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International Journal of Cardiology 74 (2000) 39–46
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Primary congenital anomalies of the coronary arteries: a coronary
arteriographic study
Naveen Garg DM, DNB, Satendra Tewari MD, DM, Aditya Kapoor MD, DM,
Deepak Kumar Gupta MD, Nakul Sinha MD, DM*
Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow, India
Received 8 June 1999; received in revised form 1 February 2000; accepted 11 February 2000
Abstract
Geographic variations in the incidence of different congenital coronary anomalies are well known, but infrequently studied in the Indian
population. Among 4100 adult patients who underwent diagnostic coronary arteriography, 39 (0.95%) patients (34 males, 5 females) had
one or more anomalous coronary arteries. Their mean age was 46.468.2 years (range, 26–68 years). Thirty-five (89.74%) had anomalies
of origin and distribution, while the remaining four (10.25%) had coronary artery fistulae. Right coronary artery was the commonest
anomalous vessel, involved in 19 (48.74%) patients. It was originating from the left sinus of Valsalva in 15 and from the non-facing aortic
sinus in four patients. Anomalous left circumflex artery was the second commonest anomaly, seen in 14 (35.89%) patients. Anomalous
left anterior descending artery and anomalous left coronary artery from pulmonary artery were seen in one patient each. Among patients
with coronary fistulae, two had fistulae between the left anterior descending artery and the main pulmonary artery, one between the conal
artery and the right atrium, while the fourth patient had fistulae from the right coronary as well as from the left anterior descending artery
to the left atrium. Atherosclerotic plaques in the anomalous arteries were seen in only 13 (33.33%) patients, much less than the overall
incidence of coronary artery disease in patients with congenital coronary anomalies in this series (66.66%). In four (10.25%) patients,
only the anomalous vessels were involved in coronary artery disease. Thus, in a small subgroup there does not appear to be an increased
risk for development of atherosclerotic coronary artery disease in anomalous coronary arteries. Recognition of coronary anomalies is
important in patients undergoing coronary arteriography, coronary interventions and cardiac surgery. Variations in the frequency of
primary congenital coronary anomalies may possibly have a genetic background.  2000 Elsevier Science Ireland Ltd. All rights
reserved.
Keywords: Coronary anomalies; Congenital; Coronary arteriography; Coronary artery disease
1. Introduction
Primary congenital anomalies of the coronary
arteries occur as isolated anomalies and are not
necessarily associated with other types of congenital
heart diseases [1]. These could be haemodynamically
*Corresponding author. Tel.: 91-552-440-700 / 900, ext. 2223; fax:
191-522-440-973 / 440-017.
E-mail address: [email protected] (N. Sinha).
significant by leading to abnormal myocardial perfusion, while haemodynamically insignificant
anomalies are principally those of abnormal aortic
origin or distribution of coronary arteries, and
myocardial perfusion is usually not altered in these
individuals.
Widespread application of coronary arteriography
has resulted in more frequent detection of patients
with anomalous coronary arteries, and their clinical
significance is becoming better appreciated [2–9].
0167-5273 / 00 / $ – see front matter  2000 Elsevier Science Ireland Ltd. All rights reserved.
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N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
40
Unrecognised coronary anomalies may lead to errors
in clinical diagnosis and surgical problems [10–12].
When planning coronary angioplasty on anomalous
coronary arteries, there is an even greater need to
accurately define the origin and course of these
vessels [13,14]. Certain anomalous coronary arteries
are associated with sudden cardiac death, myocardial
infarction and anginal syndrome [2,5,6,15,16].
Primary congenital coronary anomalies are detected in about 0.6–1.3% of adult patients undergoing
coronary arteriography [2,4,6–9]. Geographic variations in the frequency of different coronary anomalies
are well known [7,17–21]. There is paucity of Indian
data on this aspect. In this report we present our
experience with primary coronary anomalies detected
during routine coronary arteriography.
diagnostic coronary arteriography during the study
period. Thirty-nine patients had primary congenital
coronary anomalies. The indication for arteriography
was evaluation of coronary artery disease in all the
patients.
