53
Coronary Artery Anomalies
JOHN F. KEANE AND DONALD C. FYLER
DEFINITION
With increasing use and extraordinary sophistication of
noninvasive imaging techniques such as echocardiography
and magnetic resonance imaging (MRI), the number of
coronary anomalies identified is increasing. The vast
majority, such as minor degrees of origin eccentricity,
separate origins of circumflex, and left anterior descending,
are of no significance. This chapter discusses the signifi-
cant anomalies: (1) origin of a coronary from a pulmonary
artery, and (2) both coronary arteries from a single sinus,
without other structural cardiac defects. Other anomalies,
such as left anterior descending from the right coronary in
tetralogy of Fallot, variations encountered in transposition,
are described in the relevant chapters.
PREVALENCE
Among more than 1400 anomalies diagnosed by
echocardiography between 1988 and 2002, there were 51
with origin of a coronary artery from a pulmonary artery,
7 with a single coronary, and a similar number with both
from the same cusp (Exhibit 53-1). In three other patients,
all with other cardiac defects, only the circumflex branch
arose anomalously from the pulmonary artery.
ANOMALOUS CORONARY FROM A
PULMONARY ARTERY
Of the 51 patients with this anomaly, the left originated
from the pulmonary artery in 50 and the right in the other.
Pathology
The anomalous coronary artery arises from the low-
pressure main pulmonary artery and follows its usual
course over the heart. Because the other high-pressure
coronary artery is the source of perfusion for the
myocardium, it is bigger and takes over the other coronary
circulation through collateral connections, and retrograde
flow to the pulmonary artery occurs in most. Because the
myocardium is generally poorly perfused, the heart may be
dilated, and histologic evidence of inadequate myocardial
perfusion may be found. At postmortem examination,
there may be obvious myocardial infarction.
Physiology
Before birth, the pulmonary artery pressure is at
systemic levels, allowing for satisfactory myocardial perfu-
sion from the pulmonary artery through the anomalous
coronary. With birth and falling pulmonary artery pres-
sure, the antegrade perfusion of the anomalous coro-
nary gradually decreases, the circulation being taken over
by collateral vessels from the other coronary. In many
patients, the flow in the anomalous coronary artery
reverses, and, effectively, a coronary artery steal develops
from the myocardium to the pulmonary artery. The size
of this left-to-right shunt is rarely enough to be a signifi-
cant hemodynamic burden except that it deprives the
myocardium of perfusion. Sometime in the first week of
reversed coronary flow, myocardial ischemia becomes
sufficient to be recognizable on an electrocardiogram. The
heart enlarges, and congestive heart failure becomes mani-
fest. With ischemic damage to the left papillary muscles,
805
806 Congenital Heart Disease

Exhibit 53–1
Children’s Hospital Boston Experience
Coronary Artery Anomalies
1973–1987 1988–2002
LCA from PA 29 50
RCA from PA 0 1
Female 55% 66%
Age at operation
0–1 yr 36% 65%
1–10 yr 38% 23%
10 yr 26% 12%
Ligation 19% 0%
Takeuchi/implant/graft 81% 100%
FIGURE 53–1 Electrocardiogram from a 3-month-old infant
Early deaths 8% 7% with anomalous origin of left coronary artery from pulmonary
Single RCA 3 artery showing evidence of extensive anterolateral infarction
Single LCA 4 (deep Q waves leads 1, aVL, V6; diminished anterior forces V1–V4;
RCA from left coronary sinus 6 and left atrial enlargement V1).
LCA from right coronary sinus 1
LCA from noncoronary sinus 1
LCA, left coronary artery; PA, pulmonary artery; RCA,
right coronary artery. Chest X-Ray
The heart is enlarged, sometimes grossly, without an
increase in pulmonary vascularity.

