1. Comparable Systemic Ventricular Function in
Healthy Adults and Patients With Unoperated
Congenitally Corrected Transposition Using MRI
Dobutamine Stress Testing
Ali Dodge-Khatami, MD, Igor I. Tulevski, MD, Ger B. W. E. Bennink, MD, PhD,
J. Franc¸ois Hitchcock, MD, PhD, Bas A. J. M. de Mol, MD, PhD,
Ernst E. van der Wall, MD, PhD, and Barbara J. M. Mulder, MD, PhD
Divisions of Cardiothoracic Surgery and Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, Division of
Cardiothoracic Surgery, Wilhelmina Children’s Hospital, University of Utrecht, Utrecht, Department of Cardiology, Leiden
University Medical Center, Leiden, The Netherlands
Background. Failure of the systemic right ventricle
(RV) often complicates adult survival in unoperated or
physiologically repaired congenitally corrected transpo-
sition of the great arteries (CCTGA). Healthy controls
schematically represent an optimal outcome of anatomic
repair, which is increasingly performed to treat CCTGA.
Magnetic resonance imaging dobutamine stress testing
measures cardiac reserve, and sets to compare the left
ventricle of controls with the systemic RV of unoperated
and physiologically repaired patients with CCTGA.
Methods. Baseline and stress magnetic resonance im-
aging (maximum dobutamine dose, 15 ␮g/kg/min) as-
sessed systemic RV function in 13 minimally or asymp-
tomatic adult patients with CCTGA (unoperated, n ‫؍‬ 7;
physiologically repaired, n ‫؍‬ 6). The left ventricles of 11
healthy age-matched adults served as controls.
Results. Baseline and stress end-diastolic volumes
were similar between the systemic RV of unoperated
patients and the left ventricle of controls, as well as base
line end-systolic volumes. Stress ejection fraction was
lower in unoperated and physiologically repaired pa-
tients (70 ؎ 6% and 60 ؎ 5%, respectively, vs healthy
controls (84 ؎ 8%). However, comparable with healthy
controls, both subsets of CCTGA patients responded
appropriately to dobutamine stress, as illustrated by
similar RV stroke volume, heart rate, mean blood pres-
sure, and cardiac index.
Conclusions. Compared with the left ventricles of
healthy controls, both patient groups had larger systemic
RV volumes, diminished ejection fraction, but an appro-
priate response to dobutamine stress. Values of unoper-
ated patients are closer to normal than physiologically
repaired patients. Magnetic resonance imaging dobut-
amine may help to define the subgroups of CCTGA
patients with favorable anatomy, whereby asymptomatic
adult survival could be anticipated without the need for
an operation.
(Ann Thorac Surg 2002;73:1759–64)
© 2002 by The Thoracic Society of Surgeons
Right ventricular failure, tricuspid valve insufficiency,
and complete heart block are established causes of
morbidity and mortality in patients with congenitally
corrected transposition of the great arteries (CCTGA)
[1–7]. This may occur either in unoperated patients, or
after variants of the so-called “classic” or physiologic
repair, in which the morphologic right ventricle (RV) and
atrioventricular valve remain in the systemic circulation.
The disappointing natural history and surgical results of
physiologic repair [1, 8–10] led to the concept of anatomic
repair, either the double switch procedure, or an atrial
switch plus Rastelli repair, which is increasingly pro-
posed to treat symptomatic young patients with CCTGA.
Although the midterm follow-up of the anatomic repair
has shown encouraging results when performed early on
[6, 11], the indication to do so remains uncertain in adults.
Issues pertaining to left ventricular retraining before
anatomic repair are paramount, and the extent to which
this may be achieved is a major determinant of operative
success [11]. Furthermore, anatomic repair has met with
its own set of complications, namely atrial arrhythmias,
baffle obstructions, neoaortic valve insufficiency after
pulmonary artery banding, and the need for repeat
conduit changes if a Rastelli repair is involved [6, 11, 12].
In patients with CCTGA who are asymptomatic or older,
or both, a large undertaking such as the anatomic repair
would seem questionable.
Magnetic resonance imaging (MRI) coupled with do-
butamine stress testing can accurately assess cardiac
reserve and represent an ideal noninvasive follow-up
modality in all patient groups with right ventricular
overload [13–15]. It allows for early detection of ventric-
Presented at the Poster Session of the Thirty-eighth Annual Meeting of
The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2002.
