Odegard Kirsten C, Zurakowski David, Hornykewycz Stephan, DiNardo James A, Castro Robert A, Neufeld Ellis J, Laussen Peter C
Department of Anesthesiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Ann Thorac Surg. 2007 May;83(5):1797-803. doi: 10.1016/j.athoracsur.2006.12.030.
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).
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.
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.
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.
单心室生理的儿童中已描述了涉及促凝血和抗凝血蛋白的多种凝血因子异常。本研究使用年龄匹配的对照来评估双心室先天性心脏病（CHD）患儿的凝血因子。
对120例CHD患者的凝血因子进行检测，分为四个年龄组：第1组，0至3个月；第2组，3至12个月；第3组，12至48个月；第4组，48个月以上。将无CHD的健康儿童作为对照。通过标准检测方法测量因子II、V、VII、VIII、IX和X；蛋白C和S、纤溶酶原和抗凝血酶III的浓度。正常范围通过经验性95%置信区间确定。
与对照组相比，第1、2和3组患者的促凝血和抗凝血因子平均水平均显著降低，但第4组未发现差异。在第1组中，除纤维蛋白原和蛋白S外，所有变量的浓度均显著降低；在第2组中，除纤维蛋白原、因子VIII和IX、纤溶酶原和蛋白S外，所有变量的浓度均显著降低；在第3组中，除纤维蛋白原、因子VIII和IX、抗凝血酶III、纤溶酶原和蛋白S外，所有变量的浓度均显著降低。
与年龄匹配的对照组相比，双心室CHD的新生儿和婴儿促凝血和抗凝血因子水平较低，4岁以上儿童的水平接近正常。这些凝血因子异常与单心室生理患者中描述的异常相似。
