DOI:10.2214/AJR.05.2098
AJR 2007; 188:W138-W146
© American Roentgen Ray Society
Congenital Anomalies of Coronary Artery Origin in Adults: 64-MDCT Appearance
Jonathan D. Dodd1,
Maros Ferencik1,
Richard R. Liberthson2,
Ricardo C. Cury1,
Udo Hoffmann1,
Thomas J. Brady1 and
Suhny Abbara1
1 Department of Radiology and Cardiac MR-PET-CT Program, Massachusetts General
Hospital and Harvard Medical School, 55 Fruit St., Boston, MA 02114.
2 Cardiology Division, Massachusetts General Hospital and Harvard Medical
School, Boston, MA 02114.
Received December 5, 2005;
accepted after revision February 14, 2006.
Address correspondence to J. D. Dodd
(jddodd{at}partners.org).
WEB This is a Web exclusive article.
Abstract
OBJECTIVE. The purpose of this pictorial essay is to review the
64-MDCT appearance of congenital anomalies of the origins of the coronary
arteries in adults.
CONCLUSION. Increasing use of MDCT for cardiac imaging of adults
requires familiarity with the cross-sectional appearance of congenital
coronary artery anomalies visualized with noninvasive imaging techniques. Many
of these anomalies are benign, but a small number are associated with
myocardial ischemia and sudden death. Increasing use of MDCT in cardiac
imaging may yield diagnostic information not obtained with coronary
angiography. Axial, multiplanar, and 3D volume-rendered reconstructions should
aid in detection and improve interpretation of such anomalies.
Keywords: angiography cardiac imaging coronary angiography/methods CT coronary angiography tomography X-ray computed
Introduction
Primary congenital anomalies of the coronary arteries have an
incidence of 1-2% in the general population
[1]. Noninvasive techniques
such as MRI, electron-beam CT, and MDCT depict these anomalies with high
accuracy [2,
3]. MDCT in particular provides
high-resolution 3D data sets that allow precise definition of 3D spatial
relations of the anomalies. Comprehensive reviews of 16-MDCT of primary
congenital coronary anomalies have been limited
[2-5].
To our knowledge, this is the first review to provide comprehensive data from
64-MDCT. Recognition of coronary anomalies, particularly those associated with
a malignant course, increased morbidity, and sudden death, is important. The
aim of this article is to illustrate the appearance and course of congenital
coronary anomalies on 64-MDCT with coronary angiographic correlation in
selected cases.
Normal Anatomic Features
The left main coronary artery (LMCA) originates from the left sinus of
Valsalva (Fig. 1A). It can
bifurcate into the circumflex and left anterior descending (LAD) branches or
trifurcate with an additional intermediate ramus branch. The LAD coronary
artery usually descends in the anterior interventricular groove. Septal
branches arise from the LAD coronary artery and course into the
interventricular septum, supplying the anterior two thirds of the septum.
Diagonal branches arise from the LAD coronary artery and descend toward the
lateral margin of the left ventricular wall. The circumflex branch enters the
left atrioventricular groove to supply obtuse marginal branches to the lateral
and posterolateral walls of the left ventricle. In left coronary dominant
patients (10%), the circumflex branch travels all the way around the
ventricle, supplying both the posterior descending coronary artery and the
posterior left ventricular branch. More commonly, however, the circumflex
branch terminates as a small branch in the atrioventricular groove or
continues as a small terminal posterior ventricular branch.

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Fig. 1A 48-year-old man with intermittent palpitations and abnormal exercise
stress test result. Three-dimensional volume-rendered reformation shows normal
left main coronary artery originating from left sinus of Valsalva
(straight white arrow) and passing obtusely downward before
dividing into left anterior descending coronary artery (black arrow)
and circumflex artery (curved arrow). Left main coronary artery
occasionally trifurcates with additional ramus intermedius branch
(open arrow). Left anterior descending coronary artery
usually extends to apex and may even supply inferior apical region. Diagonal
branches (arrowhead) vary in number and supply lateral wall of left
ventricle.