mitral regurgitation is often added to an already deteriorat- Echocardiography

ing situation.
If, for any reason, the patient maintains systemic pres-
sure in the pulmonary arteries, this sequence of events will
not occur, and the anomalous coronary will be perfused by
blood originating from the pulmonary artery. Despite the
fact that this blood may have substantially lower oxygen
saturation than that of the other coronary, evidence of
myocardial ischemia is not seen.
Some patients with uncorrected anomalous origin from
the main pulmonary artery survive to adulthood.1 The
right coronary artery arose from the pulmonary artery in
1 of our 51 patients; even more rarely, both coronary arter-
ies may arise from the pulmonary artery.2,3
Clinical Manifestations
These patients appear to be normal at birth; approxi-
mately 60% of the patients seen at Children’s Hospital
Boston developed congestive heart failure weeks later.
Occasionally, a history of irritability, perhaps related to
angina, is elicited, but this is more of a retrospective expla-
nation than a useful fact. Older patients are discovered
because of symptoms, a systolic murmur of mitral regurgi-
tation and cardiomegaly on chest x-ray, echocardiography,
tomography, or angiography.1,4
The electrocardiogram almost always shows evidence
of an anterolateral myocardial infarction (Fig. 53-1), even
in the asymptomatic patient.
Any patient with unexplained myocardial disease should
have the origin of the coronary arteries identified. An
enlarged, poorly functioning left ventricle is characteristic,
as is mitral regurgitation. The anomalous origin of this
coronary artery from the pulmonary artery is associated
with an inequality of the coronary artery size.5
The parasternal short-axis view usually identifies the
origins of the coronary arteries from the aorta (Fig. 53-2).
Scanning in a parasternal, short- or long-axis view may
identify clearly the orifice of the coronary artery in the
pulmonary root (Fig. 53-3). Color-flow Doppler mapping
is extremely valuable in demonstrating the direction of
flow in the coronary, particularly into the pulmonary root
when the pulmonary resistance is low.5,6 Clearly, demon-
stration of antegrade flow in the coronary artery and
branches by color-flow Doppler mapping excludes anom-
alous origin of that vessel.
Careful imaging and color-flow Doppler mapping
allow accurate diagnosis in the majority such that surgery
without cardiac catheterization can be undertaken. When
the diagnosis is uncertain, cardiac catheterization with
selective angiography is the usual option in the first few
years of life, whereas MRI in the older child is currently
an effective alternative.
Cardiac Catheterization
Cardiac catheterization is rarely necessary in the infant
because echocardiographic anatomy details are excellent.
 Coronary Artery Anomalies
FIGURE 53–2 Short-axis echocardiographic view at the level of
the aortic root (Ao) demonstrating the normal origin of the right
coronary artery (RCA), the left coronary artery (LCA), and ante-
rior descending (AD) coronary artery.
If undertaken, the diagnosis is best established by injecting
contrast in the ascending aorta or selectively in the
normally originating coronary artery. The latter is seen to
be large and often tortuous and fills through collaterals
the left coronary artery, which in turn is seen to drain into
the pulmonary artery (Fig. 53-4). In addition, findings of a
dilated cardiomyopathy are usual with left ventricular
dysfunction, as evidenced by an elevated left ventricular
end-diastolic pressure and elevated left atrial pressure,
usually demonstrable.
Management
After the diagnosis has been recognized and the infant
stabilized with anticongestive medications, surgery is
undertaken as soon as possible to provide a normal two-
coronary circulation. Years ago, ligation of the anomalous
artery was used to eliminate the left-to-right shunt to
prevent a steal from the myocardium with some success but
with significant early and late mortality. In the past couple
of decades, a number of surgical techniques have been used
to connect the anomalous artery to the aorta with consider-
able success. These include reimplanting, grafting, and espe-
cially the operation proposed by Takeuchi.7 However it is
807
FIGURE 53–3 Parasternal short-axis color Doppler echocardio-
gram of anomalous origin of the left coronary artery from the
pulmonary artery. Flow in the anterior descending (AD) coro-
nary artery is seen as a blue signal, indicating flow away from the
transducer, which in this case is retrograde. Similarly, flow in the
circumflex coronary artery (CIRC) is seen as a red signal, consis-
tent with flow toward the transducer, which is also retrograde.
The red jet entering the main pulmonary artery just above the
level of the pulmonary valve (PV) is the flow signal from the left
main coronary artery into the pulmonary root.
accomplished, the desired effect is perfusion of the coronary
from the aorta. Survival rates have improved significantly,
and most patients become asymptomatic. The electro-
cardiogram generally improves, cardiomegaly on x-ray
decreases, and striking left ventricular function improve-
ment is seen echocardiographically.8–13
Course
The remarkable patient, electrocardiogram, and func-
tional improvement are impressive, suggesting that the
infant myocardium has a capacity for repair after ischemia
and infarction that exceeds that seen in the adult.
Continued observation is necessary because some require
revision of operative connections or mitral regurgitation
relief. At present, the future is very promising.
Among older patients, any evidence of cardiac embarrass-
ment is a reason to connect the coronary artery to the aorta.
808 Congenital Heart Disease
FIGURE 53–4 Cineangiogram, lateral projection, showing
dilated right coronary artery (RCA) arising normally from the
aorta with contrast then passing retrograde into the anterior
descending (AD) and circumflex (CIRC) branches to the main left
coronary artery (LCA), which in turn empties through its anom-
alous origin into the main pulmonary artery (MPA).
SINGLE SINUS CORONARY
ARTERY ORIGIN
Abnormal origin of a coronary artery from another sinus
or the other coronary artery remains an extremely rare
anomaly, with only 15 being detected in our recent 14-year
experience (see Exhibit 53-1). Sudden death, often with
FIGURE 53–5 Parasternal short-axis view of anomalous origin of
the right coronary artery from the left sinus of Valsalva, obtained
by transesophageal echocardiography. The right coronary is seen
to pass between the aorta (Ao) and pulmonary artery (PA), and
flow into the coronary is documented by color-flow mapping.
A B
FIGURE 53–6 Cineangiogram in the left coronary cusp (LCC)
in anteroposterior (A) and long atrial oblique (B) projections,
showing origin of both right (RCA) and left (LCA) coronary
arteries arising from the LCC, with the RCA passing anteriorly
between the aorta and main pulmonary artery.
exercise, has been reported in some with this anomaly but
does seem to occur only in those in whom the ectopic
coronary (right or left) passes high between the aorta and
pulmonary artery (“interarterial type”)14 (Figs. 53-5 and
53-6). Obstruction due to stenosis or intramural course
may also be present and may be visualized by magnetic
resonance imaging or intravascular ultrasound.15 The
passage of the ectopic left coronary artery from the right
sinus through the conal septum (“septal type”) is consid-
ered in some to be a benign anomaly.16 Except for the
latter, surgery is recommended for those with symptoms or
whenever the interarterial type is identified.17–19
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 Coronary Artery Anomalies
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