Protein C activity in severely ill newborns with congenital heart disease

Abstract

Levels of protein C functional activity were studied in twenty-nine full-term infants with symptomatic congenital heart disease, who presented in the neonatal period. Protein C levels on admission ranged from < 10% to 61% (mean 37.7% S. D. 14.1%). Eight of the twenty-nine babies had protein C levels between 1.5 and > 3.0 S. D. below the normal neonatal mean with no parental evidence of familial deficiency. Of these infants with low protein C two developed thrombotic complications and four had evidence of coagulation factor consumption. Critically ill infants were over-represented in the group with low protein C. Severely ill newborn infants with protein C at or below the lower limit of the normal neonatal range may be at increased risk of either a consumptive coagulopathy or major thrombosis.

Thrombotic occlusion of Blalock-Taussig shunt in a patient with unnoticed protein C deficiency

“…4 In our case, the combination of hereditary protein C defi ciency and the usage of foreign material (EPTFE tube graft) predisposed to thrombus formation inside the graft.…”

Abundance of Microembolic Signals Detected by Transcranial Doppler Ultrasound in a Patient with Eisenmenger’s Syndrome

“…days 3 and 10 in table 1) is not in favour of spontaneous erythrocyte aggregates as the correlate of the MES. Decreased protein C activity [3], spontaneously altered aPTT [5], low platelet counts and low vitamin-K-dependent clotting factors affecting the PT [4]have been described in patients with congenital cyanotic heart disease. The low platelet count may be due to shortened platelet survival, but the mechanisms of altered blood coagulation are not fully understood [5].…”

“…Patients with congenital cyanotic heart disease have numerous haematological and haemostatic abnormalities [3, 4, 5, 6, 7, 8]and probably a mildly increased risk of cerebral infarction [9, 10]. We report a case with congenital cyanotic heart disease, haematological and haemostatic abnormalities, a transient ischaemic attack and numerous microembolic signals on repeated transcranial Doppler recordings.…”

Neonatal Thrombosis: Are We Doing the Right Studies?

“…Experimental designs were used in only 10 reports ( Fig. 1): There were two prospective cohort studies to determine the incidence of thrombosis associated with central venous catheters, 5,6 one retrospective cohort study on the same topic 7 ; one retrospective cohort study on the incidence of the empty delta sign in cranial computed tomography 8 (a sign that has been interpreted to be suggestive of dural sinus thrombosis); one prospective cohort study on the incidence of venous sinus thrombosis detected by magnetic resonance imaging in infants considered at high risk for neurologic delay 9 ; two prospective cohort studies on the risk of thrombotic events in infants with low protein C activity 10,11 ; one prospective study on the risk of thrombotic events in stressed neonates with activation of the fibrinolytic system 12 ; one case-control study on the frequency of renovascular hypertension and leg growth abnormalities after aortic thrombosis in the neonatal period 13 ; and one prospective study to determine the risk of portal vein thrombosis after umbilical vein catheterization and exchange transfusion. 14…”