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The right coronary artery (RCA) originates from the right coronary sinus of
Valsalva at a slightly lower level than the origin of the left main coronary
artery (Fig. 1B). The RCA
descends in the anterior right atrioventricular groove. It usually gives off
the conus branch as its first branch and then two or three large right
ventricular wall branches. The acute marginal branch is the first large
branch, which occasionally continues to the apex. In 70% of patients the RCA
passes down the atrioventricular groove to the crux of the heart, where it
gives off the posterior descending artery and posterior left ventricular
branches. Approximately 20% of patients have a codominant supply, in which the
posterior descending artery originates from the RCA, but branches from the
circumflex artery also supply this territory.

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Fig. 1B 48-year-old man with intermittent palpitations and abnormal exercise
stress test result. Three-dimensional volume-rendered reformation shows right
coronary artery originating from right sinus of Valsalva (straight
white arrow), passing down right atrioventricular groove, and giving
off acute marginal branch (curved arrow) before dividing into
posterior descending artery (open arrow) and posterior
lateral ventricular branch (black arrow). Posterior descending artery
courses in posterior interventricular groove to supply posterior
interventricular septal branches.
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Classification of Coronary Artery Anomalies
Various classification systems exist for primary congenital coronary
anomalies. For this review we divided coronary anomalies into the following
three broad categories [6]:
anomalous aortic origin (Fig.
2A,
2B,
2C,
2D,
2E), anomalous aortic origin
with anomalous proximal course (Fig.
3A,
3B,
3C,
3D,
3E), and anomalous origin from
the pulmonary artery (Fig. 4A,
4B,
4C,
4D,
4E). The third category in
this classification (anomalous coronary origin from the pulmonary artery) is
not discussed or illustrated further in this article.

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Fig. 2A Schematic of primary coronary anomalies shows group 1, anomalous
aortic origin. Solid line = left main coronary artery/left anterior descending
coronary artery; dashed line = right coronary artery; dotted line = left
circumflex artery. Types 1a-1e. Type 1a (A) is illustrated in Figure
7A,
7B, type 1c (C) in
Figure 10A,
10B, and type 1e (E) in
Figure 6A,
6B,
6C. RVOT = right ventricular
outflow tract.
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Fig. 2B Schematic of primary coronary anomalies shows group 1, anomalous
aortic origin. Solid line = left main coronary artery/left anterior descending
coronary artery; dashed line = right coronary artery; dotted line = left
circumflex artery. Types 1a-1e. Type 1a (A) is illustrated in Figure
7A,
7B, type 1c (C) in
Figure 10A,
10B, and type 1e (E) in
Figure 6A,
6B,
6C. RVOT = right ventricular
outflow tract.
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Fig. 2C Schematic of primary coronary anomalies shows group 1, anomalous
aortic origin. Solid line = left main coronary artery/left anterior descending
coronary artery; dashed line = right coronary artery; dotted line = left
circumflex artery. Types 1a-1e. Type 1a (A) is illustrated in Figure
7A,
7B, type 1c (C) in
Figure 10A,
10B, and type 1e (E) in
Figure 6A,
6B,
6C. RVOT = right ventricular
outflow tract.
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Fig. 2D Schematic of primary coronary anomalies shows group 1, anomalous
aortic origin. Solid line = left main coronary artery/left anterior descending
coronary artery; dashed line = right coronary artery; dotted line = left
circumflex artery. Types 1a-1e. Type 1a (A) is illustrated in Figure
7A,
7B, type 1c (C) in
Figure 10A,
10B, and type 1e (E) in
Figure 6A,
6B,
6C. RVOT = right ventricular
outflow tract.
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Fig. 2E Schematic of primary coronary anomalies shows group 1, anomalous
aortic origin. Solid line = left main coronary artery/left anterior descending
coronary artery; dashed line = right coronary artery; dotted line = left
circumflex artery. Types 1a-1e. Type 1a (A) is illustrated in Figure
7A,
7B, type 1c (C) in
Figure 10A,
10B, and type 1e (E) in
Figure 6A,
6B,
6C. RVOT = right ventricular
outflow tract.
