We evaluated the preoperative prevalence of risk factors for liver disorders and the relationship between the liver disorders and perioperative outcomes in adult congenital heart disease (ACHD) patients. This retrospective study included 32 ACHD patients who underwent reoperative cardiac surgery. Preoperatively, 38% of the study patients had risk factors, including congestive liver (CL) due to right heart failure (31%), chronic hepatitis C (HC) (22%), and both CL and HC (16%). The numbers of patients with Child-Pugh scores 5, 6, 7 and 8 were 22, 7, 2 and 1. Median (range) preoperative platelet count and fibrinogen values were 155 (61-330) x 10(3)/mu l and 250 (145-367) mg/dl, respectively. The patients with higher Child-Pugh scores tended to have longer duration of anesthesia and surgery (p = 0.078, 0.078, respectively), and had significantly higher platelet transfusion (p = 0.031). Lower platelet count was associated with longer duration of anesthesia, surgery and cardio pulmonary bypass (CPB), and larger amount of blood loss and platelet transfusion (p = 0.01, 0.011, 0.024, 0.033, 0.021). Lower fibrinogen value was associated with longer duration of anesthesia, surgery and CPB, and larger amount of platelet transfusion (p = 0.015, 0.009, 0.009, 0.023). ACHD patients who underwent reoperative cardiac surgery had a high prevalence of risk factors for liver disorders preoperatively, and liver disorders aggravated some intraoperative outcomes. These findings suggest that the prevention of liver disorders is important for reducing the occurrence of poor outcomes, and that ACHD patients with liver disorders need attentive perioperative management.