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

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.

A Novel Perspective on Histopathology Provides Novel Insights into Surgical Effects in Pulmonary Atresia,Ventricular Septal Defect,and Major Aortopulmonary Collateral Arteries:A Case-Series Study

Background:Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries(MAPCAs)and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and MAPCAs.Methods:This is a case-series study.Thirteen patients were included.Angiographybased assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative ligature.Specimens were collected and stained by HE and ET+VG.Results:Twelve patients underwent one-stage unifocalization at a median age of 37 months(range:6–228 months)and a median weight of 14.0 kg(range:5.0–49.0 kg),which produced a favorable right ventricle to aortic systolic pressure ratio of no more than 0.5 except in one patient who died.Patients were divided into three groups:Group 1(n=6),had no native pulmonary arteries,and collateral arteries supplied all pulmonary blood;Group 2(n=6)presented dysplastic native pulmonary arteries on one or both sides,and in some lung lobes or segments,blood was supplied only by collateral arteries;Group 3(n=1)had well-developed left and right pulmonary arteries,and collateral arteries,and pulmonary arteries provided blood flow to the same segments.Pathological reports demonstrated two types of collateral arteries:Elastic arteries presented an arborization distribution similar to native pulmonary artery walls,while muscular arteries showed high resistance and distortion.We selectively unifocalized single-supply collateral arteries with morphologic features based on the arborization distribution.Conclusions:We found that there were two kinds of MAPCAs with different histology,and we performed selective UF for MAPCAs that might belong to the elastic artery.Selective unifocalization achieved a low right ventricle to aortic systolic pressure ratio and favorable surgical effects.