| BackgroundA therapeutic strategy in patients with left to right shunt congenital heart disease with pulmonary arterial hypertension was lack uniform treatment standards.ObjectiveWe were analyzed treatment results of 93 left to right shunt congenital heart disease combined with pulmonary arterial hypertension patients,to provide clinical basis for the treatment of such patients in the future,MethodPatients with left to right shunt congenital heart disease complicated with pulmonary arterial hypertension who were diagnosed from January 2011 to April 2019 were included in the study group.According to pulmonary vascular resistance index(PVRI),it was divided into "PVRI<4 Wood.m2" group、"4 Wood.m2≤PVRI≤8 Wood.m2" group and "PVRI>8 Wood.m2" group.Statistical analysis was performed on the treatment methods and follow-up results of the three groupsResult1、There were 93 cases in the study group,including 36 males and 57 females Median age was 51 months.Cardiac function class I has 19 cases and 38 cases in class Ⅱ、33 cases in class Ⅲ、3 cases in class Ⅳ.Cardiothoracic ratio was 0.60±0.06.Median percutaneous blood oxygen saturation was 98%.Pulmonary arterial systolic pressure was 83.99±20.50 mmHg and mean pressure was 59.64±16.34 mmHg.Median PVRI was 5.86 Wood.m2.Median Rp/Rs was 0.34.Median Qp/Qs was 2.37.The positive rate of acute pulmonary vasodilation test was 10.91%.2、There were 35 cases in "PVRI<4 Wood.m2”group.34 cases were closed defect directly.During follow-up,93.94%patients had "favorable" outcome.3、There were 31 cases in the "4 Wood.m2≤PVRI≤8 Wood.m2" group,including 13 cases in the "favorable" group and 13 cases in the "unfavorable" group.There were 5 patients in the "treat and repair" group.The Rp/Rs(0.37±0.13)in the "repair" group were lower than that of the "treat and repair" group(0.62±0.22).During follow-up,all patients in "treat and repair" group had "favorable" outcome.The rate of "favorable"outcome in the "treat and repair" group(5/5,100%)were better than "repair" group(13/26,50%).4、There were 27 cases in "PVRI>8 Wood.m2" group.15 cases were closed defect directly and 14 patients were followed-up,l case withdraw.58.33%patients had "favorable" outcome during follow-up.There were 5 cases in the "treat and repair" group and 100%patients had "favorable" outcome during follow-up.5、There were 15 cases of residual pulmonary hypertension after operation in this study.12 patients were oral PAH-specific drugs after operation.There pulmonary arterial pressure decreased to normal during follow-upConclusionHeart catheterization and pulmonary angiography were had significance in the assessment of the condition and treatment options of patients with congenital heart disease complicated with pulmonary arterial hypertension.The positive rate of pulmonary vasodilation test in patients with congenital heart disease and pulmonary arterial hypertension was 10.91%.For patients with congenital heart disease and pulmonary arterial hypertension,if "PVRI<4 Wood.m2",closing the defect directly were recommend.If "PVRI≥4 Wood.m2","treat and repair" strategy may become a new treatment model.For pulmonary hypertension after congenital heart disease operation,PAH-specific drug can reduce pulmonary artery pressure. |