Integrated Strategies Of Perinatal Diagnosis And Treatment Of PA/IVS And CPS/IVS

[Objective]This retrospective study on pulmonary atresia with intact ventricle septum?PA/IVS?and critical pulmonary stenosis with intact ventricle septum?CPS/IVS?patients aimed to summarize fetal echocardiographic features,to evaluate the outcome of transcatheter intervention during neonatal period,and to analyze the impact of prenatal diagnosis on early clinical manefestations and near of mid-term outcomes of the disease in order to explore the effectivess of integrated strategies of perinatal diagnosis and treatment of PA/IVS and CPS/IVS and to provide a reference for the clinical treatment of the disease.[Methods]From June 2009 to Feburary 2013,all the fetuses and neontates diagnosed as PA/IVS or CPS/IVS who admitted by Shanghai Xinhua Hospital were included.Clinical data,medical history records and imaging data were collected.Medical information,including gender,age,body weight,height,treatment and complications,transcutaneous oxygen saturation?SpO₂?,days of hospitalization and ventilation,follow-up time and outcomes,was analyzed.Imaging parameters of all subjects,including pulmonary valve annulus diameter?PV??cm?and Z score,tricuspid annulus diameter and Z score?TV Z score?,tricuspid annulus diameter/mitral annulus diameter?TV/MV?in four-chamber view,right ventricular pressure?mmHg?,right ventricle end-diastolic length/left ventricle end-diastolic length?RV/LV length ratio?in four-chamber view,were measured and calculated.Right ventricular morphyology and right ventricular development evaluating parameters were summarized and compared in PA/IVS and CPS/IVS.ROC curve and logistic analysis were used to explore the effectivess of integrated strategies of perinatal diagnosis and treatment of PA/IVS and CPS/IVS and to provide a reference for the clinical treatment of the disease.[Results]32 fetuses with CPS/IVS or PA/IVS were admitted to Xinhua hospital with varying degrees of right ventricular dysplasia,tricuspid valve regurgitation,pulmonary valve stenosis,et al.Accurate diagnosis could be made during mid-tremester of pregnancy.6 cases chose termination fo pregnancy and other 26fetuses were included in out perinatal congenital heart disease diagnosis and treatment system.Logistic analysis and ROC curve were used to identify predictors of early intervention after birth,which showed absence of pulmonary valve regurgitation?OR 0.012,95%CI 0.001-0.224?and tricuspid valve Z score<-2.1?sensitivity 100%,specificity 83%,AUC 0.97?had the best capability of predicting early intervention during neonatal period.20 neonates with prenatal diagnosis and 18without prenatal diagnosis were compared.Patients with prenatal diagnosis were adimmitted and treated earlier than those who were diagnosed after birth.On the other hand,patients without prenatal diagnosis had higher incidence of complications?OR 3.33,95%CI 0.59-18.89?.38 patients underwent percutaneous balloon pulmonary valvuloplasty?PBPV?during neonatal period.Right ventricular systolic pressure decreased significantly right after procedures,also the SpO₂ all had improved.No serious intra-and post-operative complications happened.All patients achieve biventricular circulation.Right ventricle showed no catch-up development during near of mid-term of follow-up.Pulmonary valve had significant development.Postoperative infusion of prostaglandin E1 over 5 days had good correlation of reintervention?sensitivity 83%,specificity 72%,AUC 0.82?.[Conclusions]Accurate diagnosis could be made during mid-tremester of pregnancy.Logistic analysis and ROC curve were used to identify predictors of early intervention after birth,which showed absence of pulmonary valve regurgitation and tricuspid valve Z score<-2.1 had the best capability of predicting early intervention during neonatal period.Patients without prenatal diagnosis had higher incidence of complications.Right ventricular systolic pressure decreased significantly and the SpO₂ all had improved after PBPV during neonatal period.Postoperative infusion of prostaglandin E1 over 5 days had good correlation of reintervention.