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Romanian Journal of Morphology and Embryology
2010, 51(3):569–572
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Coexisting of aortic arch variation of the
left common carotid artery arising from
brachiocephalic trunk and absence of
the main branches of right subclavian
artery: a review of the literature
ÖZLEN KARABULUT1), KENAN İLTIMUR2), M. CUDI TUNCER1)
1)
Department of Anatomy
2)
Department of Cardiology
Medical School, “Dicle” University, Diyarbakır, Turkey
Abstract
The variations in the aortic arch and its branches are of surgical interest. Many anomalies of the aortic arch and great vessels are
uncovered as serendipitous findings on imaging studies, in the anatomy laboratory, or at surgery. A 56-year-old woman had an arch
angiogram as part of an evaluation for cardiovascular disease. A two-vessel left aortic arch was identified consisting of the brachiocephalic
trunk and left subclavian artery. In this study, we present a case of the left common carotid (LCC) artery arising from the brachiocephalic
trunk (BT) and absence of the main branches of right subclavian artery in her angiographic imaging findings. There is no previous report
about LCC artery arising from BT and absence of the main branches of right subclavian artery in the same person.
Keywords: aortic arch variations, common origin, carotid arteries, angiography.
Introduction
Many variations occur in the human vascular
pattern, not only in the peripheral circulation, but also in
large vessels including the aorta. Arterial variations of
the aortic arch appear in a large number of possible
combinations with various frequencies.
In about 80% of individuals, three branches arise
from the aortic arch: the brachiocephalic trunk, left
common carotid artery, and left subclavian artery [1].
Adachi first classified this branching pattern as type A.
Another 11% of reported cases have a common trunk
incorporating the left common carotid artery and the
brachiocephalic leaving only two branches originating
from the aortic arch, Adachi’s type B. The third most
common pattern, type C, has the left vertebral artery,
a fourth branch of the aortic arch, originating proximal
to the left subclavian artery [2]. Numerous other
variations of the branching pattern of the aortic arch
are found in less than 1% of cases [1]. In all reported
cases, however, the most distal branch arising from the
arch, or the descending thoracic aorta, was either a
subclavian or thyroid ima artery [3, 4].
The position of the aortic arch may vary across the
population. The possible variation of aortic arch can be
seen in Figure 1. Common origin of the carotid arteries
(COCA) is a normal aortic arch variant found in
approximately 11% of whites [5] with an even higher
incidence, up to 25%, reported in the African-American
population [6]. In COCA, the right innominate and the
left carotid arteries arise from a single origin or trunk of
the aorta (Figure 2). Thus, the right and left common
carotid arteries both arise from a short “innominate”
trunk [7]. Whereas previously it was reported that this
defect had no associated anomalies or adverse consequ-
ences to the patient, more recent studies have indicated
an increased incidence of COCA with certain congenital
anomalies including DiGeorge syndrome, esophageal
atresia-tracheoesophageal fistula [8] and anomalous origin
of the left coronary artery from the pulmonary artery [9].
Figure 1 – Schematic diagram of variations of aortic
arch (1: right brachiocephalic trunk, 2: left brachio-
cephalic trunk, 3: right subclavian artery, 4: left sub-
clavian artery, 5: right common carotid artery, 6: left
common carotid artery, 7: left vertebral artery).
Özlen Karabulut et al.
570
Figure 2 – Schematic dia-
gram o
f
LCA arisin
g
f
rom
brachiocephalic trun
k
(1: right brachiocephalic
trunk, 3: ri
g
ht subclavian
arter
y
, 4: le
f
t subclavian
arter
y
, 5: ri
g
ht common
carotid artery, 6: left com-
mon carotid arter
y
, 7: le
ft
vertebral artery).
In addition, multiple congenital heart defects have
been associated with COCA [5]. An increased incidence
of stridor has been noted in infants with the COCA ano-
maly. Respiratory distress in the neonate with COCA has
been attributed to superior mediastinal crowding [10].
The anomalous right subclavian artery is thought to
develop because of the involution of the fourth aortic
arch, which normally forms the innominate artery, and
persistence of the seventh inter-segmental artery [11].
On the right side, the subclavian artery arises from the
innominate artery behind the right sternoclavicular
articulation; on the left side, it springs from the arch of
the aorta. The two vessels, therefore, in the first part of
their course, differ in length, direction, and relation with
neighboring structures. In order to facilitate the
description, each subclavian artery is divided into three
parts. The first portion extends from the origin of the
vessel to the medial border of the scalenus anterior; the
second lies behind this muscle; and the third extends
from the lateral margin of the muscle to the outer border
of the first rib, where it becomes the axillary artery.
