临床研究与实践-先天性心脏病并肺动脉高压患儿内源性二氧化硫与同型半胱氨酸的相关性

Correlation between endogenous sulfur dioxide and homocysteine in children with pulmonary arterial hypertension associated with congenital heart disease

目的 探讨先天性心脏病（CHD）相关性肺动脉高压（PAH）患儿血浆内源性二氧化硫（s02）、同型半胱氨酸（Hcy）的水平及其与肺动脉压力之间的关系，并分析其在疾病中的作用。

Objective To determine the relationship between the serum sulfur dioxide, homocysteine and the pulmonary arterial pressure in children with congenital heart defects who generated a pulmonary arterial hypertension syndrome （PAH-CHD） , and analyze their role in the pathological process of the disease. Method This was a prospective cohort study, children with systemic pulmonary shunt CHD were selected. The patients were devided into three groups ： the CHD with no PAH group ： n = 20,10 males, 10 females,5 with ventricular septal defect （VSD） , 8 with atrial septal defect （ASD） and 7 with patent ductus arteriosus （PDA） , mean age （ 1.9 ± 1.8 ） years ; the CHD with mild PAH group ： n = 20,10 males,10 females,12 with VSD,6 with ASD,and 2 with PDA,mean age （1.0 ±0. 8） year; the CHD with moderate or severe PAH group：n =20,8 males,12 females,12 with VSD,6 with ASD,and 1 with PDA,1 with ASD ＋ VSD,mean age （1.8 -＋ 1.6） year. Twenty healthy children were enrolled from outpatient department as the control group [ included 8 males, 12 females, mean age （ 1.9 ±1.5 ） years]. The homocysteine and SO2 concentrations in the serum samples were detected by a modified high performance liquid chromatographic method with fluorescence detection （HPLC-FD）, then, multiple comparisons among the groups were performed with analysis of variance, and the pearson correlation. Result The serum homocysteine concentrations were respectively （ 11.0 ± 2. 7 ）, （ 11.7 ± 2. 5 ）, （ 12. 0 ± 2. 1 ）, （ 14. 3 ± 3.2 ） μmol/L in the control group, CHD with no PAH group, CHD with mild PAH group ,and CHD with moderate or severe PAH group. According to the multiple comparisons, the CHD with moderate or severe PAH group had the highest level（P all 〈 0. 05 ）. While the comparison within the control group, CHD with none PAH group, and CHD with mild PAH group, the differences were not significant （P all 〉 0. 05 ）. The serum sulfur dioxide strength （concentrated asSO32-） were respectively （10.6 ±2.4）, （8.9 ±2.3）, （7.3 ±2.9）, （4.3 ±2.1） txmoL/L in the control group, CHD with none PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. CHD with moderate or severe PAH group had the highest level of serum sulfur dioxide （ P 〈 0. 05 ）. The pearson correlation analysis indicated that in the CHD children, the serum homoeysteine were positively correlated with the pulmonary arterial pressure （ r = 0. 481, P 〈 0. 01 ） , while, the sulfur dioxide were negatively correlated with pulmonary arterial pressure （r = -0. 553, P 〈 0. 01 ）. In all children,the serum homocysteine levels were negatively correlated with the sulfur dioxide（ r = -0. 231 ,P = 0. 039）. Conclusion The PAH-CHD children had higher homocysteine levels and lower sulfur dioxide levelsl,which demonstrated the disturbance of homocysteine-suffur dioxide pathway in the sulfur containing amino acids metabolish in the disease. The homocysteine may become a biological marker which reflecting the severities of the PAH-CI-ID, while the sulfur dioxide can be a new target for the therapy of PAH-CHD.