Reprint requests to Dr Dodge-Khatami, Division of Cardiothoracic
Surgery, Academic Medical Center, University of Amsterdam, Postbus
22660, 1100 DD Amsterdam, The Netherlands; e-mail: a.dodgekhatami@
amc.uva.nl.
© 2002 by The Thoracic Society of Surgeons 0003-4975/02/$22.00
Published by Elsevier Science Inc PII S0003-4975(02)03553-1

2. ular dilation and impending failure, and may guide
medical or surgical management before symptoms ap-
pear [13]. Our study uses dobutamine stress MRI in an
attempt to define and predict the eventual subsets of
patients who will never need an operation rather than an
anatomic repair.
Patients and Methods
Thirteen asymptomatic or minimally symptomatic adults
with CCTGA (mean age, 28.1 Ϯ 11.5 years) and 11
age-matched healthy volunteers were enrolled in our
study. Six patients with congenitally corrected transposi-
tion had undergone multiple operations along the phys-
iologic repair pathway between 1978 and 1995. Preoper-
atively, 2 patients had presented with cyanosis, and 4 had
been in intractable heart failure. All were in New York
Heart Association classes III-IV preoperatively, and all
were in sinus rhythm. Palliative procedures were per-
formed in 3 patients at a mean age of 1.1 years (range, 0.4
to 2 years), including pulmonary artery banding (n ϭ 2),
and a modified left Blalock-Taussig shunt (n ϭ 1). Intra-
cardiac physiologic repair was performed at a mean age
of 15.8 years (range, 3 to 61 years), and included closure
of a ventricular septal defect (n ϭ 3) or an atrial septal
defect (n ϭ 3), tricuspid valve replacement (n ϭ 2) or
repair (n ϭ 1; reoperation), pulmonary valve commissur-
otomy (n ϭ 1), and insertion of a left ventricular-to-
pulmonary artery valved homograft conduit (n ϭ 1).
There was no operative mortality and no incidence of
postoperative complete heart block. At the time of the
MRI dobutamine study, mean age was 29.5 Ϯ 18 years
(range, 17 to 65 years). Three patients had residual 2 to
3ϩ tricuspid valve regurgitation, as assessed by
echocardiography.
Equally studied were 7 asymptomatic adult patients
with unoperated CCTGA, with a mean age of 26.7 years
(range, 22 to 35 years). One patient was in spontaneous
complete heart block without a pacemaker, and the other
6 were in sinus rhythm. Five of these patients had a
combination of 9 associated intracardiac anomalies,
namely an atrial septal defect in 2, a ventricular septal
defect in 3, pulmonary valve stenosis in 2, and Ebstein’s
anomaly of the tricuspid valve in 2. Tricuspid valve
regurgitation (2ϩ to 3ϩ), as assessed by echocardiogra-
phy, was present in 4 patients.
Eleven age-matched healthy adults underwent the
same study protocol and served as controls (mean age,
31 Ϯ 11 years).
Magnetic Resonance Imaging
Study subjects were placed supine in a 1.5 Tesla MRI
scanner (Vision, Siemens, Erlangen, Germany) with high
power gradients. Electrocardiogram triggered T1-
weighted turbo spin echo axial images were acquired,
followed by four-chamber views of the heart. An electro-
cardiogram-triggered, ultrafast, breath-hold gradient-
echo cine sequence with the following measurements:
repetition time ϭ R-R interval; time of echo ϭ 4.8 ms;
slice thickness, 10 mm; imaging matrix ϭ 256 ϫ 256; field
of view ϭ 350 mm; lip angle ϭ 20° was then used to
acquire images in the short axis plane in contiguous
10 mm slices. End-systolic volumes and end-diastolic
volumes were calculated from this multi-slice, mul-
tiphase image set. Velocity maps were acquired with a
flip angle of 30°, Time to Echo ϭ 5.0 ms, slice thickness ϭ
6 mm, field of view ϭ 320 mm, and imaging matrix ϭ
256 ϫ 256, velocity encoding ϭ 250 cm per second. The
MRI protocol was repeated during dobutamine infusion
with an initial dose of 5 ␮g per kg per minute. The
infusion rate was increased by 5 ␮g per kg per minute
every 3 minutes to a maximum of 15 ␮g per kg per
minute. The MRI protocol during the dobutamine study
started 3 minutes after the maximum dose. During each
MRI measurement, the electrocardiogram, heart rate,
and systolic and diastolic blood pressures were
monitored.