The first portions of the two vessels require separate
descriptions; the second and third parts of the two
arteries are practically alike. Clinically, most important
branch of the subclavian artery is the vertebral artery.
Absence of the vertebral artery is rare, and incidentally
encountered in radiological imaging techniques. Verte-
bral segmental agenesis is seldom reported [12]. A rete
vertebralis is rare in animals [13]. Hyogo T et al. [14]
and Karasawa J et al. [15] reported cases in Asian
individuals. Although, Hachem K et al. [16] and
Woodcock RJ et al. [17] reported a case of bilateral
vertebral arteries agenesis. Kao CL et al. [18] reported
a case of unilateral vertebral artery agenesis. Up to now,
it has been not reported that LCC artery arising from BT
and absence of RVA or other main branches of right
subclavian artery in the same person. In terms of
coexistence of these variations, this case report is so
important knowledge of the embryonic aortic arch
system is required to understand the development of
anomalous branches arising from the arch of the aorta.
Patient, Methods and Results
A 56-year-old woman presented to Cardiac Catheteri-
zation Center at the Cardiology Department. The patient
had history of the chest pain on exertion and dyspnea
occurring over three to four months. The pain was
located on the left side of her chest with radiation to the
left arm. Occasionally, the patient had sweating, stable
angina and syncope. Physical exam, baseline electro-
cardiogram, electrocardiogram Holter and X-ray were
normal. However, treadmill (electrocardiogram) test
was positive. Coronary angiography was subsequently
accomplished utilizing a 6F Judkins catheter to cannu-
late a single, ectopic ostium. There was no evidence of
any coronary stenosis. Here, we present a case of the
left common carotid artery arising from the brachio-
cephalic trunk (usually from the arch of the aorta) and
absence of the main branches of right subclavian artery
(Figure 3). Brachiocephalic trunk coursed towards on
the right side, approximately 4 cm, and than gave off the
left common carotid artery towards on the left side.
At this level, there was explicit bulge in brachiocephalic
trunk before dividing the right common carotid and sub-
clavian arteries. After it coursed approximately 2 cm,
it divided as the right common carotid and subclavian
arteries. In addition, the remarkable finding was the
right subclavian artery gave off no branches. On the
other hand, the left subclavian artery gave off the
vertebral, internal thoracic, thyrocervical, and costo-
cervical arteries (Figure 4).
Figure 3 – LCCA arising from brachiocephalic trunk
and left subclavian artery gave off no branches in
her angiographic imaging (BT: brachiocephalic
artery, LCCA: left common carotid artery, RCCA:
right common carotid artery, RSA: right subclavian
artery, LSA: left subclavian artery).
Figure 4 – Main branches of left subclavian artery
are shown (sa: left subclavian artery, 1: thyrocervical
artery, 2: vertebral artery, 3: costocervical artery,
4: internal thoracic artery).
Coexisting of aortic arch variation of the left common carotid artery arising from brachiocephalic trunk and absence…
571
Discussion
There are several reports of blood vessel malforma-
tions associated with aortic arches [19–27]. Non-recog-
nition of a critical aortic arch branch variation at surgery
may lead fatal consequences [28]. Knowledge of the
embryonic aortic arch system is required to understand
the development of anomalous branches arising from
the arch of the aorta. In this case report, we present a
case of the left common carotid artery arising from the
brachiocephalic trunk and absence of the main branches
of right subclavian artery in her angiographic imaging
findings. The remarkable point is these two anomalies
being together. Although, left common carotid artery
arising from brachiocephalic trunk [19–21, 23, 24] and
absence of the vertebral artery [12, 15, 18] were
reported individually in the literatures, it is the first case
report in terms of LCC artery arising from BT and
absence of the main branches of right subclavian artery
together. Therefore, we discuss these anomalies deve-
lopmental aspects.