3.2. Incidence
The incidence of primary coronary anomalies was
0.95% (39 out of 4100 patient) in our angiographic
population. Thirty-five (89.74%) patients had
anomalies of origin and distribution, while four
(10.25%) had coronary artery fistulae (Table 1).
There were 34 (87.17%) males and only five
(12.85%) females. The mean age was 46.468.2 years
(age range, 26–68 years).
3.3. Classification
2. Material and methods
We reviewed the database of our cardiac catheterisation laboratory. All patients who underwent coronary arteriography from January 1990 to December
1998 were included. Informed consent was obtained
from each patient. The catheterisation reports were
analysed, and those with anomalous coronary arteries
were selected for further assessment. Angiograms
were reviewed by at least two experienced independent observers, who reached a consensus on the
origin and course of the anomalous coronary arteries.
Patients with separate origin of conus artery from
right sinus of Valsalva, separate ostia for left anterior
descending artery and left circumflex artery in left
sinus of Valsalva and high ‘take-off’ of coronary
arteries were excluded. Patients with coronary
anomalies occurring as part of congenital heart
disease were also not included in this study.
Patients were categorised as having single, double
or triple vessel disease when a significant lesion
(defined as more than 50% narrowing of intraluminal
diameter) was present in one or more coronary
arteries or in a major branch [7].
3. Results
3.1. Patient population
The study included 4100 patients who underwent
Table 2 depicts incidence of different coronary
anomalies in our angiographic population.
3.3.1. Anomalous right coronary artery
Anomalous right coronary artery was the commonest coronary anomaly and was present in 19 (48.71%)
patients with angiographic incidence of 0.46%. In 15
patients, this vessel originated from left sinus of
Valsalva and coursed between the aorta and the
pulmonary artery (Fig. 1). In four patients it originated from the non-facing aortic sinus and coursed
retroaortically (Fig. 2). Final distribution was normal
in all cases. Origin and distribution of the left
coronary artery was normal in all these cases.
3.3.2. Anomalous left circumflex coronary artery
The second commonest anomaly was an anomalous left circumflex coronary artery, and was present
in 14 (35.89%) patients with angiographic incidence
Table 1
Incidence of congenital coronary anomalies (n54100)
Total coronary arteriograms
Total coronary anomalies
Anomalies of coronary origin
and distribution
Coronary artery fistulae
Number of
patients
Angiographic
incidence (%)
Anomaly
incidence
(%)
4100
39
35
0.95
0.85
89.74
4
0.09
10.25
N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
41
Table 2
Incidence of different congenital coronary anomalies in angiographic population (n54100)a
Serial Number
Coronary anomaly
Number of
patients
(n539)
Angiographic
incidence (%)
Anomaly
incidence
(%)
(A)
Anomalies of origin
and distribution
Anomalous origin of
RCA from LCS / NAS
Anomalous origin of
LCX from RCS / RCA
Anomalous LAD from
RCS
ALCAPA
35
0.85
89.74
19
0.46
48.74
14
0.34
35.89
1
0.02
2.56
1
0.02
2.56
Coronary artery fistulae
4
0.09
10.25
1.
2.
3.
4.
(B)
a
RCA, right coronary artery; LCS, left coronary sinus; NAS, non-facing aortic sinus; LCX, left circumflex artery; RCS, right coronary sinus; LAD, left
anterior descending artery; ALCAPA, anomalous left coronary artery from pulmonary artery.
of 0.34%. It originated from the right sinus in 12
patients (Fig. 3) and from the right coronary artery in
two patients. Its initial course was retroaortic in all
cases. Peripheral distribution of the left circumflex
artery was normal in all of them. The left anterior
descending coronary artery in all of them originated
from a separate ostium in the left sinus and had a
normal distribution.
3.3.3. Anomalous left anterior descending coronary
artery
Anomalous left anterior descending coronary artery
Fig. 1. Left anterior oblique view showing anomalous right coronary artery originating from the left coronary sinus near the origin of the left coronary
artery. Note the position of the Amplatz catheter tip between the ostia of the right and left coronary artery.
