Introduction
Platelets in congenital cyanotic heart disease (CCHD) were occasionally characterized by significantly enhanced expression of P-selectin in resting circulating platelets as well as their augmented activation in response to stimulating agents. P-selectin is present in the alpha granule membrane as well as Weibel-Palade body membrane of endothelial cells. It is translocated to platelet membrane after activation; thus, its expression on platelet surface is an indicator of platelet activation in vivo. The occurrence of the prothrombotic variant of GPIIIa in cyanotic children may be associated with enhanced activation of circulating platelets.
Purpose
To study the platelet functions in patients with CCHD by determination of Pselectin expression and the GPIIIa polymorphism and to correlate these findings with other clinical, radiological, and laboratory parameters in these patients.
Methods
This study included 47 patients, with cyanotic congenital heart disease with decreased pulmonary blood flow, attending the Pediatric Cardiology Clinic. They were divided into two groups: Group I: 31 patients with congenital cyanotic heart disease before cardio surgery Group II: 16 patients with congenital cyanotic heart disease 3–6 months after palliative or corrective cardio surgery, Group III: 12 age and sex matched normal children were included in the study as control group. They were subjected to Flow-cytometric evaluation of platelet activation by Pselectin expression: P-selectin expression was estimated using monoclonal antibody.-Typing of GPIIIa gene polymorphism was done.
Results
Statistically significant higher P-selectin levels were found in patients compared with controls being significantly higher in patients <3 years of age. There was significant negative correlation between P-selectin level and O2 in patients. The incidence of prothrombotic variant A2 was described as phenotype positive. Statistically significant higher levels of P-selectine were found in positive phenotype patients than positive phenotype controls
Conclusion
Enhanced platelet activation may be an important contributor in the high thrombotic liability in congenital cyanotic heart disease. This may be attributed in part to genetic factors
Funding Acknowledgement
Type of funding source: None
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