Mar 7, 1985 - of situs solitus and dextrocardia, in which. CCT is known to be present in 50% of cases. Index terms: Angiocardiography, radionuclide,. 50.1242.
Congenitally
Gerard L. Guit, M.D. Herman M. Kroon, M.D. Jan G. Chin, M.D. Ernest K. Pauwels, DSc. Ad
E. van
Voorthuisen,
Corrected
Transposition
Detection
M.D.
in the Adult:
by Radionuclide
Angiocardiography”
C
Congenitally corrected transposition (CCT) of the great vessels is an uncommon anomaly usually detected in children because of associated severe cardiac malformations. When these are absent, patients may be hemodynamically normal, but associated cardiac abnormalities are usually present in CCT, leading to symptoms in adulthood. When CCT is suggested in an adult, diagnosis by means of ultrasound may be difficult. Radionuclide angiocardiography (RA) is a simple, noninvasive method by which to diagnose CCT. We found consistent morphologic scintigraphic results in 13 patients with proved CCT, leading to establishment of reliable diagnostic criteria. In all instances of situs solitus the aorta ascends and descends on the left with vertical onentation. In the case of situs inversus, the aorta ascends and descends on the right with vertical orientation. Our criteria are independent of the situs and cardiac position, unlike earlier reports by others. We believe images obtained in the anterior projection are sufficient for the study. The practical application of RA study in patient diagnosis is demonstrated, giving special attention to patients referred because of situs solitus and dextrocardia, in which CCT is known to be present in 50% of cases.
corrected transposition of the great vessels (CCT) is an uncommon cardiac anomaly in which the atnioventnicular (AV) and ventniculoartenial (VA) connections are discordant. This means that the right atrium communicates with the morphologic left ventricle. From this ventricle the transposed pulmonary trunk arises. The left atrium is connected to the morphologic right ventricle. From the infundibulum of this chamber, the ascending aorta originates; this is left and anterior in its relation to the pulmonary trunk in situs solitus and right and anterior to the pulmonary trunk in situs invemsus. In CCT, the ventricular septum is almost perpendicular to the frontal plane; therefore, the chambers show a side-by-side relationship (1). Most cases are detected in the patient’s early childhood owing to the presence of severe associated cardiac malformations such as ventricular septal defect, pulmonary valvular stenosis, and dysplasia of the left AV valve (1, 2). When malformations are absent, patients may be hemodynamically normal. However, abnormalities of the left AV valve and conduction tissues almost invaniably are present, which may lead to symptoms in adulthood (1-4). When CCT is suspected in an adult, the application of ultrasound (US) in diagnosing this cardiac anomaly is limited by two factors. US diagnosis of CCT may be technically impossible because of interposition of the lung or the thomacic cage, resulting in a too poor window. Also, interpretation of US study findings may be difficult for a cardiologist who has limited or no experience with this uncommon cardiac anomaly (1, 5). This will often be the mule in adult
Index terms: Angiocardiography, 50.1242 #{149} Transposition of great Ultrasound, comparative studies
establish the morphologic scintigmaphic findings in this congenital heart disease. We found consistent criteria leading to the diagnosis of CCT in this relatively large series. Our criteria are not entirely in accordance with those mentioned in earlier reports (6, 7), which reviewed smaller patient groups, and are in our opinion easier to interpret. The practical application of RA study in diagnosing on excluding CCT is demonstrated by three cases we were able to study prospectively. Scintigraphic findings in patients with proved CCT and in those who were suspected of having CCT are presented and discussed. Additionally, the potential role of RA in the diagnosis of CCT in early childhood is considered.
radionuclide, 50.163
vessels,
#{149}
Radiology 1985; 157:521-527
1 From the Departments of Diagnostic Radiology (G.L.G., H.M.K., E.K.P., A.E.V.) and Cardiology (J.G.C.), University Hospital Leiden, Rijnsburgerweg, The Netherlands. Received March 7, 1985; accepted and revision requested May 28; revision received June 28. C RSNA, 1985
ONGENITALLY
cardiologic In the
practice. literature,
two
reports
have
drawn
attention
to
radionu-
clide angiocardiography (RA) as an alternative method for diagnosing CCT (6, 7). Sequential images obtained in this manner allow a morphologic study of the configuration, location, and relations of the different cardiac compartments and great vessels (8, 9) (Fig. 1). We performed RA study in 14 adult patients with proved CCT to
521
MATERIALS From hospital
cases
and
phy, 14
AND
the
we
proved
were
cases.