Image Analysis
A Unix workstation (Sun Microsystems, Palo Alto, CA)
was used for analysis of the MR images. The MASS
image analysis software (Medis, Leiden, The Nether-
lands) was used to display multi-slice, multiphase im-
ages, both individually and in a movie loop mode.
Frames for end-diastolic and end-systolic volumes were
determined by manual outlining of a midventricular
slice. Papillary muscles and the moderator band were not
included in the ventricular volume. The enclosed RV and
left ventricle cross-sectional areas were measured by
computer, multiplied by section thickness, and summed
up according to Simpson’s rule to provide RV and left
ventricle volumes.
Calculations
Stroke volume was defined as end-diastolic volume mi-
nus end-systolic volume. The RV ejection fraction was
calculated as stroke volume divided by end-diastolic
volume. The dobutamine stress values were calculated as
a percentage increase from values at rest. All volumetric
measurements were corrected for body surface area.
Statistical Analysis
Differences between groups were compared with the
unpaired t test. The effects of dobutamine within groups
were compared with the paired t test. A p value less than
0.05 was considered statistically significant.
Results
All patients tolerated and completed the protocol. Details
of results are given in Table 1.
At rest and at dobutamine stress, heart rate was com-
parable between patients with physiologic repair, unop-
erated patients with CCTGA, and controls. Mean arterial
blood pressure was similar among all three groups at
rest, but remained lower in unoperated CCTGA patients
at stress, as compared with controls. While assessing
systemic ventricles, the right ventricle of patients with
CCTGA, unoperated or after physiologic repair, was
compared with the systemic left ventricle of controls.
1760 DODGE-KHATAMI ET AL Ann Thorac Surg
MRI EVALUATION OF UNOPERATED CONGENITALLY CORRECTED TRANSPOSITION 2002;73:1759–64

3. Baseline mean systemic ventricular end-diastolic vol-
umes were larger in the physiologically repaired group
per body surface area compared with controls. After
dobutamine infusion, there was no significant difference
in end-diastolic volumes between the three groups (Fig
1). At rest the indexed mean systemic ventricle end-
systolic volume was similar among unoperated and con-
trols, but larger with dobutamine stress in unoperated
patients as compared with controls. Mean systemic ven-
tricle end-systolic volume was significantly larger in the
physiologically repaired group, both at rest and with
dobutamine stress, as compared with controls. Mean
systemic ventricular ejection fraction was lower at base
line (p Ͻ 0.002) and at stress (p Ͻ 0.001) in physiologically
repaired patients as compared with controls. Base line
mean systemic ventricular ejection fraction was similar in
unoperated patients as compared with controls, but was
lower after dobutamine stress (p Ͻ 0.005). Mean indexed
systemic ventricular stroke volume and cardiac index
were similar at baseline values among the three groups,
and increased appropriately during dobutamine infusion
(Figs 2, 3).
Comment
This is the first study to evaluate cardiac reserve in
patients with congenitally corrected transposition, either
without previous operations or after physiologic repair,
using stress dobutamine MRI. The MRI dobutamine
provides for an accurate and reproducible quantitative
assessment of RV volumes and function, and its routine
use in the follow-up of patients with RV overload has
been validated [13–15] to detect right ventricular dilation,
or impending failure, or both. Disadvantages include the
cost and the general contraindications to MRI, namely
the presence of metal implants, claustrophobia, and
arrhythmia [13–15].
Assuming that the perfect result after anatomic repair
entails normalization of systemic left ventricular function
and reserve, the left ventricle of healthy controls was
Fig 1. Variation of systemic ventricular end-diastolic volume
(SVEDV) between baseline values and after dobutamine stress.
(ns ϭ not significant; PHYS ϭ physiologically repaired; UN ϭ
unoperated.)
Fig 2. Variations in percentage between baseline values (0) and after
dobutamine stress testing. (BP ϭ blood pressure; CI ϭ cardiac index;
EDV ϭ end-diastolic volume; EF ϭ ejection fraction; ESV ϭ end-
systolic volume; HR ϭ heart rate; PHYS ϭ physiologically re-
paired; SV ϭ systemic ventricle; SV sv ϭ systemic ventricle stroke
volume; UN ϭ unoperated.)
