BACKGROUND Pulmonary arterial hypertension (PAH) caused by congenital heart disease (CHD) is very common in clinics. Some studies have shown that PAH is related to the number of endothelial progenitor cells (EPCs), but there is no report on the relationship between PAH and the number of EPCs in children with CHD. METHODS In this study, a total of 173 cases with CHD (from 0 to 6 years old) were collected. According to the mean pulmonary arterial pressure (mPAP) measured by right heart catheterization, these cases were divided into PAH groups (including high PAH group, mPAP> 25 mmHg, n = 32, and the middle PAH group, 20 mmHg ≤ mPAP≤25 mmHg, n = 30) and non-PAH group (mPAP< 20 mmHg, n = 111). Peripheral blood was taken for flow cytometry, and the number of EPCs (CD133+/KDR+ cells) was counted. The number of EPCs /μL of peripheral blood was calculated using the following formula: EPCs /μL = WBC /L × lymphocytes % × EPCs % × 10- 6. RESULTS The median EPCs of the non-PAH group, middle PAH group and high PAH group is 1.86/μL, 1.30 /μL and 0.98/μL, respectively. The mPAP decreases steadily as the level of EPCs increases (P < 0.05). After adjustment of gender, age and BMI, the number of EPCs was significantly associated with a decreased risk of high PAH (OR = 0.37, 95% CI: 0.16-0.87, P < 0.05). However, EPCs was not significantly associated with middle PAH (P > 0.05). CONCLUSION The findings revealed that the EPCs and high PAH in patients with CHD correlate significantly and EPCs may become an effective treatment for PAH in patients with CHD. EPCs may be a protective factor of high PAH for children with CHD.

中文翻译：

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先天性心脏病患儿内皮祖细胞与肺动脉高压的关系。

背景技术由先天性心脏病（CHD）引起的肺动脉高压（PAH）在临床中非常普遍。一些研究表明，PAH与内皮祖细胞（EPC）的数量有关，但尚无关于CHD儿童PAH与EPC数量之间关系的报道。方法在本研究中，共收集了173例CHD（0至6岁）的病例。根据右心导管检查测得的平均肺动脉压（mPAP），将这些病例分为PAH组（包括高PAH组，mPAP> 25 mmHg，n = 32和中间PAH组，20 mmHg≤mPAP≤25 mmHg，n = 30）和非PAH组（mPAP <20 mmHg，n = 111）。抽取外周血进行流式细胞术，并计数EPC（CD133 + / KDR +细胞）的数量。EPC数/μL外周血的计算公式如下：EPCs /μL= WBC / L×淋巴细胞％×EPCs％×10-6。结果非PAH组，PAH中等组和高PAC组的中值EPC PAH组分别为1.86 /μL，1.30 /μL和0.98 /μL。随着EPC水平的提高，mPAP稳步下降（P <0.05）。调整性别，年龄和BMI后，EPC数量与高PAH风险降低显着相关（OR = 0.37，95％CI：0.16-0.87，P <0.05）。然而，EPCs与中间PAH无关（P> 0.05）。结论研究结果表明，冠心病患者的EPCs与高PAH呈显着相关，EPCs可能成为冠心病患者PAH的有效治疗方法。EPC对CHD儿童可能是高PAH的保护因素。