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Fig. 3A Schematic of primary coronary anomalies shows group 2, anomalous
aortic origin and anomalous proximal course. Solid line = left main coronary
artery/left anterior descending coronary artery; dashed line = right coronary
artery; dotted line = left circumflex artery. Types 2a-2e. Type 2a (A)
is illustrated in Figure 12A,
12B, type 2b (B) in
Figure 13A,
13B,
13C, type 2c (C) in
Figure 14A,
14B, type 2D (D) in
Figure 8, and type 2e
(E) in Figure 15A,
15B,
15C. RVOT = right ventricular
outflow tract.
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Fig. 3B Schematic of primary coronary anomalies shows group 2, anomalous
aortic origin and anomalous proximal course. Solid line = left main coronary
artery/left anterior descending coronary artery; dashed line = right coronary
artery; dotted line = left circumflex artery. Types 2a-2e. Type 2a (A)
is illustrated in Figure 12A,
12B, type 2b (B) in
Figure 13A,
13B,
13C, type 2c (C) in
Figure 14A,
14B, type 2D (D) in
Figure 8, and type 2e
(E) in Figure 15A,
15B,
15C. RVOT = right ventricular
outflow tract.
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Fig. 3C Schematic of primary coronary anomalies shows group 2, anomalous
aortic origin and anomalous proximal course. Solid line = left main coronary
artery/left anterior descending coronary artery; dashed line = right coronary
artery; dotted line = left circumflex artery. Types 2a-2e. Type 2a (A)
is illustrated in Figure 12A,
12B, type 2b (B) in
Figure 13A,
13B,
13C, type 2c (C) in
Figure 14A,
14B, type 2D (D) in
Figure 8, and type 2e
(E) in Figure 15A,
15B,
15C. RVOT = right ventricular
outflow tract.
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Fig. 3D Schematic of primary coronary anomalies shows group 2, anomalous
aortic origin and anomalous proximal course. Solid line = left main coronary
artery/left anterior descending coronary artery; dashed line = right coronary
artery; dotted line = left circumflex artery. Types 2a-2e. Type 2a (A)
is illustrated in Figure 12A,
12B, type 2b (B) in
Figure 13A,
13B,
13C, type 2c (C) in
Figure 14A,
14B, type 2D (D) in
Figure 8, and type 2e
(E) in Figure 15A,
15B,
15C. RVOT = right ventricular
outflow tract.
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Fig. 3E Schematic of primary coronary anomalies shows group 2, anomalous
aortic origin and anomalous proximal course. Solid line = left main coronary
artery/left anterior descending coronary artery; dashed line = right coronary
artery; dotted line = left circumflex artery. Types 2a-2e. Type 2a (A)
is illustrated in Figure 12A,
12B, type 2b (B) in
Figure 13A,
13B,
13C, type 2c (C) in
Figure 14A,
14B, type 2D (D) in
Figure 8, and type 2e
(E) in Figure 15A,
15B,
15C. RVOT = right ventricular
outflow tract.
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Fig. 4A Schematic of primary coronary anomalies shows group 3, anomalous
origin from pulmonary artery. RVOT = right ventricular outflow tract. Solid
line = left main coronary artery/left anterior descending coronary artery;
dashed line = right coronary artery; dotted line = left circumflex artery.
Types 3a-3e.
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Fig. 4B Schematic of primary coronary anomalies shows group 3, anomalous
origin from pulmonary artery. RVOT = right ventricular outflow tract. Solid
line = left main coronary artery/left anterior descending coronary artery;
dashed line = right coronary artery; dotted line = left circumflex artery.
Types 3a-3e.
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Fig. 4C Schematic of primary coronary anomalies shows group 3, anomalous
origin from pulmonary artery. RVOT = right ventricular outflow tract. Solid
line = left main coronary artery/left anterior descending coronary artery;
dashed line = right coronary artery; dotted line = left circumflex artery.
Types 3a-3e.
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Fig. 4D Schematic of primary coronary anomalies shows group 3, anomalous
origin from pulmonary artery. RVOT = right ventricular outflow tract. Solid
line = left main coronary artery/left anterior descending coronary artery;
dashed line = right coronary artery; dotted line = left circumflex artery.
Types 3a-3e.
