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.

Figure 1

Axial T2-weighted MR image shows a persistent trigeminal artery (arrow) coursing from the cavernous left internal carotid artery posteriorly and medially to anastomose with the midbasilar artery.

Figure 2

Camera in the AP plane. A contrast injection reveals anomalous origin of the left common carotid artery from the roof of the main pulmonary artery.

Figure 3

(a) Camera in the AP plane. An angiogram (early phase) is performed in the transverse aorta, revealing a right aortic arch with aberrant origin of the left subclavian artery and absence of the left common carotid artery. (b) Camera in the AP plane. An angiogram (late arterial phase) reveals retrograde opacification of the left common carotid artery (arrow) draining into the left pulmonary artery.