Liver function monitoring in Fontan-procedure patients: audit of current practice across South Wales.

The Fontan procedure provides a palliative surgical repair for complex congenital heart disease, but it is associated with many long-term problems, including liver cirrhosis, and hepatocellular carcinoma. The current suggestion from international guidance is that end-organ surveillance should be carried out, with a particular focus on regular blood tests and imaging for liver function. In this study, retrospective analysis was performed on adult patients who had previously had a Fontan operation performed to determine the regularity of end-organ surveillance in regards to their liver function covering the three calendar years from 2016 to 2018, and the first six months of 2019. Eighty-six patients were identified in South Wales monitored by the adult congenital heart disease unit. We found that the number of investigations performed in the first six months of 2019 was comparable to other calendar years in their entirety. Liver function tests had been performed in 57% of patients throughout 2018, with only 8% having had an alpha-fetoprotein taken, and only 9% having had imaging of the liver performed. Over the course of their lifetime, 97% of patients had had a liver function blood test performed at some point, with 17% having had an alpha-fetoprotein taken, and 49% having their liver imaged. In conclusion, end-organ surveillance is an important follow-up for patients with a Fontan circulation, with guidelines proposing yearly blood test and imaging investigations. This study shows the opportunities to improve surveillance in this group of patients to highlight the development of liver cirrhosis, and/or hepatocellular carcinoma.