Complete interruption of the aortic arch

The clinical, hemodynamic, angiographic, and pathologic findings in three patients with complete interruption of the aortic arch are presented. In addition, data derived from published reports of 52 previous patients are summarized. This malformation is never an isolated one, since a patent ductus arteriosus is always present and a ventricular septal defect is almost invariably found as well. The diagnosis of absence of the aortic arch is difficult, since differential cyanosis is uncommon, relative systolic hypertension of the arms is rare, and the heart murmur, when present, is of a nonspecific nature. At catheterization the pressure in the pulmonary artery, patent ductus arteriosus, and descending aorta is usually similar to that in the ascending aorta and in its branches. Selective angiocardiography has been shown to be the only means of establishing the presence of the malformation prior to operation or autopsy. Since the majority of patients with absence of the aortic arch die within the month following birth, early recognition of the lesion is imperative if surgical correction is to be of benefit.