The six pairs of aortic arches are a series of vessels
that connect on each side the aortic sac with the
corresponding dorsal aorta. At a later developmental
stage, the aortic arches are both reduced in number and
extensively transformed, and finally an asymmetric
blood supply system is achieved. The first and second
aortic arches largely disappear by the time the third to
sixth arches develop, but the remaining parts of the1st
arch form the maxillary arteries. The common carotid
arteries and proximal portions of the internal carotid
arteries are embryonic derivatives of the third aortic
arches [29, 30], which appear around the end of the
fourth week of gestation; the distal portions of the
internal carotid arteries are formed by the cranial
portions of the dorsal aorta. The external carotid arteries
are derived from portions of the ventral aortic roots
[30]. The left limb of the aortic sac normally forms the
part of the arch that intervenes between the origins of
the brachiocephalic trunk and the LCC artery. If the
aortic sac fails to bifurcate into right and left limbs, then
the LCC artery will connect to the aortic sac directly.
That results in a common origin of the carotid arteries
(COCA). Gürbüz J et al. [31] reported that variations of
the aortic sac and branchial artery system, from an
embryologic standpoint, can be explained by the disap-
pearance of normally persisting vessels or from the per-
sistence of channels that normally disappear; however,
reasons for subtle variations are not understood [32].
According to Anson BI et al., the normal three-
branched arrangement of the aortic arch is found in
64.9%. An arrangement distinguished by reduction in
the number of stems to two, both common carotid arte-
ries arising from the innominate occurs in 27.1% [33].
More rarely, the left common carotid and subclavian
arteries arise from a left brachiocephalic or right
common carotid and subclavian arteries arise separately,
in which case the latter more often branches from the
left end of the arch and passes behind the esophagus.
This anomaly assumes some importance in the adult as
well as in the child, as a cause of esophageal compre-
ssion. The abnormal course of the “recurrent” laryngeal
nerve, which accompanies this anomaly, is also impor-
tant [34]. Bhatnagar KP et al. reported that aortic arch
passed to the right side of the esophagus and trachea and
had four branches, from proximal to distal: left common
carotid, right common carotid, right subclavian, left
subclavian arteries. The ligamentum arteriosum connec-
ted the pulmonary trunk to the left subclavian artery and
lay to the left of the esophagus and trachea. The left
recurrent laryngeal nerve passed under the ligamentum
arteriosum while the right recurrent laryngeal nerve
passed under the aortic arch [35].
Adachi classified the branching pattern of the aortic
arch into seven groups (groups A through G) based on
his experience with the dissection of 516 Japanese
cadavers. The arch vessel anomalies most frequently
encountered were common brachiocephalic trunk
(type B), isolated left vertebral artery (type C), and
aberrant right subclavian artery (type G). According to
his report, the frequency was 10.9% for common
brachiocephalic trunk, 4.3% for isolated left vertebral
artery, and 0.2% for aberrant right subclavian artery [2].
Williams GD and Edmonds HW reported their findings
about arch vessel anomalies after dissecting 407 human
cadavers: 191 American whites and 216 blacks. Accord-
ing to their report, the frequencies of arch vessel anoma-
lies in white and black populations were 17.8% and
37.8% for common brachiocephalic trunk, 2.6% and
2.0% for isolated left vertebral artery, and 1.0% and 0%
for aberrant right subclavian artery, respectively [6].
Our case report was classified as type B arch.
The incidence of congenital atresia or hypoplasia of
the left vertebral artery is 3.1%, and of the right verte-
bral artery (RVA) it is 1.8% [36]. Woodcock RJ et al.
reported a case report of bilateral proatlantal arteries,
both vertebral arteries were absent [17]. Similarly,
Hachem K et al. presented a case report of the absence
of cervical segments of both vertebral arteries. MR-
angiography performed on a 3T machine confirmed the
bilateral absence of cervical segments and the presence
of normal intracranial segments arising from the occipi-
tal arteries, branches of the external carotid arteries [16].
On the other hand, Kao CL et al. described the case of a
40-year-old woman who presented with a large
aneurysm of the left vertebral artery in the angiographic
absence of a right vertebral artery [18]. Above we
mentioned case reports; there were no any congenital
anomalies of aortic arches except for our case report.
Conclusions
Even if congenital anomalies of aortic arch and
absence of branches of subclavian artery are rare
individually, it is so important that these two congenital
anomalies being together. If a precise preoperative
diagnosis cannot be established, unexpected results may
be encountered in surgery or medical approaches.
The possibility of their presence should be recognized
for surgeons and anatomists.
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Corresponding author
Mehmet Cudi Tuncer, Associate Professor, MD, PhD, Department of Anatomy, “Dicle” University of Medicine,
21280 Diyarbakır, Turkey; Phone +90 412 2488001 ext. 4539, e-mail: drcudi@hotmail.com
Received: February 3rd, 2010
Accepted: March 31st, 2010