Sex,
METHODS
of CCT
registered
by
in our
angiocardiogra-
able
to perform
age,
situs,
RA
study
cardiac
in
position,
and associated cardiac lesions in these patients are listed in Table 1. Another three patients suspected of having CCT were mefemmed
to our
ysis. Studies
department
were
for
carried
field-of-view
out
gamma
further
anal-
with
a large-
camera
equipped
with a parallel hole collimator, and results were stored in a 64 X 64 matrix on disc. Patients were examined in the supine position
with
the
region. A bolus mCi) of Tc-99m in
the
basilic
detector
above
injection citrate
of 277.5 MBq was administered
vein
of
radiopharmaceutical more
the
was
rapidly
eliminated
the
right
cardiac
arm.
chosen by
This
as it
the
the
maceutical study
is not (10).
tion
choice
was
with
second
the
disc.
took
place
A detailed
morphologic
dose;
this the
Images
of one
30-second
type
of
scintillawere
ob-
image
per
period
by
data
-a
-
and
nuclear hemody-
,
study
of the
scm-
findings
was
per-
4 .....
retrieval
‘I,
,
..
..
‘. .
All studies but one were of technically good quality, allowing momphologic evaluation. In case 8, the injection of the nadiopharmaceutical was performed poorly, indicated by persistent activity in the superior vena cava. Dilution of the madiophanmaceutical resulted in too low activity in the cardiac compartments, not allowing morphologic evaluation. Therefore, this case was excluded from further study. Situs and cardiac position were detemmined by penetrated posteroanterion (PA) chest films. Results were divided in five groups: situs solitus with laevocandia, situs solitus with mesocardia, situs solitus with dextrocardia, situs inversus with dextrocardia, and one group in which the prospective studies were performed.
Solitus
With laevocardia (n 7).-The scmtigmaphic morphologic findings of situs solitus with laevocardia in case 3 are shown in Figure 2. These findings are similar to those of cases 1, 2, 4-6, and 7. The right atrium and ventricle (morphologic left ventricle) are tniangular in configuration. The pulmonary trunk is central in position. The left-sided ventricle (morphologic
522
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formed.
Situs
axis is vertically orientated. The aorta ascends on the left, and its course is almost vertical, not crossing the midline. The ventricular septum is perpendicular to the plane of imaging and is vertically orientated. Wit/i dextrocardia (ii 1)-The momphologic findings of situs solitus with dextmocardia in case 14 are shown in Figure 4. The right-sided atrium and ventnicle (night atrium and morphologic left ventricle) have an oval configuration. The pulmonary trunk is central in position. The left-sided ventricle (momphologic right ventricle) is oval. Its long axis is vertically orientated. The aorta ascends on the left, is vertical in position, and does not cross the midline. The ventricular septum is penlong
radiophar-
on transparent Review of the
pattern
tigraphic
for
started. a
were recorded medicine film. from
critical
an exposure
during
namic
either
Simultaneously,
camera
tamed
of
is
kidneys
than Tc-99m diethylenetriaminepentaacetic acid, thus minimizing radiation however,
(7.5
night ventricle) is oval in shape. Its long axis is more vertically orientated than in normal heart anatomy. The aorta ascends on the left, is vertically orientated, and does not cross the midline. The ventricular septum is perpendicular to the antemoposteniom (AP) plane of imaging. Wit/i mesocardia (ii 4)-The momphologic scintignaphic findings of situs solitus with mesocardia in case 9 are shown in Figure 3. These findings are similar to those in cases 10, 1 1, and 12. The night-sided atrium and ventnicle (might atrium and morphologic left ventricle) are oval in configuration. The pulmonary trunk is central in position. The left-sided ventricle (momphologic might ventricle) is oval. Its
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a.
