Isolated left common carotid artery in an infant with pulmonary atresia and intact ventricular septum

Abstract

Isolation of the left common carotid artery (LCCA) is a very rare congenital aortic arch anomaly. We present this finding in a female infant with pulmonary atresia and intact ventricular septum, with a clinical diagnosis of CHARGE syndrome. Cardiac catheterization revealed an anomalous origin of the LCCA from the pulmonary trunk, with retrograde filling of the pulmonary trunk seen during left subclavian artery injection. The LCCA was ligated during central shunt placement.

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156 © 2019 Annals of Pediatric Cardiology | Published by Wolters Kluwer - Medknow
Address for correspondence: Dr. Jennifer L Cohen, 3959 Broadway, CHN 2-253, New York 10032, USA. E-mail: jenniferlaurencohen@gmail.com
INTRODUCTION
Anomalous origin of the left common carotid artery
from the pulmonary artery is exceedingly rare, with
only nine prior cases reported in the literature.[1-9]
Many of these cases are associated with congenital
heart disease, most commonly Tetralogy of Fallot. We
present the rst reported case of an anomalous origin
of the left common carotid from the main pulmonary
artery in an infant with pulmonary atresia with intact
ventricular septum.
CASE REPORT
A neonate girl with a prenatal diagnosis of pulmonary
atresia with intact ventricular septum was born at our
institution, with postnatal echocardiogram conrming
this diagnosis. The right ventricle was noted to be
severely hypoplastic, and color Doppler suggested the
presence of the possible right ventricle to coronary artery
stulas. The postnatal echocardiogram also showed a
right aortic arch with an unclear branching pattern. She
was started on prostaglandin immediately after birth for
ductal-dependent pulmonary blood ow. In addition, she
was also found to have multiple congenital anomalies,
including abnormal ears, a right eye coloboma, and
unilateral choanal atresia. Given this constellation of
ndings, genetics evaluation conrmed clinical diagnosis
of CHARGE syndrome. Her karyotype, microarray, and
whole-exome sequencing were all negative.
Cardiac catheterization was performed on the 2nd day
of life to assess her coronary artery anatomy and
rule out the right ventricular-dependent coronary
circulation (RVDCC). She was found to have connections
between her right ventricle and right coronary artery;
however, there was no coronary artery stenosis. She
had normal antegrade lling of her coronary arteries
from aortic injection, therefore ruling out RVDCC.
The ascending aorta angiogram revealed a right aortic
arch with abnormal head vessel branching, including
an aberrant left subclavian artery. In addition, the
left common carotid artery (LCCA) could not be seen
arising from the aortic arch [Figure 1a]. There was a
left-sided arterial duct that was long and somewhat
tortuous. A pulmonary artery angiogram obtained
with a catheter advanced through the arterial duct
demonstrated the origin of the LCCA from the superior
Isolated left common carotid artery in an infant with pulmonary
atresia and intact ventricular septum
Jennifer L Cohen, Nicole Stanford, Alejandro Torres
Department of Pediatrics, Division of Pediatric Cardiology, New York-Presbyterian Hospital, Columbia University Medical Center, New York, USA
ABSTRACT
Isolation of the left common carotid artery (LCCA) is a very rare congenital aortic arch anomaly. We present
this nding in a female infant with pulmonary atresia and intact ventricular septum, with a clinical diagnosis
of CHARGE syndrome. Cardiac catheterization revealed an anomalous origin of the LCCA from the pulmonary
trunk, with retrograde lling of the pulmonary trunk seen during left subclavian artery injection. The LCCA
was ligated during central shunt placement.
Keywords: Aortic arch anomaly, CHARGE syndrome, isolated carotid artery, pulmonary atresia with intact
ventricular septum
CASE REPORT
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How to cite this article: Cohen JL, Stanford N, Torres A. Isolated left
common carotid artery in an infant with pulmonary atresia and intact
ventricular septum. Ann Pediatr Card 2019;12:156-8.
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Cohen,
et al
.: Isolated left common carotid artery
157
Annals of Pediatric Cardiology / Volume 12 / Issue 2 / May-August 2019
DISCUSSION
This is the rst case of an isolated LCCA arising from the
pulmonary trunk described in a patient with pulmonary
atresia with intact ventricular septum. This demonstrates
the importance of fully delineating aortic arch anatomy
in patients with a diagnosis of pulmonary atresia and
intact ventricular septum, as it certainly changed surgical
management in this case. Previous case reports of an
anomalous origin of the LCCA are summarized in Table 1.
All of these patients, similar to our patient, had a right
aortic arch and an aberrant left subclavian artery. Six
out of the nine cases (67%) had associated congenital
heart disease; three of whom had Tetralogy of Fallot, one
had Ebstein’s anomaly, one had a primum atrial septal
defect, and one patient had a patent arterial duct and
large secundum atrial septal defect. Three patients (33%)
had no associated congenital heart disease.[1-9]
Genetic abnormalities, especially CHARGE and DiGeorge
