First Department of Paediatrics, Medical School, National and Kapodistrian University of Athens, “Aghia Sophia” Children’s Hospital, 115 27 Athens, Greece
Ioannis Papassotiriou
First Department of Paediatrics, Medical School, National and Kapodistrian University of Athens, “Aghia Sophia” Children’s Hospital, 115 27 Athens, Greece
Zoi Iliodromiti
Neonatal Department, Aretaieio Hospital, Medical School, National and Kapodistrian University of Athens, 115 28 Athens, Greece
Rozeta Sokou
Neonatal Department, Aretaieio Hospital, Medical School, National and Kapodistrian University of Athens, 115 28 Athens, Greece
Elena Bouza
2nd Neonatal Intensive Care Unit, “Aghia Sophia” Children’s Hospital, 115 27 Athens, Greece
Marianna Politou
Blood Transfusion Department, Aretaieio Hospital, Medical School, National and Kapodistrian University of Athens, 115 28 Athens, Greece
Nicoletta Iacovidou
Neonatal Department, Aretaieio Hospital, Medical School, National and Kapodistrian University of Athens, 115 28 Athens, Greece
Theodora Boutsikou
Neonatal Department, Aretaieio Hospital, Medical School, National and Kapodistrian University of Athens, 115 28 Athens, Greece
Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.