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N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
Fig. 2. Left anterior oblique view showing anomalous right coronary artery originating from non-facing aortic sinus. Note the abnormal hooking position
of the Amplatz catheter.
was present in one (2.56%) patient with angiographic
incidence of 0.02%. This anomalous left anterior
descending artery was originating from the right sinus
and coursing anterior to the right ventricular outflow
tract with normal peripheral distribution. The left
circumflex artery was originating from the left sinus
through separate ostium with normal peripheral distribution.
3.3.4. Anomalous left coronary artery from
pulmonary artery
Anomalous left coronary artery from pulmonary
artery (ALCAPA) was present in one (2.56%) patient
with angiographic incidence of 0.02%. During coronary arteriography, the left coronary artery could not
be hooked while the right coronary artery was grossly
dilated. The left coronary artery filled retrogradely
through the right coronary artery and was draining
into the main pulmonary artery. Coronaries were free
of atherosclerotic coronary artery disease. On oximetry, there was no significant step-up.
3.3.5. Coronary artery fistulae
Coronary artery fistulae were present in four
(10.25%) patients with angiographic incidence of
0.09%. In two patients fistulae were present between
the proximal left anterior descending artery and the
main pulmonary artery (Fig. 4). In one patient there
was a fistulous connection between the conal artery
and the right atrium. This conal artery was originating separately from the right sinus and was connected to the right atrium through an atrial branch.
The fourth patient had multiple small fistulae from
the proximal left anterior descending artery as well as
from the proximal right coronary artery to the left
atrium. There was no significant step-up on oximetry
in any case.
3.4. Coronary artery disease in anomalous
coronaries
Table 3 depicts incidence of atherosclerotic coronary artery disease in patients with coronary
anomalies. The overall incidence was 66.66% (26 of
N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
43
Fig. 3. Left anterior oblique view showing an anomalous left circumflex artery originating from the right coronary sinus traversing in a retroaortic course.
Note the severe coronary stenosis in its proximal part.
39 patients). The anomalous coronary arteries were
involved in coronary artery disease in 33.33% (13 of
39 patients). Coronary artery disease was restricted to
anomalous vessel in only four (10.25%) patients —
two of the anomalous left circumflex and one each of
the anomalous right coronary artery and the anomalous left anterior descending coronary artery.
Out of 19 cases of anomalous right coronary
artery, six (31.57%) had significant coronary artery
disease in the anomalous vessel, while this was
28.57% (four of 14 patients) for patients with
anomalous left circumflex artery. Proximal segment
was involved in three (15.78%) patients with anomalous right coronary artery and three (21.42%) with
anomalous left circumflex artery. The solitary patient
with anomalous left anterior descending artery had
coronary artery disease restricted to the anomalous
vessel only. There was only one patient with anomalous left coronary artery from the pulmonary artery
(ALCAPA), and he was free from coronary artery
disease. Out of four patients with coronary artery
fistulae, three had obstructive coronary artery disease
and out of these three patients two had coronary
artery disease in the vessels involved in fistulous
connections (both left anterior descending artery to
main pulmonary artery), while in none of them
coronary artery disease was restricted to the left
anterior descending coronary artery.
4. Discussion
We found an overall incidence of 0.95% among
patients undergoing diagnostic coronary arteriography; this is in agreement with 0.6–1.3% incidence
as reported in different series [2,4,6–9]. More men
than women had anomalous coronaries, possibly
because more angiograms were done in male patients.
However, it is to be noted that we have not included
patients with congenital heart disease and patients
with common innocuous variations in the coronary
arterial pattern (separate conal artery, separate ostia
for left anterior descending and left circumflex artery
and high ‘take-off’ of coronary arteries). These
N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
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Fig. 4. Left coronary angiogram in right anterior oblique view showing coronary artery fistulae in between proximal left anterior descending artery and
main pulmonary artery. Note severe coronary stenosis in mid left anterior descending artery.
variations have been included in a few studies [8,9],
while excluded in others [6,7,22].
