Exploration Of New Surgical Procedures For The Diagnosis And Treatment Of Severe Pulmonary Atresia And Exploration Of Independent Risk Factors For Tetralogy Of Fallot Based On A Large Retrospective Cohor

PART Ⅰ.Exploring a new surgical approach for severe pulmonary atresia:a multicenter "selective" unifocalization operation efficacy analysis studyBackground and Objective 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.PART Ⅱ.A histopathological perspective provides new insights into the surgical outcomes of "selective" unifocalization in patients with PA/VSD/MAPCAs:a case series studyBackground and Objective 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.Conclusion 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.PART Ⅲ.Association of Pulmonary Valve Morphology Differences With Outcomes in Tetralogy of Fallot Repair With Right Ventricular Outflow Tract Incision:a clinical risk factor study based on the largest retrospective cohort of tetralogy of Fallot in ChinaBackground and Objective Current observational studies may not have large samples to investigate the relationship between pulmonary valve(PV)morphology differences and outcomes after complete repair for tetralogy of Fallot(TOF)by right ventricular outflow tract(RVOT)incision.This study aimed to assess the impact of PV morphology differences on outcomes after complete repair for TOF.Methods This is a retrospective cohort study.Consecutive patients who underwent TOF repair with RVOT incision at Fuwai Hospital from January 2012 to December 2017 were included and compared according to PV morphology differences(unicuspid or bicuspid was abnormal morphology,while the tricuspid valve was normal morphology).The primary outcome was defined as a composite of death,or reintervention,or significant annular peak gradient(APG),or significant pulmonary regurgitation(PR),whichever occurred first.Multivariable Cox model analysis was used to assess the relationships between PV morphology differences and outcomes.Subgroup analysis and Propensityscore analysis were performed as sensitivity analyses to assess the robustness of our results.Results The cohort included a total of 1,861 patients with primary diagnosis of TOF,with 1,688 undergoing CR-TOF with RVOT incision.The median age was 318 days[interquartile range(IQR):223-534 days],a median weight of 8.9 kg(IQR:7.6-10.5 kg)and 60.0%(1,011)were male.Complete follow-up data were available for 1,673 CR-TOF patients with a median follow-up duration of 49 months.Adjusted risks for the primary outcome and significant APG were lower for patients with normal PV morphology at follow up[adjusted hazard ratio(HR):0.68;95%CI:0.46-0.98;adjusted HR:0.22;95%CI:0.07-0.71,respectively].The trend for the primary outcome during follow-up remained unchanged,even in subgroups and propensity score matching analyses.Conclusions In this analysis of data from a large TOF cohort,patients with normal tricuspid PVs were associated with a decreased risk of the primary outcome and a lower risk of significant APG,as compared with patients with abnormal unicuspid or bicuspid PVs.