Abstract

BackgroundPulmonary 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.MethodsIn 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.ResultsThe 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).ConclusionThe 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.

Highlights

  • Pulmonary arterial hypertension (PAH) caused by congenital heart disease (CHD) is very common in clinics

  • – Pulmonary arterial hypertension (PAH) caused by congenital heart disease (CHD) is very common in the clinic, about 4–10% of CHD patients develop PAH, the prevalence of CHD-related PAH is about 25 people per million in the general population; patients with CHD and PAH have at least a doubled mortality risk compared with patients with CHD without PAH. – To our knowledge, there were few studies of the relationship between endothelial progenitor cells (EPCs) and CHD-related PAH

  • We adjusted age and gender, and examined the associations between the number of EPCs and mean pulmonary arterial pressure (mPAP) levels, and the results showed that the number of EPCs was significantly associated with a decreased risk of high PAH (OR = 0.38, 95%CI: 0.17–0.87, P < 0.05)

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Summary

Introduction

Pulmonary arterial hypertension (PAH) caused by congenital heart disease (CHD) is very common in clinics. Pulmonary arterial hypertension (PAH) is a common clinical disease which can exist independently. It is mainly the manifestation of many diseases when they progress to a certain stage, namely secundum PAH [1]. Left-to-right shunt congenital heart disease (CHD) will increase the right cardiac blood flow, damaging the pulmonary artery and causing PAH [2]. The statistics show that about 4–10% of CHD patients develop PAH [3,4,5,6,7]. PAH is very common in children, mainly due to CHD [2, 7, 8]

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