The classification system (Types A, B, and C) were defined by Celoria and Patton in 1959.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. The first successful repair of a Type A interrupted aortic arch in an infant was in a 12-day-old infant in 1969, in which the left subclavian artery was connected to the descending thoracic aorta, the patent ductus arteriosus was closed, and the main pulmonary artery was banded.The first successful repair of a Type B interrupted aortic arch was in 1954, in which the 16 year-old, female patients own aorta was grafted from the arch to the descending thoracic aorta and the left subclavian artery was ligated. The first successful repair on a type B interrupted aortic arch in an infant was in 1973, in which a vein was used to connect the ascending aorta and the descending aorta.The first successful repair of a Type C interrupted aortic arch was in 1964, in which the 16-year-old female patients ascending aorta was grafted to the descending thoracic aorta. As of 1984, there had been no successful repairs on infants under one year old.The use of PGE1 dramatically improved the mortality rate after its introduction in 1976.
As a result, the kidneys fail and the blood becomes acidic, resulting in death.With modern surgical techniques, 81% of children with an interrupted aortic arch survive to be 15 years-old. The fate of survivors in the long-term is still unclear. Surgical complications
The most common, early complication of surgery is bleeding, the risk of which can be increased by prematurity, prolonged acidosis prior to surgery, and excessive tension on the anastamosis due to inadequate mobilization of the ascending and descending aorta. Other early complications include damage to the left recurrent laryngeal nerve and the phrenic nerve. Late complications include obstruction of the graft and obstruction of the left main bronchus (which passes underneath the aortic arch). Epidemiology
The incidence of an interrupted aortic arch is extremely rare, occurring between three and twenty times per 1,000,000 births. In the context of other congenital cardiac abnormalities, interrupted aortic arch represents about 1.5% of cases. History
The condition was first identified by Dr. Raphael Steidele, Professor of Obstetrics at the University of Vienna, in 1778. In the case Steidele described, the infant had a type A interrupted aortic arch, and survived only for "a few hours." In homage to the discoverer, the terminology of "Steideles complex" has been used to describe an interrupted aortic arch. The first type B interrupted aortic arch was reported by Seidel in 1818, and the first type C was reported by Weisman and Kesten in 1948.