We evaluated the utility of intravenous digital subtraction angiography (IV DSA) for diagnosing lesions of the aortic arch and great vessels in 25 patients. Digital subtraction angiography (DSA) was found useful in evaluating congenital and acquired lesions of the arch and great vessel origins, and it proved adequate for follow-up of patients who had graft replacement. Cases examined included: right aortic arch, double arch, aortic coarctation, aberrant vascular origins, aortic aneurysm and pseudoaneurysm, changes in atherosclerotic great vessels, and revascularization procedures for patients with pulmonary atresia and aortic interruption. In our experience, DSA is a useful tool for screening and following patients with aortic arch or great vessel lesions; it is often the only diagnostic imaging examination necessary.