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Fig. 4E Schematic of primary coronary anomalies shows group 3, anomalous
origin from pulmonary artery. RVOT = right ventricular outflow tract. Solid
line = left main coronary artery/left anterior descending coronary artery;
dashed line = right coronary artery; dotted line = left circumflex artery.
Types 3a-3e.
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Anomalous Aortic Origin
The coronary artery ostia may have abnormal origins in the aorta and then
course in a normal direction. The most common is a high origin of the RCA
above the sinotubular junction
[7] (Fig.
5A,
5B,
5C). This anomaly usually
occurs a few millimeters above the sinotubular junction, but distances of 2.0
cm have been reported [8]. It
is important for cardiac surgeons to be aware of this anomaly, because during
cardiac bypass surgery when the aorta is cross-clamped, high cannulation is
needed to avoid accidental crossclamping or transection of the RCA.

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Fig. 5A 49-year-old man with intermittent atypical chest pain and strong
family history of heart disease. Five-millimeter oblique maximum intensity
reconstructions show anomalous right coronary artery with high origin
(straight arrow, A) above sinotubular junction
(straight arrow, B) relative to origin of left main
coronary artery (curved arrows).
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Fig. 5B 49-year-old man with intermittent atypical chest pain and strong
family history of heart disease. Five-millimeter oblique maximum intensity
reconstructions show anomalous right coronary artery with high origin
(straight arrow, A) above sinotubular junction
(straight arrow, B) relative to origin of left main
coronary artery (curved arrows).
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Fig. 5C 49-year-old man with intermittent atypical chest pain and strong
family history of heart disease. Three-dimensional volume-rendered reformation
shows high origin of right coronary artery (straight solid
arrow) above sinotubular junction (open arrow) and
clockwise rotation of right sinus of Valsalva. Myocardial bridge (curved
arrow) of left anterior descending coronary artery is evident.
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The LAD coronary artery can arise from the right coronary sinus or RCA and
take a course anterior to the right ventricular outflow tract (RVOT) or it may
take an anomalous proximal course (see next section and Fig.
6A,
6B,
6C). The former anomaly is not
associated with myocardial ischemia. Anomalous origin of the LAD coronary
artery should be suspected when injection of contrast material into a coronary
vessel originating in the right sinus of Valsalva reveals a long, nonbranching
proximal segment that courses anteriorly in the intraventricular groove.
Cardiac surgeons need knowledge of such anatomic features to avoid
intraoperative complications during valve surgery.

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Fig. 6A 77-year-old woman after myocardial infarction. Five-millimeter axial
maximum intensity reconstruction shows anomalous origin of left anterior
descending coronary artery (arrow) originating from right coronary
artery, which passes between aorta and right ventricular outflow tract to
interventricular groove.
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Fig. 6B 77-year-old woman after myocardial infarction. Five-millimeter axial
maximum intensity reconstruction shows circumflex artery originating from
right coronary artery (white arrow) and passing posteriorly behind
aorta to left atrioventricular groove. Stent (black arrow) is evident
in proximal portion.
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Fig. 6C 77-year-old woman after myocardial infarction. Coronary angiogram
shows anomalous origin of left anterior descending coronary artery
(straight white arrow) and circumflex artery (black
arrow), both originating from right coronary artery (curved
arrow).
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The circumflex artery can arise from the right sinus of Valsalva or the
RCA. In the Coronary Artery Surgery Study
[9], which included coronary
angiograms of 24,959 patients, the most common anomaly (60%) involved the
circumflex artery. In 69% of these cases, the circumflex artery arose from a
separate ostium in the right coronary sinus of Valsalva, and in 31% of cases
it originated as a branch of the RCA. The circumflex artery always takes a
posterior course. It may be an isolated anomaly with the LAD coronary artery
originating normally from the LMCA (Fig.
7A,
7B), or may be associated with
other branch anomalies, such as origin of the LAD coronary artery from the
anomalous circumflex artery (Fig.
8).

