Tomita H, Yamada O, Ohuchi H, Ono Y, Arakaki Y, Yagihara T, Echigo S
Department of Pediatrics, National Cardiovascular Center, Suita, Osaka, Japan.
Cardiol Young. 2001 Jan;11(1):62-6. doi: 10.1017/s1047951100012439.
We analyzed retrospectively the relationship between coagulation profile, and either hepatic function or hemodynamics, in patients who had undergone a Fontan-type procedure, comparing them, first, with a control group of 12 patients without significant hemodynamic abnormality, and, second, with a group of 14 patients who had not undergone a Fontan procedure, but whose mean right atrial pressure exceeded 8 mmHg. Follow-up catheterization had been performed in all 30 patients submitted to the Fontan-type operation. Prothrombin time, and factor XIII, were significantly lower in those who had undergone the Fontan procedure than in the other groups. Those submitted to the Fontan operation also had lower levels of protein C than controls, and their levels of plasminogen were lower than the patients with high right atrial pressure. Both aspartate aminotransferase and alanine aminotransferase were higher in those undergoing the Fontan procedure than in the other groups, while gamma-glutamyltranspeptidase in these patients was higher than in the control group. Mean right atrial pressure was highest in those undergoing the Fontan procedure, while cardiac index was lowest. Prothrombin time was correlated to some extent with aspartate aminotransferase, mean right atrial pressure, and cardiac index. Protein C correlated with both aspartate aminotransferase and mean right atrial pressure, while factor XIII correlated with alanine aminotransferase, mean right atrial pressure, and cardiac index. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltranspeptidase, parameters of hepatic function, correlated significantly with mean right atrial pressure. In those who had undergone the Fontan procedure, decreased synthesis of pro-and anti-coagulant factors is a risk factor for both thrombosis and bleeding. Abnormal hemodynamics, in the absence of a right sided pumping chamber, may predispose to subclinical hepatic dysfunction, leading to selective disturbances of protein synthesis.
我们回顾性分析了接受Fontan类手术患者的凝血指标与肝功能或血流动力学之间的关系，首先将他们与12名无明显血流动力学异常的对照组患者进行比较，其次与14名未接受Fontan手术但平均右心房压力超过8 mmHg的患者进行比较。所有接受Fontan类手术的30例患者均进行了随访导管检查。接受Fontan手术患者的凝血酶原时间和因子XIII显著低于其他组。接受Fontan手术患者的蛋白C水平也低于对照组，其纤溶酶原水平低于右心房压力高的患者。接受Fontan手术患者的天冬氨酸转氨酶和丙氨酸转氨酶均高于其他组，而这些患者的γ-谷氨酰转肽酶高于对照组。接受Fontan手术患者的平均右心房压力最高，而心脏指数最低。凝血酶原时间在一定程度上与天冬氨酸转氨酶、平均右心房压力和心脏指数相关。蛋白C与天冬氨酸转氨酶和平均右心房压力均相关，而因子XIII与丙氨酸转氨酶、平均右心房压力和心脏指数相关。作为肝功能指标的天冬氨酸转氨酶、丙氨酸转氨酶和γ-谷氨酰转肽酶与平均右心房压力显著相关。在接受Fontan手术的患者中，促凝血和抗凝血因子合成减少是血栓形成和出血的危险因素。在没有右侧泵血腔室的情况下，异常的血流动力学可能易导致亚临床肝功能障碍，从而导致蛋白质合成的选择性紊乱。
