Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: primary repair

Primary repair of pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collaterals based on single-stage unifocalization was first reported in 1995. From a midline approach, all collaterals are extensively dissected, translocated in front of the oesophagus and/or the trachea, when required, and directly anastomosed to each other or to the native pulmonary arteries, whenever present, without interposition of prosthetic material. The need for concomitant VSD closure is assessed intraoperatively with a pulmonary flow study according to a standardized protocol. Pulmonary blood supply is established by valved conduit interposition in all patients, regardless of the suitability for VSD closure. Palliation with systemic-pulmonary shunt is reserved for selected cases. Between 1994 and 2015, 94 patients with a median age of 1.09 years (range 0.03-19) underwent single-stage unifocalization at our institution. In 78 (82.1%) of them, an intraoperative pulmonary flow study was utilized to assess acceptability for concomitant VSD closure, which was accomplished in 69 cases (73%). Intraoperatively, following VSD closure, the mean right ventricle-to-aortic pressure ratio was 0.49 ± 0.14. The overall mortality rate was 11.2% (n = 10), with an 82% survival at 12.5 years. At a median follow-up interval of 5.8 years, the right ventricle-to-aortic pressure ratio did not differ significantly from the early postoperative phase. The surgical results of primary repair of PA with VSD and major aortopulmonary collaterals based on single-stage unifocalization and an intraoperative pulmonary flow study are satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions.