Type B interrupted aortic arch associated with other extracardiac and intra
cardiac congenital lesions is a lethal defect. Surgical management with a o
ne- or two-stage approach is complex, difficult and associated with high mo
rbidity and mortality. Between January 1996 and December 1999, 5 consecutiv
e patients with type B interrupted aortic arch were operated on. The group
included 3 girls and 2 boys weighing from 2.4 kg to 3.5 kg (mean 2.84 kg) a
nd aged from 3 to 35 days (mean 15 days). All patients underwent a primary
reconstruction of the aortic arch and one-stage repair of the associated he
art defects. Two patients developed symptoms of mild aortic arch narrowing
7-12 months postoperatively, Balloon angioplasty of the obstructed site was
successfully carried out in one of the patients, In the second child, angi
oplasty was not successful and surgery was used, All the patients survived
and are being followed-up. This experience supports a strategy of primary o
ne-stage repair of IAA type B, including direct anastomosis for aortic arch
reconstruction, and all associated heart anomalies in the neonatal period.