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Physiologic Versus Anatomic Repair of
Congenitally Corrected Transposition of the Great
Arteries: Meta-Analysis of Individual Patient Data
Abdullah A. Alghamdi, MD, Brian W. McCrindle, MD, and Glen S. Van Arsdell, MD
Division of Cardiac Surgery and Congenital Cardiac Surgery Program, Department of Surgery, University of Toronto, Toronto,
Ontario, Canada
The objective of this meta-analysis of individual pa-
tients’ data was to compare the immediate outcomes of
anatomic and physiologic repair of congenitally cor-
rected transposition of the great arteries. Eleven nonran-
domized studies, involving 124 patients, met the inclu-
sion criteria for this review. The Rastelli type anatomic
repair and the era of surgery were significantly related to
C ongenitally corrected transposition of the great ar-
teries (CCTGA) is a congenital cardiac anomaly that
is characterized by atrioventricular (AV) and ventriculo-
arterial (VA) discordant connection [1]; thus, physiologi-
cally corrected. It accounts for less than 1% of congenital
heart diseases [2] and is almost always associated with
coexistent cardiac anomalies [3]. Ventricular septal defect
(VSD) is the most common coexisting anomaly and is
present in about 80% of the cases [1]. Other coexisting
anomalies include the following: pulmonary valve steno-
sis or atresia, atrial septal defect, systemic (tricuspid)
valve abnormalities, and heart block [2, 4].
The classical surgical approach to CCTGA has been to
repair the associated lesions without addressing the AV
and VA discordance. In this approach, the morphologi-
cally right ventricle remains as the systemic ventricle and
the morphologically right AV valve (tricuspid) remains as
the systemic AV valve [5]. Concerns about the long-term
function of the morphologic right ventricle and the sys-
temic AV valve (tricuspid) have led to the concept of
anatomic repair that incorporates the morphologically
left ventricle and morphologically left AV valve (mitral)
in the systemic circulation [6].
To date, there are no studies comparing the immediate
outcomes of these surgical approaches. Reports of small,
single-center studies assessing immediate results of ei-
ther anatomic or physiologic surgical approaches were
encouraging so that it appeared to be of scientific and
practical importance to collect the individual patients’
data into one large database. Such an approach may yield
a more precise estimate of the effect of each surgical
approach on immediate outcomes over a wide range of
the spectrum of CCTGA patients. The objective of this
Address correspondence to Dr Alghamdi, Division of Cardiac Surgery
and Congenital Cardiac Surgery Program, The Hospital for Sick Children,
555 University Ave, Suite 1525, Toronto, ON, Canada M5G 1X8; e-mail:
abdullah.alghamdi@utoronto.ca.
© 2006 by The Society of Thoracic Surgeons
Published by Elsevier Inc
the outcome in different tested models. Entering all
variables into the logistic regression model showed a
significant protective effect of the Rastelli type anatomic
repair (odds ratio ⴝ 0.05, 95% confidence interval: 0.01,
0.50, p ⴝ 0.02).
(Ann Thorac Surg 2006;81:1529 –35)
© 2006 by The Society of Thoracic Surgeons
review was to meta-analyze individual patients’ data to
compare the immediate outcomes of anatomic and phys-
iologic repair of CCTGA.
Material and Methods
Inclusion and Exclusion Criteria
All published studies that included patients with CCTGA
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who underwent a definitive repair procedure were in-
cluded. The intervention was the definitive repair of
CCTGA (defined as physiologic or anatomic repair).
Physiologic repair entails utilizing the morphologic right
ventricle (RV) as a systemic ventricle and addressing the
associated intracardiac lesions such as a VSD. Anatomic
repair entails addressing the anatomic correction of
CCTGA; therefore, the morphologically left ventricle
supports systemic circulation. In the anatomic repair, the
atrial switch procedure (Senning or Mustard) is com-
bined with either an arterial switch or ventricular (Ras-
telli) level repair. The primary outcome was the incidence
of postoperative all-cause in-hospital mortality. Studies
that met any one of the following criteria were excluded:
failure to measure the outcome of interest (ie, in-hospital
mortality), unspecified period of follow-up, insufficient
patients’ data, and inability to extract individual patients’
data (eg, when a study provides aggregate data only).
Literature Search and Data Extraction
Studies were identified by searching MEDLINE, EM-
BASE, and the Cochrane controlled trial register (CCTR)
on the Cochrane library from the earliest achievable date
of each database to May 2005, supplemented by manual
search of reference lists of retrieved studies. The follow-
ing terms and keywords were used: [transposition of
great vessels OR congenitally corrected transposition of
the great arteries OR CCTGA OR (corrected AND trans-
position)]. The highly sensitive search strategy for iden-
0003-4975/06/$32.00
doi:10.1016/j.athoracsur.2005.09.035
 1530 REVIEW ALGHAMDI ET AL Ann Thorac Surg
PHYSIOLOGIC VERSUS ANATOMIC REPAIR OF CCTGA 2006;81:1529 –35

