Hepatic venous wedge pressure gradient measurements in intestinal failure associated liver disease

Historically, in children with intestinal failure associated liver disease (IFALD), the presence of splenomegaly and moderate bridging fibrosis would be considered as evidence of advanced liver disease and portal hypertension and be recommended for liver-inclusive intestinal transplant graft. In our experience, the assessment of portal hypertension based on conventional investigations, which are well established in other chronic liver diseases, could be misleading in some children with IFALD, and further investigations could help in assessing the severity of liver disease. Hepatic venous wedge pressure gradient (HVWPG) is used in chronic liver diseases for objectively assessing the severity of portal hypertension. We postulated that HVWPG may be useful to assess the severity of portal hypertension in children with IFALD and, therefore, help in the decision-making process for the need for a liver-inclusive intestinal graft.

Retrospective analysis of children with IFALD who had HVWPG measured between 2005 and 2020. Demographic details, laboratory parameters, liver biopsy, HVWPG and clinical outcomes were reviewed. Children were grouped into two categories based on HVWPG gradient: HVWPG ≥ 10 mmHg (significant portal hypertension) and HVWPG < 10 mmHg.

Between 2005 and 2020, 23 children (median age: 33 months, interquartile range: 11-54) had 27 HVWPG measurements (4 children had repeat measurements). No procedural complications were documented. 16/23 children had HVWPG < 10 mmHg, 7/23 children had HVWPG ≥ 10 mmHg. Of the 16 children with HVWPG < 10 mmHg, 10 children were referred to the local team for intestinal rehabilitation, while 6 children were recommended for transplantation (4 for isolated intestinal transplant and 2 for liver-inclusive intestinal transplant) as they fulfilled other indications for intestinal transplantation (impaired venous access, etc.). Of the seven children who had significant portal hypertension (HVWPG ≥ 10 mmHg), six were recommended for liver-inclusive intestinal transplant. There was a cohort of four children with at least bridging fibrosis and HVWPG < 10 mmHg who had repeat measurements due to failed intestinal rehabilitation strategies to wean from parenteral nutrition and worsening clinical signs (increasing splenomegaly, etc.). Two children were recommended for liver-inclusive intestinal transplant in view of increase in HVWPG to ≥10 mmHg.

HVWPG measurements can guide in the decision-making process in children with IFALD, especially those with bridging fibrosis without significant clinical evidence of portal hypertension for deciding on the need for liver-inclusive intestinal transplantation.