A useful review on this topic: Hepatology 2012; 56: 1160-69.

As a result of the successes of surgery for congenital heart disease (CHD), the majority of patients (~85%) with complex CHD survive into adulthood.  Hepatic complications are common in these patients either from the primary cardiac defect, from the surgical procedure, from transfusion or from medications.

Several liver diseases also have congenital heart disease manifestations include the following:

• Alagille syndrome: 25% with CHD including peripheral pulmonic stenosis and tetralogy of Fallot
• Abernethy malformation: ASD, VSD, and PDA
• Biliary atresia splenic malformation syndrome: numerous forms of CHD
• Mitochondrial fatty acid oxidation disorders: cardiomyopathy

CHD defects associated with hepatic dysfunction:

• Right-sided failure: single ventricle physiology after Fontan, d-Transposition, Eisenmenger syndrome, Ebstein’s anomaly, tetralogy of Fallot with pulmonary regurgitation.  These disorders lead to passive venous congestion of liver with hepatic zone 3 sinusoidal dilation and sometimes zone 3 necrosis due to ischemia.  Biochemically, a cholestasis pattern predominates.
• Left-sided failure: left ventricular outflow obstruction/coarctation of aorta, VSD, repaired complete atrioventricular septal defect.  This is associated with hypoperfusion and can lead to ischemia with increased transaminases as well as fibrosis.

Specific issues reviewed include the following:

• Acute cardiac dysfunction
• Cardiac dysfunction after Fontan
• Complications of portal hypertension
• Imaging (there have been reports of HCC following Fontan)
• Transplantation: isolated cardiac, isolated liver transplantation, combined heart-liver transplantation

Related blog entry:

Fontan and PLE | gutsandgrowth