Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation.

Abstract

Over time, long-term survival has dramatically increased for patients with complex congenital heart disease who undergo the Fontan operation. With this increased survival, it has become apparent that such a circulation has important consequences for other organ systems, particularly the liver and kidney. The adverse milieu created by chronic venous hypertension, low cardiac output, and an inflammatory state contribute to the pathologic changes observed in the liver and kidneys over the long term in Fontan patients. The clinical importance of these hepatic and renal comorbidities have only recently begun to be recognized in the context of increasing life expectancy in this population. The objectives of this review are to provide an overview of the pathophysiology of the Fontan circulation and how liver and kidney disease evolve in this setting; to summarize the current evidence base as it relates to the diagnostic approach to liver and kidney disease in Fontan patients; and to discuss the therapeutic approaches to Fontan- associated liver and kidney disease. Given that this is a very active area of research in congenital heart disease, we have identified knowledge gaps and priority research areas to improve the care of Fontan patients. These include establishing the optimal diagnostic tests to detect and track liver and kidney disease change over time, determining which treatable risk factors contribute to the development of liver and kidney disease, and evaluating therapies to prevent or slow progression of liver and kidney disease.