The commonest anomaly in our series was an
anomalous right coronary artery. Only Topaz et al.
[7] had similar findings, but they had also included
patients with congenital heart diseases, and their
study group was predominantly of Hispanic origin.
All other reports [6,22,23] have placed the anomalous
Table 3
Incidence of atherosclerotic coronary artery disease in patients with congenital coronary artery anomalies (n539)a
Serial Number
Congenital coronary
artery anomaly
Patients with
CAD
1.
Anomalous RCA
(n519)
Anomalous LCX
(n514)
Anomalous LAD
(n51)
ALCAPA (n51)
Coronary artery
fistulae (n54)
14 (73.68%)
6 (31.57%)
1 (5.2%)
8 (57.14%)
4 (28.57%)
2 (14.28%)
1 (100%)
1 (100%)
1 (100%)
2 (50%)
–
–
2.
3.
4.
5.
Total (n539)
a
–
Anomalous
vessel with
CAD
–
3 (75%)
26 (66.66%)
13 (33.33%)
Only
anomalous
vessel with
CAD
4 (10.25%)
CAD, coronary artery disease; RCA, right coronary artery; LCX, left circumflex artery; LAD, left anterior descending artery; ALCAPA, anomalous left
coronary artery from pulmonary artery.
N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
left circumflex as the commonest anomaly. The high
incidence of the anomalous right coronary artery in
our study is a cause of some surprise. This discrepancy may reflect the inevitable nature and bias of
a retrospective study. Other possible reasons may be
a smaller number of patients [3,23], a selective
collection of cases from diverse sources [24] or the
artery originating from the non-facing aortic sinus
misdiagnosed as a total occlusion at the ostium [25].
Another possible explanation is genetic and geographic variations in the coronary arterial pattern
which are well known [17–21]. Our study is the
largest from this part of the world and the study
population may be genetically different from that in
other series.
The second commonest anomaly, in concurrence
with Topaz et al. [7], was an anomalous left circumflex artery. Its course was retroaortic, as reported in
all other previous series [6–9,22,23]. The third
commonest anomaly in our series was coronary artery
fistulae (10.25%), quite similar to the Cleveland
clinic series [8], which is the largest study of
anomalous coronaries to date. We encountered only
one patient with an anomalous left coronary artery
from the pulmonary artery, a potentially serious
coronary artery anomaly. Since 90% of these patients
die in infancy of myocardial infarction, congestive
heart failure or sudden cardiac death [26], it is not
surprising that in our predominantly adult population
we found only one such patient. Anomalous left
anterior descending artery is one of the rarest
anomalies, and we found it in only one patient. The
functional significance of this anomaly is unknown. It
was reported to occur more commonly in association
with tetralogy of Fallot [2]. There was not a single
patient with this anomaly in the Hungarian series of
primary congenital anomalies [9]. However Topaz et
al. reported an 11% incidence of this anomaly in their
series, but associated congenital heart diseases were
present in 44% of patients [7].
Involvement of anomalous coronaries in atherosclerotic disease is at best controversial. Previous
studies have reported a greater degree of atherosclerotic involvement of anomalous left circumflex
artery, especially in its proximal segment, and it is
suggested to be due to its retroaortic course [27]. But
in our study, only 28% of these patients had evidence
of atherosclerosis (significant stenosis of proximal
45
vessel in 21% patients only), thus corroborating
Topaz et al. [7] and Page et al. [3]. Our study is in
agreement with others [7,27], in that there is no
predisposition for atherosclerotic involvement of the
proximal segment of the anomalous right coronary
artery.
From a clinical standpoint, there are reports of
chest pain, myocardial infarction and sudden cardiac
death related to coronary anomalies [2,5,6,15,16].
