Surgical Therapy for Anomalous

Aortic Origin of the Coronary Arteries

James Jaggers and Andrew J. Lodge

Anomalous aortic origin of the coronary arteries is a rare defect and is associated with an
increased risk of cardiac symptoms including sudden death. The most important subtypes
are those in which the anomalous coronary artery arises from the wrong sinus as in
anomalous left main coronary artery from the right sinus and anomalous right coronary
artery from the left sinus. Ischemia can result when the flap-like orifice becomes ob-
structed, usually during exercise. Indications for surgical intervention are evolving. Cur-
rently, the favored approach for repair is an unroofing type of procedure that results in
relocation of the coronary artery into the appropriate sinus of Valsalva.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 8:122-127 2005 Elsevier Inc. All
rights reserved.

KEYWORDS: Anomalous aortic origin of a coronary artery (AAOCA), right coronary artery from
the left sinus of Valsalva (ARCA), left main coronary artery arising anomalously from the
right sinus of Valsalva (ALMCA)

A nomalous aortic origin of a coronary artery (AAOCA)

from the incorrect coronary sinus of Valsalva is a rare
congenital cardiac defect that is associated with increased
risk of sudden death and cardiac morbidity.1-3 The most
common anomaly of this type is that in which the circumflex
coronary artery arises from the right sinus or the right coro-
nary artery, with an incidence of 0.37% to 0.6%.4,5 The next
most common and pathologically significant anomalies are
the right coronary artery from the left sinus of Valsalva
(ARCA) and the left main coronary artery arising anoma-
lously from the right sinus of Valsalva (ALMCA). The com-
bined incidence of these defects approximates 0.17% in au-
topsy series and 0.1% to 0.3% in patients undergoing
catheterization or echocardiography.1-3,6 In adults referred
for coronary angiography, the prevalence of all congenital
coronary artery anomalies is 0.6% to 1.3%.7,8 Most of the
anomalies of coronary arteries are relatively benign, but sud-
den death is associated with defects that involve anomalous
origination of the coronary artery from the wrong sinus and
that pass in an intramural course between the aorta pulmo-
nary artery. This risk is most pronounced in young, compet-
itive athletes.1,2 Coronary anomalies are the second leading
From the Department of Pediatric Cardiovascular Surgery, Duke University
Medical Center, Durham, NC.
Address reprint requests to James Jaggers, MD, Pediatric Cardiovascular
Surgery, Duke University Medical Center, Box 3474, Durham, NC
27710.
cause of cardiac death in young athletes. In a postmortem
study, the incidence of sudden death was 54% among 49
cases of ALMCA and 25% among cases of ARCA. Increased
risk of sudden death associated with exercise is well de-
scribed with ALMCA and ARCA.2,3,9 In this article we confine
our discussion to those that involve anomalous aortic origin
of the left main coronary artery from the right sinus (ALMCA)
or anomalous right coronary artery from the left sinus
(ARCA) and that take an intramural course between the aorta
and pulmonary artery. It is in these two lesions that there
seems to be an increased risk of major cardiovascular symp-
toms or sudden death.
Pathology and Anatomy
Roberts10 has described four subtypes of anomalous origin of
the left coronary artery from the right sinus. The anomalous
left coronary artery can arise from the right coronary artery or
right sinus and travel across the infundibulum of the right
ventricle, take a posterior retro-aortic course, an intramuscu-
lar or septal course, and finally an interarterial course. In
patients who had an interarterial, intramural course between
the great vessels, ischemic symptoms were common. In an-
other series, Roberts11 reports on 10 necropsy cases in which
an anomalous origin of the right coronary from the left sinus
was associated with death. In ALMCA or ARCA, the anoma-
lous artery may share a common orifice with the other coro-
nary artery or may originate from a separate origin in the

122 1092-9126/05/$-see front matter 2005 Elsevier Inc. All rights reserved.
doi:10.1053/j.pcsu.2005.01.004
Surgical therapy for AAOCA 123

