CASE REPORTS
Coronary Artery Fistula with Anomalous
Coronary Artery Origin: A Case Report
Sahar S. Abdelmoneim, MD, Farouk Mookadam, MD, MSc, FRCPC, FACC,
Sherif E. Moustafa, MD, MSc, and David R. Holmes, MD, Rochester, Minnesota,
and Ottawa, Ontario, Canada
Congenital anomalies of the coronary arteries occur
in 0.2% to 1.2% of the general population. Most
coronary artery anomalies are found incidentally
during angiographic evaluation for other cardiac
diseases. We describe the case of a young woman
presenting with chest discomfort and fatigue where
echocardiography identified a coronary artery fis-
tula. Successful embolization of the coronary artery
CASE REPORT
A 22-year-old female Jehovah’s Witness presented with
chest discomfort and exertional dyspnea for many years.
No symptoms of heart failure were noted. She had a
history of stress-related left-sided chest tightness. Medical
history was positive for possible pericarditis. She was
referred to our facility for clinical evaluation and counsel-
ing before a planned pregnancy.
Physical examination revealed stable vital signs, normal
first and second heart sounds, and a continuous murmur
at midleft sternal border that increased during expiration.
The remainder of the examination was unremarkable.
Electrocardiographic, chest radiographic, and laboratory
findings were all normal.
Transthoracic echocardiography (TTE) Doppler con-
firmed the presence of a coronary artery fistula (CAF) with
a continuous left-to-right shunt noted in the right ventric-
ular (RV) outflow tract. In addition, an anomalous origin of
the right coronary artery (RCA) from the left coronary
system was noted. Marked dilatation (17 mm) of the distal
RCA segment was noted (Figure 1 and Video 1). The left
ventricle showed normal size and function with an ejec-
tion fraction of 64%. Mild RV enlargement with normal
systolic function was noted with an estimated RV systolic
pressure of 26 mm Hg. Cardiac valves were normal.
From the Departments of Cardiology at Mayo Clinic College of
Medicine and University of Ottawa Heart Institute (S.E.M.).
Reprint requests: Farouk Mookadam, MD, MSc, FRCPC, FACC,
Cardiovascular Diseases, Mayo Clinic Scottsdale, 3rd Floor Mayo
Bldg., 13400 E. Shea Blvd., Scottsdale, AZ 85259. (E-mail:
Mookadam.farouk.@mayo.edu).
0894-7317/$32.00
Copyright 2007 by the American Society of Echocardiography.
doi:10.1016/j.echo.2006.09.012
fistula was performed. To our knowledge, this is a
unique case of an anomalous coronary artery and a
fistulous anomaly in the same patient. This report
describes the echocardiographic diagnosis, and the
feasibility of a transcatheter management decision
to avoid cardiopulmonary bypass in a patient in
whom blood products are contraindicated. (J Am
Soc Echocardiogr 2007;20:333.e1-e4.)
Cardiac catheterization revealed a dominant single left
coronary artery with a dilated left main coronary artery and
a normal left anterior descending coronary artery. The right
coronary ostium was absent at the site of the right sinus of
Valsalva. Three separate fistulous tracts arising from the left
anterior descending coronary artery supplying an aneurys-
mal RCA were identified. The most proximal RCA aneurysm
drained into the RV. There was no atherosclerosis of the
native coronary arteries (Figure 2, A and Video 2, A).
The patient underwent percutaneous coil embolization of
the fistulae. The distal fistula was embolized using 3 Tornado
microcoils (Cook Inc., Bloomington, Ind): two 5-mm coils
and one 8-mm coil were deployed. The middle fistula was
embolized with 4 fiber coils: one 2 ⫻ 20 mm, one 3 ⫻ 30
mm, and two 2 ⫻ 20 mm. The proximal fistula was emboli-
zed with three 2 ⫻ 20 mm fiber coils. Postembolization
angiography revealed no residual communication. There
were no periprocedural complications (Figure 2, B and
Video 2, B).
At 1-year follow-up, the patient was asymptomatic and
had an uneventful pregnancy. Follow-up TTE revealed the
original anomaly of the RCA arising from the left anterior
descending coronary artery. No residual CAF was identi-
fied. At 2-year follow-up, the patient remained asymptom-
atic and coronary angiography revealed a trivial flow
around 2 of the 3 fistulae.
DISCUSSION
CAF is an aberrant connection between a coronary
artery and a cardiac chamber, pulmonary artery,
coronary sinus, or pulmonary vein, occurring in
0.1% to 0.2% of all patients undergoing coronary
angiography. The fistulae may be single or multiple,
isolated or associated with accompanying congeni-
tal defects. The hemodynamic effect of CAF depends
333.e1
 Journal of the American Society of Echocardiography
333.e2 Abdelmoneim et al March 2007

