MURAI ET AL

CASE REPORT
Radiation Medicine: Vol. 22 No. 3, 186189 p.p., 2004

Evaluation of Major Aortopulmonary Collateral Arteries (MAPCAs)

Using Three-dimensional CT Angiography: Two Case Reports
Sachiko Murai,* Seiki Hamada,* Shuji Yamamoto,** Azzam A. Khankan,*
Hiromitsu Sumikawa,* Atsuo Inoue,* Mitsuko Tsubamoto,* Osamu Honda,*
Noriyuki Tomiyama,* Takeshi Johkoh,*,*** and Hironobu Nakamura*

Pulmonary atresia (PA) and ventricular septal defect (VSD) are usually associated with major
aortopulmonary collateral arteries (MAPCAs). Preoperative evaluation of MAPCAs is essential
for effective planning of unifocalization. Multidetector-row computed tomography (MDCT)
with three-dimensional volume rendering (3D-VR) demonstrates MAPCAs as well as
conventional angiography. We report two cases of PA and VSD associated with MAPCAs
examined pre- and postoperatively by means of angiography and MDCT with the 3D-VR
technique. MDCT with the 3D-VR technique had the potential to elucidate all MAPCAs
demonstrated by invasive angiography. This technique is useful with preoperative conventional
angiography as a standard examination for the planning of staged surgery.

Key words: multidetector-row CT (MDCT), three-dimensional volume rendering technique

