THE INTERRUPTED AORTIC-ARCH - AN OVERVIEW AFTER 20 YEARS OF SURGICAL-TREATMENT

Objective and methods: The records of 95 patients with interrupted aor tic arch. admitted to our center from 1975 to 1995, were reviewed. We were particularly interested in the long term results and evaluated th e impact of the preoperative state on the outcome after surgery. Resul ts: Using the 'Celoria and Patton' classification, 13% were type A, 84 % type B and 3% type C. Among various associated anomalies were ventri cular septal defects and left ventricular outflow tract obstructions, either subvalvular or due to a hypoplastic annulus or a bicuspid valve . We have also seen complex malformations such as truncus arteriosus c ommunis. double outlet right ventricle and transposition of the great arteries. Preoperative neurological disorders, among them the Di Georg e's syndrome, were found in 29% of the cases. Our long tem-m results s how 52 patients to be alive, of which 89% are in good clinical conditi on. Due to improved operative techniques and changes in the management of neonates respectively, early mortality was 17% between 1985 and 19 95 compared to 42% between 1975 and 1985. Reoperations were necessary due to arch stenosis, compression of the bronchus or left ventricular outflow tract obstruction. Conclusions: Nevertheless, mortality after surgical repair of an interrupted aortic arch has dropped significantl y and the preoperative condition plays an important role in the outcom e. Sepsis, low output, low weight (under 2400 g), severe left ventricu lar outflow tract obstruction and complex malformations impeded surger y in 13% of cases. Immediate surgical intervention is the only therapy . Arch continuity and repair of associated anomalies could be achieved in the remaining collective. Most of the children have a good quality of life. The preoperative condition stems to influence late neurologi cal disorders. (C) 1997 Elsevier Science B.V.