b. Figure
1. Normal appearance of heart on radionuclide angiogram, anterior view. (a) Right side of heart is U-shaped. Note superior vena cava, right atrialventricular complex, and pulmonary trunk, which arises on the left from the right ventricle. (b) Left side of heart shows left ventricle and aorta. Aorta oniginates on the right, crosses the midline, and descends on the left. Due to the
oblique
position
right-sided
of the ventricular
septum,
the left ventricle
Table 1 Data of Patients
with
Proved
Cardiac
No.
Sex
Age
S1tUS
Position
1
M
26
SS
L
2
M M
4
M M F
55 SS SS 55 SS
L
3
50 48 33 25 22
11 12
F F M F F M
37 64 27 21 38 23
13
M
14
F
5
6 7
8 9
10
-
D -
on
CCT
Case
Note.-M mesocardia; tence; ASD
is superimposed
structures.
VSDclosed ...
L
LeftAV,valvel
L L L
LeftAV,valvel LeftAV,valvel
SS 55 SS SS SS SS
L L Mc Mc Mc Mc
VSD closed Left AV, valve
28
SI
D
21
SS
D
male; F female; SS situs dextrocardia; VSD ventricular atrial septal defect. -
Associated Lesions
...
I
...
Small VSD LeftAV,valvel ASD closed ...
VSD
closed
solitus; SI situs inversus; L laevocardia, Mc septal defect; AV atrioventricular; I incompe-
November
1985
to
pendiculam
the
AP
plane
of
imag-
In
situs
and
ing.
Situs Inversus Dextrocardia
inversus,
descends
the
on
the
ascends
aorta
right.
with (n
1)
=
Prospective
Studies
3)
(n
The scintigmaphic morphologic findings of situs inversus with dextnocandia in case 13 are shown in Figume 5. The right atrium-characterized by its connection to the left-sided supemi-
Case 15.-A 12-year-old boy was mefemred because of recurrent episodes of syncope. Electrocardiographic (ECG) findings were normal. Chest madiognaphs (Fig. 6) showed a long, convex left upper mediastinal border
or vena cava-is on the left, consistent with situs invemsus. The morphologic findings are the mirror image of situs solitus with mesocandia (Fig. 3).
with aomtic diologic formed,
a.
an indistinct knob. CCT grounds. showing
pulmonary was suggested RA study normally
and mawas penrelated
b.
Figure 2. dia, anterior
Radionuclide
view,
angiogram
right-sided (morphologic trunk. (b) Left side of heart left ascending aorta. Note
plane
of imaging,
side-by-side
study
in case 3. (a) Right
with
of CCT,
of heart
situs
shows
solitus
and
triangular
cranial
of the
to left
caudal
deviation,
laevocar-
shape
left) ventricle and central position shows left atrium (arrows), left-sided perpendicular position of ventricular
right
relationship
side
of the
of pulmonary ventricle, and septum to the
indicated
on
chambers and great vessels (as in Fig. 1). CCT was excluded. Case 16.-A 32-year-old man was referred because of an unexplained cardiac murmur. ECG examination showed a first-degree AV block. Situs solitus and dextrocardia were noted on chest nadiographs (Fig. 7a). CCT could not be excluded on clinical and nadiologic grounds. RA study showed a central position of the pulmonary trunk, vertical position of the ventniculam septum perpendicular to the AP plane of imaging, and a left ascending aorta, consistent with a diagnosis of CCT (Fig. 7b and c). This was confirmed by US examination and cineangiocardiogmaphy. No associated cardiac abnormalities could be demonstrated. Case 17.-A 14-year-old girl was mefenred because of a heart murmur. Radiogmaphic studies of the chest showed dextmocamdia and situs solitus (Fig. 8a). CCT could not be excluded. RA study showed an oval configumation of the might-sided atrium and ventricle. The pulmonary trunk deviated to the left (Fig. 8b). The left-sided ventricle was triangular, with the apex pointing to the right. The leftsided ventricle was almost completely superimposed on the right-sided yentmicle. The aorta ascended on the right, crossed the midline, and descended on the left (Fig. 8c). CCT was excluded by RA study. No shunt could be detected, and a diagnosis of uncomplicated dextrovemsion was made. This was confirmed by US examination.
