Open AccessA clinical case of protein C deficiency revealed in a patient during surgery for cyanotic heart defect
Author(s) -
А.А. Гончаров,
М.М. Рыбка,
Д.Я. Хинчагов,
Е А Рогальская
Publication year - 2020
Publication title -
gematologiâ i transfuziologiâ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.126
H-Index - 5
eISSN - 2411-3042
pISSN - 0234-5730
DOI - 10.35754/02345730-2020-65-1-61-69
Subject(s) - medicine , hypoxemia , heart disease , heart failure , cardiology , thrombosis , cardiac surgery , surgery
Background . Protein C deciency 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 deciency in a patient operated for cyanotic heart disease. General ndings . 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 deciency of natural anticoagulants, which can occur as a result of genetically conditioned protein C deciency, prematurity, immaturity of the liver or its reduced synthetic function, heart failure and hypoxemia. Additional pre-operative screening is required for detecting a deciency of natural anticoagulants in this group of patients. The hospitals that perform such surgery should be equipped with protein C concentrates.