Biventricular Correction of Aortic Atresia/ Severe Aortic Valve Hypoplasia with Ventricular Septal Defect Using “Norwood-Rastelli Operation” after Hybrid Palliation

Background: Aortic atresia/ severe aortic valve hypoplasia with ventricular septal defect (VSD) in case of two adequate ventricles can be managed by a primary biventricular correction or by a Norwood-type palliation followed by a biventricular repair. We applied a biventricular repair in four patients with the diagnosis of aortic atresia/ severe aortic valve hypoplasia, interrupted aortic arch (IAA) and VSD following an initial hybrid approach.

Patients and methods: Between 2002–2015 two patients with the diagnosis of aortic atresia and VSD and two patients with severe aortic valve hypoplasia, IAA and VSD were primarily treated using hybrid Norwood approach. Patients age at the time of the first operation were 15, 3, 6 and 8 days and at the time of the biventricular correction were 263,140, 274, 290 days respectively. Weights of the patients at the time of the first operation were 3310, 2600, 2960 and 3520 g and at the time of the second operation were 6230, 6000, 7100, 6400 g respectively. Following initial hybrid stage 1 procedure (bilateral pulmonary artery banding and ductal stenting) patients underwent to a biventricular correction via Norwood-Rastelli operation. After pulmonary artery de-banding, patch augmentation of the pulmonary arteries and removal of the ductal stent a Norwood-Rastelli operation was performed using selective cerebral perfusion. A valved conduit was used between the right ventricle and the pulmonary arteries to accomplish the repair.

Results: All patients are alive after follow-up times of 155, 125, 34 and 6 months after biventricular repair, respectively. The sternum was left open after biventricular repair in all cases for secondary closure. One patient received left bronchial stent one month after the operation due to left bronchial compression. There was no early and late mortality. Two patients received interventions on pulmonary arteries and no patients required a reoperation until now.

Conclusion: Biventricular repair can be successfully performed in aortic atresia/ severe hypoplasia with VSD after initial palliation with bilateral pulmonary artery banding and ductus stenting. This method can avoid the neonatal major surgical complications with the use of cardiopulmonary by-pass at primary correction or Norwood palliation. Delayed biventricular repair with Norwood-Rastelli like operation has no mortality and low morbidity rates.