by the
ventricles.
DISCUSSION
a.
b.
Figure 3. dia, anterior
shape trunk.
aorta,
of the (b)
157
morphologic
Left
side
of
superimposed
of ventricular
Volume
Radionuclide view, in case
indicated
2
study side
left ventricle heart
shows
on descending
septum,
Number
angiogram 9. (a) Right
of CCT, of heart
and left
aorta. by the
central
and
vertical
situs solitus and clearly demonstrates
position
of the
orientation
Note
perpendicular
relationship
of the
pulmonary of
vertical chambers.
mesocaroval ascending
position
Cardiac two-dimensional real-time ultrasonogmaphy and angiocandiogmaphy are established methods for the diagnosis of CCT (1, 5). The principle underlying the diagnosis in both methods is based on direct identification of the morphology of atnia and ventricles and the demonstration of the discordant AV and VA connections (1, 5). Two earlier reports (6, 7) in which the mole of RA in the diagnosis of CCT are discussed mention the same pninciple for the diagnosis of this cardiac anomaly. The place of the might atmium can be detected by its connection to the superior vena cava, and the great arterial vessels can be easily identified. According to these communications, identification of the morphology of the ventricles is more difficult. These that the triangular sided ventricle
previous shape is indicative
reports state of the nightof a mom-
Radiology
523
#{149}
phological left ventricle. However, in our study this triangular configuration was found only in cases with situs solitus and laevocardia. The other patients-those with situs solitus with mesoand dextrocandia as well as situs inversus-showed an oval shape of this right-sided ventricle, which did not differ significantly from the shape of the left-sided yentmicle. Therefore, ventricular configunation is highly influenced by cardiac position and rotation in the thomacic cavity. In our opinion, it seems unmeliable to correlate a certain shape of the chambers with the morphology of those ventricles. This implies that RA examination strate directly VA connections identification
is not able to demonthe discordant AV and because positive of the morphology of
ventricles is not possible. Indirect information concerning the location of the morphologic might ventricle should be obtained by the “looprule of van Pnaagh” (11, 12), which states that a left and anterior position of the aorta in situs solitus suggests that the morphologic might ventricle is situated on the left and that the aorta emerges from this chamber. A right and anterior position of the aorta in situs invemsus implies that the morphologic might yentmicle is on the left. Unfortunately, theme are many exceptions to this rule, as for instance in congenitally connected malposition, AV discordant and VA concordant hearts, and univentricular hearts (1). Therefore, this mule also does not allow an appnopniate identification of abnormal AV and VA connections. Although the location of the momphologic left and right ventricle cannot be seen with certainty in RA study, we found other constant momphologic scintigraphic features of CCT in our studies that are unrelated to the situs and cardiac position, namely: (a) left ascending aorta in situs solitus and night ascending aorta
.2
4:’.
-
a.
.-.
b. Figure
dia,
4.
anterior
logic
Radionuclide view, in case
left ventricle
angiogram 14. (a) Right
and central
study of CCI, situs side of heart shows
position
is poorly visualized due to inadequate ascending aorta is seen (arrow). There tion of the ventricular septum, indicated
of pulmonary
solitus and dextrocaroval shape of morpho-
trunk.
(b) Left side of heart
injection technique; however, a left is a perpendicular, almost vertical, posiby the relationship of the chambers.
the
in situs invemsus, of the pulmonary pendiculan position septum in the AP
(b) central position trunk, and (c) perof the ventricular projection.
