99

LETTER TO THE EDITOR

Anomalous origin of coronary arteries: When one sinus ts all
Antonios N . Pavlidis , George K. Karavolias , John S. Malakos , Eftihia Sbarouni , Panagiota Georgiadou
& Vasillis V. Voudris
Second Department of Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece
lef anterior descending (LAD) coronary artery, while LM and
lef circumfex (LCX) coronary arteries were free of obstructive
disease (Figure 1A). Right coronary artery (RCA) was ectopic,
arising from the lef sinus of Valsalva (LSOV), and was free of
signifcant atherosclerotic disease (Figure 1B, Supplementary
Movie 1 to be found online at http://www.informahealthcare.
com/abs/doi/10.3109/17482941.2012.683797). Further evalua-
tion of the coronary anatomy with multislice computed tomog-
raphy confrmed the anomalous origin of the RCA which
aroused from the LSOV and coursed interarterially between
the pulmonary trunk and the aortic root into the right atrio-
ventricular groove (Figure 2). Photon emission myocardial
perfusion scan was negative. Patient was treated conservatively
and was discharged the following day.
Patient 2
A 60-year-old man was referred for coronary angiography
and possible revascularization therapy afer sustaining a
non-ST elevation acute coronary syndrome. He was a heavy
smoker and was under treatment for hypertension and
hyperlipidemia. Echocardiography depicted lef ventricular
hypertrophy with preserved lef ventricular ejection fraction.
Coronary angiography was performed via the femoral
approach and multiple attempts with diferent diagnostic
catheters failed to engage the lef coronary ostium. Cannula-
tion of the right coronary system was achieved with an 6F
Amplatz lef 1 (AL1) diagnostic catheter (Cordis) and con-
trast injection demonstrated that the RCA and LM origi-
nated from two separate ostia within the right sinus of
Valsalva (RSOV) (Figure 3A). Afer a long course, the LM
gave rise to the LCX and LAD (Supplementary Movie 2 to be
found online at http://www.informahealthcare.com/abs/doi/
10.3109/17482941.2012.683797). A tight lesion was detected
in the distal part of the RCA, while the other coronary arter-
ies were free of signifcant narrowing.
Te RCA was selectively cannulated with a 6F AL1 guid-
ing catheter (Cordis) with side holes and the stenotic lesion
was successfully crossed with a 0.014 inch BMW wire (Abbott
Vascular) and dilated with a 2.5 10 mm Falcon balloon
Correspondence: Antonios N. Pavlidis, 26 Phoenix Lodge Mansions, London W6 7BG, UK. Fax: 30 210 6205330. E-mail: antonispav@yahoo.com
(Received 13 February 2012; accepted 2 April 2012)
Acute Cardiac Care, September 2012; 14(3): 99102
Copyright 2012 Informa UK, Ltd
ISSN 1748-2941 print/ISSN 1748-295X online
DOI: 10.3109/17482941.2012.683797
A right coronary artery origin from the left coronary sinus and a
left coronary origin from the right sinus although rarely encoun-
tered during routine cardiac catheterization, they represent two
relatively common autopsy fndings in young patients sufering
sudden cardiac death. The interarterial course of the aberrant
artery, between the aortic root and the pulmonary artery has
been considered as a malignant variant, because of the higher
risk of myocardial ischemia and sudden death. We present two
rare cases of ectopic coronary origin from the opposite sinus of
Valsalva.
Keywords: Ectopic coronary , congenital coronary anomalies ,
sudden death
Introduction
Coronary artery anomalies (CAA) are rare congenital abnor-
malities that are usually seen in patients with other coexis-
tent congenital cardiac malformations, such as a bicuspid
aortic valve or transposition of the great vessels. Although
usually asymptomatic, CAA are considered as the second
most common cause of sudden cardiac death in young ath-
letes (1). Te origin of both coronary arteries from a single
sinus of Valsalva is an extremely rare abnormality in which
the interarterial course of the ectopic arteries between
the great vessels has been linked to a high incidence of
cardiovascular events and sudden death.
Cases presentation
Patient 1
A 63-year-old man with atypical chest pains was referred for
coronary angiography following a positive exercise treadmill
test. He had a history of treated hypertension and hyperlipi-
demia. Coronary angiogram was performed via the femoral
approach and the lef main stem (LM) was cannulated with
a 6F Judkins lef 4 (JL4) diagnostic catheter (Cordis). Tere
was a moderate narrowing in the middle segment of the
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100 A. N. Pavlidis et al.
Acute Cardiac Care
(Invatec). Afer predilatation of the narrowed segment, a
3.0 18 mm Nobori stent (Terumo) was deployed at 16
atm. A fnal diagnostic angiogram showed an excellent
angiographic result (Figure 3B). Patient made an uneventful
recovery and he was discharged the following day on dual
antiplatelet therapy.
