Cлучай интраоперационного выявления дефицита протеина C у больного, оперированного по поводу цианотического порока сердца

Background . Protein C deficiency is a rare condition. However, this pathology occurs more frequently in premature babies with cyanotic heart disease. Aim . To present a clinical case of an intraoperative detection of protein C deficiency in a patient operated for cyanotic heart disease. General findings . A clinical case of an intraoperative recurrent thrombosis of systemic-to-pulmonary shunts in a newborn patient with cyanotic heart disease is described. Newborn and premature babies with cyanotic heart defects belong to a high risk group of developing intraoperative thrombosis due to a deficiency of natural anticoagulants, which can occur as a result of genetically conditioned protein C deficiency, prematurity, immaturity of the liver or its reduced synthetic function, heart failure and hypoxemia. Additional pre-operative screening is required for detecting a deficiency of natural anticoagulants in this group of patients. The hospitals that perform such surgery should be equipped with protein C concentrates.