Evaluation of the coagulation system in children with two-ventricle congenital heart disease.
BACKGROUND
Multiple coagulation factor abnormalities involving both procoagulant and anticoagulant proteins have been described in children with single-ventricle physiology. This study used age-matched controls to evaluate coagulation factors in children with two-ventricle congenital heart disease (CHD).


METHODS
Coagulation factors were assayed in 120 patients with CHD, divided into four age groups: group 1, 0 to 3 months; group 2, 3 to 12 months; group 3, 12 to 48 months; and group 4, older than 48 months. Healthy children without CHD were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X; protein C and S, plasminogen, and antithrombin III, were measured by standard assays. Normal ranges were determined by the empirical 95% confidence intervals.


RESULTS
Significant reductions were found in mean levels of both procoagulant and anticoagulant factors in patients in groups 1, 2, and 3 compared with controls, but no differences were found in group 4. In group 1, all variables had significantly lower concentrations except fibrinogen and protein S; in group 2, all variables had significantly lower concentrations except for fibrinogen, factors VIII and IX, and plasminogen and protein S; and in group 3, all variables had significantly lower concentrations except fibrinogen, factors VIII and IX, and antithrombin III, plasminogen, and protein S.


CONCLUSIONS
Neonates and infants with two-ventricle CHD have lower levels of procoagulant and anticoagulant factors compared with aged-matched controls approaching normal levels in children aged older than 4 years. These coagulation factor abnormalities are similar to those described in patients with single-ventricle physiology.
Odegard
Kirsten C
KC
Department of Anesthesiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA. kirsten.odegard@childrens.harvard.edu
Zurakowski
David
D
Hornykewycz
Stephan
S
DiNardo
James A
JA
Castro
Robert A
RA
Neufeld
Ellis J
EJ
Laussen
Peter C
PC
D001777:Blood Coagulation / Q000502:physiology*; D001779:Blood Coagulation Factors / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011446:Prospective Studies
D001779:Blood Coagulation Factors