Long-term survival in preductal atresia of the aortic arch

Atresia of the aortic arch is distinguished from complete interruption of the aorta by the presence of a fibrotic strand bridging the 2 discontinuous segments of aorta. Most patients with aortic arch atresia have associated intracardiac defects. There is a high neonatal mortality in this condition, with 95% of the patients dying in the first year of life. The case described herein is the first long-term survivor with atresia of the aortic arch, a patent ductus arteriosus, and an atrial septal defect to reach adulthood. In this patient an extensive collateral network existed in spite of the presence of a patent ductus arteriosus.