AJR 2005; 184:S28-S32
© American Roentgen Ray Society
Demonstration of Complex Coronary-Pulmonary Artery Fistula by MDCT and Correlation with Coronary Angiography
Monica S. M. Chan1,2,
Ivan Y. F. Chan1,
K. H. Fung1,
Gilbert Lee1,
K. L. Tsui3 and
T. C. Leung3
1 Department of Radiology, Pamela Youde Nethersole Eastern Hospital, Hong Kong
SAR, China.
2 Present address: Department of Diagnostic Radiology and Organ Imaging, The
Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing
St., Shatin NT, Hong Kong SAR, China.
3 Department of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong
SAR, China.
Received April 18, 2004;
accepted after revision June 22, 2004.
Address correspondence to M. S. M. Chan
(drmonicachan{at}hotmail.com).
Introduction
Coronary artery anomalies are rare, with an incidence of 0.2% to
1.2% [1], among which
coronary-pulmonary artery fistula is usually detected in 0.1% to 0.2% of
coronary angiograms
[2-4].
Although not all coronary-pulmonary artery fistulas are clinically or
hemodynamically significant, some can result in serious consequences including
myocardial ischemia, myocardial infarction, or sudden death
[5]. When complex anatomy or
intervention is contemplated, coronary angiography may not be sufficient. An
ideal investigation technique should be noninvasive and provide a quality
anatomic description of the fistula.
We report a case of complex coronary-pulmonary artery fistula with two
feeding vessels of separate origins: one from the left coronary artery via the
left anterior descending artery and another arising from the right coronary
sinus. The complex anatomy of the fistula was demonstrated in detail by an
MDCT scanner using multiplanar reconstruction and different 3D reconstruction
techniques.
Case Report
A 60-year-old woman was referred to our hospital for management of heart
failure. She had a history of hypertension and heart murmur, the latter of
which had not been investigated. Chest radiography showed moderate
cardiomegaly. ECG showed persistent T wave inversion over V5 and V6 leads but
the creatine kinase level was normal.
Transthoracic and transesophageal echocardiography were performed and
showed findings suspicious for coronary-pulmonary artery fistula. A coronary
angiogram was obtained that confirmed coronary fistula draining into pulmonary
trunk (Figs. 1A and
1B). Despite various
projections, the exact anatomic course of the suspected fistula could not be
clearly shown by the coronary angiogram.

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Fig. 1A. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. Left
anteroposterior projection of left coronary angiogram shows an abnormal
complex vascular structure composed of a plexus of fine tortuous vessels
(open arrow) arising from the proximal left anterior descending
artery (LAD) and a main draining vessel (1). Abnormal early opacification of
main pulmonary artery (MPA) was present, suggestive of a coronary pulmonary
artery fistula (F). Left main coronary artery (LCA) and LAD were dilated while
the left circumflex artery (CFA) was of normal caliber.
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Fig. 1B. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. Right
anterolateral projection with caudal tilting of the left coronary angiogram
has similar findings as in A but failed to resolve the complex anatomy.
On this projection, an abnormally dilated branch of left anterior descending
artery (asterisk) is seen.
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Contrast-enhanced CT of the heart and great vessels, including coronary
arteries, was performed in an attempt to demonstrate the course of the
coronary-pulmonary artery fistula.
CT coronary angiography was performed using a 16-slice MDCT scanner
(Aquilion TSX 101A M16, Toshiba). Imaging parameters of 120 kV, 250 mAs, and
0.5-mm slice collimation were preset for the scan. Given the patient's heart
rate was between 65-70 beats per minute and was electrocardiogram gated (ECG
gated), the CT scanner could automatically optimize the scanning parameters
for the examination. In this case, gantry rotation speed at 0.4 sec per
revolution and a helical pitch of 3.2 were applied. A multisegment
reconstruction algorithm was also selected automatically, which effectively
improved the temporal resolution to 116 msec from the 200 msec, if a
half-reconstruction algorithm was to be used.
The examination was performed using a single breath-hold technique to cover
120 mm from the cardiac outflow tract to the apex of heart within a total
scanning time of about 30 sec. A total of 120 mL nonionic water-soluble
iodinated contrast medium at 370 mg I/mL concentration followed by 30 mL of
normal saline IV was administered at a rate of 4 mL/sec. An automated contrast
medium tracing program was applied to trigger the scan when the attenuation at
the ascending aorta reached 180 H. Retrospective ECG-gated multisegment
reconstruction was performed at a 0.4-mm interval (i.e., 20% overlapping),
with a predefined temporal offset at 70% R-R wave interval (at diastole) of
each cardiac cycle to demonstrate the anomaly, using multiplanar
reconstruction and different 3D reconstruction techniques. Postexamination
image processings were performed via a commercial workstation using 3D
visualization software (Vitrea 2, version 3.4.5, Vital Images). Reconstruction
at every 10% of R-R intervals of cardiac cycles allowed cardiac function
analysis to be performed using the same set of image data.