Figure 1 (A) Autopsy specimen of a 32-year-old woman who died suddenly while jogging. Specimen shows ALMCA
from the right sinus of Valsalva originating at the level and immediately to the right of the intercoronary commissure.
(B) Histologic specimen of a 22-year-old man who died suddenly during a soccer game. This specimen demonstrates
the intramural course of the left main coronary artery as it travels between the aorta (A) and the pulmonary artery (P).

sinus. The anomalous coronary artery takes an intramural
course between the great arteries and leaves the aortic wall
from the appropriate coronary sinus of Valsalva (Fig 1 A,B).
The pathophysiology of compromised coronary blood
flow with AAOCA has many possible mechanisms. The most
likely explanation for insufficient coronary flow is the acute
angle of the coronary orifice. Because the luminal pressure of
the aorta increases (eg, with exercise), the intimal flap of the
acute take-off of the coronary can obstruct the coronary ar-
tery during diastole. In patients in whom the intramural seg-
ment is at the level of the intercoronary commissure, the
increased luminal pressure may result in displacement of the
commissure into the intramural segment. The previously
popular explanation of compression between the great ves-
sels seems to be an unlikely mechanism for ischemia. Occa-
sionally, the anomalous artery originates from the wrong si-
nus and travels between the great vessels but does not have an
intramural course.12 In this situation there does not seem to
be a slit-like orifice, and symptoms may be be related to
compression between the great vessels.
In an autopsy study, Taylor et al13 examined 30 cases of
ALMCA and ARCA. Of these 30 individuals, 12 died after
exertion. The authors found no relationship between the size
or angle of the slit-like orifice or the length of the intramural
segment and the risk of sudden death. However, there did
seem to be a relationship between the age of the patient and
the risk of anomaly related death. Patients dying of causes
other than anomaly-related sudden death were significantly
older (43 17 years v 22 12 years). With the possible
exception of age, risk stratification on the basis of anatomy
and prediction of which patients will benefit most from re-
pair may not be possible based on the current information.
Clinical Presentation
A common presentation is sudden unexplained death; however,
angina, syncope, and arrhythmia can be the presenting symp-
toms. The prevalence of this anomaly is impossible to know
because most patients are asymptomatic. The diagnosis may be
made in patients who are having echocardiograms or catheter-
izations for other reasons. With increased index of suspicion and
awareness of the diagnosis, the incidence of these anomalies
may increase. The diagnosis is being made more frequently in
patients undergoing provocative testing for other cardiovascular
124
Figure 2 Two-dimensional echocardiographic image of unrepaired ALMCA. There is a clear intramural segment with a
common orifice with the right coronary artery and a slit-like opening. ICC, intercoronary commissure; AV, aortic valve;
PA, pulmonary artery.
disease with catheterization or echocardiography.6,8 Autopsy se-
ries have shown that although some patients who die with
anomalous coronary arteries exhibit myocardial ischemia, oth-
ers have no evidence of ischemic injury and may have suffered a
lethal arrhythmia. In an Italian study in which 3,504 elite com-
petitive athletes were screened for cardiovascular disease by
trans-thoracic echocardiography, the prevalence of AAOCA was
0.09% in asymptomatic patients.14
In an autopsy study, Taylor et al13 examined 30 cases of
ALMCA and ARCA. Of these 30 individuals, 12 died after exer-
tion. The authors found no relationship between the size or
angle of the slit-like orifice and the risk of sudden death. Thus,
risk stratification for this defect and prediction of which patients
will benefit most from repair may not be possible. In a series of
27 athletes that had sudden death, 23 had ALMCA, and 4 had
ARCA. Each athlete died during or shortly after intense exercise.
Cardiovascular symptoms were present before death in 10 of 27.
In this series, 9 of 27 had had a previous ECG (all normal), and
6 of 27 patients had an exercise stress test a mean of 10 months
before death (all normal).15 Therefore, screening exercise testing
may not be a reliable method of detection.
Diagnosis
The diagnosis of anomalous aortic origin of the coronary
arteries can be made with several techniques. Often the diag-
J. Jaggers and A.J. Lodge
nosis is made serendipitously at the time of cardiac catheter-
ization for other pathology (eg, coronary artery disease). In
children, the diagnosis is most frequently made with echo-
cardiography (Fig 2).6 More recently, computed tomography
angiograms and cardiac MRI have been used. These tech-
niques can provide clear images but are not superior to echo-
cardiography. Coronary angiography is a useful technique to
diagnose this defect and to determine the presence or absence
of coexisting atherosclerotic coronary artery disease. We tend
to reserve the use of coronary catheterization for patients in
whom the diagnosis is in question or in adult patients with
risk factors for coronary artery disease. If catheterization is
necessary, the best method of determining the origin and
course of the anomalous coronary artery is to simultaneously
inject in the orifice of the coronary artery and the pulmonary
artery.16 This technique accurately identifies the course of the
intramural of the coronary artery.
Surgical Repair
In patients with AAOCA and symptoms referable to coronary
ischemia, there is no debate regarding indication for opera-
tive repair. The management of the asymptomatic patient
with AAOCA remains somewhat controversial. The risk of
sudden death or cardiac ischemia must be weighed against
the risk of the operation and the risk of potential late com-
Surgical therapy for AAOCA 125

