| 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.
Keywords: Aortic arch anomaly, CHARGE syndrome, isolated carotid artery, pulmonary atresia with intact ventricular septum
|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
|How to cite this URL:|
Cohen JL, Stanford N, Torres A. Isolated left common carotid artery in an infant with pulmonary atresia and intact ventricular septum. Ann Pediatr Card [serial online] 2019 [cited 2019 May 26];12:156-8. Available from: http://www.annalspc.com/text.asp?2019/12/2/156/257403
| 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.,,,,,,,, Many of these cases are associated with congenital heart disease, most commonly Tetralogy of Fallot. We present the first 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 confirming 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 fistulas. 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 flow. 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 findings, genetics evaluation confirmed 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 filling 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 1]a. 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 portion of the pulmonary trunk near the site of bifurcation [Figure 1]b and [Figure 1]c. A selective left subclavian angiogram showed a normal left vertebral artery and retrograde filling of the LCCA into the pulmonary trunk [Figure 1]d.
|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 fills 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 flow seen in the left common carotid artery|
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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.
| Discussion|| |
This is the first 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.,,,,,,,,
|Table 1: Previously reported cases of anomalous origin of the left carotid artery from the pulmonary artery|
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Genetic abnormalities, especially CHARGE and DiGeorge syndromes, appear to have an association with an isolated LCCA.,,, 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 filled 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 spectroscopy, with no change noted on clamping and then ligation of the LCCA. Ligation appears to be a reasonable option when reimplantation is technically difficult, as long as collateralization is ensured.,,,,,,,,
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 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 identified.
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.
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Conflicts of interest
There are no conflicts of interest.
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Dr. Jennifer L Cohen
3959 Broadway, CHN 2-253, New York 10032
Source of Support: None, Conflict of Interest: None