Table 1. Effects of Dobutamine Stressa
Controls
Physiologically
Repaired
Congenitally
Corrected
Transposition of
the Great Arteries
Unoperated
Congenitally
Corrected
Transposition of
the Great Arteries
Controls Versus
Physiologically
Repaired
Controls
Versus
Unoperated
Rest Stress Rest Stress Rest Stress p Rest/p Stress
p Rest/p
Stress
Heart rate (bpm) 65 (12) 100 (14) 68 (15) 90 (9) 65 (14) 98 (27) NS/NS NS/NS
Mean BP (mm Hg) 82 (8) 95 (12) 70 (3) 88 (13) 70 (5) 87 (10) NS/NS p Ͻ 0.03/NS
SVEDV/BSA (mL/m2
) 62 (17) 63 (19) 92 (12) 88 (13) 78 (27) 76 (35) p Ͻ 0.02/NS NS/NS
SVESV/BSA (mL/m2
) 19 (9) 10 (4) 43 (5) 35 (4) 26 (12) 22 (10) p Ͻ 0.001/p Ͻ 0.001 NS/p Ͻ 0.005
SVsv/BSA (mL/m2
) 43 (10) 52 (16) 49 (11) 53 (11) 52 (15) 53 (26) NS/NS NS/NS
SV EF (%) 71 (8) 84 (8) 53 (8) 60 (5) 68 (4) 70 (6) p Ͻ 0.002/p Ͻ 0.001 NS/p Ͻ 0.005
CI (L/min/m2
) 2.7 (0.6) 5.3 (1.7) 3.2 (0.1) 4.7 (0.6) 3.4 (1.3) 4.8 (1.8) NS/NS NS/NS
a
Values in parentheses represent standard deviation.
BP ϭ blood pressure; bpm ϭ beats per minute; BSA ϭ body surface area; CI ϭ cardiac index; EDV ϭ end-diastolic volume; EF ϭ
ejection fraction; ESV ϭ end-systolic volume; NS ϭ not significant; SV ϭ systemic ventricle; SVsv ϭ stroke volume.
1761Ann Thorac Surg DODGE-KHATAMI ET AL
2002;73:1759–64 MRI EVALUATION OF UNOPERATED CONGENITALLY CORRECTED TRANSPOSITION

4. used to compare with the systemic RV of CCTGA pa-
tients. In our study, the systemic right ventricle of asymp-
tomatic adults with unoperated CCTGA showed similar
ejection fraction at rest, and lesser values at stress,
compared with the systemic left ventricle of age-matched
healthy controls. Suboptimal results were noted in pa-
tients after physiologic repair. In these patients, both at
baseline and at stress testing, we found larger systemic
ventricular end-systolic volumes. Also, baseline systemic
ventricular end-diastolic volumes were larger after phys-
iologic repair, compared with controls. As a snapshot
value, the significance of this is unknown and difficult to
interpret. Is this the result of surgical insult to the right
ventricle, the result of long-standing volume overload of
the RV, or the normal response of a morphologic right
ventricle when chronically faced with systemic pres-
sures? Furthermore, what are the true implications of
these morphometric deviations in light of an apparent
proper response to effort? Indeed, with stress testing, we
found cardiac index to increase appropriately in both
subsets of patients with CCTGA, without undue increase
in heart rate or mean blood pressure, as compared with
controls.
Symptomatic patients with CCTGA were originally
treated with a combination of surgical procedures, either
palliative or corrective, known as the “classic” or physi-
ologic repair of CCTGA, leaving the right ventricle and
tricuspid valve in the systemic circulation. In general, the
results of physiologic repair have been disappointing,
despite an acceptable operative mortality rate of 2% to
15% [1, 8–10, 16, 17]. In a 40-year review from the Toronto
Hospital for Sick Children, including 118 patients having
undergone a physiologic repair for CCTGA, Yeh and
colleagues [8] reported a 6% operative mortality. How-
ever, 10 years after repair, survival was only 74%, and at
20 years it was an unsatisfactory 48% [8]. Termignon and
colleagues [16] reported an even more concerning 55%
survival rate at 10 years, when obstruction to the pulmonary
ventricle was associated. Major concerns with physiologic
repair include the nonnegligible incidence of complete
heart block (14% to 38%) [8, 10, 16, 18] and the frequent
need for reoperation on the tricuspid valve (Fig 4), but more
importantly, the long-term failure of the right ventricle,
which must chronically sustain systemic pressures.
