Fontan Associated Liver Disease

Cincinnati Children's Hospital Medical Center

Status: Active, not recruiting

Conditions: Fontans

Treatments: Diagnostic Test: Liver Stiffness Ultrasound

Study type: Observational

Funder types: Other

Identifiers

NCT03914196

2017-7816

Details and patient eligibility

About

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.

Enrollment: 50 estimated patients

Sex: All

Ages: Under 5 years old

Volunteers: No Healthy Volunteers

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.