Liver disease secondary to congenital heart disease in children

Abstract

Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.

Fontan-associated liver disease and hepatocellular carcinoma in adults

“…Cardiac cirrhosis results from prolonged passive liver venous congestion secondary to right-sided congestive heart failure and is defined as stage-4 fibrosis on liver biopsy 38 . At this stage, obtaining liver samples is hampered by the risk of bleeding and ascites accumulation.…”

Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction

“…The continuous advancements in surgical technique and post-operative management have contributed to a consistent improvement in early and long-term survival. Since the introduction of the Fontan's operation in the early 1970s, a growing number of patients with Fontan circulation is now reaching adulthood, and they present with unexpected complications and with unfavorable outcome [21].…”

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

“…Hepatic fibrosis and HCC are known complications of patients who have undergone Fontan procedures as children, secondary to hepatic congestion and low cardiac output [137,138]. Ten years after Fontan palliation is the expected time point for development of cirrhosis and HCC, although cases have been published with cirrhosis occurring as early as 4-5 years post Fontan [139].…”