Table 1. Characteristics of Included Studies

Year of Year of Surgery Sample
Author Publication (Range) Country Size

Metcalfe and colleagues [72] 1983 1970–1980 United Kingdom 19

Di Donato and colleagues [73] 1992 1990–1991 Italy 02
Yamagishi and colleagues [74] 1993 1989–1991 Japan 11
Yagihara and colleagues [75] 1994 1987–1990 Japan 10
Imai and colleagues [71] 1994 1989–1993 Japan 09
Stumper and colleagues [76] 1995 1991–1993 United Kingdom 04
Delius and colleagues [77] 1996 1993–1994 United Kingdom 03
Reddy and colleagues [5] 1997 1993–1996 United States 17
Metras and colleagues [78] 1997 1985–1997 France 08
Sharma and colleagues [79] 1998 1994–1998 India 14
Imamura and colleagues [80] 2000 1993–1998 United States 27

tifying clinical controlled trials in MEDLINE was also
used [7]. No language restrictions were applied.
The studies retrieved by the search strategy were
reviewed by one reviewer (AA) and relevant studies were
selected according to the definitions in the inclusion and
exclusion criteria. A bibliographic software (Reference
Manager V.10; Thomson Scientific, Philadelphia, PA) was
used to download all references and ensure the absence
of duplication of references. Data were extracted by the
above reviewer. Data collected from each study included
individual patients’ data on the following: age at the time
of definitive repair, type of repair, presence of ventricular
septal defect, preoperative pulmonary valve abnormality,
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covariates age at definitive repair, preoperative pulmo-
nary valve abnormality, preoperative tricuspid valve ab-
normality, preoperative arrhythmia, and preoperative
shunt procedures were entered one at a time. Thereafter,
the full model including the main model and all other
covariates was built. Results were expressed in odds
ratios with physiologic repair as a reference category. The
final model was assessed by quantifying the area under
the receiver operating characteristic (ROC) curve and the
probability associated with Hosmer-Lemeshow goodness
of fit. An area under ROC curve of 0.5 indicates no
predictive discrimination (equivalent to chance alone)
and an area of 1.0 indicates perfect separation of patients

preoperative tricuspid valve abnormality, preoperative
arrhythmia, preoperative shunting procedures, and in-
hospital mortality. Reason(s) for exclusion were docu-
mented for all excluded studies. Data were extracted onto
predesigned data abstraction forms.
with different outcomes [8, 9]. Hosmer-Lemeshow good-
ness-of-fit statistics compares the predicted probability
with actual probability within population subgroups; ie,
the larger the p value the better the fit [10].