The anatomy of the coronary artery-pulmonary artery fistula was complex but
was well demonstrated after detailed analysis. The aortic and coronary sinuses
were well shown with segmentation techniques and interactive sectioning on the
3D image to remove the overlying right and left atria. The left main coronary
artery arising from the left coronary sinus was dilated, measuring up to 12 mm
in caliber. The dilated left coronary artery immediately branched into the
left anterior descending and left circumflex arteries. The left circumflex
artery was of normal caliber and ran along the left atrioventricular groove.
The left anterior descending artery passed posteroinferior to the left main
pulmonary artery, running along the interventricular grove to emerge at the
cardiac surface to the left of the main pulmonary artery, where it gave rise
to a few branches (Figs. 1C and
1D). The continuation of the
left anterior descending artery ran along the interventricular groove and
became abruptly terminated at the mid interventricular groove (Figs.
1E and
1F). A plexus of fine tortuous
vessels arose from the proximal left anterior descending artery and its side
branches at the level of the atrioventricular groove. This plexus formed a
network that encircled the left anterior descending artery and eventually
coalesced to form a major tortuous dilated draining vessel that ran
horizontally across the anterior aspect at the base of the main pulmonary
artery (Figs. 1E and
1F). This large draining vessel
in turn ran superiorly on the right side of the main pulmonary artery, where
it formed an irregular C-shaped tubular structure, measuring 7.3 mm, on the
surface of the root of the main pulmonary artery
(Fig. 1E). Finally, it ended by
joining to a 20-mm bulbous dilatation over the anterior aspect at the base of
the main pulmonary artery before draining into the main pulmonary artery
(Figs. 1E,
1F and
1G).

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Fig. 1C. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. CT
coronary angiogram by 2D normal rendering thick-slab oblique axial
reconstruction shows the dilated left main coronary artery, which immediately
branched into left anterior descending (LAD) and left circumflex arteries. A
plexus of fine tortuous vessels (open arrow) arises from the proximal
LAD and its side branches (asterisk) at the level of atrioventricular
groove. A smaller anonymous vessel (2) arises from the left side of the right
coronary sinus, another feeding vessel of the coronary-pulmonary artery
fistula. AA = ascending aorta, DA = descending aorta, SVC = superior vena
cava, LA = left atrium, RA = right atrium, RCA = right coronary artery, MPA =
main pulmonary artery.
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Fig. 1D. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. CT
coronary angiogram by 2D normal rendering thick-slab oblique axial
reconstruction at a more cranial level to C shows exact site of fistula
draining into the main pulmonary artery (MPA).
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Fig. 1E. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. Right
anterolateral projection of the heart by 3D tissue tone volume rendering shows
the surface anatomy of the complex coronary pulmonary artery fistula and its
relationship with the adjacent structures. The left anterior descending artery
(LAD) continues to run along the interventricular groove and is abruptly
terminated at the mid interventricular groove. A plexus of fine tortuous
vessels (open arrow) arises from the proximal LAD, and its side
branches at the level of the atrioventricular groove form a network encircling
the LAD and eventually coalesce to become a major tortuous dilated draining
vasculature (1), running horizontally across the anterior aspect at base of
the main pulmonary artery (MPA). Note the irregular C-shaped tubular structure
formed by the draining vasculature (1) on the surface of the root of the main
pulmonary artery (MPA). Similar plexus of fine tortuous vessels (open
arrow) supplied by the anonymous vessel arising from the right coronary
sinus (2) is found along the surface of the ascending aorta, arch of aorta,
and MPA. The anonymous vessel arising from the right coronary sinus had
separate origin from the right coronary artery (RCA). AA = ascending aorta, LA
= left atrium, RV = right ventricle, LV = left ventricle, LPA = left pulmonary
artery, F = coronary pulmonary artery fistula.
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Fig. 1F. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. Left
anteroposterior projection of the heart by 3D tissue tone volume rendering
shows clearly the nondilated right coronary artery and the anonymous vessel
(2) arising from the right coronary sinus. AA = ascending aorta, SVC =
superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, arrow = plexus of
fine vessels along the surface of the root and proximal ascending aorta, RV =
right ventricle, LV = left ventricle.
