The formal pathogenesis of isolated common carotid or innominate arteries: the concept of malseptation of the aortic sac

Abstract

 Deficient connections (= isolation) of the innominate artery or the common carotid artery to the aorta are rare congenital anomalies of the human aortic arch complex that are usually associated with a patent vascular connection between the isolated artery and a pulmonary artery. In the present study we demonstrate chick fetuses with a corresponding anomaly, the isolation of the brachiocephalic artery. In our chick fetuses the left brachiocephalic artery did not arise from the aortic arch, but was connected to the pulmonary trunk proximal (upstream) to the patent left and right ductus arteriosus. These cases are of interest because the presence of a congenital pulmonary-systemic arterial connection proximal (upstream) to the ductus arteriosus cannot be explained by the traditional concept of the morphogenesis of the aortic arch complex. The development of the normal and abnormal branching patterns of the aortic arch arteries is traditionally explained by transformation of the primitive embryonic pharyngeal arch arterial system due to obliteration of some of its vascular segments. Based on this concept, the isolation of an aortic arch artery can be explained by obliteration of vascular segments proximal and distal to this artery, whereas its connection to a pulmonary artery can be explained only by deficient obliteration (persistence) of the distal portion of the right or left sixth pharyngeal arch artery. The connecting ”vascular segment” between an isolated aortic arch artery and the pulmonary circulation, therefore, is traditionally interpreted as a patent ductus arteriosus. The formal pathogenesis of congenital pulmonary-systemic arterial connections proximal (upstream) to the ductus arteriosus is discussed. The presented cases of isolation of the brachiocephalic artery are explained by disturbances in the partition of the embryonic aortic sac, possibly due to abnormal development of the ”cardiac” neural crest.