Background: Pulmonary arterial hypertension (PAH) is a serious condition that causes right heart failure. A persistent inflammatory process is present in the vascular endothelial tissue, which releases circulating CD34+,133+ endothelial progenitor cells (EPCs), possibly as a mechanism of response to vascular damage.

Material and methods: Using flow cytometry with MACS Quant Analyzer 10 equipment, the number and percentage of CD34+,133+ EPCs were determined in a cohort of 30 pediatric patients with PAH associated with congenital heart disease with right heart failure (n = 16) and without (n = 14).

Results: The population were male (56.5%), aged 8.4 ± 5.9 years old, and 4% carried Down syndrome. The EPCs showed a significant correlation with pulmonary mean arterial pressure (rho = 0.49, P = 0.04) and a negative correlation with oxygen saturation (rho = −0.75, P < 0.01) in the subgroup with right ventricle failure. Finally, the percentage and total number of CD34+,133+ EPCs showed significant diagnostic ability to discriminate right ventricle maladaptation (area under the curve = 0.85, P = 0.006 and 0.86, P = 0.004, respectively). The differences between the increase in EPCs CD34+,133+ and ProBNP levels also had a significant correlation (rho = −0.6, P < 0.001).

Conclusion: CD34+,133+ EPCs showed a strong and positive correlation with parameters reflecting the severity of right heart failure and could be useful as a clinical tool for early identification of right ventricle maladaptation in pediatric patients with secondary PAH.

KEY CONTRIBUTORS
Humberto García Aguilar, Juan Antonio Suárez Cuenca, Jose Luis Aceves Chimal, Centro Médico Nacional 20 de Noviembre ISSSTE, México City, Mexico

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