A better understanding of the current gaps in knowledge, clinical care and research of Fontan-associated liver disease (FALD) is critical to the development of a standardized prevention, screening and treatment approach for Fontan patients, according to a report published Dec. 18 in the Journal of the American College of Cardiology.

Curt J. Daniels, MD, FACC, et al., summarize findings from an earlier meeting of FALD stakeholders and establish a platform to better understand and address FALD. As the majority of patients born with single-ventricle anatomy who had the Fontan operation as children have reached adulthood, FALD has emerged as a late extracardiac complication. FALD and other late complications have a negative effect on patients’ quality of life and could lead to premature death. Although survival rates to adulthood for patients with congenital heart disease (CHD) is close to 90 percent, survival rates to a similar age for patients with severe CHD, including those with single-ventricle anatomy, is much lower at 56 percent. In addition, long-term follow up of patients who had the Fontan procedure suggests 59 percent freedom from adverse cardiovascular events five years post-operation, but only 29 percent freedom after 15 years. This suggests an “important emerging medical crisis,” the authors write.

According to the report, an accurate definition of FALD that is widely recognized is necessary for investigators to conduct research that will lead to improved understanding of FALD epidemiology, causation, pathophysiology, prevention and treatment. In addition, research into cardiac and extracardiac mechanisms that contribute to patients’ risk for FALD could help identify those at higher risk of the disease and enable earlier screening. A longitudinal registry of patients who have had the Fontan procedure could help advance these research efforts, according to the report.

The report committee recommends several practices for prevention, screening and treatment of FALD. For prevention, the authors advise that providers focus on optimizing underlying Fontan physiology and preventing liver injury before and after a Fontan operation. Screening for FALD should, at a minimum, include baseline liver function tests five years post-Fontan operation for children, or sooner if a liver injury or Fontan failure is suspected. Adults and adolescents who have had the Fontan procedure should have liver function tests every one to three years. Routine physicals should include laboratory studies; evaluation of Hepatitis A, B and C exposure; and liver imaging. In addition, the report outlines considerations for Fontan patients undergoing evaluation for heart or liver transplantation or combined heart and liver transplantation.

Collaboration between health care societies and organizations, research institutions, and subspecialists involved in treatment of Fontan patients will be critical to mobilizing resources necessary to improve understanding and treatment of FALD. The authors conclude that moving forward, “We need an investment toward research and technologies post-Fontan operation that matches our past and current commitment to pre-Fontan patients.”

Keywords: Child, Adolescent, Adult, Fontan Procedure, Liver Function Tests, Liver Transplantation, Survival Rate, Mortality, Premature, Investments, Follow-Up Studies, Hepatitis A, Quality of Life, Liver Diseases, Research, Heart Defects, Congenital, Registries

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