The origin of the left coronary artery from the
contralateral sinus or artery, with subsequent passage
between the aorta and right ventricular outflow tract
has been clearly shown to be a dangerous lesion
[5,28]. The magnitude of the ischemic risk depends
on the angulation of the coronary artery after its
origin from the aorta, with acute angulation often
associated with a slit-like ostium [28,29]. With increased cardiac output (i.e., exercise), the aorta
dilates and, upon aortic wall stretching, this slit-like
ostium further becomes severely narrowed leading to
myocardial ischemia. Acute myocardial infarction as
well as sudden death have been reported, even when
the course of the artery was anterior to the pulmonary
trunk or posterior to the aorta [30,31]. The origin of
the right coronary artery from the contralateral sinus
or artery with passage between the aorta and right
ventricular outflow tract is somewhat less dangerous
[32], though presumably through the same mechanism. Other anomalies which can lead to these events
include single coronary artery, anomalous coronary
origin from pulmonary artery, coronary artery fistula
and atresia of coronary ostium [1,16,26].
Angiographic recognition of coronary anomalies
prior to cardiac surgery is of great importance.
Surgical problems may follow if an anomalous vessel
is excluded from perfusion during cardiopulmonary
bypass or if the surgeon unwittingly incises the
anomalous vessel [10]. Failure to recognise them can
also lead to inadequate or prolonged procedures [33].
During valve replacement surgery, the ostium of the
anomalous vessel can be inadvertently obstructed or
the anomalous vessel may be compressed along its
course by a valvular prosthesis [11,12].
Accurate identification of origin and course of
anomalous coronaries is mandatory before planning
coronary interventions also [13,14], so that an appropriate guiding catheter, wire advancement and balloon systems may be selected.
46
N. Garg et al. / International Journal of Cardiology 74 (2000) 39 – 46
5. Conclusions
In summary, the incidence of isolated congenital
coronary anomalies in our population is mostly the
same as in others but, contrary to them, an anomalous
right coronary artery is the commonest anomaly in
our series, as in Hispanics. They do not appear to be
associated with an increased risk for development of
coronary atherosclerosis. Angiographic recognition of
these vessels is important because of their clinical
significance and applied importance in patients undergoing coronary angioplasty or cardiac surgery.
References
[1] Levine DC, Fellow KE, Abrams HL. Haemodynamically significant
primary anomalies of the coronary arteries: angiographic aspects.
Circulation 1978;58:25–34.
[2] Liberthson RR, Dinsmore RE, Bharti S et al. Aberrant coronary
artery origin from the aorta. Circulation 1974;50:774.
[3] Page Jr. HL, Engle HJ, Campbell WB, Thomas Jr. CS. Anomalous
origin of left circumflex coronary artery: recognition, angiographic
demonstration and clinical significance. Circulation 1974;50:768.
[4] Engle HJ, Torres C, Page HL. Major variations in anatomic origin of
the coronary arteries. Cathet Cardiovasc Diagn 1975;1:157.
[5] Chietlin MD, De Castro CM, Mc Allister HA. Sudden death as a
complication of anomalous left coronary origin from the anterior
sinus of Valsalva. Circulation 1974;50:780.
[6] Chitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical
angiographic,and haemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 1976;53:122.
[7] Topaz O, De Marchena EJ, Perin E, Sommer LS, Mallon SM,
Chahine RA. Anomalous coronary arteries: angiographic findings in
80 patients. Int J Cardiol 1992;34:129–38.
[8] Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595
patients undergoing coronary arteriography. Cathet Cardiovasc
Diagn 1990;21:28–40.
[9] Kardos A, Balsai L, Rudas L et al. Epidemiology of congenital
coronary artery anomalies: a coronary arteriographic study on a
Central European population. Cathet Cardiovasc Diagn
1997;42:270–5.
[10] Longenecker CG, Reentsma K, Creech Jr. O. Surgical implications
of single coronary artery: a review and two case reports. Am Heart J
1961;61:382.
[11] Robert WC, Morrow AG. Compression of anomalous left circumflex
coronary arteries by prosthetic valve rings. J Thorac Cardivasc Surg
1969;57:834–7.