Data Analysis Results

The statistical software package SAS (version 8.2, SAS
Institute, Cary, NC) was used for all statistical analyses.
Categorical variables were summarized as frequencies
and percentages, and continuous variables as means and
standard deviations. Categorical variables were com-
pared using the Pearson ␹2 test for independent propor-
tions, and the Student t test was used to compare con-
tinuous variables. The sample population was divided
into three groups: physiologic repair, anatomic repair
(Rastelli type), and anatomic repair (with arterial switch
procedure).
For comparison of surgical approaches for the primary
outcome, the logistic regression statistical technique was
used. The reference group was selected to be protective
compared with other categories for each variable under
consideration. To adjust for the fact that the patient
population was different between studies, the study era
was entered into the model as a binary variable (before
1995 and 1995 or after).
Due to the large number of covariates and the small
number of events, a main model containing surgical
approach and study era was designed; subsequently, the
Sixty-five references were identified from the search
strategy. There were no randomized studies. Fifty-four
studies were excluded after examining the entire manu-
scripts. The reasons for exclusion were the following:
outcome of interest (in-hospital mortality) was not mea-
sured (five studies) [11–15], different target population
(eleven studies) [3, 16 –25], insufficient patients’ data (four
studies) [6, 26 –28], patients’ data were presented in an
aggregate form (twenty-four studies) [29 –52], and multi-
ple reasons (ten studies) [2, 53– 61].
Eleven studies, involving 124 patients, met the inclu-
sion criteria for this review. In these studies, sample sizes
ranged from 2 to 27 patients. In total, 69 patients under-
went anatomic repair (Rastelli type), 25 patients under-
went anatomic repair (with arterial switch), and 30 pa-
tients underwent physiologic repair. All studies were
from single centers: France (1), India (1), Japan (3), Italy
(1), United Kingdom (3), and United States (2). Table 1
summarizes the characteristics of the included studies.
The age of included patients at the time of surgery
ranged from 0.25 to 55 years. One hundred and twelve
(90%) patients had an associated ventricular septal de-
Ann Thorac Surg
2006;81:1529 –35
Table 2. Characteristics of Included Patients in Each Surgical Approach
Physiologic Repair
Variable (n ⫽ 30)
Age in years (mean ⫾ SD) 19.2 (15.8)
Preoperative pulmonary valve abnormality 18 (60%)
Preoperative shunt procedure 6 (20%)
Preoperative tricuspid valve abnormality 14 (47%)
Preoperative arrhythmia 9 (30%)
Surgery performed before 1995 19 (63%)
Surgery performed in 1995 or after 11 (37%)
fect, 80 (65%) had an associated pulmonary valve abnor-
mality (stenosis or atresia), 53 (43%) underwent a previ-
ous shunt procedure, 51 (46%) had a preoperative
tricuspid valve abnormality, and 13 (12%) had a preop-
erative arrhythmia. Fifty-one (41%) patients underwent
the definitive repair procedure before 1995, whereas 73
(59%) patients underwent the definitive repair in 1995 or
after. The characteristics of the included patients in each
surgical approach are summarized in Table 2.
In total, 13 patients died during their hospitalization.
Of those, 7 (23%) patients were in the physiologic repair
group, whereas 3 (4%) and 3 (12%) were in the anatomic
(Rastelli type) and anatomic (with arterial switch), re-
spectively. Unadjusted mortality difference was statisti-
cally significant (␹2 ⫽ 8.11, degrees of freedom [df] ⫽ 2, p
⫽ 0.01).
The results of multivariable logistic regression of the
main model (surgical approach and era of surgery), and
the main model with other covariates (age of the patient
at the time of surgery, preoperative pulmonary valve
abnormality, preoperative tricuspid valve abnormality,
preoperative arrhythmia, and previous shunting proce-