This scintigraphic pattern is in our opinion reliable in suggesting the diagnosis of CCT. However, there are other congenital cardiac diseases in which a left ascending aorta (situs solitus) may be encountered (1). These anomalies, listed in Table 2, are virtually always associated with a shunt (1). RA study performed in patients with these cardiac conditions theometically may show scintigraphic findings comparable to those obtained in patients with CCT complicated by a shunt. Moreover, the presence of an
524
Radiology
#{149}
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.,.‘.. ‘,,.‘
..
. .
.
.
..
a.
b. Figure 5. Radionuclide angiogram study of CCT, situs inversus and dextrocardia, anterior view, in case 13. (a) Hemodynamic right side of heart still shows vaguely ventricle, of heart mirror
intracardiac
the superior and central shows a right image of Figure
shunt
may
vena cava on the left, oval shape of the morphologic position of pulmonary trunk. (b) Hemodynamic ascending and descending aorta in situs inversus. 3.
result
in poor
Table 2 Cardiac Conditions That May Be Associated with a Left Ascending Aorta (situs solitus)
image quality, not allowing adequate morphological interpretation. This is also reflected in literature (13). For these reasons, we do not recommend this method for the diagnosis of CCT in patients who are seen in early childhood because of severely altered hemodynamic function. In our hands, RA study has proved to be an important method of examination in adult patients in whom CCT is suspected. Application of US as a diagnostic tool in these patients is limited. Conventional angiocandiography often requires hospitalization and is potentially hazardous. Moreover, cardiac diseases (Table 2) that may potentially mimick the scintigraphic findings encountered in CCT have never been reported as seen in adolescence or adulthood (1). RA
left left side This is a
Double outlet Univentricular
ventricle
heart
Atrioventricular discordant and ventriculoarterial concordant heart Complete transposition of great vessels Congenitally corrected malposition
study
is
tential tion one
PA
of
findings
lows In
performed,
easily
hazard, and dose, comparable chest
film is
the
criteria
our
opinion,
be obtained
not
in the
uses
lacks a low to that
pomadiafrom
(8). Interpretation difficult if one folstated here. images only have to anterior
projection.
November
1985
5.
of patients with congenital corrected transposition of the great arteries. N EngI Med 1970; 282:1053-1059. Keutel J, Hagenmuller H. Echocardio-
graphy
6.
7.
in diagnosis
giocardiography
8.
in the diagnosisof
DS, Mason
Cox
PH,
cal
behaviour
van
with particular In: Cox PH,
cology. 11.
12.
Figure convex arteries
6. PA radiograph of the chest type of CCI. Radionuclide images (as in Fig. 1). CCT was excluded.
in case 15. Cardiac silhouette demonstrate normal related
is compatible ventricles
and
with a great
13.
14.
earlier reports (6, 7) lateral projections were also obtained to show the anterior position of the ascending aorta. However, if RA study shows a left ascending aorta in situs solitus or a right ascending aorta in situs inverIn
sus in the AP projection, this implies the anterior position of the aorta. Latenal projections, therefore, provide no supplementary information, affect the simplicity of the method, and increase the radiation dose. The practical application of RA study for diagnosing on excluding CCT is demonstnated by the three cases we were able to study prospectively. Chest films of two of these patients showed situs solitus and dextmocandia. In this situation CCT is known to be present in more than 50% of cases (1, 14). Our studies in these patients indicate that RA examination can easily differentiate cases of situs solitus and dextnocardia with normal related great vessels and normal AV and VA connections, from situs solitus and dextnocamdia complicated by CCT. Although clinical and radiographic findings were
Volume
157
Number
2
compatible cases, RA the correct
with CCT in all three study was able to establish diagnosis.
Acknowledgments: for reproducing the Soons for manuscript
Send
correspondence
Gerard L. Guit, M.D., Radiology. University burgerweg 10. 2333 lands.
We thank illustrations preparation.
and
reprint
Department Hospital AA Leiden,
Mr. and
DT, eds. Clinical
nude-
der
in congenital 1980; Pompe
of technetium reference ed. Progress
Amsterdam:
heart
dis-
46:1247-1255. WB. The biologi-
complexes
to brain tumours. in radiopharma-
Elsevier/North
Holland, 1979; 45-62. Van Praagh R, van Praagh S. VIad P, et al. Anatomic types of congenital dextrocardia: diagnostic and embryologic implications. Am J Cardiol 1964; 13:510-531. De la Cruz MV, Berrazueta JR, Artenaga M, et al. Rules for diagnosis of arterioventricular discordances and spatial identification of ventricles: crossed great arteries and transposition of the great arteries. Br Heart J 1976; 38:341-354. Kriss JP, Enright LP, Haydan WG, et al. Radioisotopic angiocardiography: findings in congenital heart disease. J NucI Med 1972; 13:31-40. Anselmi C. Munoz 5, Blanco P. Machado I, De La Cruz MW. Systematization and clinical study of dextroversion, mirror-image dextrocardia and levoversion. Br Heart
J 1972; 34:1085-1098.
M. Popkes Ms. Y. E. J.
requests
con-
ar cardiology. New York: Grune & Stratton, 1981; 285-317. Treves S. Fogle R, Lang P. Radionuclide
angiocardiography ease. Am J Cardiol 10.
transposi-
genitally corrected transposition of the great arteries. Clin NucI Med 1984; 9:383-388. Howman-Ciles RB, Gilday DL, Mason DT, Berman DS. Nuclear cardiology in paediatrics: evaluation of intracardiac shunts and additional congenital disorders. In:
Berman
9.
of corrected
tion. In: Anderson RH, ed. Paediatric cardiology. Edinburgh: Churchill Livingstone, 1977:516-523. Attie F, Cuaron A, Marquez M, Buendia A, Zamora C, Acero A. Qualitative radionuclide angiocardiography in the diagnosis of corrected transposition. Am Heart 1979; 97:141-146. Brendel AJ, Wynchank MB, Choussat A, Barat JL, Ducassou D. Radionuclide an-
Figures
7-8 on the following
pages.
to:
of Diagnostic Leiden, RijnsThe Nether-
References 1.
2.
3.
4.
Losekoot TG, Anderson RH, Becker AE, Danielson GK, Soto B. Congenitally corrected transposition. In: Anderson RH, ed. Modern paediatric cardiology. Edinburgh: Churchill Livingstone, 1983; 21-47, 68-79, 155-173. Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol 1976; 38:910-923. Guit GL, Kroon HM, van Voorthuisen AE, Steiner RM, Chin JG, Buis-Liem T. Congenitally corrected transposition in adults with left atrioventricular valve incompetence. Radiology 1985; 155:567-570. Friedberg DZ, Nadas AS. Clinical profile
Radiology
525
#{149}
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.
:
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PA chest radiograph is difficult to determine. Radionuclide CCT (compare with Fig. 3).
Radiology
#{149}
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c. Figure
526
.
#{149}:j7’
.
a.
‘
7.
(a) shows images
situs solitus and dextrocardia of the right (b) and left
in case 16. The (c) sides of the heart
position show
of the ascending typical characteristics
November
aorta of
1985
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Figure
8.
PA chest
radiograph
(a) shows
situs
solitus
and
dextrocardia
in case
17. The
position
of the
ascending
aorta
can hardly he determined. Radionuclide image of the right side (b) of the heart shows an oval shape of the right-sided ventricle and deviation of the pulmonary trunk to the left. Radionuclide image of the left side (c) of the heart shows a right ascending and left descending aorta. The apex of the left ventricle points to the right. There is superposition of the left ventricle on right-sided structures, indicating oblique position of the ventricular septum. CCI was excluded by RA study.
Volumel57
Number2
Radiology
#{149} 527
