Abstract: AbstractObjectiveTo explore the influencing factors in forming the secondary pulmonary hypertension of congenital heart disease. MethodsRandomly select 30 healthy people as healthy group.Randomly select 30 patients of left to right congenital heart disease (CHD) with normal pulmonary arterial pressure (PAP) and with pulmonary hypertension (PH).Compare the serum concentration of LArg,NO and plasma concentration of ET1 in different groups. ResultsSerum concentration of LArg: Control group(group A):(72.00±18.01)nmol/mL,Patients with normal PAP(group B),(30.74±8.97)nmol/mL,Patients with PH(group C): (23.51±12.37)nmol/mL.Serum concentration of NO: group A:(76.10±17.10)nmol/mL,group B: (90.55±26.57)nmol/mL,group C:(60.05±17.60)nmol/mL.Plasma concentration of ET1: group A:(50.82±7.58)pg/mL,group B: (64.9±16.28)pg/mL,group C: (69.64±10.66)pg/mL. ConclusionSerum concentration of NO and plasma concentration of ET1 and the balance relationship between them corporately affect the formation and the extent of PH of congenital heart disease.The increase of plasma concentration of ET1 is the direct factor,the decrease of the serum concentration of NO is the indirect factor,while the decrease of the serum concentration of NO is caused by the decrease of the serum concentration of LArg.

Key words: Endothelin , Larginine, Nitric oxide

摘要： 目的探讨先天性心脏病(CHD)患儿肺动脉高压(PH)形成的影响因素。 方法研究对象均为200306—200502于北京大学深圳医院收集病例，以健康者30名为对照组(A组)，以肺动脉压正常和并发PH的左向右分流的CHD患儿各30例为观察组(B组、C组)。以高效液相色谱法、硝酸还原法及放射免疫法测定其血清精氨酸(LArg)、一氧化氮(NO)、血浆内皮素(ET1)的浓度。 结果血清LArg浓度：对照组(A组)为(72.00±18.01)nmol/mL，肺动脉压正常的患儿(B组)为(30.74±8.97)nmol/mL，伴PH的患儿(C组)为(23.51±12.37)nmol/mL。血清NO浓度：A组为(76.10±17.10)nmol/mL，B组(90.55±26.57)nmol/mL，C组(60.05±17.60)nmol/mL。血浆ET1浓度：A组(50.82±7.58)pg/mL，B组(64.90±16.28)pg/mL，C组(69.64±10.66)pg/mL。 结论血清NO浓度和血浆ET1浓度及其之间的平衡关系共同影响PH的形成及其程度。血浆ET1浓度的升高是肺动脉压升高的直接因素，血清NO浓度的降低是间接因素，而血清NO浓度降低是由血清LArg浓度的降低引起。

关键词: 肺动脉高压, 左旋精氨酸, 一氧化氮, 内皮素