Clinical Study On Evaluation Of Children With Congenital Heart Disease Associated With Pulmonary Hypertension

ObjectiveTo investgate the epidemiology,clinical characteristics and prognosis of children with congenital heart disease associated with pulmonary hypertension(CHD-PH)by the review of 7 years cases in our hospital.To investigate the value of red cell distribution width(RDW)?serum uric acid(SUA)in the prognosis and assessment of pediatric congenital heart disease associated pulmonary hypertension.MethodsCHD-PH patients were admitted into our hospital from January 2009 to December 2016.The clinical features,treatment,baseline data of patients,laboratory examination,echocardiography and results of right heart catheterization were recorded and the CHD-PH patients were followed up for prognosis.There were 70 cases of 0-16 year-old pediatric congenital heart disease associated pulmonary hypertension patients were enrolled and 76 patients with congenital heart disease(CHD)without pulmonary hypertension treated in the same period as the reference group.The follow-up data were statistically analyzed in the two groups.According to the persistence of pulmonary hypertension after operation,the patients were divided into two groups: postoperative pulmonary hypertension group and no postoperative pulmonary hypertension group.The data of the two groups were analyzed.To expand the sample,CHD-PH patients over 16-year-old were admitted in the same period.The difference of serum uric acid level and red cell distribution width in CHD-PH were analyzed.Results1.There were 70 children with CHD-PH enrolled in this study.Ventricular septal defect is the most common disease(63%)in children with congenital heart disease associated with pulmonary hypertension,followed by over 2 kinds of compound(28.5%),atrial septal defect(4%),patent ductus arteriosus(4%).2.Surgical treatment is the main treatment(72%),percutaneous intervention and drug treatment accounted for a low proportion.3.RDW,NYU PHFI score,the ratio of bidirectional shunt was higher than thatof the control group(P <0.05),LVEF in CHD-PH patients was significantly lower than that in CHD patients(P<0.01).4.The age and systolic pressure of pulmonary artery in postoperative pulmonary hypertension group were higher than those in control group(P < 0.05).Multivariate logistic regression analysis showed operative age was the risk factor of postoperative pulmonary hypertension in children with CHD-PH(P < 0.05).5.RDW in died patients was higher than the survival patients(P < 0.05).ROC analysis showed that RDW was the effective predicter of all-cause mortality in CHD-PH patients,the area under the ROC curve was 0.724(95% CI: 0.535-0.913).6.Uric acid was not correlated with pulmonary arterial systolic pressure,pulmonary arterial diastolic pressure and pulmonary arterial mean pressure in children with CHD-PH,respectively(P >0.05).Conclusion1.Ventricular septal defect is the most common cardiac anomalies in congenital heart disease associated pulmonary hypertension.Surgery repair is the main treatment.The age at operation is an independent risk factor for postoperative pulmonary hypertension in children with CHD-PH and early surgical treatment with most good prognosis suggest that such patients should be early diagnosis,timely correction of cardiac malformation.2.RDW in the CHD-PH death group was significantly higher than that in the survival group,and a certain extent could indicate the prognosis of the CHD-PH children.Serum uric acid is positively related to pulmonary arterial pressure.3.Serum uric acid was not correlated with pulmonary artery pressure.It may not reflect the severity of the disease in patients with congenital heart disease associated with pulmonary hypertension.