Objective To assess the serum level of stromal-cell-derived factor 1 (SDF-1) in patients with congenital heart disease (CHD) and pulmonary arterial hypertension. Methods The heart color Doppler ultrasound was conducted to measure the tricuspid regurgitation velocity, in order to estimate pulmonary artery systolic pressure. According to the existing standards, a total of 86 patients were divided into CHD without pulmonary hypertension group (n=16), mild pulmonary hypertension group (n=18), moderate pulmonary hypertension group (n=17), severe pulmonary hypertension group (n=15). Another 20 healthy chil-dren were recruited as control group (n=20), The serum level of SDF-1 was detected by the ELISA method. Results The serum SDF-1 levels were signiifcantly lower in CHD groups than in control group (F=27.793, P<0.001). In CHD groups, CHD with se-vere pulmonary hypertension group had the lowest SDF-1 level, and CHD without pulmonary hypertension group had the highest SDF-1 level. There were signiifcant differences between CHD groups (P<0.05). The pulmonary artery systolic pressure was cor-related with serum SDF-1 levels (r=-0.737, P<0.001). Conclusions The serum level of SDF-1 is decreased in patients with CHD, and is negatively correlated with the severity of pulmonary arterial hypertension.%目的：探讨先天性心脏病（CHD）伴肺动脉高压患儿血清基质细胞衍生因子1（SDF-1）水平的变化。方法66例CHD患儿，采用心脏彩色多普勒超声测算三尖瓣返流速度，并估算肺动脉收缩压；根据肺动脉压，分为无肺高压组16例，轻度肺高压组18例，中度肺高压组17例，重度肺高压组15例；另20例健康儿童作为对照组。以ELISA法检测并比较各组患儿血清SDF-1水平。结果各组CHD患儿与对照组的血清SDF-1水平差异有统计学意义（F=27.793，P<0.001）。各组CHD患儿的SDF-1水平均低于对照组；CHD患儿中，重度肺高压组的血清SDF-1水平最低，无肺高压组的血清SDF-1水平最高，各组间的差异均有统计学意义（P均<0.05）。CHD患儿的肺动脉收缩压与血清SDF-1水平呈负相关（r=-0.737，P<0.001）。结论血清SDF-1水平在CHD患儿中下降，并且与肺动脉高压的严重程度呈负相关。
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