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Background:  This study aims to explore the efficacy of selective unifocalization (UF) for major aortopulmonary collateral arteries (MAPCAs) unifocalization in children with pulmonary atresia with ventricular septal defect (PA/VSD).  Methods:  A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019. Sex, age, preoperative cardiovascular angiography test results and McGoon ratio were collected. The properties of the collateral arteries were evaluated by angiography, and selective UF for the “dendritic” MAPCAs and ligation of MAPCAs demonstrating distortion and resistance.  Results:  A total of 13 severe patients underwent one-stage repair, of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO support. The median age was 37 months, and a median weight was 14.0 kg. A right ventricular to aortic systolic pressure ratio (pRV/pAo) of no more than 0.5 was achieved in 12 living patients.  Conclusion:  Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early stage. This surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.

The Clinical Application Value of Selective Unifocalization in the Treatment of Severe Pulmonary Artery Atresia with Ventricular Septal Defect