(3D-VR), major aortopulmonary collateral artery (MAPCA), tetralogy of Fallot

INTRODUCTION Recently, multidetector-row CT (MDCT) has been

considered the standard noninvasive imaging modality

P ULMONARY ATRESIA ( PA) AND VENTRICULAR SEPTAL

defect (VSD) are generally associated with major
aortopulmonary collateral arteries (MAPCAs). The total
surgical correction of this congenital anomaly is usually
staged and consists of two different surgical approaches.
In the first stage of repair, complete unifocalization is
performed by unifying intrapulmonary arteries at the
hilum, while the second stage consists of establishing
right ventricular pulmonary arterial continuity with
closure of the ventricular septal defect. In order to
completely unifocalize the multifocal pulmonary blood
sources, preoperative evaluation of the anatomic details
of MAPCAs is necessary. Although conventional
angiography is an accepted diagnostic modality for the
evaluation of MAPCAs, it is invasive and sometimes
misses the central and hilar pulmonary arteries when
they are not confluent in patients with tetralogy of Fallot.1
Received September 18, 2003; revision accepted December 18,
2003.
Departments of *Radiology and ***Medical Physics, Osaka
University Graduate School of Medicine
**Department of Radiology, Osaka University Hospital
Reprint requests to Sachiko Murai, M.D., Department of
Radiology, Osaka University Graduate School of Medicine, 2-2
Yamadaoka, Suita, Osaka 565-0871, JAPAN.
186
for evaluating the aorta and its branches.2 By using
MDCT with thin spatial resolution of the vascular
structures and the three-dimensional volume rendering
(3D-VR) technique, the architecture of vascular
structures can be visualized.
We describe two cases of PA and VSD associated
with MAPCAs evaluated pre- and postoperatively using
both angiography and MDCT with the 3D-VR technique.
Visualization of MAPCAs on 3D-VR images was
compared with that of conventional angiography and
was found to provide all the anatomic details needed for
planning and following up the surgical repair. MDCT
with 3D-VR, a noninvasive modality, could demonstrate
MAPCAs and their anatomic relationships with other
structures and assist with conventional angiography for
future staged surgical repair.
To our knowledge, this is the first report comparing
MDCT with the 3D-VR technique with conventional
angiography for the evaluation of MAPCAs.
CASE REPORTS
Case 1
A 24-year-old man had been diagnosed with PA and
VSD with MAPCAs at the age of 3 years. One year ago,
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Fig. 1. Case 1.
Left MAPCA originating from the descending thoracic aorta bifurcates into two branches as shown on the three-dimensional volume
rendering image (a) and angiography (b). The upper branch (arrow) runs upward and then turns caudally to supply the upper lobe,
while the lower branch (arrowhead) runs directly toward the lower lobe.
he developed severe congestive heart failure symptoms,
and echocardiography showed significant aortic
regurgitation. After he underwent aortic valve
replacement, a staged unifocalization was decided, and
pulmonary arteriography was requested for planning the
surgical repair. Both MDCT with 3D-VR and
angiography of the pulmonary arteries were performed
preoperatively.
MDCT scanning was performed on a four-detector
row Aquilion (Toshiba Medical Systems Corp., Tokyo,
Japan) from the aortic arch to the diaphragm with a
slice thickness of 2 mm, a pitch of 5.5, rotation time of
0.5 second, 120 kV, and 200 mA. Ninety milliliters of
iohexol 300 mgI/ml (Omnipaque 300, Daiichi
Pharmaceutical Co., Ltd., Tokyo, Japan) was injected
through the median cubital vein at a rate of 2 ml/s. The
enhanced helical scan was initiated automatically with
aortic enhancement of 150 HU at the level of the right
pulmonary artery. All the scans were done during a single
breath hold of about 20 seconds.
The raw scan data were then reconstructed with a 2
mm slice thickness and 1 mm interval. About 300 axial
image slices were reconstructed covering the whole
thoracic aorta. The reconstructed image data were then
transferred to an independent workstation (Plugn View
ZEUS, KCO, Ltd., Voxar, Edinburgh, UK) for 3D-VR
reformation.
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CASE REPORT
a b
3D-VR images showed three right MAPCAs and one
left MAPCA, all arising from the descending thoracic
aorta (Fig. 1a). Of the three right MAPCAs, one was
relatively large and supplied both middle and lower
lobes, while the others were relatively small and supplied
the upper and middle lobes. The left MAPCA bifurcated
a short distance after its origin into two branches: an
upper branch running upward then turning caudally to
supply the upper lobe, and a lower branch running to
the lower lobe. These findings were compared and
confirmed with thoracic arteriography (Fig. 1b), and both
modalities showed similar images. The patient was
advanced for staged surgical repair. The first stage of
repair was done in two steps: first right pulmonary
unifocalization and then left pulmonary unifocalization.
Operative findings confirmed both the CT and
angiographic findings.
One month later, second-stage repair, reconstruction
of the right ventricular outflow tract, was performed,
followed by CT and cardiac catheterization, which also
showed similar images.
Case 2
A 31-year-old woman with PA, VSD, and MAPCAs
underwent left brachio-thoracic shunt at the age of 14
years. Three years ago, she developed progressive heart
failure symptoms, and surgical correction was decided.
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MURAI ET AL
She underwent MDCT and thoracic angiography. The
same CT scan parameters as those of case 1 were used
for this patient. CT axial images showed the absence of
apical and posterior segments (S1 and S3) of the upper
lobe and the entire middle lobe (S4 and S5) of the right
lung. 3D-VR images (Fig. 2a, b) depicted two MAPCAs
arising from the left side of the descending thoracic aorta.
The upper MAPCA was small and ran toward the right
to the posterior segment of the right upper lobe (S2).
The lower MAPCA was large and bifurcated into two
branches: one ran upward and supplied the whole left
lung, and the second branch ran toward the right and
supplied the right lower lobe contralaterally. These
vascular findings of 3D-VR images were confirmed by
angiography (Fig. 2c, d). She was advanced for staged
188
a b
c d
Fig. 2. Case 2.
Two left MAPCAs are shown on three-
dimensional volume rendering images of
the descending thoracic aorta (a, b). The
upper artery (arrow) is small and runs
toward the right to the posterior segment
of the right upper lobe, while the lower
artery is large and bifurcates into an
upper branch (large arrowhead)
supplying the whole left lung and a lower
branch (small arrowhead) supplying the
right lower lobe contralaterally. Their
courses and branches are shown on
selective arteriographies of the upper (c)
and lower (d) left MAPCAs.
surgical repair, which was performed using a technique
similar to that of case 1. Both CT and angiography were
done after each stage and showed no differences.
DISCUSSION
The presence of PA as a component of tetralogy of Fallot
is designated as an extreme form. In such patients, the
lungs are supplied by extracardiac sources such as patent
ductus arteriosus, previous systemic-pulmonary shunts,
and, most commonly, MAPCAs. These discrete arteries
arise commonly from the descending thoracic aorta, but
they may arise from the aortic arch branches and, rarely,
from the abdominal aorta.3-9 MAPCAs transmit blood
to the pulmonary arterial circulation at variable
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pressures. Long-standing systemic blood flow into the
pulmonary circulation leads to pulmonary hypertension
and MAPCA stenosis. Complete unifocalization of
MAPCAs (unifying the intrapulmonary arteries at the
hilum) is the first stage and most important part of
complete surgical repair of this cardiac anomaly, while
the complete correction (establishment of right
ventricular-pulmonary arterial continuity and closure of
the ventricular septal defect) is performed as a second
stage. 3,4,7,9 Accurate visualization of MAPCAs is
necessary before unifocalization. Angiography is
considered the standard modality for evaluation of
MAPCAs, but it is invasive, time-consuming, not
suitable for multiple follow-up examinations, and has
moderate to severe complication rates.11,12 CTs role as
a noninvasive imaging modality for evaluation of the
aorta and its branches has been proven.2 Currently,
MDCT with 3D-VR through administration of a single
bolus of contrast material constitutes a significant
advantage over invasive angiography in the diagnosis
of vascular diseases. In our cases, the specific anatomic
details of MAPCAs required for surgical repair were
well depicted by 3D-VR images, which act as an
operative road map for the surgeon in spite of the
obscurity of peripheral pulmonary arteries. Additionally,
MDCT was useful in demonstrating the comorbid
anomalies of non-vascular thoracic structures in case 2.
It assists invasive angiography in the diagnosis, planning
of surgical approach, and postoperative follow-up of
tetralogy of Fallot. Although magnetic resonance
imaging (MRI) was not performed in our cases, it is
noteworthy that images similar to pulmonary
angiography are obtained on MRI, but MRI has some
imitations. The time allotted for MRI examination is
longer than that for MDCT, and MRI examination is
largely dependent on the effective time of sedatives in
pediatric cases.13,14
The advantages of MDCT with 3D-VR indicate that
it will become a routine procedure with selective
angiography for the preoperative evaluation of MAPCAs
in tetralogy of Fallot.
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