Sildenafil for Liver Fibrosis in Adolescents and Adults After Fontan Operation
Status:
Recruiting
Trial end date:
2023-07-01
Target enrollment:
Participant gender:
Summary
Fontan operation is currently the most common procedure performed in patients with
single-ventricle physiology. This surgery allows for passive caval blood flow to the
pulmonary arteries in the absence of a subpulmonary pump and therefore separating the
pulmonary circulation from systemic circulation. However, late hepatic complications such as
liver fibrosis, cirrhosis, or even hepatocellular carcinoma are increasingly described in
patients with Fontan circulation. The Fontan associated liver disease (FALD) is related to
the decreased cardiac output and elevated central venous pressure after Fontan operation.
Although the prevalence of FALD is higher than 90% in patients with Fontan circulation. There
are no specific medications can reverse the liver fibrosis or prevent the progression of FALD
at present. Sildenafil had been used in patients after Fontan operation not only to decrease
their pulmonary vascular resistance and central venous pressure, increased systemic cardiac
output. Comparing with other oral pulmonary vasodilators, sildenafil is with less possibility
of liver toxicity. Therefore, we will conduct a prospective, open-labeled,
randomized-controlled study in at least 90 Fontan patients with age > 12 years receiving
follow -up at our institute. These patients will be in accordance with the ratio of 1: 1
distribution randomized into two groups, group 1 will receive sildenafil 20 mg three times
daily for 3 years and the group will not receive any pulmonary vasodilator as a control.
Patients in both group will receive the examinations of liver fibrosis markers, transient
ultrasound elastrography (FibroScan), diffusion-weighted magnetic resonance imaging (DW-MRI)
and cardiopulmonary function test at baseline, 1 year, 2 years and 3 years after initiating
treatment. Comparing the results of two groups, we may further clarify the treatment effect
of sildenafil on FALD in patients after Fontan procedure.