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NCT Number: NCT03914196

Fontan Associated Liver Disease

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.

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This study is active but is not currently recruiting participants.

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Key information

Conditions

Fontans

Age range

0 year–5 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, 45229, United States

Who can participate

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Single ventricle physiology congenital heart disease undergoing Fontan operation;
  • ≤5 years of age at the time of stage 3 Fontan operation;
  • Ability to return for follow-up imaging.

Exclusion criteria

  • Inability to undergo ultrasound imaging.
  • Non-English speaking patients, as breath-hold is required for US shear wave elastography in older children due to excursion of the liver with breathing.

Treatment and study plan

Liver Stiffness Ultrasound

Diagnostic Test

Research imaging will include liver assessments of stiffness (ultrasound shear wave elastography), tissue attenuation (if available), and blood flow (gray-scale and Doppler imaging).

Other names: shear wave elastography (also called ARFI)

Primary outcomes

  1. Pre/Post Fontan Liver Stiffness Changes

    Time frame: 5 years

    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.

Secondary outcomes

  1. Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation

    Time frame: 10 years

    • 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)
    • Lymphatic abnormalities (e.g., protein losing enteropathy, plastic bronchitis, intractable ascites)
    • 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

Sponsors and collaborators

Lead sponsor

Children's Hospital Medical Center, Cincinnati

Other

Registry information

Official study title

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

Important dates

Study start
2018
Primary completion
2027
Study completion
2028
First posted
Apr 16, 2019
Registry last updated
Apr 8, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.