These imperfections led to the development of the
anatomic repair concept, combining an atrial and arterial
switch, also known as the double switch, or an atrial
switch with a Rastelli procedure in patients with left
ventricular outflow tract obstruction [19]. The current
operative mortality after anatomic repair varies between
0% to 15% [6, 11, 12, 19–22]. However, anatomic repair is
not exempt of its own set of complications, and increas-
ing concern is present as to the long-term development
of atrial dysrhythmias and venoatrial obstructions, which
are well-documented after the atrial switch procedures
[6, 12, 19, 21, 22]. Furthermore, certain timing issues
remain unanswered. The proponents of the anatomic
repair insist on the better outcome of the procedure
when it is performed in a younger age group, namely
before 15 to 16 years of age [6, 11]. This presumably
relates to the degree to which the left ventricle may be
retrained as the neosystemic ventricle by pulmonary
artery banding [11]. Indeed, older patients have higher
early and late mortality when submitted to the anatomic
repair protocol, and the results of left ventricle retraining
have been more uncertain when performed after the age
of 15 years [11].
The driving impetus towards the development of the
anatomic repair of CCTGA stems from the preformed
concept that a left ventricle, with or without retraining,
will ultimately perform better in the systemic circulation
than the right ventricle. However, there is no long-term
data to support this [6, 12, 23]. In a series of 27 patients
undergoing anatomic repair without early or late mortal-
ity, Imamura and colleagues [20] reported normal post-
operative ejection fractions of both left and right ventri-
cles, as assessed by echocardiography at the time of
hospital discharge without further follow-up. Imai and
colleagues [6] reported an operative mortality of 7.9% in
a series of 76 patients with CCTGA who underwent
anatomic repair at less than 16 years of age. At a mean
Fig 3. Variation of cardiac index between baseline values and after
dobutamine stress. (PHYS ϭ physiologically repaired; UN ϭ
unoperated.)
Fig 4. Gradient echo magnetic resonance imaging at rest. End-dia-
stolic 4-chamber view of a patient with physiologically repaired
congenitally corrected transposition of the great arteries. (LV ϭ left
ventricle; RV ϭ right ventricle; TP ϭ tricuspid valve prosthesis.)
1762 DODGE-KHATAMI ET AL Ann Thorac Surg
MRI EVALUATION OF UNOPERATED CONGENITALLY CORRECTED TRANSPOSITION 2002;73:1759–64

5. postoperative follow-up of 4.9 years, they actually ob-
served a slight decrease in left ventricular ejection frac-
tion, no increase in RV ejection fraction, and unchanged
left ventricular end-diastolic volumes [6]. Yagihara and
colleagues [22] found subnormal postoperative left ven-
tricular ejection fractions as assessed by cardiac catheter-
ization, at a mean follow-up of 11 months after anatomic
repair in their series of 10 patients.
Frequent failure of the right ventricle to chronically
function as a systemic ventricle is another driving force to
promote anatomic repair. However, findings across the
literature differ, and multiple reports of normal adult
survival without limitation to effort or arrhythmia exist,
in unoperated CCTGA without associated intracardiac
anomalies (Fig 5) [2, 7, 24, 25]. Peterson and colleagues [4]
compared 17 children after atrial baffle procedures for
complete transposition of the great arteries, with 8 unop-
erated patients with uncomplicated CCTGA, and 10
normal controls. Using radionuclide angiocardiography,
stress testing revealed a normal increase in pulmonary
ventricular ejection fraction in patients with CCTGA, as
compared with controls. However, the systemic ventri-
cles of patients with CCTGA did not significantly in-
crease their ejection fractions with exercise, and both
end-diastolic and end-systolic volumes were larger than
controls. Cardiac index augmented appropriately in all
groups, mainly because of an increase in heart rate in the
CCTGA group [4]. In accordance with these findings,
Parrish and colleagues [5] found subnormal increases in
right and left ventricular ejection fractions in response to
an effort in 5 children with CCTGA, although their
exercise capacity, heart rate, and blood pressure re-
sponded appropriately. Supporting our findings, Benson
and colleagues [3] found normal systemic right ventric-
ular ejection fractions at exercise in 8 patients with
uncomplicated and unoperated CCTGA, whereas the
function of the pulmonary ventricle was subnormal. In
their study using radionuclide angiocardiography, end-
diastolic and systolic volumes of the systemic right ven-
tricle decreased appropriately from rest to exercise [3].
