Congenital heart disease and the liver

Abstract

There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug‐related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart‐liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long‐term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. (HEPATOLOGY 2012;56:1160–1169)

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

“…Other possible causes for impaired hepatic venous outflow are BuddChiari syndrome or long-term survival after successful Fontan surgery [52]. In the latter, decreased cardiac index together with increased right atrial pressure (termed central venous hypertension), which results from direct attachment of the right atrium to the pulmonary artery, leads to liver damage and fibrosis [51].…”

Extra-cardiac manifestations of adult congenital heart disease

“…There are rare primary hepatic abnormalities that carry an association with CHD [42,43], but the majority of hepatic manifestations in CHD occurs secondary to hemodynamic perturbations and is not usually specific to particular CHD malformations. Cardiac disease of any cause can cause hepatic dysfunction through elevated hepatic vein pressure (congestive hepatopathy), decreased hepatic blood flow (ischemic hepatitis), or hypoxemia [44].…”

“…Rather, it can complicate any CHD presentation that includes poor cardiac output, including cardiomyopathy, hypotension, and arrhythmia (or combination thereof). The biochemical hallmark of ischemic hepatitis is elevation of aminotransferases to the thousands within 24 hours, along with elevations in bilirubin that lag in their rise and fall [42]. Management of ischemic hepatitis is aimed at increasing hepatic perfusion.…”

“…Hepatic dysfunction in CHD can result from iatrogenesis, especially in the context of medications (drug-related hepatitis) [42]. Of particular concern in ACHD are antiarrhythmic medications, especially amiodarone.…”

“…Encouragingly, amiodarone-induced hepatotoxicity does not appear to be increased in non-CHD-related right heart failure [48]. Iatrogenic hepatic injury in ACHD can also occur as a result of transfusion-related infections [42]. CHD patients who underwent heart surgery before 1992, when routine hepatitis C virus (HCV) blood screening was instituted, have higher rates of HCV infection [49].…”