ObjectiveThe total cavopulmonary connection (TCPC) offers a palliation for the hemodynamic derangements associated with congenital heart lesions characterized by a single functional ventricle, but it may cause acute hepatic injury because of the special physiology. The objective of this study was to characterize hepatic function and its relationship to cardiac function in children who had undergone the Fontan procedure. MethodsWe retrospectively analyzed 114 children who had undergone TCPC operation in Shanghai Children's Medical Center between January 2013 and March 2014. There were 65 males and 49 females with a median age of 3.8 years (range 2.5 to 13.2) and a median weight of 14.8 kg (range 12.0 to 33.0). The study cohort was further divided into three groups according to the Child-Pugh classification. The total scores were calculated regarding to ascite, bilirubin, albumin, and international normalized ratio (INR). The scores from 4 to 5 were classified in Child A group, from 6 to 8 classified in Child B group, from 9 to 11 classified in Child C group. Thirty-four patients met criteria for Child Class A, 53 patients for Child Class B, and 27 patients for Child Class C. The univariate analysis and multivariable logistic regression model were used to compare demographic, anatomic, and physiological variables among the three groups. ResultsWithin the study population, 80 patients of Child B group and Child C group met criteria for acute hepatic injury. Univariate risk factors for acute hepatic injury included longer total bypass time (P=0.044), longer aortic cross-clamping time (P=0.005), longer ventilation time (P=0.000), higher postoperative mean pulmonary arterial pressure (P=0.000), elevated N-terminal pro-brain natriuretic peptide (P=0.001), higher vasoactive inotropic score (P=0.000), lower mixed venous oxygen saturation (SvO2, P=0.000) and arterial oxygen saturation (P=0.001), higher incidence of arrhythmia (P=0.000), and low cardiac output syndrome (P=0.003), the need of peritoneal dialysis (P=0.000). In the multivariable logistic model, the need for peritoneal dialysis (OR=17.018, 95%CI 5.117-56.602) and the lower postoperative SvO2 (OR=0.922, 95%CI 0.871-0.976) were two independent risk factors for acute hepatic injury after the TCPC. ConclusionThe need for peritoneal dialysis and lower postoperative SvO2 may represent the compound effects of multiple risk factors including preoperative hemodynamic and a marked hepatic vascular inflammatory response to surgery and cardiopulmonary bypass, which in turn may mediate acute hepatic injury.