Aortic arch interruption presenting with absence of all limb pulses

Abstract

Complete interruption of the aortic arch is a rare congenital anomaly which normally presents in infancy with heart failure and acidaemia. There is almost always a ventricular septal defect and persistence of the ductus arteriosus. When the interruption is between the left common carotid artery and the left subclavian artery, the descending aorta is supplied from the pulmonary artery via the ductus arteriosus and also by retrograde flow from the left vertebral artery, which also supplies the left subclavian artery. When an infant presents with cyanotic congenital heart disease, heart failure, acidaemia, and reduced or absent upper and lower limb pulses, the diagnosis of aortic atresia is likely. When type B interruption of the aortic arch, when the right subclavian artery arises aberrantly from the descending aorta, and the ductus arteriosus constricts, a similar clinical picture is found. In this case, however, there will be normal or bounding carotid pulses. This is now a surgically correctable lesion and hence the importance of its recognition. Three patients with aortic arch anomalies presented with severe heart failure, acidaemia, and poor or absent upper and lower limb pulses. Prominent carotid arterial pulsations were detected which distinguished the condition clinically from aortic atresia. In 2 patients with a left aortic arch, interruption was distal to the left common carotid artery and there was an aberrant right subclavian. In the 3rd patient with a right aortic arch, interruption was distal to the right common carotid and there was an aberrant left subclavian artery. By dilating the narrowed ductus, prostaglandin E2 infusion corrected the acidosis. Successful surgical correction was achieved in one patient by direct anastomosis of descending aorta to ascending aorta and the base of the left common carotid artery.