Chaloupecký V, Svobodová I, Hadacová I, Tomek V, Hucín B, Tláskal T, Janousek J, Reich O, Skovránek J
Kardiocentrum, University Hospital Motol, V úvalu 84, 150 06 Prague 5, Czech Republic.
Heart. 2005 Jan;91(1):73-9. doi: 10.1136/hrt.2003.026419.
To examine coagulation factors and liver function test abnormalities in patients after total cavopulmonary connection (TCPC).
Cross sectional study comprising clinical and echocardiographic evaluation, and biochemical and coagulation profile screening.
Tertiary referral centre.
102 patients aged 4-24 years (median 10 years) at one to eight years (median five years) after TCPC were examined. All patients were maintained on a low dose of aspirin. 96% of patients were in a good clinical condition (New York Heart Association class I or II). No intracardiac thrombi were detected on echocardiography and ventricular function was good in 91% of patients.
Total bilirubin was increased in 27% and gamma glutamyltransferase in 54% of patients. Serum total protein, albumin, and prealbumin were normal in almost in all patients. Compared with the control group, patients after TCPC had significantly lower fibrinogen, factor V, factor VII, and protein C concentrations, prolonged international normalised ratio, and increased antithrombin III concentration. Factor V concentration was abnormally decreased in 35%, factor VII in 16%, and protein C in 28% of patients. Antithrombin III was increased in 23% of patients. Factor VII, factor V, protein C, and antithrombin III correlated significantly with serum prealbumin. There was also a significant correlation between procoagulant factor VII and both anticoagulant protein C and antithrombin III.
Almost half of patients after TCPC had laboratory signs of mild cholestasis. Decreased liver synthesis of procoagulant and anticoagulant factors was observed but overall coagulation homeostasis appeared to be in balance in this selected group of patients with a good clinical outcome.
研究全腔静脉肺动脉连接术（TCPC）后患者的凝血因子及肝功能检查异常情况。
横断面研究，包括临床及超声心动图评估，以及生化和凝血指标筛查。
三级转诊中心。
对102例年龄在4至24岁（中位数10岁）、在TCPC术后1至8年（中位数5年）的患者进行检查。所有患者均服用低剂量阿司匹林。96%的患者临床状况良好（纽约心脏协会心功能分级为I或II级）。超声心动图检查未发现心内血栓，91%的患者心室功能良好。
27%的患者总胆红素升高，54%的患者γ-谷氨酰转移酶升高。几乎所有患者的血清总蛋白、白蛋白和前白蛋白均正常。与对照组相比，TCPC术后患者的纤维蛋白原、凝血因子V、凝血因子VII和蛋白C浓度显著降低，国际标准化比值延长，抗凝血酶III浓度升高。35%的患者凝血因子V浓度异常降低，16%的患者凝血因子VII异常降低，28%的患者蛋白C异常降低。23%的患者抗凝血酶III升高。凝血因子VII、凝血因子V、蛋白C和抗凝血酶III与血清前白蛋白显著相关。促凝血因子VII与抗凝血蛋白C和抗凝血酶III之间也存在显著相关性。
几乎一半的TCPC术后患者有轻度胆汁淤积的实验室检查迹象。观察到肝脏促凝血和抗凝血因子合成减少，但在这组临床结局良好的特定患者中，总体凝血稳态似乎保持平衡。
