NCT03914196

Fontan Associated Liver Disease

Can Ultrasound Liver Stiffness Pre-Fontan, Post-Fontan, or Change Over Time Predict Onset and Severity of Fontan-Related Complications, Including Fontan-Associated Liver Disease

• Status: Active Not Recruiting
• Phase: —
• Study type: Observational
• Enrollment: 50 (estimated)
• Sponsor: Children's Hospital Medical Center, Cincinnati · Academic / Other
• Sex: All
• Age: 0 Years – 5 Years
• Healthy volunteers: —

Summary

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.

Conditions

• Fontans

Interventions

Type	Name	Description
DIAGNOSTIC_TEST	Liver Stiffness Ultrasound	Research imaging will include liver assessments of stiffness (ultrasound shear wave elastography), tissue attenuation (if available), and blood flow (gray-scale and Doppler imaging).

Timeline

• Start date: 2018-01-18
• Primary completion: 2027-12-31
• Completion: 2028-12-31
• First posted: 2019-04-16
• Last updated: 2026-04-08

Locations

1 site across 1 country: United States