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Fig. 7A 65-year-old man with atypical chest pain. Eight-millimeter
maximum-intensity-projection reconstruction shows anomalous circumflex artery
(white arrow) originating from right sinus of Valsalva and passing
posteriorly between aorta and left atrium (black arrow) to reach left
atrioventricular groove (open arrow). In this case, left
anterior descending coronary artery originated from left sinus of
Valsalva.
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Fig. 7B 65-year-old man with atypical chest pain. Coronary angiogram shows
anomalous circumflex artery traveling backward from right sinus of Valsalva
(black arrow). Early filling of right coronary artery (white
arrow) is evident.
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Fig. 8 39-year-old man with hyperlipidemia after myocardial infarction.
Five-millimeter maximum intensity reconstruction shows anomalous origin of
circumflex artery (black arrow) from right sinus of Valsalva, which
passes posterior to aorta (white arrow). Left anterior descending
coronary artery (open arrow) originates from anomalous
circumflex artery.
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In rare instances, all three coronary arteries arise from the right
coronary sinus (Fig. 9A,
9B). Only a small number of
case reports [10] have
described this anomaly. The arteries have a normal course once they have
reached their respective atrioventricular and interventricular grooves.

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Fig. 9A 82-year-old woman with shortness of breath and elevated cardiac
enzyme levels. Five-millimeter axial maximum intensity reconstruction shows
entire coronary system originating from right sinus of Valsalva. Left anterior
descending coronary artery (white arrow) passes anterior to right
ventricular outflow tract. Circumflex artery (black arrow) passes
posteriorly between left atrium and aortic root to resume its normal position
in left atrioventricular groove. Right coronary artery (open
arrow) has normal configuration.
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Fig. 9B 82-year-old woman with shortness of breath and elevated cardiac
enzyme levels. Angiogram from coronary catheterization shows entirely
right-sided coronary arterial system with left anterior descending coronary
artery (black arrow) and circumflex artery (white arrow)
arising from right coronary sinus (open arrow).
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Anomalous Aortic Origin with Anomalous Proximal Course
The RCA can originate from the left side of the aorta above the commissure
between the right and left coronary sinuses, from the left sinus of Valsalva,
or from the LMCA. The origin can be anterior to or immediately adjacent to the
left coronary sinus and may dictate the degree of RCA opposition between the
aorta and the RVOT (Figs. 10A,
10B and
11A,
11B). Less often the RCA
arises entirely from a single left coronary sinus (Fig.
12A,
12B). The anomalous RCA can
take a course anterior or posterior to the great vessels. This coronary
anomaly is also the most common to course between the great vessels
[9].

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Fig. 10A 40-year-old man with congenital aortic stenosis and atrial
fibrillation. Five-millimeter maximum intensity reconstruction shows anterior
origin of right coronary artery (arrow) with no compression between
aorta and right ventricular outflow tract.
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Fig. 10B 40-year-old man with congenital aortic stenosis and atrial
fibrillation. Coronary angiogram shows bend (arrow) in proximal right
coronary artery characteristic of this anomaly but no evidence of
stenosis.
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Fig. 11A 30-year-old woman with chest pain. Five-millimeter maximum intensity
reconstruction illustrates right coronary artery originating from left sinus
of Valsalva. Compression (arrow) is evident where artery passes
between aortic root and right ventricular outflow tract.
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Fig. 12A 52-year-old man with atypical chest pain. Five-millimeter axial
maximum intensity reconstruction shows right coronary artery (white
arrow) originating entirely from left sinus of Valsalva. Stent (black
arrow) is evident in distal left anterior descending coronary artery.
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Fig. 12B 52-year-old man with atypical chest pain. Three-dimensional
volume-rendered reformation shows origin of right coronary artery (white
arrow) from left sinus of Valsalva. Anomaly and stent position (black
arrow) are more easily appreciated than in A.
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The LMCA can arise from the right coronary sinus and take a course between
the aorta and the RVOT (Fig.
13A,
13B,
13C); anterior to the RVOT,
which can result in long (5-6 cm) arteries (Fig.
14A,
14B); to the right and
posterior to the RCA and the aortic root; and in an intramyocardial direction
through the crista supraventricularis (Fig.
15A,
15B,
15C). It has been suggested
that the last anomaly may be more easily visualized with MDCT than with
angiography [3].

