Abstract Body:
Introduction
Single ventricle heart disease (SVHD) includes tricuspid atresia (TA), mitral atresia (MA), hypoplastic left heart syndrome (HLHS), and hypoplastic right heart syndrome (HRHS), managed through staged surgical palliation, culminating in the Fontan procedure. While this procedure has significantly improved survival rates, it is associated with long-term complications, particularly Fontan-associated liver disease (FALD). Despite growing awareness, data on FALD’s prevalence and progression remain limited, highlighting the need for better surveillance and management.

Methods
We conducted a retrospective analysis of the National Inpatient Sample (NIS) database (2018–2020) to identify adult (>18 years) hospitalizations with a history of surgically corrected TA, HLHS, and HRHS. Hospitalizations with concurrent diagnoses of of liver fibrosis (LF) and liver cirrhosis (LC) were identified using appropriate ICD-10 codes. Multivariable logistic regression analysis was performed to evaluate the association between SVHD hospitalizations and hepatic complications while adjusting for potential confounders.

Results
Between 2018 and 2020, there were 399 TA, 94 HRHS, and 380 HLHS adult hospitalizations following surgical correction. The mean age of these hospitalizations was significantly lower (HRHS: 28, HLHS: 23, TA: 33 years) compared to the general adult hospitalizations. Multivaribale logistic regression analysis revealed surgically corrected adult SVHD hospitalizations had significantly increased odds of liver cirrhosis (TA: OR 17, HLHS: OR 11, HRHS: OR 14) and liver fibrosis (HLHS: OR 498, TA: OR 55, HRHS: OR 619) when compared to other adult hospitalizations.

Conclusion
Our study supports existing evidence that patients with Fontan circulation have a significantly increased risk of liver disease. Further research is needed to develop optimal strategies for surveillance, screening of FALD, and determining the appropriate timing for liver transplantation. Additionally, emerging innovations like the cavopulmonary pump aim to address Fontan-related hemodynamic challenges.