plications such as neo-ostial stenosis. In asymptomatic pa-

tients with ALMCA, there seems to be a consensus that sur-
gical intervention is indicated to prevent the risk of sudden
death. Because the risk of sudden death in patients with
ARCA is significantly less than with ALMCA, the decision for
elective surgical intervention is more difficult but in our
opinion is warranted. If surgical repair is declined, avoidance
of strenuous physical activity and competitive athletics
should be prescribed.
In patient with symptoms, repair should not be delayed. In
the asymptomatic patient, timing of surgical intervention is
controversial. It is our recommendation to delay elective sur-
gical repair until late puberty or approximately 10 years of
age in the asymptomatic patient. This recommendation is
based on the fact that sudden death in patients with AAOCA
is rare in children before adolescence. It is also with adoles-
cence that children begin to be involved in competitive
sports, which places them at greater risk. Many surgical strat-
egies have been suggested to treat this defect, including cor-
onary reimplantation, unroofing the intramural segment,
coronary artery bypass grafting, and transvascular intracoro-
nary stenting.
In a series of 14 patients treated with intracoronary stent,
12 had intramural course between the pulmonary artery and
the aorta. All but one had acceptable patency at 6 months
after stent placement. It is unclear whether this therapy, with
its attendant risk of decreased long-term patency and the Figure 3 The modified unroofing procedure as described by Mus-
tafa.21 The intramural segment of the anomalous coronary artery is
potential for ostial stenosis at the origin of the stent, is a
incised from within the lumen of the aorta to the point at which the
durable solution.17 This therapy may be appropriate for an coronary artery leaves the aortic wall in the appropriate sinus. If
older patient with medical problems that would increase the reflection of the commissure is necessary, then it is resecured to the
risk of surgery or if they have co-existent atherosclerotic cor- aortic wall.
onary artery disease that otherwise would be best treated
with angioplasty and stent therapy.
Coronary artery bypass grafting usually with internal Mustafa21 was the first to report an unroofing of the intra-
mammary artery bypass graft to the anomalous coronary ar- mural segment of the anomalous coronary (Fig 3). This pro-
tery eliminates the need to open the aorta and manipulate the cedure has been adopted by many surgeons with good short-
intercoronary commissure but subjects the patient to a by- term results. In this procedure, the patient is placed on
pass graft and the potential need for reintervention. Because cardiopulmonary bypass via median sternotomy. Cardiople-
the flow through the anomalous coronary artery is likely gic arrest is initiated. This is typically accomplished with
minimally obstructed at rest, an internal mammary bypass antegrade aortic root infusion, but consideration could be
graft may have decreased patency secondary to competitive made to retrograde cardioplegia via the coronary sinus in
flow. This has led some authors to recommend ligation of the patients with significant ostial stenosis. Then a transverse
coronary artery proximal to the insertion of the graft.18 This aortotomy or aortic transection is carried out. Care must be
may be an acceptable therapy in an elderly patient with co- taken to make this incision so as to avoid the intramural
existent coronary artery disease, but it essentially condemns coronary if it is at a level above the commissure. The tract of
the patient to grafted coronary artery disease and its associ- the intramural segment is probed to be sure of its course, and
ated risks and seems imprudent in a young patient. then with fine-angled scissors the luminal wall of the coro-
A more anatomic approach to this problem is to return the nary is incised to the point at which the coronary artery leaves
coronary artery orifice to the appropriate sinus where it exits the aortic wall adjacent to the appropriate sinus of Valsalva.