Figure 1 Transthoracic echocardiogram parasternal long-axis view. A, Very dilated right coronary artery
(RCA) (dashed lines). B, Continuous left-to-right shunt noted in right ventricular (RV) outflow tract. Ao,
Aorta; AV, Ao valve; IVS, interventricular septum; LA, left atrium; LV, left ventricle; LVOT, LV outflow
tract; MV, anterior mitral valve leaflet.

on the site of drainage and resistance within the
fistula. CAF may be caused by deviations of normal
embryologic development or acquired from trauma
or iatrogenesis during invasive cardiac procedures
such as pacemaker implantation, endomyocardial
biopsy, or coronary angiography.1 Congenital CAF
most frequently arises from the RCA system with the
majority exiting into the right heart.2 Patients with
CAF present with palpitations, chest discomfort,
heart failure, or bacterial endocarditis, or they may
be asymptomatic, presenting with an incidental
continuous murmur, characteristically heard over
the left sternal border and apex.3
TTE is an important noninvasive tool for identify-
ing the anomalous origin of coronary arteries.4 Con-
tinuous turbulent systolic and diastolic flow pattern
characterizes the shunt entry site.5,6 However, TTE
may not detect flow in the distal site of a CAF.
Transesophageal echocardiography more accurately
defines the origin, course, and drainage site of CAF.7
Journal of the American Society of Echocardiography
Volume 20 Number 3 Abdelmoneim et al 333.e3

Figure 2 Coronary angiogram pre- and post-coil embolization. A, Single left coronary artery. Dilated left
main and proximal left anterior descending coronary artery (LAD). No right coronary artery (RCA)
ostium. Three separate fistulous (F) tracts arise from LAD supplying markedly aneurysmal RCA. B, Left
coronary to RCA F, coil embolization of F to RCA (3 coils).

333.e4 Abdelmoneim et al
Contrast-enhanced electron-beam tomography is
newer, more sensitive diagnostic tool with excellent
spatial resolution and identifies most coronary anoma-
lies including origin, course, and insertion; this is at a
cost of ionizing radiation and potentially nephrotoxic
or allergenic contrast agents.8 Magnetic resonance
imaging holds the greatest appeal because it avoids
radiation and contrast agents and yields excellent
images at expert centers.9 Coronary angiography
still remains the gold standard for diagnosing coro-
nary anomalies. Selective coronary arteriography
can be used to reliably identify the size and anatomic
features of the fistulous tract and can be both
diagnostic and therapeutic.1
Management of CAF remains controversial. Small
asymptomatic fistulae are usually asymptomatic and
managed conservatively. They generally run a benign
course and may close spontaneously.10,11 Surgical liga-
tion of fistulae is limited to larger symptomatic fistulae
with large branch vessels that may be compromised by
occlusion devices. Surgical correction may also be
used for coronary fistulae with multiple communica-
tions. Surgical management is safe and effective with a
high closure rate and survival but periprocedural myo-
cardial infarction or fistula recurrences have been
described. Furthermore, operation requires a median
sternotomy and bleeding can be significant.12
Catheter-based closure of the fistulous connection
is an alternative strategy in selected patients. Early
complications included transient S-T segment and
arrhythmias.13 In this case presentation, blood prod-
ucts were contraindicated for religious purposes,
hence percutaneous embolization of the fistulae
were undertaken.
Conclusion
A dilated coronary artery can usually be detected by
2-dimensional echocardiography. Coronary angiogra-
phy remains the gold standard for imaging the coro-
nary arteries and for determination of the relation of
CAF to other structures. New imaging modalities such
as contrast-enhanced computed tomography with 3-di-
mensional reconstruction or magnetic resonance im-
aging can be used as an adjunct to coronary angiogra-
phy and identify the anatomy of CAF. Transcatheter
closure of CAF is a safe alternative in anatomically
suitable vessels, with a lower risk than sternotomy and
surgical correction.
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SUPPLEMENTARY DATA
Supplementary data associated with this article can
be found, in the online version, at 10.1016/j.echo.
2006.09.012.