The interrupted aortic arch: an overview after 20 years of surgical treatment

The records of 95 patients with interrupted aortic arch, admitted to our center from 1975 to 1995, were reviewed. We were particularly interested in the long term results and evaluated the impact of the preoperative state on the outcome after surgery.

Using the 'Celoria and Patton' classification, 13% were type A, 84% type B and 3% type C. Among various associated anomalies were ventricular 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 communis, double outlet right ventricle and transposition of the great arteries. Preoperative neurological disorders, among them the Di George's syndrome, were found in 29% of the cases. Our long term results show 52 patients to be alive, of which 89% are in good clinical condition. Due to improved operative techniques and changes in the management of neonates respectively, early mortality was 17% between 1985 and 1995 compared to 42% between 1975 and 1985. Reoperations were necessary due to arch stenosis, compression of the bronchus or left ventricular outflow tract obstruction.

Nevertheless, mortality after surgical repair of an interrupted aortic arch has dropped significantly and the preoperative condition plays an important role in the outcome. Sepsis, low output, low weight (under 2400 g), severe left ventricular outflow tract obstruction and complex malformations impeded surgery 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 seems to influence late neurological disorders.