from the aortic wall at right angles. This can be accomplished Small gauge monofilament suture is used to tack down the
by excision and reimplantation19,20 or by using a modified rough disrupted intimal edges at the neo-orifice. If the origin
unroofing technique.21 Reimplantation has been successful, of the anomalous coronary artery is at a level higher or ceph-
but the coronary artery must be large enough move directly, alad to the commissure, there is little risk to unroofing that
or a button of aortic wall must be excised with the coronary segment and tacking the endothelium down with monofila-
artery and reimplanted in the appropriate sinus. This neces- ment suture. In patients in whom the intramural course is at
sitates reconstruction of the aorta where the button was ex- or below the level of the commissural attachments of the
cised. Reimplantation may necessitate the reconstruction of aortic valve, the commissure may require detachment and
the commissural attachments. reflection into the lumen of the aorta so that unroofing can be
126
Figure 4 In patients in whom the intramural path of the anomalous
coronary artery is at or below the level of the commissure, then a
neo-ostia can be created by passing a probe or instrument through
the intramural segment to a point within the appropriate sinus and
unroofing the coronary at the point at which it exits the aorta.
accomplished. The commissure should be secured to the
aortic wall at the appropriate level to prevent prolapse of the
aortic leaflets and aortic insufficiency. An alternative proce-
dure can be performed that avoids manipulation of the com-
missural attachment (Fig 4). A probe or right-angle clamp
can be passed from the anomalous origin through its intra-
mural course to the point at which it leaves the aorta from the
appropriate sinus. The neo-orifice is created. This orifice
should be trimmed and debrided of excessive or ragged inti-
mal tissue, and the residual intramural course can be obliter-
ated to avoid dual source of this coronary.22,23
Outcomes
Few data exist regarding the long-term outcomes of surgi-
cally repaired patients with AAOCA. Patients treated with
CABG require long-term follow-up like any patient with cor-
onary artery disease and a coronary artery bypass graft. Pa-
tients treated with coronary artery reimplantation or unroof-
ing procedure require long-term surveillance to detect
potential ostial stenosis and, in the case of manipulation of
the aortic commissure, surveillance for aortic insufficiency.
In patients who are symptomatic before surgery, monitoring
for persistence or recurrence of symptoms is required. In our
recently reported series of nine patients treated surgically for
J. Jaggers and A.J. Lodge
anomalous origin of the left main coronary artery or the
anomalous right coronary artery, unobstructed patency of
the coronary artery orifices and initial proximal path was
demonstrated by echocardiography in eight patients after re-
pair with modified unroofing procedures. All of these pa-
tients had negative stress test or stress echocardiography at a
mean of 29 months after repair.22 In another series, surgical
repair of AAOCA was carried out in eight patients. A modi-
fied unroofing procedure was used in seven patients. All of
the patients treated in this series had demonstrated patency
of the coronary artery by echocardiography and had negative
exercise stress test at a mean of 1.5 years.24 The results of
these series seem reassuring that no patient had signs of isch-
emia with exercise by symptoms, electrocardiogram, or stress
tests after surgical repair. However, in a series of athletes who
had sudden death, 9 of 27 patients had a normal ECG, and 6
of 27 patients had a negative exercise stress test a mean of 10
months before death.15 There is some limitation to the con-
clusions that can be drawn from the results of these tests. It is
encouraging that this potentially lethal defect can be repaired
with low surgical risk, but the durability of these repairs is yet
to be determined.
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