Anomalous Origin of the Left Common Carotid Artery from the Main Pulmonary Artery: A Rare Association in an Infant with CHARGE Syndrome

Abstract

Case Report. Isolated carotid artery originating from the pulmonary trunk is an exceedingly rare anomalous origin of head and neck vessels. We present this finding, along with a persistent embryonic trigeminal artery, in a male infant with multiple cardiac defects and other congenital anomalies associated with CHARGE syndrome. After extensive investigations, cardiac catheterization revealed the anomalous left common carotid artery arising from the cranial aspect of the main pulmonary artery. There was retrograde flow in this vessel, resulting from the lower pulmonary pressure, essentially stealing arterial supply from the left anterior cerebral circulation. The persistent left-sided trigeminal artery provided collateral flow from the posterior circulation to the left internal carotid artery territory, allowing for safe ligation of the anomalous origin of the left common carotid artery, thereby reversing the steal of arterial blood flow into the pulmonary circulation and resulting in a net improvement of cerebral perfusion. Conclusion. The possibility of this vascular anomaly should be considered in all infants with CHARGE syndrome. Surgical repair or ligation should be tailored to the specific patient circumstances, following a careful delineation of all sources of cerebral perfusion.

The closing process of the ductus arteriosus connecting the left common carotid artery and main pulmonary artery

“…To the best of our knowledge, only 13 cases have been reported so far. [1][2][3][4] All the reported cases showed three characteristics, which are connection of the isolated left common carotid artery to the pulmonary artery, presence of right aortic arch, and presence of aberrant left subclavian artery. It was first reported by Fong and Venables in 1987.…”

“…Significant associated congenital cardiac defects were also often present in cases with isolated left common carotid artery from the pulmonary artery: primum atrial septal defect, 5 secundum atrial septal defect, tetralogy of Fallot, 6 persistent left supra vena cava, 7 aortic atresia, 8 Ebstein anomaly, 4 CHARGE syndrome, 1 , 4 DiGeorge syndrome, 9 or hemifacial abnormality 7 . Our case had anovulvar fistula, without any other abnormality including coloboma, genital anomaly, and ear abnormality, and she had normal karyotype and fluorescence in situ hybridisation result for 22q11 deletion.…”

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

“…Genetic abnormalities, especially CHARGE and DiGeorge syndromes, appear to have an association with an isolated LCCA. [3478] 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.…”

“…Ligation appears to be a reasonable option when reimplantation is technically difficult, as long as collateralization is ensured. [123456789]…”

“…Anomalous origin of the left common carotid artery from the pulmonary artery is exceedingly rare, with only nine prior cases reported in the literature. [123456789] 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.…”