Development and Evaluation of a Bristol Surveillance Guideline into Liver Disease amongst the Adult Fontan Population

Summary

Some children are born with heart defects that need surgery. One type of surgery is called a Fontan procedure. This surgery is not a permanent fix, but as we learn more, patients are living longer and with better quality of life. However, a long term consequence of the surgery can be liver disease. This happens because of changes in blood flow in and out of the liver after surgery.  The Bristol Heart Institute cares for a large number of patients who have had Fontan procedures.

The Heart and Liver teams are working together to understand how best to look after these patients at risk of liver disease.  I am setting up a joint clinic with the Heart and Liver teams, looking for liver disease in Fontan patients.  I am focusing on the development of high pressure in the blood vessels around the liver (portal hypertension) and potentially cancerous spots in the liver (lesions).  I have chosen to focus on these as they cause complications that can impact patient's quality of life.  To find these, I use two common tests, an endoscopy (camera test of the gullet and stomach) and an ultrasound scan.

Introducing new health concerns can be quite scary for patients.  I will carry out patient interviews to understand patients' views and to find out what we can do to reduce anxiety.  With help from patients, I will design a guideline that is acceptable to both healthcare professionals and patients to manage liver disease in the adult Fontan population.