Discussion
CAA are ofen asymptomatic and are usually encountered as
coincidental fndings during coronary angiography or at
autopsy with an estimated incidence of 0.9% and 0.3%
respectively (1). However, it is speculated that 20 31% of
patients with CAA experience life threatening cardiovascu-
lar complications such as angina, myocardial infarction,
arrhythmias, syncope and sudden death (2). CAA account
for up to one third of sudden cardiac deaths in the young
population (3). Anomalies in the origin and distribution of
the coronary arteries are responsible for 90 95% of CAA,
while coronary fstulae for the rest of the cases (2,4). Separate
ostia of the LCX and LAD within the LSOV in the absence
of LM has been described as the most common anatomic
variant, with an incidence of 0.6% (5). Rigatelli et al.
proposed a classification of CAA based on angiographic
appearance and clinical significance: benign (class I);
relevant-associated with fixed myocardial ischemia (class
II); severe-related to sudden death (class III); and critical-
associated with CAD (class IV) (6). Lipton et al. classified
Figure 1. Coronary angiography in anteroposterior (A) and right anterior oblique (B) projections demonstrating ectopic origin of the RCA arising
from the lef sinus of Valsalva. Tere is a moderate narrowing in the middle segment of the LAD (arrow). RCA, right coronary artery; LAD, lef
anterior descending; LCX, lef circumex.
Figure 2. Reconstructed multislice cardiac computed tomography showing the interarterial course of the RCA between the aorta and the pulmonary
trunk. RCA, right coronary artery; LAD, lef anterior descending; LCX, lef circumex; PA, pulmonary artery; Ao, aorta.
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Anomalous origin of coronary arteries 101
2012 Informa UK, Ltd.
coronary artery variations according to the origin and ana-
tomical course related to the ascending aorta and the pul-
monary artery (7).
Anomalous origin of RCA from the LSOV is a rare
congenital abnormality found in 0.03 0.17% of adult
patients undergoing coronary angiography (1). The course
of the aberrant RCA can be retro-aortic, interarterial
(between the aorta and the pulmonary artery) or anterior
to the pulmonary trunk. The interarterial or malignant
subtype is the most common form and has been linked
to a higher incidence of myocardial infarction, sudden
death and exercise induced angina pectoris (8). Potential
mechanisms of myocardial ischemia in those patients
include compression of the artery between the aorta and
the pulmonary artery, ostial obstruction due to slit-like
coronary orifice, acute take-off angle of the RCA, coro-
nary stretching or angulation with distention of the
ascending aorta or the pulmonary trunk (9). Slit-like ori-
fice structure and acute angle take-off are seen more fre-
quently in patients who suffer sudden cardiac death (9).
Surgical decompression (unroofing procedure), PCI, re-
implantation of the anomalous artery and coronary artery
bypass grafting (CABG) are all acceptable as potential
therapeutic approaches (10).
Te incidence of an anomalous origin of the LM from
the RSOV among patients who undergo angiography has
been estimated between 0.008 and 0.017% (2). Te LM
either arises independently to the ostium of the RCA, or
less frequently the two arteries share a common ostium
(12). Four diferent types of this extremely rare coronary
abnormality have been described based on the course of the
ectopic LM. Type A: Anterior to the right ventricular out-
fow tract, Type B: Between the aorta and the pulmonary
trunk, Type C: Cristal, through the supraventricular crest
and interventricular septum, Type D: Dorsal or posterior to
the aorta (5,12). Te anomalous origin of the LCA from the
RSOV is the most frequent and has been consistently related
to myocardial infarction and sudden cardiac death. Te
incidence is signifcantly higher in patients with interarte-
rial course of the LM (13). Although few cases of sudden
death and myocardial ischemia associated with a posterior
course of the LM have been described, this type of anomaly
is considered mostly benign (14). Anginal symptoms are
usually related to exercise and are caused by compression of
the proximal part of the LM between the expanded aortic
root and the pulmonary trunk. Terapeutic approach must
be individualized according to symptoms, age and the anat-
omy of the aberrant LM. Surgical correction is generally
indicated in young symptomatic patients who are at high
risk of sudden death.
Multidetector computed tomography scan (MDCT), car-
diac magnetic resonance (CMR) and transesophageal
echocardiography (TEE) are commonly used for the diagno-
sis and imaging of the origin and course of CAA. However,
coronary angiography remains the gold standard for the
diagnosis. MDCT can provide various multiplanar image
reconstructions and valuable anatomic information that are
usually dif cult to assess during angiography. Tree dimen-
sion (3D) multiplane TEE is a minimally invasive imaging
modality that can portray directly the proximal and interar-
terial course of the LM (15). Cannulation of the ectopic
coronary arteries during angiography can be extremely chal-
lenging and success depends mostly on physicians experi-
ence. Intravascular ultrasound (IVUS) has also been used in
order to obtain cross-sectional luminal images.
In conclusion, anomalous origins of coronary arteries
from the opposite coronary sinus are extremely rare entities
during angiography. Although most patients are usually
asymptomatic, certain types of these congenital anomalies
have been linked to myocardial ischemia and sudden car-
diac death. Cannulation of ectopic coronaries can fre-
quently be problematic and time consuming; therefore,
other imaging modalities, such as MDCT may have a com-
plementary role.
Figure 3. (A) Coronary angiography in lef anterior oblique projection demonstrating ectopic origin of the LM arising from the right sinus of
Valsalva. Tere is a signicant distal lesion of the RCA (arrow). (B) Selective cannulation of the RCA afer successful treatment of the culprit
lesion (arrow).
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102 A. N. Pavlidis et al.
Acute Cardiac Care
Declaration of interest: Te authors report no conficts of
interest. Te authors alone are responsible for the content
and writing of the paper.
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Supplementary material available online
Movies 1 & 2
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