Principal Investigator: Daniel R. Ganger, MD

Many babies born with heart problems grow up and mature well into adulthood thanks to modern surgical advancements. In fact, one million adults currently live with some form of congenital heart disease in the United States alone. A rare congenital heart condition, single ventricle disease usually requires surgical intervention early on, with most children undergoing a common surgery known as the Fontan procedure. While it helps these young patients to overcome their heart problem, unfortunately the Fontan procedure can lead to liver problems—from cirrhosis and liver cancer to even liver failure—over time.

Even with this knowledge, current hepatic laboratory testing, imaging tools, and/or liver biopsies are woefully inaccurate predictors of the development of serious liver disease. Awarded a grant from the Digestive Health Foundation, Northwestern Medicine investigators see a potential solution to providing effective screening and treatment for this chronically-ill population: the HepQuant-SHUNT test. Safe and non-invasive, the HepQuant-SHUNT test has shown promise by yielding easily reproducible and accurate measurements of liver function. In studies involving Hepatitis C patients, the novel test has begun demonstrating its strength for predicting important hepatic clinical outcomes, including liver-related death. Outdoing the results of the current gold standard of care, invasive liver biopsy, the HepQuant-SHUNT test may offer a more accurate as well as tolerable test for patients.

GI and hepatology faculty member Daniel R. Granger, MD and chief medical resident Alexander Lemmer, MD, hope to determine if the HepQuant liver function test can accurately predict significant hepatic clinical outcomes in the post-Fontan population. Recruiting a total of 50 Fontan patients (ages 18-65) from Northwestern Memorial Hospital to undergo HepQuant-SHUNT testing, the research team will monitor the liver health of these participants over one to two years. If the test succeeds in providing predictive information, next steps will involve mathematical modelling. Ultimately, evidence-based guidelines could be developed for the first time to direct the care of these seriously-ill patients, from increased screening to closer monitoring for those at highest risk of liver damage.