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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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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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). |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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. | 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Correlate liver stiffness measurements with clinical outcomes at 3-, 5-, and 10-years post stage 3 Fontan operation |
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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Inclusion Criteria:
Exclusion Criteria:
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All patients that are undergoing a Fontan Palliation during cardiothoracic surgery.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Cincinnati Children's Hospital Medical Center | Cincinnati | Ohio | 45229 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 27752831 | Background | 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. |
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| 10 years |