FontansNCT03914196
Fontan Associated Liver Disease
An observational study of Fontans, run by Children's Hospital Medical Center, Cincinnati at 1 site in United States.
Trial 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. Read more about this study
- Who is it for?
- Ages 0 to 5 years
- Conditions the study is for
- Fontans
- What is being tested?
- Liver Stiffness Ultrasound
- How many people
- 50 participants
- Where
- 1 siteUnited States
- Study dates
- 18 Jan 2018to 31 Dec 2028
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 0 to 5 years
- All sexes
- Child
Study population: All patients that are undergoing a Fontan Palliation during cardiothoracic surgery.
Who may be able to join
- 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.
Who cannot join
- 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.
Trial locations and contacts
1 site in 1 country.
United States1 site
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio, 45229
About this study
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
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
- Being tested
- Liver Stiffness Ultrasound
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Liver Stiffness UltrasoundDiagnostic test
Also called shear wave elastography (also called ARFI)
Research imaging will include liver assessments of stiffness (ultrasound shear wave elastography), tissue attenuation (if available), and blood flow (gray-scale and Doppler imaging).
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 5 years
Secondary outcomes (1)
Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation
- 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
- 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
Measured: 10 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted16 Apr 2019
- Study start18 Jan 2018
- Primary completion31 Dec 2027 (estimated)
- Study completion31 Dec 2028 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for FontansStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 50 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Children's Hospital Medical Center, Cincinnati
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT03914196
- Sponsor's protocol ID
- 2017-7816
- Status last verified
- Apr 2026
- First submitted
- 11 Apr 2019
- Last updated
- 8 Apr 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Undecided
Publications
DiPaola FW, Schumacher KR, Goldberg CS, Friedland-Little J, Parameswaran A, Dillman JR. Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol. 2017 Jun;27(6):2434-2442. doi: 10.1007/s00330-016-4614-x. Epub 2016 Oct 17.
PubMed 27752831
Study at a glance
From the registry record, last updated by the sponsor on 8 Apr 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- 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
- ClinicalTrials.gov ID
- NCT03914196
- Acronym / sponsor ID
- 2017-7816
- Conditions
- Fontans
- Study type
- Observational
- Recruitment status
- Active, not recruiting
- Enrolment
- 50 participants (estimated)
- Lead sponsor
- Children's Hospital Medical Center, CincinnatiUniversity, hospital or other
- Study start
- 18 Jan 2018 (actual)
- Primary completion
- 31 Dec 2027 (estimated)
- Study completion
- 31 Dec 2028 (estimated)
- First posted
- 16 Apr 2019 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
Who may qualify
- Ages
- 0 to 5 years
- Sex
- All
- Age groups
- Child
- Study population
- All patients that are undergoing a Fontan Palliation during cardiothoracic surgery.
Key inclusion criteria (3 listed)
- 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.
Key exclusion criteria (2 listed)
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Liver Stiffness UltrasoundAlso: shear wave elastography (also called ARFI)Research imaging will include liver assessments of stiffness (ultrasound shear wave elastography), tissue attenuation (if available), and blood flow (gray-scale and Doppler imaging). | Diagnostic test | – |
Primary outcome
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.
1 secondary outcome
Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation
Time frame: 10 years
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 8 Apr 2026.
- Background: PubMed 27752831DiPaola FW, Schumacher KR, Goldberg CS, Friedland-Little J, Parameswaran A, Dillman JR. Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol. 2017 Jun;27(6):2434-2442. doi: 10.1007/s00330-016-4614-x. Epub 2016 Oct 17.
- Individual participant dataSharing planned: Undecided
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
The record lists no contact. Use the official record to reach the sponsor.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Fontans, run by Children's Hospital Medical Center, Cincinnati at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: under way, not looking for participants, as of the sponsor's last update on 8 Apr 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Pre/Post Fontan Liver Stiffness Changes (time frame: 5 years). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT03914196, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 8 Apr 2026.