Functional hepatic deterioration determined by C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults.

Background: Despite improved survival a substantial number of Fontan patients eventually develop late failure. Fontan-associated liver disease (FALD) is the most frequent end-organ dysfunction. Although impaired hemodynamics and Fontan failure correlate with FALD severity, no association between hepatic functional metabolic impairment and Fontan hemodynamics has been established.

Morphologic Alterations Precede Functional Hepatic Impairment as Determined by C-Methacetin Liver Function Breath Test in Adult Fontan Patients.

Fontan-associated liver disease (FALD) is the most common end-organ dysfunction affecting up to 70-80% of the Fontan population. The clinical significance of FALD is incompletely understood and no unambiguous correlation between hepatic function and FALD severity has been established. In this study, we sought to evaluate maximal liver function capacity with liver maximum function capacity test (LiMAx®) in adult Fontan patients.