Background: The precise mechanisms of the increased incidence of hemostatic abnormalities in congenital heart disease (CHD) have not been determined. The aim of the study was to evaluate some indicators of activation of platelets and vascular endothelial cells in patients with CHD, evaluation of bleeding liability of these patients, and correlation with the clinical presentation of these patients.

Methods: This work was carried out on 20 patients with cyanotic congenital heart diseases (CCHD), 20 patients with acyanotic congenital heart diseases (ACHD), and 20 healthy children who served as the control group, aged between 1 and 10years. All were subjected to full clinical examination, complete blood count, oxygen saturation, echocardiography, bleeding and coagulation times, PT, PTT, FDPs, plasma soluble P-selectin, E-selectin, and platelet factor 4 (PF4).

Results: There was significant prolongation of PT and PTT, and there was a significant lowering of platelet counts. These results were obtained in CCHD and ACHD, but were more significant in CCHD patients. There was a significant elevation in PF4 (55.0±25.5ng/mL), P-selectin (128.9±42.44ng/dL), and E-selectin (9461.5±1701.24pg/mL) levels in children with CCHD as compared to those with ACHD (PF4, 21±7.94ng/mL; P-selectin, 80.1±13.2ng/mL; E-selectin, 7969.6±2127.5pg/mL), and significant increase in both groups when compared to the control group (PF4, 8.1±4.7ng/mL; P-selectin, 27.83±9.73ng/mL; E-selectin, 6750.00±3204.00pg/mL). There was a significant negative correlation between oxygen saturation, plasma P-selectin (r=-0.865), E-selectin (r=-0.401), and PF4 (r=-0.792) in patients with CCHD.

Conclusion: Patients with CHD-both cyanotic and acyanotic-have variable degrees of increased liability for both thrombosis and hemorrhage that represents some sort of adaptation to preserve hemostasis and to protect these patients against the clinical presentation of both thrombosis and bleeding. This is to say that CHD patients have their own point of balance between thrombogenicity and bleeding liability. Wide-scale studies are needed to detect the normal levels of different thrombohemorrhagic parameters of these patients.

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http://dx.doi.org/10.1016/j.hemonc.2017.07.001DOI Listing

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