dure) entered one at a time are summarized in Table 3.
In the main model, anatomic repair (Rastelli type) was
associated with a significant reduction in the in-hospital
mortality (odds ratio [OR] ⫽ 0.20, 95% confidence interval
[CI]: 0.05, 0.85, p ⫽ 0.02), and era 1 (operations before
1995) was associated with a significant risk of mortality
(OR ⫽ 5.70, 95% CI: 1.30, 24.70, p ⫽ 0.02). Entering the
other covariates into the main model did not dramatically
change the estimates or level of significance of the effect
of era. Similarly, the estimates and levels of significance
of the protective effect of anatomic (Rastelli type) repair
did not change with the addition of the other covariates
into the main model with the exception of preoperative
pulmonary valve abnormality (OR ⫽ 0.24, 95% CI: 0.05,
1.20, p ⫽ 0.16).
Entering all variables into the final model revealed
findings similar to the main model with a significant
protective effect of the anatomic repair (Rastelli type),
and significant increased risk with era 1 (before 1995);
(OR ⫽ 0.05, 95% CI: 0.01, 0.50, p ⫽ 0.02) and (OR ⫽ 9.66,
95% CI: 1.54, 60.4, p ⫽ 0.01), respectively (Table 4). The
area under the receiver operating characteristics curve of
the final (all variables) model was 0.87 and the probabil-
REVIEW ALGHAMDI ET AL 1531
PHYSIOLOGIC VERSUS ANATOMIC REPAIR OF CCTGA
Anatomic Repair Anatomic Repair
(Rastelli Type) (With Arterial Switch)
(n ⫽ 69) (n ⫽ 25) p Value
5.3 (3.8) 2.1 (1.7) ⬍0.001
60 (87%) 2 (8%) ⬍0.001
39 (57%) 8 (32%) ⬍0.001
21 (36%) 16 (70%) 0.02
2 (3%) 2 (11%) 0.001
27 (39%) 5 (20%) 0.02
42 (61%) 20 (80%) 0.02
ity associated with Hosmer-Lemeshow goodness of fit
statistics ⫽ 0.54.
Comment
The pathological anatomy of CCTGA was first described
by Rokitansky in 1875 [1]. The clinical features were
recognized later. In CCTGA, the systemic venous blood
enters into the right atrium, which drains through a
bicuspid mitral valve into a left ventricle, which ejects
blood into the pulmonary artery. Pulmonary venous
blood returns to a left atrium, which drains through a
tricuspid valve into a morphological right ventricle that
ejects into the aorta. This double anatomical discordance
is physiologically corrected.
Only 1% to 2% of CCTGA patients have no coexisting
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anomalies [3]. A few studies reported that such patients
can live up to 80 years without surgical intervention [12,
24, 25]. However, in these reports patients with isolated
CCTGA presented with heart failure and varying degrees
of AV valve regurgitation. In a series of 111 patients with
CCTGA with a follow-up reaching up to 20 years, Lund-
strom and colleagues [43] reported that 20 patients of the
series who were over the age of 20 years did not require
surgical intervention [43]. Three women among them
became pregnant and gave birth to four healthy babies
and one miscarriage [43].
Surgical intervention is indicated when CCTGA is
associated with clinically important intracardiac anoma-
lies such as a VSD or pulmonary valve abnormalities. The
classical surgical approach has been to repair the associ-
ated intracardiac anomalies without addressing the ana-
tomic discordance (ie, physiologic repair). However, a
number of studies have demonstrated depressed right
ventricular function, especially during exercise [11, 14, 34,
46]. Concerns about the right ventricular function, along
with frequent dysfunction of the tricuspid valve have led
to the concept of anatomic repair, which was originally
suggested by Ilbawi and colleagues [6] in 1990 for pa-
tients with CCTGA, VSD, and pulmonary stenosis. In this
setting, the atrial switch procedure (Senning or Mustard)
is combined with either an arterial switch or ventricular
(Rastelli) level repair.
The focus of this review of individual patients’ data
was to address the following question: Which surgical
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PHYSIOLOGIC VERSUS ANATOMIC REPAIR OF CCTGA 2006;81:1529 –35