syndromes, appear to have an association with an
isolated LCCA.[3,4,7,8] This suggests that aortic arch
abnormalities, including the anomalous origin of the
LCCA, should be ruled out in patients with these genetic
syndromes, especially in the presence of associated
congenital heart disease.
Three of the previously reported patients underwent
carotid ligation, four had reimplanation of the carotid, one
has had no intervention, and one died before intervention.
It was clear from cardiac catheterization that the left
common carotid was being supplied by the left subclavian
artery via the vertebral artery, given that the left carotid
artery lled during left subclavian injection. Therefore,
it was not felt necessary to pursue further cerebral
vasculature imaging. In patients, where the supply of
the isolated head vessel is not as clearly delineated, then
further cerebral vascular assessment may be warranted.
Our patient underwent carotid ligation with cerebral
oxygen consumption monitoring through near-infrared
portion of the pulmonary trunk near the site of
bifurcation [Figure 1b and c]. A selective left subclavian
angiogram showed a normal left vertebral artery and
retrograde lling of the LCCA into the pulmonary
trunk [Figure 1d].
She went to the operating room on the day of life ten at
which time a central aortopulmonary shunt was placed.
Cerebral near-infrared spectroscopy was monitored
throughout the procedure, with no change after ligation
of the LCCA. Carotid reimplantation was not attempted
due to the long distance between the origin of the
anomalous left carotid and the transverse aortic arch.
She did well postoperatively with appropriate oxygen
saturations and a normal neurologic exam.
Figure 1: Angiograms demonstrating anomalous origin of the left
common carotid artery from the main pulmonary artery. (a) Aortic
arch injection demonstrating a right aortic arch with the right
common carotid artery arising from the transverse arch. The
main pulmonary artery lls via the patent ductus arteriosus, and
the left common carotid artery origin form the main pulmonary
artery is also seen. (b and c) Anteroposterior and lateral
projections demonstrating the left common carotid artery arising
from the superior portion of the main pulmonary artery near the
bifurcation. (d) Left subclavian injection with retrograde ow seen
in the left common carotid artery
ab
cd
Table 1: Previously reported cases of anomalous origin of the left carotid artery from the pulmonary
artery
Author Associated
congenital
heart disease
Cardiac
anatomy
Arch
sidedness
Aberrant left
subclavian?
Genetic
diagnosis
Outcome
Fong
et al
.[1] Yes Primum ASD Right Yes Primordialdwarsm carotid ligation
Fouilloux
et al
.[2] No Normal Right Yes None carotid re‑implantation
Ghalili
et al
.[3] Yes PDA and large
secundum ASD
Right Yes CHARGE syndrome carotid re‑implantation
Huang
et al
.[4] Yes Tetralogy of Fallot Right Yes DiGeorge syndrome patient death prior to
intervention
Hurley
et al
.[5] No Normal Right Yes None no intervention
Kaushik
et al
.[6] No Normal Right Yes Dysmorphisms
withnospecic
diagnosis
carotid re‑implantation
Oppido
et al
.[7] Yes Tetralogy of Fallot Right Yes DiGeorge syndrome carotid re‑implantation
Osakwe
et al
.[8] Yes Ebstein’s anomaly Right Yes CHARGE syndrome carotid ligation
Tozzi
et al
.[9] Yes Tetralogy of Fallot Right Yes None carotid ligation
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Cohen,
et al
.: Isolated left common carotid artery
158 Annals of Pediatric Cardiology / Volume 12 / Issue 2 / May-August 2019
spectroscopy, with no change noted on clamping and then
ligation of the LCCA. Ligation appears to be a reasonable
option when reimplantation is technically difcult, as long
as collateralization is ensured.[1-9]
The embryologic origin of an isolated left carotid
artery is not fully understood. A proposed mechanisms
utilizing the hypothetical double aortic arch of Knight
and Edwards[2] would suggest that regression of the
arch between the LCCA and left subclavian artery, as
well as regression of the arch between the ascending
aorta and LCCA, could lead to the left common carotid
connected to the pulmonary artery via the arterial duct.
It is interesting that an anomalous origin of the carotid
artery from the pulmonary artery has only been reported
in regard to the left carotid artery, universally in the
setting of a right aortic arch and aberrant left subclavian,
perhaps because of the arterial duct anatomy associated
with a right aortic arch.
We report an unusual case of an isolated LCCA arising
from the pulmonary trunk in a patient with CHARGE
syndrome and pulmonary atresia with intact ventricular
septum. Infants with suspected CHARGE or DiGeorge
syndrome associated with congenital heart disease should
have head-and-neck vessel anatomy clearly identied.
Declaration of patient consent
The authors certify that they have obtained all
appropriate patient consent forms. In the form the
patient(s) has/have given his/her/their consent for
his/her/their images and other clinical information to
be reported in the journal. The patients understand
that their names and initials will not be published and
due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conicts of interest.
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