The development of cardiosurgical care for paediatric and neonatal patients is undergoing the rapid growth. Complex, multi-stage reconstructive operations and the use of invasive monitoring are associated with high risk of venous and arterial thrombosis.The cardiac surgery patient is inherently unique, since it requires controlled anticoagulation during cardiopulmonary bypass. Moreover, the most cardiovascular pediatric patients require antithrombotic measures over the perioperative period. In addition to medication support with the use of various groups of antithrombotic agents, vascular access management is justified in order to minimize the risk of thromboembolic complications, which can affect both the functional status, and common and inter-stage mortality.The purpose of this review was to systematize the available data on risk factors contributing to the development of thrombotic complications in patients with congenital heart disease.An information search was carried out using Internet resources (PubMed, Web of Science, eLibrary.ru); literature sources for period 2015–2020 were analysed. As a result of the analysis of the literature data age-dependent features of the haemostatic system, and associated with the defect pathophysiology, and undergone reconstructive interventions were described. The issues of pathophysiology of univentricular heart defects and risk factors associated with thrombosis were also covered.Moreover, aspects of intraoperative anti-thrombotic support are discussed, as well as measures to prevent thromboembolic complications in this population.Coordinated actions of haematologists, cardiologists, anaesthesiologists, intensivists, and cardiac surgeons will allow achieving a fine balance between risks of bleeding and thrombosis in the population of paediatric patients undergoing cardiovascular surgery.

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