Table 3. Statistical Details of the Multivariable Logistic Regression

Odds 95% 95%
Model Ratio LCL UCL p Value

Main model
A vs P 0.20 0.05 00.85 0.02
D vs P 0.90 0.18 04.80 0.35
Era of surgery 5.70 1.30 24.70 0.02
Main model ⫹ age at the time of surgery
A vs P 0.12 0.02 00.54 0.01
D vs P 0.42 0.07 02.70 0.76
Era of surgery 6.02 1.33 27.21 0.02
Age 0.94 0.86 01.02 0.14
Main model ⫹ Preoperative pulmonary valve abnormality
A vs P 0.24 0.05 01.20 0.16
D vs P 0.66 0.11 04.05 0.75
Era of surgery 5.75 1.32 25.12 0.01
Preoperative pulmonary valve abnormality 0.51 0.10 02.45 0.40
Main model ⫹ preoperative tricuspid valve abnormality
A vs P 0.19 0.03 01.07 0.02
D vs P 1.64 0.24 11.11 0.15
Era of surgery 7.35 1.39 38.68 0.01
Preoperative tricuspid valve abnormality 0.69 0.17 02.79 0.60
Main model ⫹ preoperative arrhythmia
A vs P 0.16 0.02 00.91 0.01
D vs P 1.54 0.25 09.27 0.12
Era of surgery 6.93 1.32 36.45 0.02
Preoperative arrhythmia 0.76 0.14 03.99 0.74
Main model ⫹ preoperative shunt procedure
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A vs P 0.16 0.03 00.82 0.02

D vs P 0.87 0.16 04.64 0.34
Era of surgery 5.57 1.27 24.31 0.02
Preoperative shunt procedure 1.44 0.36 05.61 0.59

A ⫽ anatomic repair (Rastelli type); D ⫽ anatomic repair (with arterial switch); LCL ⫽ lower confidence limit of the odds ratio; P ⫽ Physiologic
repair; UCL ⫽ upper confidence limit of the odds ratio.

approach is superior in reducing the incidence of in-
hospital mortality in patients with CCTGA? This study
addresses this question in a systematic way. Our results
were derived from 11 studies encompassing 124 patients,
of whom 56.65% underwent anatomic (Rastelli) level
repair, 20.16% underwent anatomic (with arterial switch)
repair, and 24.19% underwent physiologic repair.
The results of this study suggest that the Rastelli type
anatomic repair is probably superior to the other surgical
approaches in terms of the in-hospital mortality. This
finding could be explained in part by the technical
advantages of the Rastelli type anatomic repair approach
which includes the following: first, closing the VSD
through the right ventriculotomy allows a more ready

Table 4. Statistical Details of the Multivariable Logistic Regression of the Full Model
Parameter Odds Ratio 95% LCL 95% UCL p Value

Anatomic (Rastelli) vs physiologic repair 0.05 0.01 0.50 0.02

Anatomic (double switch) vs physiologic repair 0.22 0.01 02.97 0.98
Era of surgery 9.66 1.54 60.40 0.01
Age at the time of surgery 0.91 0.82 1.01 0.09
Preoperative pulmonary valve abnormality 0.31 0.05 01.81 0.19
Preoperative tricuspid valve abnormality 1.64 0.27 09.69 0.58
Preoperative arrhythmia 0.56 0.08 03.67 0.55
Preoperative shunt procedure 4.67 0.84 25.76 0.07

