Interrupted aortic arch: natural history and operative results.

The chances of survival for patients with interruption of the aortic arch depend mainly on early recognition and medical treatment before symptoms of cardiogenic shock appear, and specifically on early operative repair after the exact diagnosis has been established. Accurate angiography with visualization of the interrupted aortic arch and delineation of associated cardiac malformations is of prime importance for the surgical management and prognosis. Among our 36 patients with interrupted aortic arch, 27 were operated on. The operative mortality in 22 patients with associated ventricular septal defect (VSD) and persistent ductus arteriosus (PDA) was reduced from 43% (in seven patients seen up until 1979) to 7% (in 15 patients seen since 1980). One patient with interrupted aortic arch type C and another infant with associated truncus arteriosus were successfully corrected on day 9 and day 17 of life, respectively. In the patients with associated VSD and PDA as well as in the one patient with associated truncus arteriosus, the primary correction--direct anastomosis of the interrupted segments without prosthesis and VSD closure and in the case with truncus, the additional positioning of a valve-bearing allograft conduit from the right ventricle to the pulmonary artery--has been more successful than a two-step approach with initial pulmonary artery banding.