Study Of Morphological Correlation And Preoperative Risk Factors For Mortality Of Total Anomalous Pulmonary Venous Connection

Purpose:To summarize the morphological features and regularities of pulmonary venous drainage of total anomalous pulmonary venous connection(TAPVC).To find the relationship between morphological features.To assess survival rates and to find the preoperative risk factors for mortality of TAPVC.It can deepen our understanding of TAPVC and it is helpful to provide a comprehensive preoperative evaluation for the surgeons.Materials and Methods:Patients with total anomalous pulmonary venous connection were successively collected between May 2014 and June 2017 in Hunan Children,s Hospital.All available clinical data were collected and computed tomography imaging were reviewed.The CT angiography(CTA)features of TAPVC were summarized.The survival time was followed up by telephone.The clinical and CTA data were analyzed by two experienced radiologists.Life Tables analysis was used to estimate survival rates.Patient survival was described with Kaplan-Meier curves.T test and chi-square test were used to compare the parameters between groups.Cox Regression model was used to test the potential risk factors.Results:In this study,70 patients with TAPVC were collected.There was no significant difference in average birth weight between infant with TAPVC and normal.Pulmonary venous obstruction(PVO)was found in 30(42.9%)of 70 patients in this group.The incidence of PVO was highest in infracardiac type and lowest in cardiac type.PVO occurs in the vertical vein in 95%of patients with supracardiac type.PVO often occurs in the orifice of the vertical venous or resulted from the vertical venous drainage to the portal vein in patients with infracardiac type.In this study,all the patients with TAPVC were complicated with atrial septal defect(ASD),and 44.3 percent of patients with TAPVC complicated with patent ductus arteriosus,but the other concurrent deformities of TAPVC are multifarious.Correlation analysis demonstrated that PVO was correlated with admission age of patient,the incidence of PDA,PA/AA value and ASD size.ASD size was correlated with admission age of patient,RT/LT value and the incidence of PDA.The incidence of PDA was correlated with admission age of patient,PVO and ASD size.MGI value was correlated with admission age of patient,the incidence of PVO and PDA and ASD size.In our study,estimates of 1-month,3-month,6-months,and 1-year survival rates of patients with TAPVC were 76%,61%,49%,and 38%respectively without surgical intervention.The median survival time was 4.8 months.Patients with TAPVC had a higher mortality in the neonatal period.The mortality tends to be stable after one year old.There were significant statistical differences in overall survival rates between PVO and non-PVO groups,PDA and non-PDA groups,natural delivery and caesarean groups,different types of TAPVC,large and small MGI value groups.The univariate analysis showed that the PVO,PDA,caesarean,infracardiac type and smaller MGI value were associated with the risk of mortality in patients with TAPVC.The ratios of risk were 5.602,6.918,2.144,3.231 and 3.157 respectively.The multivariate analysis showed that the PVO,caesarean and smaller MGI value were associated with the risk of mortality.The ratios of risk were 3.333、2.448 and 6.452 respectively.Conclusions:The existence of malformation of TAPVC had no significant effect on fetal physical development.The morphology of TAPVC was complex,and the incidence of PVO was high,but the site of connect,drainage path and the site of obstruction were certain regular.The concomitant malformations were complex and multifarious.ASD and PDA had a high incidence,but ASD should be regarded as part of TAPVC.PVO seriously affected the hemodynamics and pathophysiology of patients with TAPVC,and thereby affected ASD size,the incidence of PDA,admission age and survival time of patient.The ASD size affected the clinical and pathological physiology of patient in various aspects,and it could affect the left vent:ricular development,pulmonary artery pressure,PDA occurrence,physical development,admission age and survival time.The presence of PDA was a reflection of high pulmonary vascular resistance and serious conditions,and its occurrence was affected by ASD size and PVO.MGI ratio was related to ASD size,PDA,PVO and pulmonary artery pressure.It was a comprehensive evaluation indicator that could reflect the hemodynamics and pathophysiology of patients with TAPVC.Patients with TAPVC had a high mortality in the first year of life without surgical intervention.PVO,caesarean and smaller MGI ratio were risk factors for mortality of patients with TAPVC.