Objective:
To analyze the results of one-stage unifocalization (UF) performed through the median sternotomy in the surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs).

Methods:
Since 2007 we operated on 16 consecutive patients with pulmonary atresia, ventricular septal defect, hypoplastic central pulmonary arteries and MAPCAs. Median age was 8 months. Median weight was 7.1 kg. Median MAPCAs quantity was 4. All patients received one stage UF through the median sternotomy. Only native tissues were used. Total repair preferentially was done at the same operation either central shunt was performed.

Results:
There were no early or late deaths. Total repair was performed at the time of UF in 13 of 16 patients, in 1 more patient - 6 months later. Two more patients with UF and central shunt are awaiting total repair. Early postrepair peak systolic right to left ventricular pressure ratio ranged from 0.45 to 0.8 (median - 0.5). All patients were followed postoperatively with routine CT angiography and echocardiography. Median follow-up period was 9 months (range - 2-18 months). Two patients required balloon dilation of the pulmonary arteries at 5 and 13 months after surgery and 1 patient required conduit replacement at 9 months.

Conclusions:
Complete UF was achieved in all patients through the median sternotomy and was followed by successful total repair in 88% of them. Presently, one-stage UF through the median sternotomy is our preferred strategy in pulmonary atresia with ventricular septal defect, hypoplastic central pulmonary arteries and MAPCAs.