CRITERIA

• Research type

  Research Study

• Full title

  Hepatic Stress In Fontan Associated Liver Disease

• IRAS ID

  352553

• Contact name

  Michael Quail

• Contact email

  michael.quail@gosh.nhs.uk

• Sponsor organisation

  University College London

• Clinicaltrials.gov Identifier

  Z6364106/2025/07/245, UCL Data protection registration number

• Duration of Study in the UK

  3 years, 0 months, 0 days

• Research summary

  Normally, the heart has two pumping chambers - one sends blood to the lungs, the other to the body. Some babies are born with only one working pump chamber - called single ventricle heart disease. Doctors fix this with several heart surgeries in childhood. The final one (Fontan operation) connects veins directly to the lungs, so the single pump only needs to send blood to the body. While this helps people live, the changed blood flow can damage the liver over time - called Fontan-associated liver disease (FALD). FALD is a major concern for patients, because it is associated with a worse long-term outlook and patients can do worse after a heart transplant.

  Most patients with a Fontan circulation have detectable liver disease within 10 years of their final Fontan operation, however some patients have more severe FALD that others, and we do not understand the basis of this variation.
  Based on observations from pilot data we hypothesise that FALD is due to variation in the mechanical stress in the liver (stretching and compressing the liver tissue) experienced by the Fontan liver on a daily basis. Daily activities such as eating and exercise have the potential to cause large increases in mechanical forces within the liver, which may explain the variability seen in FALD.

  In this study we will use a novel imaging technique called MRI elastography, which can measure the stiffness of the liver non-invasively. We would like to determine whether we can reduce the mechanical liver stress during these activities: 1. During eating by modifying the macronutrient composition of the food eaten (high-fat and low-fat), and 2. During exercise by giving a medicine called Sildenafil which dilates the lung arteries and may reduce hepatic stress by lowering vein pressure during exercise.
  

• REC name

  Yorkshire & The Humber - Leeds East Research Ethics Committee

• REC reference

  26/YH/0021

• Date of REC Opinion

  5 Mar 2026

• REC opinion

  Further Information Favourable Opinion