[12] Dc Morchena EJ, Russo CD, Wazniak PM, Kessler KM. Compression of an anomalous left circumflex coronary artery by a bioprosthetic valve ring. J Cardiovasc Surg 1990;31:52–4.
[13] Bass TA, Miller AB, Rubin MR, Stowers SA, Perryman RA.
Transluminal angioplasty of anomalous coronary arteries. Am Heart
J 1986;112:610–3.
[14] Topaz O, Di Sciascio G, Goudrean E et al. Coronary angioplasty of
anomalous coronary arteries: notes on technical aspects. Cathet
Cardiovasc Diagn 1990;21:106–11.
[15] Benson PA. Anomalous aortic origin of coronary artery with sudden
death: case report and review. Am Heart J 1970;79:254.
[16] Pachinger OM, Vanden HP, Judkins MP. Single coronary artery — a
cause of angina pectoris. Eur J Cardiol 1974;2:161.
[17] Symmers WSC. Note on accessory coronary arteries. J Anat
1907;41:141.
[18] Chaudhry MS. Some observations on coronary artery pattern and
intercoronary anastomoses in human hearts. Medicus Karachi
1965;30:162–72.
[19] Schlesinger MG, Zoll PM, Wessler S. The conus artery: a third
coronary artery. Am Heart J 1949;38:823–36.
[20] Topaz O, Di Sciascio G, Cowley MJ et al. Absent left main
coronary artery; Angiographic findings in 83 patients with separate
ostia of left anterior descending and circumflex arteries at the left
aortic sinus. Am Heart J 1991;12:447–52.
[21] Kurja HJ, Chaudhry MS, Olsen TR. Coronary artery variations in a
native Iraqi population. Cathet Cardiovasc Diagn 1986;12:386–90.
[22] Kimbris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic
origin coronary arteries. Circulation 1978;58:606–15.
[23] Ogden JA. Congenital anomalies of coronary arteries. Am J Cardiol
1970;25:474–9.
[24] Click RL, Holmes Jr. DR, Vlietstra RE, Kosinski AS, Kronmal RA.
Anomalous coronary arteries: location, degree of atherosclerosis and
effect on survival — a report from the coronary artery surgery study.
J Am Coll Cardiol 1989;13:531–7.
[25] Cohen DJ, Kim D, Baim DS. Origin of left main coronary artery
from the non-coronary sinus of Valsalva. Cathet Cardivasc Diagn
1991;22:190–2.
[26] Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the
left coronary artery from the pulmonary trunk; its clinical spectrum,
pathology, pathophysiology, based on a review of 140 cases with
seven further cases. Circulation 1968;38:403–25.
[27] Wilkins CE, Betancourt B, Mathur VS et al. Coronary artery
anomalies. Texas Heart J 1988;15:166–73.
[28] Mustafa I, Gula G, Radley Smith R, Durrer S, Yacoub M. Anomalous origin of the left coronary artery from the anterior aortic sinus: a
potential cause of sudden death. J Thorac Cardiovasc Surg
1981;82:297–300.
[29] Robert III CW, Robert WC. Left main coronary artery originating
from the right sinus of Valsalva and coursing between aorta and
pulmonary trunk. J Am Coll Cardiol 1986;7:366–8.
[30] Murphy DA, Roy DDL, Sohal M, Chandler BM. Anomalous origin
of left main coronary artery from the anterior sinus of Valsalva with
myocardial infarction. J Thorac Cardiovasc Surg 1978;75:282–5.
[31] Robert WC, Kragel AH. Anomalous origin of either the right or left
main coronary artery from the aorta without coursing of the
anamolistically arising artery between aorta and pulmonary trunk.
Am J Cardiol 1988;62:1263–7.
[32] Robert WC, Siegel RJ, Zipes DP. Origin of the right coronary artery
from the left sinus of Valsalva and its functional consequences:
analysis of 10 necropsy patients. Am J Cardiol 1982;49:863–8.
[33] Donaldson RM, Raphael MJ. Missing coronary artery. Br Heart J
1982;47:62–70.