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Fig. 13A 54-year-old man with positive stress test result. Five-millimeter
axial maximum intensity reconstruction shows left main coronary artery
originating entirely from right sinus of Valsalva and compression
(arrow) of left main coronary artery as it passes between aorta and
right ventricular outflow tract.
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Fig. 13B 54-year-old man with positive stress test result. Five-millimeter
axial maximum intensity reconstruction shows left anterior descending coronary
artery (black arrow) and circumflex artery (white arrow)
originating from left main coronary artery in normal configuration.
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Fig. 13C 54-year-old man with positive stress test result. Coronary angiogram
shows abnormal course of left main coronary artery and compression
(arrow) of portion between aorta and right ventricular outflow
tract.
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Fig. 14A 63-year-old woman with chest pain and hyperlipidemia.
Five-millimeter maximum intensity reconstruction shows anomalous origin of
left main coronary artery from right sinus of Valsalva and passage anterior to
right ventricular outflow tract (arrow).
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Fig. 14B 63-year-old woman with chest pain and hyperlipidemia.
Three-dimensional volume-rendered reformation shows left main coronary artery
continues around right ventricular outflow tract (straight solid
arrow) and branches into left anterior descending coronary artery
(open arrow) and circumflex artery (curved
arrow).
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Fig. 15A 48-year-old woman with atypical chest pain and family history of
coronary artery disease. Five-millimeter axial maximum intensity
reconstruction shows left anterior descending coronary artery (arrow)
originating from right sinus of Valsalva and passing between aorta and right
ventricular outflow tract.
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Fig. 15B 48-year-old woman with atypical chest pain and family history of
coronary artery disease. Five-millimeter axial maximum intensity
reconstruction shows anomalous left anterior descending coronary artery passes
intramyocardially (arrow) before reentering epicardial fat in
interventricular groove.
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Fig. 15C 48-year-old woman with atypical chest pain and family history of
coronary artery disease. Five-millimeter axial maximum intensity
reconstruction shows circumflex artery (arrow) originating from left
sinus of Valsalva.
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Many congenital coronary anomalies are benign, but a small number cause
symptoms ranging from chest pain and dyspnea to cardiorespiratory arrest and
sudden death [1,
11]. The most common
abnormalities include anomalous origin of the left coronary artery from the
pulmonary artery (Bland-Garland-White syndrome), coronary artery fistulas, and
coronary arteries coursing between the great vessels
[12]. The presence of an
anomalous course between the great vessels is more relevant than is ectopic
origin of the coronary artery
[13]. The LMCA can arise from
the RCA or right coronary sinus of Valsalva and pass between the aorta and
RVOT, where it can become compressed, a condition that results in myocardial
ischemia [14]. This anomaly
appears to have a closer association with sudden death than does origin of the
RCA from the left sinus of Valsalva or LMCA passing between the aorta and the
RVOT [15]. The LAD coronary
artery can arise from the RCA or right coronary sinus of Valsalva and course
between the great vessels, where it can be compressed
[16].
Whether ischemia results from coronary artery anomalies coursing between
the great vessels depends on several factors. These anomalies often originate
at an acute angle from a slitlike hypoplastic ostium, which can become
compressed between the great vessels during exercise
[17]. It is hypothesized that
during exercise, expansion of the aortic and pulmonary roots increases
angulation of the slitlike ostium with subsequent luminal compromise of the
anomalous coronary artery. In addition, whether the aberrant artery exhibits
dominance strongly influences the clinical presentation.
Finally, there is a close association with strenuous exercise, sudden death
classically occurring during or immediately after exercise
[15,
17]. Any young patient with
angina pectoris, myocardial infarction, or cardiac syncope needs noninvasive
imaging to rule out such anomalies.
Conclusion
Knowledge of the appearance of anomalies of congenital coronary origin in
adults is important. Most of these anomalies are benign, but a small number,
principally those with a course between the great vessels, are associated with
myocardial ischemia and even sudden death. Increased use of MDCT in cardiac
imaging highlights the value of recognizing such anomalies on cross-sectional,
multiplanar, and volume-rendered reconstructions.
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