论文部分内容阅读
目的探讨左向右分流型先天性心脏病(先心病)合并肺动脉高压(PAH)的发病机制。方法 2010年6月至2010年12月中国医科大学附属盛京医院住院的32例年龄1个月至5岁的左向右分流型先心病患儿,其中24例为先心病未合并PAH组(A组);8例为先心病合并PAH组(B组)。均收集血清并行心脏彩超检查。同时收集31例年龄1个月至5岁的健康儿童血清作为对照组(C组)。测定并比较3组血清中一氧化氮(NO)、一氧化氮合酶(NOS)、L-精氨酸(L-arg)及L-低氨酸(L-cit)。结果 A组NO为(29.30±24.22)μmol/L、B组为(18.95±12.54)μmol/L,均明显低于C组[(54.94±40.80)μmol/L],差异有统计学意义(P<0.05),尤以B组下降更为明显。A组NOS活力为(13.33±6.31)U/mL、B组为(14.00±6.25)U/mL,均明显低于C组[(31.04±10.02)U/mL],差异有统计学意义(P<0.05);A组与B组间NOS活力差异无统计学意义(P>0.05)。B组L-arg[(11.31±3.38)μmol/L]明显低于C组[(15.99±4.04)μmol/L]和A组[(16.36±3.20)μmol/L],差异有统计学意义(P<0.05);A组与C组间L-arg差异无统计学意义(P>0.05)。3组间L-cit差异无统计学意义(P>0.05)。结论先心病患儿因内皮系统功能障碍,导致血清内NO含量和NOS活力明显降低。随疾病进展,一旦合并PAH,作为内源性NO生成底物的L-arg消耗过多,机体不能完全代偿时,血清内L-arg浓度也会明显降低。
Objective To investigate the pathogenesis of left-sided shunt congenital heart disease (CHD) complicated with pulmonary hypertension (PAH). Methods From June 2010 to December 2010, 32 children aged 1 month to 5 years with left-to-right shunt congenital heart disease admitted to Shengjing Hospital affiliated to China Medical University were enrolled. Among them, 24 cases were CHD without PAH A group); 8 cases of congenital heart disease with PAH group (B group). Serum were collected for cardiac echocardiography. At the same time, 31 healthy children aged from 1 month to 5 years old were collected as the control group (C group). Serum levels of nitric oxide (NO), nitric oxide synthase (NOS), L-arg and L-cit were determined and compared. Results The NO level in group A was (29.30 ± 24.22) μmol / L and in group B was (18.95 ± 12.54) μmol / L, which were significantly lower than those in group C [(54.94 ± 40.80) μmol / L, P <0.05), especially in group B decreased more obviously. The activity of NOS in group A was (13.33 ± 6.31) U / mL and in group B was (14.00 ± 6.25) U / mL, which were significantly lower than those in group C [(31.04 ± 10.02) U / mL] <0.05). There was no significant difference in NOS activity between group A and group B (P> 0.05). (11.31 ± 3.38) μmol / L in group B was significantly lower than that in group C (15.99 ± 4.04 μmol / L) and in group A (16.36 ± 3.20 μmol / L), the difference was statistically significant ( P <0.05). There was no significant difference in L-arg between group A and group C (P> 0.05). There was no significant difference in L-cit between the three groups (P> 0.05). Conclusion Due to endothelial dysfunction in children with congenital heart disease, the content of NO and the activity of NOS in serum decrease significantly. As the disease progresses, once PAH is combined, L-arg, which is the substrate for endogenous NO production, is consumed excessively, and when the body can not fully compensate, the L-arg level in the serum is significantly reduced.