Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction

Background. Management of newborns with interrupted aortic arch (IAA) remai ns challenging. Associated severe left ventricular outflow tract obstructio n (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this com plex surgical problem. Methods. From May 1991 to Tune 1999, 28 neonates were treated for IAA. Thir teen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) a nd severe LVOTO (Z value -2 to -7; mean -5 +/- 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic ap lasia and chromosome 22 region q11 deletion. All 13 patients were treated i nitially with a modified Norwood procedure. Results. There were no perioperative deaths. Complications included 2 patie nts with recurrent arch stenosis treated with balloon dilatation. Two patie nts had systemic arterial shunt revision. Follow-up ranged from 2 to 99 mon ths old (mean 39 months). There were 2 late deaths unrelated to any operati on. Nine of 12 patients had a second stage palliation consisting of a bidir ectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient ha d a modified Fontan operation and 5 patients are awaiting potential biventr icular repair. Conclusions. Children with IAA and severe LVOTO may be managed by initial N orwood palliation with an excellent outcome likely. This initial "univentri cular" approach has enabled eventual successful biventricular repair despit e severe LVOTO. (C) 2001 by The Society of Thoracic Surgeons.