LCL ⫽ lower confidence limit of the odds ratio; UCL ⫽ upper confidence limit of the odds ratio.
Ann Thorac Surg
2006;81:1529 –35
accessibility to the right ventricular aspect of the septum
and tricuspid valve than the other approaches, which is
important in avoiding the conduction system and ad-
dressing tricuspid valve abnormalities [6, 62, 63]. In our
included patients, the Rastelli type anatomic repair was
associated with the lowest incidence of postoperative
heart block compared with the anatomic repair with
arterial switch and the physiologic repair (8%, 19%, and
65%, respectively; p ⬍ 0.001). Additionally, the Rastelli
type repair was associated with a lower incidence of
postoperative systemic AV valve regurgitation compared
with the physiologic repair (6% and 53%, respectively; p
⬍ 0.001). These two variables were not included in the
logistic regression models as the proportions of missing
values were high; 22% and 31% for the postoperative
heart block and postoperative AV valve regurgitation,
respectively.
Second, there is no coronary transfer in the Rastelli
type anatomic repair, which decreases coronary-related
complications. Some reports have shown that the coro-
nary pattern and technique of transfer are related to
postoperative mortality [64, 65]. Furthermore, coronary
artery stenosis or obstructions are concerns after coro-
nary transfer. Some studies showed that 3% to 8% of
patients develop coronary stenosis or occlusion after
arterial switch procedure [66].
Another potential explanation of our finding is related
to the changes in hemodynamics after anatomic repair. In
the physiologic repair approach the morphologic RV
supports the systemic circulation, which leads to progres-
sive ventricular dilatation, failure, and subsequent tricus-
pid valve regurgitation [35]. The basic mechanism is
probably related to the progressive increase in the mor-
phologic RV (systemic RV) pressure, which leads to a
commitment of the ventricular septum rightward leading
to tricuspid valve (systemic AV valve) regurgitation [67].
Deterioration of the RV function with tricuspid valve
regurgitation is associated with worse clinical outcomes
in patients with CCTGA [35]. Anatomic repair, on the
other hand, utilizes the morphologic LV as the systemic
ventricle; as a result, the LV pressure will increase and
the ventricular septum will shift from a rightward to a
midline position. Subsequently, an appreciable improve-
ment of the RV pressures and tricuspid valve function
will be noticed [67].
Kreutzer and colleagues [68] reviewed a cohort of 101
consecutive patients who underwent Rastelli repair for a
simple transposition of the great arteries (TGA) between
1973 and 1998. Of this cohort 73 patients had pulmonary
stenosis and 18 had pulmonary atresia. The early mortal-
ity (defined as death within 30 days after operation) was
7%. In our included patients, 4% of the Rastelli repair
group died within the hospital stay.
The focus of this individual patients’ data meta-
analysis was on the in-hospital mortality. Data was not
available to address the long-term benefits or risks of
different surgical approaches. However, there are sug-
gested long-term benefits of the Rastelli repair. These
benefits may include the avoidance of the neoaortic valve
incompetence (ie, maintenance of the aortic valve integ-
REVIEW ALGHAMDI ET AL 1533
PHYSIOLOGIC VERSUS ANATOMIC REPAIR OF CCTGA
rity) and an apparent lower incidence of heart block. In
the arterial switch procedure, the pulmonary valve will
form the new aortic valve. Reports have indicated that an
appreciable percentage of patients develop neoaortic
valve regurgitation after the arterial switch procedure
which might reach up to 30% [69]. Another long-term
complication that is of concern is the development of
surgically induced arrhythmias as suture line and suture
load may be related to the development of postoperative
arrhythmias. Gandhi and colleagues [70] demonstrated
that atrial arrhythmias were related to the suture line in
an animal model that underwent a modified Fontan
procedure. This concept may be important in avoiding
the suture sites that are associated with the development
of arrhythmias.
This meta-analysis does not answer the question of
which patients’ subgroups may benefit from one surgical
approach or another. In the earlier time cohort (before
1995), the proportion of patients who underwent the
physiologic repair was 63%, which dropped to 37% after
1995. This may indicate increased adoption of the ana-
tomic surgical approach. Rastelli type anatomic repair
might not be the optimal approach in all patients with
CCTGA. Reports have indicated that anatomic repair in
patients with right ventricular or tricuspid valve dysfunc-
tion offers the greatest immediate benefits, in the form of
improved systemic ventricular and AV valve function [5,
71].
This meta-analysis of individual data is not a substitute
for well-designed large studies that compare different
REVIEWS
surgical approaches with regard to the immediate and
long-term clinical outcomes. The optimal means of com-
paring anatomic and physiologic surgical approaches of
CCTGA would be a randomized clinical trial. However,
given the paucity of this condition and individualized
nature of its complex repairs, such a trial is unlikely to be
performed. Well-designed large multicenter cohort stud-
ies that address early and late outcomes would be the
most practical and feasible study designs.
Conclusion
This review revealed that the anatomic (Rastelli) repair of
CCTGA was associated with a significant improvement
in the incidence of the in-hospital mortality. Further
studies are warranted to confirm the result of meta-
analysis, to explore late outcomes, and to define patients’
subgroups that could benefit from other surgical
approaches.
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