先天性心脏病合并肺动脉高压患儿57例临床分析

Clinical study on congenital heart disease with pulmonary arterial hypertension in children

目的分析先天性心脏病合并肺动脉高压患儿的临床资料,以提高对该病的临床诊治水平。方法 57例患儿均予以吸氧、抗感染以及强心、利尿、扩血管治疗。11例左向右分流型先天性心脏病以及5例复杂型先天性心脏病经内科治疗后肺部感染、心力衰竭难以控制,转我院心外科予以手术。术后8例患儿用前列地尔,6例患儿用西地那非降肺动脉压力。患儿于内科治疗前后、手术后药物治疗前后测肺动脉压力(PAP)、左室射血分数(LVEF)及血浆N端-脑钠肽前体(NT-proBNP)。结果 38例左向右分流型先天性心脏病患儿经内科治疗后PAP、血浆NT-proBNP水平均较治疗前下降(P<0.05),LVEF较治疗前升高(P<0.05)。11例左向右分流型先天性心脏病以及5例复杂型先天性心脏病患儿PAP、血浆NT-proBNP水平均高于前述38例患儿(P<0.05),LVEF低于前述38例患儿(P<0.05)。8例患儿术后予以前列地尔,6例予以西地那非治疗后,PAP、血浆NT-proBNP水平较用药前下降(P<0.05),LVEF较用药前升高(P<0.05)。结论先天性心脏病合并肺动脉高压需早治疗,可改善预后。前列地尔、西地那非在治疗肺动脉高压方面是有效的。

Objective To analyze clinical data of congenital heart disease（CHD） complicating with pulmonary arterial hypertension（PAH） in children,in order to improve the level of clinical diagnosis and treatment for the disease.Methods Fifty-seven pediatric patients were given oxygen uptake,anti-infection and cardiac tonic,diuretic,and enlarge vascular drugs.Pulmonary artery pressure（PAP）,left ventricular ejection fraction（LVEF） and NT-terminal pro-brain natriuretic peptide（NTproBNP） were monitored respectively before and after medical treatment,or before and after drug therapy after operation.Eleven cases of left to right shunt CHD and five cases of complex CHD were turned to cardiac surgery and operated after their pulmonary infection and cardiac failure were hard to control with medical treatment.Prostaglandin E1was administered to eight pediatric inpatients and sildenafil was administered to six pediatric inpatients after operation.Results PAP and NT-proBNP in 38 cases of left to right shunt CHD were dropped,and LVEF was rose after medical treatment（P0.05）.PAP and NT-proBNP in 11cases of left to right shunt CHD and 5cases of complex CHD were higher than those of the other 38cases,and LVEF was lower than that of the other 38cases（P0.05）.PAP and NT-proBNP in eight patients were dropped,and LVEF were rose,after they were administered prostaglandin E1.PAP and NT-proBNP in six cases were dropped,and LVEF were rose,after they were administered sildenafil.Conclusion CHD with PAH should be early treated to improves the prognosis.Prostaglandin E1and sildenafil were effective in therapy for PAH.