Surgery is inevitable and clearly warranted in one form
or another for symptomatic patients presenting in failure
or cyanosis. In patients with associated intracardiac
anomalies, such as pulmonary valve stenosis, Ebstein’s
malformation with severe insufficiency of the tricuspid
valve, or important degrees of intracardiac shunting,
early right ventricular dilation and failure occurs, indi-
cating early surgical intervention. For these patients, we
acknowledge the anatomic repair as the surgical proce-
dure of choice [6, 11, 12, 17, 20–22]. However, we question
the recommendation for anatomic repair in asymptom-
atic patients by some [6, 12, 17], based on echocardio-
graphic evidence of an enlarged right ventricle and
tricuspid valve insufficiency. Our findings and other
evidence of satisfactory long-term RV function in unop-
erated adults with CCTGA [3, 7, 24, 25] would suggest a
more conservative attitude in the management of asymp-
tomatic patients, which includes patients with and with-
out associated lesions. Although it is intuitive and gen-
erally agreed that asymptomatic patients with CCTGA
and intact ventricular septum require no surgery, they
represent a minority. Given the potential complications
of both surgical pathways and the relative unfavorable
long-term outcome of physiologic repair, we make a case
for surgical abstinence even in CCTGA patients with
associated lesions. Indeed, 5 out of 7 of our studied
patients with unoperated CCTGA have intracardiac
anomalies, but are otherwise asymptomatic, without
medication, have near normal ventricular volumes, and
respond appropriately to stress testing.
In conclusion, defining the eventual subsets of patients
with CCTGA who may never require an operation is of
paramount clinical importance. With current diagnostic
modalities, no such definition exists, and the therapeutic
dilemma persists. Even respectable centers with a larger
experience of the anatomic repair provide no long–term
follow-up data to demonstrate improved systemic left
ventricular function postoperatively [6, 11]. Accordingly,
the open-ended question remains as to the true benefit
and age cutoff to perform an anatomic repair in asymp-
tomatic patients with CCTGA. Robust evidence is needed
before more CCTGA patients with normally functioning
right ventricles are enrolled in anatomic repair protocols.
In those asymptomatic or minimally symptomatic pa-
tients, the use of a noninvasive follow-up modality, such
as MRI dobutamine stress, could help to define morpho-
logic and hemodynamic criteria justifying nonoperative
management. As a corollary, deviations from these crite-
ria, especially in asymptomatic patients, could serve as
an early indicator of impending cardiac failure, and
indicate timely surgical intervention, preferably in the
form of anatomic repair.
Study Limitations
Our ongoing MRI dobutamine stress protocol strives to
define noninvasive criteria, whereby asymptomatic patients
Fig 5. Gradient echo magnetic resonance imaging at rest. End-dia-
stolic 4-chamber view of a patient with unoperated congenitally cor-
rected transposition of the great arteries. Note the convex interven-
tricular septum toward the left ventricle. (LV ϭ left ventricle; RV ϭ
right ventricle.)
1763Ann Thorac Surg DODGE-KHATAMI ET AL
2002;73:1759–64 MRI EVALUATION OF UNOPERATED CONGENITALLY CORRECTED TRANSPOSITION

6. with CCTGA may be judged as not requiring operations, or
on the contrary, as impending failures and candidates for
timely anatomic repair. The small number of patients di-
minishes statistical power, which limits the significance of
our results, and does not allow yet for a clear-cut manage-
ment protocol according to the sought after criteria. To date,
perhaps by unfortunate serendipity, we have had no pa-
tients enrolled who either presented initially with normal
values, which then degraded over time, or presented in
failure with highly abnormal MRI dobutamine stress val-
ues. These findings would strengthen the definitions of
“normality” and deviations therefrom.
Using the left ventricle of healthy controls as a mea-
suring stick to compare with the RV of patients with
CCTGA is a potential weakness of the study. Although the
two respective ventricles perform the same function in each
group, their intrinsic morphology and geometry are clearly
different.
As a group, patients with CCTGA present with a wide
variety of morphology and resulting physiology, making
generalizations and recommendations across such a het-
erogenous group hazardous. Owing to unfavorable anat-
omy and physiology in many instances, we fully recog-
nize that anatomic repair is often inevitable and
indicated. Our data only illustrates and quantifies the
potential for a morphologic right ventricle to sustain the
systemic circulation, even in the presence of associated
intracardiac lesions. This view is supported by other
reports, which show normal exercise tolerance and qual-
ity of life, in asymptomatic and unoperated adults with
CCTGA [2, 3, 7, 24].
Doctor Tulevski is supported by The Netherlands Heart Foun-
dation (NHS) and Interuniversity Cardiology Institute of The
Netherlands (ICIN-KNAW).
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