Basic therapy efficacy in children with pulmonary hypertension related to congenital heart defects: place of endothelial dysfunction and systemic inflammation markers

Aim. To evaluate efficacy of the specific therapy by results of 4-year observation of patients with pulmonary arterial hypertension associated with congenital heart defects (PH-CHD)

Material and methods. The evaluation is provided, of endothelium
condition dynamics during one year (baseline, in 6 and 12 months) by the level of endothelium dysfunction markers, cells reparation and proinflammatory factor — interleukin-1. Dynamics of clinical status of patients and of the condition of the right heart chambers by echocardiography with Doppler study, was monitored during 4 years.

Results. In severe PH patients under bosentan complex therapy the clinical improvement was marked as exercise tolerance improvement, decreased rate of pulmonary crises and decreased functional class of the disease, tendency to increase life duration. In bosentan group, there was 10-time reduction of vascular endothelial growth factor level, double decrease of sPECAM-1 and by 70% — of proinflammatory cytokine interleukin-1. In moderate and severe PH without bosentan therapy there was statistically significant increase of sPECAM-1, increase of vascular endothelial growth factor by 5 and 2 times, respectively, and progression of the disease.

Conclusion. For infants with PH-CHD, complex bosentan therapy makes it for clear clinical improvement related to antiproliferative and antiaggregant effects, which are mediated by non-selective inhibition of endothelin-1 receptors. Absence of PH-specific therapy determines the progression of disease, irrelevant to functional class.

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