the systemic ventricle,
mitral valvuloplasty is usually the procedure of first choice,
although the procedure selected may vary depending on the

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valve’s form and condition [12]. As a rule, a similar
strategy should also be adopted when tricuspid valve
treatment is required in patients with ccTGA. However, the
tricuspid valve often has morphological abnormalities,
occasionally assuming an Ebstein-like form [1, 7]. Subse-
quent to our experience about the switch to valve re-
placement (Case 1 from Group C), valve replacement has
been applied as a basic procedure at our center.
We performed nine tricuspid valve replacements with a
mechanical valve, with no valve-related complications in
the present study. For valve replacement, either a biologi-
cal valve or a mechanical valve needs to be selected. After
implantation of a mechanical valve, anticoagulant therapy,
primarily using warfarin, is indispensable, and medication
and dose level adjustment are sometimes difficult in chil-
dren. Furthermore, there are many females with ccTGA,
requiring surgeons to consider the possibility of pregnancy
and delivery [13, 14]. The use of a biological valve is
beneficial in terms of the need for anticoagulant therapy,
but the durability of biological valves is poor compared to
the mechanical type, necessitating reoperation. At our
hospital, a mechanical valve is used in all cases, always
followed by the use of warfarin (sometimes combined with
antiplatelet drugs) to maintain a prothrombin time–inter-
national normalized ratio in the range 1.5–2.5. To date, no
patient has presented postoperative complications in the
form of bleeding or embolism. Furthermore, adjustment of
medication in cooperation with obstetricians/gynecologists
has enabled pregnancy and delivery by 2 women (3 times
in total). On the basis of these results, we consider the
choice of a mechanical valve to be acceptable at present.
We essentially perform TVR as soon as right ventricular
dysfunction is noted. The timing of the surgical interven-
tion into the valve is a topic of controversy. It seems likely
that control of tricuspid regurgitation as soon as possible
leads to maintenance of right ventricular function; how-
ever, surgeons tend to resist early valve replacement be-
cause of the abovementioned need for anticoagulant
therapy, or the difficulty in selecting the size of valve to be
implanted. If valvuloplasty can be judged possible intra-
operatively, it should be selected in preference to valve
replacement. However, this is not likely to be easy, because
the morphological valve abnormalities tended to involve in
each part of the annulus, leaflets, and subvalvular tissues
especially in cases with TVR in childhood, as in this series.
The function of the right ventricle (systemic ventricle)
was evaluated by echocardiography, but there was no inter-
group difference in right ventricular ejection fraction be-
tween Group C ? A and Group N, as calculated by
Simpson’s method (P \ 0.05). As stated above, no case in
the present study was complicated by bleeding or em-
bolism, including patients who had undergone valve re-
placement at age 18 or less. Taken together, these results
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Gen Thorac Cardiovasc Surg (2015) 63:496–501
suggest that if right ventricular dysfunction is noted, early
surgical intervention may be a reasonable option, even
though valve replacement may be needed in some cases.
Evaluation of a larger number of patients will be required
to confirm this, since the number of cases analyzed to date
is too small.
In the present study, although there was no case where
atrioventricular block progressed soon after valve re-
placement, one patient exhibited progression during the
chronic postoperative period. Arrhythmia is another factor
that is closely involved in patients’ long-term quality of life
[15]. After our experience with this case, we have made it a
rule to perform myocardial lead implantation concomi-
tantly when performing surgery on more mature patients.
On the other hand, none of our patients has developed
tachycardiac episodes of atrial origin.
It is not uncommon for ccTGA to be accompanied by
abnormal heart rotation, necessitating close attention to
this kind of anomaly when performing surgery such as
TVR. In the present study, according to the Van Praagh
classification, the anomaly was {I, D, D} in 1 case and {S,
L, L} in the other cases. We even encountered a case
where the left atrium was located completely on the re-
verse side, hampering the visual field for the surgeon
during surgery. In such cases, visual field expansion and
evaluation will be difficult during the leakage test (salin
injection test) at the time of valvuloplasty. The standard
approach to the tricuspid valve is from the right side of
the left atrium, but we encountered two cases where a
better visual field was obtained with an approach involv-
ing incision of the atrial septum. When dealing with pa-
tients of smaller physique, we need to consider modifying
parameters such as the size of the prosthetic valve and the
angle of prosthetic valve insertion. In this respect, the
visual information yielded from imaging techniques such
as multi-detector computed tomography and magnetic
resonance is important and its adequate evaluation before
surgery is essential.
Conclusions
An analysis of the results of physiologic repair for ccTGA,
focusing on patients who had undergone TVR, showed that
the long-term outcome was favorable overall. Some pa-
tients underwent TVR during infancy or early childhood,
but none of them developed complications related to
bleeding or the coagulation system. To maintain right
ventricular function, early TVR may be a reasonable op-
tion, even in the management of patients during childhood.
Conflict of interest All the authors have declared no competing
interest. None of the authors of this manuscript has any financial of
Gen Thorac Cardiovasc Surg (2015) 63:496–501
personal relationship with other people or organization that could
inappropriately influence their work.
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