An observational study in Fontans, sponsored by Children's Hospital Medical Center, Cincinnati. Active, not recruiting at 1 site in United States. Open to participants aged 0 Years to 5 Years. Per ClinicalTrials.gov, last updated 2026-04-08.
Sponsored by Children's Hospital Medical Center, Cincinnati · Observational
Fontan-associated liver disease (FALD), including the development of cardiac cirrhosis and liver neoplasms (benign and malignant), occurs in a majority of patients with congenital heart disease palliated with the Fontan operation. However, the specific phenotype (fibrosis only, fibrosis + lesions, etc.) of disease and severity/timing of onset are variable. Chronic passive congestion of the liver due to the absence of a functional sub-pulmonary ventricle and resultant chronic central venous hypertension is suspected to be one of the chief drivers of FALD and recent work has demonstrated that ultrasound shear wave elastography can be used to noninvasively detect and measure the degree of liver congestion. Chronic passive congestion of the liver may also be a predictor of other Fontan-related complications, such as protein losing enteropathy, plastic bronchitis, and intractable ascites.
Children's Hospital Medical Center, Cincinnati is the lead sponsor of 661 studies on the registry; 134 are open to participants now.
Of its 54 completed or terminated interventional studies of FDA-regulated products, 30 (56%) have results posted.
Counted across the registry records on this site, refreshed daily.
All patients that are undergoing a Fontan Palliation during cardiothoracic surgery.
Exclusion Criteria:
Research imaging will include liver assessments of stiffness (ultrasound shear wave elastography), tissue attenuation (if available), and blood flow (gray-scale and Doppler imaging).
Also known as: shear wave elastography (also called ARFI)
Pre/Post Fontan Liver Stiffness Changes
to determine if there are differences between patients with regard to liver stiffness (hepatic congestion) pre- and post-Fontan operation or with regard to change in liver stiffness over time. This information will be used to drive future hypothesis-driven research related to predicting Fontan-related outcomes based on measured liver stiffness.
Time frame: 5 years
Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation
1. Time to anatomic Fontan pathway obstruction or physiologically high resistance, need for Fontan pathway intervention, need for cardiac transplant evaluation or actual transplantation, and need for mechanical cardiac support evaluation or actual implementation (e.g., ventricular assist device) 2. Lymphatic abnormalities (e.g., protein losing enteropathy, plastic bronchitis, intractable ascites) 3. FALD i. MRI outcomes (e.g., increased liver stiffness \[do US and MRI measures of liver stiffness correlate?\], portal hypertension, development of focal liver lesions) ii. Liver laboratory assessments (e.g., ALT, AST, bilirubin, alkaline phosphatase, GGT) iii. PELD score iv. VAST score v. Need for liver transplant evaluation or actual transplantation d. Available biopsy (liver and cardiac) data e. Available cardiac catheterization data f. Available cardiac echocardiography and/or cardiac MRI data
Time frame: 10 years
Plan to share: Undecided
This study is active, not recruiting, as verified in Apr 2026. You cannot join it, but the record below documents what was studied.
Get an email when the registry record changes — status, dates, results — or when someone posts here.
Sign in to followQuestions and observations about this study, from anyone following it. Not medical advice, and not a channel to the study team — their contact details are on the registry record.
Sign in to join the discussion. Reading takes no account; posting does. You choose a display name, and a pseudonym is the default.
Nothing here yet. If you are running this trial, taking part in it, or weighing whether to, this is the place to say so.
Children's Hospital Medical Center, Cincinnati