| Objective:To investigate the clinical value of ventricular end-diastolic diameter ratio(VEDd R)for predicting the occurrence of adverse events in patients with congenital heart disease associated pulmonary arterial hypertension(PAH-CHD)in the long term.Methods:Through a retrospective cohort study,361 eligible adult patients with PAHCHD who visited our hospital from January 2010 to June 2020 were selected.Relevant information such as RVEDd and LVEDd of enrolled patients was collected in the Harvest Medical Record System,and the ratio between them was calculated as VEDd R,which was divided into all-cause mortality group and survival group by telephone follow-up.Cox regression analysis and survival curve model were used to analyze the predictive value of VEDd R on the prognosis of patients with PAH-CHD.Results:hirty-one patients were included,including 87 males and 274 females,and the mean age of the death group was44(35,52)years.Baseline data showed that there were statistical differences between the survival and death groups in systolic blood pressure,RVEDd,LVEDd,right ventricular outflow tract,right atrial internal diameter,pulmonary artery internal diameter,FS,SV,CO,pulmonary artery pressure,VEDd R,treatment modality,and cardiac function class(P< 0.05).One-way Cox regression analysis showed that right atrial internal diameter,right ventricular outflow tract,pulmonary artery pressure,pulmonary artery internal diameter,VEDd R,RVEDd,and treatment modality and cardiac function class were risk factors associated with all-cause mortality in patients with PAH-CHD.Multifactorial Cox regression analysis showed that VEDd R(HR=12.537,95% CI: 1.203-30.698,P=0.034).NYHA class IV(HR=6.565,95% CI: 1.436-30.028,P=0.015).Interventional treatment(HR=0.343,95% CI: 0.127-0.922,P=0.034),and surgical treatment(HR=0.490,95% CI:0.260-0.924,P=0.027)had predictive value for the prognosis of patients with PAH-CHD.ROC curve VEDd R cut-off value was 0.822,sensitivity 0.644 and specificity 0.818.Kplan-Meier survival curve analysis we found that when VEDd R ≥ 0.822,the long-term survival of patients gradually decreased with the extension of follow-up time.Conclusion:VEDd R is an index to evaluate right heart function and can be used as an independent risk factor to predict all-cause mortality in patients with PAH-CHD.NYHA class IV is a risk factor for patient death,and interventional and surgical treatment are protective factors. |