Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies. Interrupted aortic arch is often associated with DiGeorge Syndrome.
Treatment consists of open heart surgery soon after birth. Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body. Failure to treat the condition yields a mortality rate of 90% at a median age of 4 days.
^ abMessner, Greg; Reul, George J.; Flamm, Scott D.; Gregoric, Igor D.; Opfermann, Ulrich Tim (2002). "Interrupted aortic arch in an adult single-stage extra-anatomic repair". Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital29 (2): 118–21. PMC116738. PMID12075868.