Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births)[1] in which the aorta is not completely developed.

Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus.

[2] It is thought that an interrupted aortic arch occurs through excessive apoptosis in the developing, embryonic aorta.

[5] If the condition progresses, the infant may turn pale, feel cold in the lower half of the body, and have a weak pulse due to insufficient blood flow.

[10] Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body.

After successful treatment, the patient is monitored for the rest of their life by a specialist to ensure that problems do not occur.

[3] The incidence of an interrupted aortic arch is extremely rare, occurring between three[1] and twenty times per 1,000,000 births.

[13] In the case Steidele described, the infant had a type A interrupted aortic arch, and survived only for "a few hours.

[1] The first successful repair of a Type A interrupted aortic arch was reported in 1961, in which the left subclavian artery was grafted into the descending thoracic aorta in a 14-year-old male patient.

[3] While PGE1 is the standard of care for maintaining the ductus arteriosus, there is insufficient data on the proper dose, duration of therapy, safety, and long-term consequences of PGE1 on infants with ductal-dependent congenital heart defects (like interrupted aortic arch).