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Fontan Associated Liver Disease

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT03914196
Recruitment Status : Recruiting
First Posted : April 16, 2019
Last Update Posted : May 7, 2019
Sponsor:
Information provided by (Responsible Party):
Children's Hospital Medical Center, Cincinnati

Brief 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.

Condition or disease Intervention/treatment
Fontans Diagnostic Test: Liver Stiffness Ultrasound

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Study Type : Observational
Estimated Enrollment : 50 participants
Observational Model: Cohort
Time Perspective: Prospective
Official 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
Actual Study Start Date : January 18, 2018
Estimated Primary Completion Date : December 31, 2027
Estimated Study Completion Date : December 31, 2028

Resource links provided by the National Library of Medicine



Intervention Details:
  • 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).
    Other Name: shear wave elastography (also called ARFI)


Primary Outcome Measures :
  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 Outcome Measures :
  1. Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation [ Time Frame: 10 years ]
    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




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Ages Eligible for Study:   up to 5 Years   (Child)
Sexes Eligible for Study:   All
Sampling Method:   Non-Probability Sample
Study Population
All patients that are undergoing a Fontan Palliation during cardiothoracic surgery.
Criteria

Inclusion Criteria:

  1. Single ventricle physiology congenital heart disease undergoing Fontan operation;
  2. ≤5 years of age at the time of stage 3 Fontan operation;
  3. Ability to return for follow-up imaging.

Exclusion Criteria:

  1. Inability to undergo ultrasound imaging.
  2. 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.

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03914196


Contacts
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Contact: Katy Fischesser 513-803-5191 katy.fischesser@cchmc.org

Locations
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United States, Ohio
Cincinnati Children's Hospital Medical Center Recruiting
Cincinnati, Ohio, United States, 45229
Contact: Katy a Fischesser    513-803-5191    hircregulatory@cchmc.org   
Sponsors and Collaborators
Children's Hospital Medical Center, Cincinnati
Publications:
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Responsible Party: Children's Hospital Medical Center, Cincinnati
ClinicalTrials.gov Identifier: NCT03914196    
Other Study ID Numbers: 2017-7816
First Posted: April 16, 2019    Key Record Dates
Last Update Posted: May 7, 2019
Last Verified: May 2019
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: Undecided

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Liver Diseases
Digestive System Diseases