Children’s Hospital of Philadelphia

Fontan-Associated Liver Disease (FALD) Study
$200,000 over two years

Patients born with complex heart disease, specifically single ventricle heart disease, undergo palliation with a Fontan operation in which all the blood flow that would traditionally return to the heart is directed directly to the lungs. These patients are at risk for advanced liver fibrosis and cirrhosis which is known as Fontan-Associated Liver Disease (FALD).  

The mechanism is not fully understood but hypothesized to be secondary to altered blood flow to the liver because of the Fontan hemodynamics/circulation. Specifically, reliance on the right ventricle alone to manage circulation to the body and high venous pressures innate to Fontan circulation likely place the liver at significant risk. An additional challenge is that conventional liver function tests employed for primary liver disease maybe insufficient to detect FALD. 

The overall goal of this study by Dr. Yuli Kim and colleagues from the Children’s Hospital of Philadelphia is to develop a reliable means assessing severity of FALD and better understand the role of Fontan hemodynamics in developing FALD.