Abstract
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.
Highlights
Protein C deficiency is a rare condition
present a clinical case of an intraoperative detection of protein C deficiency in a patient operated for cyanotic heart disease
premature babies with cyanotic heart defects belong to a high risk group of developing intraoperative thrombosis
Summary
Активность протеина С ( %) Activity of protein С ( %) 17–53 20–64 21–65 28–80 37–81 40–92 45–93 55–111 70–140. Классификация степени тяжести дефицита протеина С [12] Table 2. Classification of the severity of protein C deficiency [12]
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