Evaluation of Fontan's Failure by Classifying the Severity of the Fontan-Associated Liver Disease—A Single-Centre Cross-Sectional Study

Objectives: The Fontan-associated liver disease (FALD) is a hallmark of the failing Fontan's circulation, but no general classification of FALD severity exists. In this study, we propose a scoring system to grade the severity of FALD and analyze its applicability for evaluation of Fontan's failure.

Methods: From 2017 to 2019, 129 successive Fontan's patients received a comprehensive hepatic assessment. FALD score was based on results from laboratory testing, hepatic ultrasound, and transient elastography by assigning scoring points for each abnormality detected. FALD severity was graded mild, moderate, and severe. Hemodynamic assessment was performed using echocardiography and cardiopulmonary exercise testing.

Result: FALD was graded absent/mild, moderate, and severe in 53, 26, and 50 patients, respectively. Cardiopulmonary capacity was significantly impaired in patients with severe FALD compared with patients with absent/mild FALD (p = 0.001). FALD score significantly correlated with pulmonary artery pressure (p = 0.001), end-diastolic ventricular pressure (p < 0.001), hepatic venous pressure (p = 0.004), and wedged hepatic venous pressure (p = 0.009). Fontan's failure was present in 21 patients. FALD was graded as moderate in 2 and severe in 19 of these patients. FALD score accurately discriminated patients with and without Fontan's failure (sensitivity: 90.5%, specificity: 71.3%).

Conclusion: FALD score significantly correlates with the impaired Fontan hemodynamics. A cut-off value of ≥6.0 has a high diagnostic accuracy to detect Fontan's failure.

Publication History

Article published online:
21 February 2021