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Fig. 1G. 60-year-old woman presenting with heart failure and heart
murmur. White arrowhead = origin of the anonymous vessel, black arrowhead =
origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior
vena cava, RA = right atrium, RCA = right coronary artery, MPA = main
pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus
of fine vessels along the surface of the root and proximal ascending aorta and
along the left atrioventricular groove, 1 = C-shaped main draining vessel to
the fistula, 2 = anonymous vessel arising from right coronary sinus. CT
coronary angiogram by 3D tissue tone volume rendering with oblique trimming
shows clearly the exact drainage of fistula (F) into the main pulmonary artery
(MPA). The anonymous vessel (2) is seen arising from the dilated right
coronary sinus (RCS), with a separate origin from the right coronary artery
(RCA).
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Bulbous dilatation of the right coronary sinus was present
(Fig. 1G). The right coronary
artery arose from the right side of the coronary sinus and was of normal
caliber (Figs. 1F and
1G). It was seen running along
the right atrioventricular groove. Another smaller anonymous vessel was found
arising from the left side of the right coronary sinus, which was another
feeding vessel of the ultimate coronary-pulmonary artery fistula. It ran
superiorly in a straight path for 15 mm, becoming a tortuous loop before
forming another plexus of fine vessels along the surface of the ascending and
arch of aorta and the main pulmonary artery. This plexus acted as a second
supply to the fistula that eventually drained into the ultimate
coronary-pulmonary artery fistula (Figs.
1F and
1G). This additional supply to
the fistula was not appreciated in the coronary angiogram because of selective
cannulation of left and right coronary arteries only.
Moderate dilatation of the left atrium and left ventricle was noted. CT of
the thorax showed dilated pulmonary vasculatures consistent with congestive
heart failure.
The ejection fraction was estimated to be 78.3% by cardiac functional
analysis, comparable with that found on conventional coronary angiography
(76%).
Discussion
Until recently, conventional coronary angiography was the diagnostic method
of choice for detecting coronary anomalies. However, it is invasive and has a
0.15% mortality rate and 1.5% morbidity rate
[6]. The diagnostic value of
coronary angiography is limited by its planar imaging nature, restricted angle
of angiographic projections, and concern for the contrast load. For complex
pathology as presented in our case, conventional coronary angiography
sometimes is inadequate for a clear demonstration of the exact anatomy.
Other noninvasive imaging techniques, including MRI and contrast-enhanced
electron beam tomography (EBT), were introduced in the past few years and have
proven to be reliable [7].
Their results are comparable to those obtained by conventional coronary
angiography. However, both MRI and EBT have their limitations.
Although MRI can demonstrate a lesion in multiple planes, the temporal
resolution only reaches 100-150 msec
[8]. Hence, its use is limited,
especially in patients with tachycardia or arrhythmia in whom significant
motion artifacts occurred and only poor image quality of limited diagnostic
value could be obtained. EBT has a temporal resolution of 100 msec
[9] and is capable of
demonstrating the lesion in multiple planes; however, its clinical application
is limited by its availability and its limited image quality.
Because of its advancements, MDCT is an emerging practical method for the
investigation of coronary artery disease. With the availability of 16-detector
row MDCT, which improves both in its temporal and spatial resolutions, it is
now possible and practical to have high-quality cardiac imaging. Although MDCT
remains inferior in temporal resolution to conventional coronary angiography
(40 msec), its spatial resolution is superior to that of MRI and EBT
[9]. The high-contrast
resolution of 16-detector row MDCT when using a 0.5-mm acquisition can achieve
a spatial resolution of 0.35 ± 0.05 mm at all planes
[10]. This spatial resolution
is approaching that of conventional coronary angiography. The radiation dose
from 16-detector row MDCT is similar to that of uncomplicated conventional
coronary angiography [8]. Using
MDCT, the data are collected after a single shot of contrast injection. As the
spatial resolution of data collected is isotropic, images can be reviewed by
multiplanar reconstruction and 3D reconstruction techniques including surface
shaded display, volume rendering, and maximum intensity projection. The
ultrafast rate of the machine allows data acquisition at different phases of
the cardiac cycles, reducing the pulsation motion artifacts of normal cardiac
cycles significantly.
The 3D reconstruction with viewing at an unlimited angle allows us to
demonstrate a lesion such as a fistula at its best projection, without
subjecting the patient to repeated radiation exposure and an additional
contrast load, and makes assessment of the size and exact location of the
lesion feasible. This could be helpful for planning future cardiovascular
intervention.
Apart from the qualitative analysis of the complex cardiac pathology,
similar to conventional coronary angiography, MDCT cardiac examination allows
quantitative cardiac function analysis.
The short investigation time (total scanning time as short as 30 sec),
relative noninvasiveness of the procedure, simple preparation, and minimal
aftercare make MDCT coronary angiography advantageous over conventional
coronary angiography.
We believe that with its increasing availability and advances in
technology, including data acquisition and postprocessing, coronary angiogram
and cardiac investigation using noninvasive MDCT could be more practical and
become the main method of choice for investigation of coronary artery
anomalies and diseases.
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