Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: a single-centre cross-sectional study

Abstract

OBJECTIVES Fontan-associated liver disease (FALD) is a hallmark of the failing Fontan circulation, but no general classification of FALD severity exists. In this study, we propose a scoring system to grade the severity of FALD and analyse its applicability for evaluation of Fontan failure. METHODS From 2017 to 2019, a total of 129 successive Fontan patients received a comprehensive hepatic assessment. The 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. Haemodynamic assessment was performed using echocardiography, cardiopulmonary exercise testing and catheterization. RESULTS 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 to patients with absent/mild FALD (P = 0.001). The 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 failure was present in 21 patients. FALD was graded moderate in 2 and severe in 19 of these patients. The FALD score accurately discriminated patients with and without Fontan failure (sensitivity 90.5%, specificity 71.3%). CONCLUSIONS The FALD score significantly correlates with impaired Fontan haemodynamics. A cut-off value ≥6.0 has a high diagnostic accuracy in detecting Fontan failure. Clinical trial registry DRKS (GCTR, German clinical trial registry) Clinical trial registration number DRKS00015039

Morphologic Alterations Precede Functional Hepatic Impairment as Determined by 13C-Methacetin Liver Function Breath Test in Adult Fontan Patients

“…In a previous study, we proposed a scoring system (FALD score) to grade FALD severity based on a combination of laboratory parameters, hepatic ultrasound and transient elastography (TE). Our results revealed that the FALD score significantly correlated with Fontan hemodynamics and reliably discriminated between patients with and without Fontan failure ( 7 ). Liver-associated morbidity and mortality are well-described in the adult Fontan population and constitute major risk factors limiting survival after cardiac transplantation ( 8 , 9 ).…”

“…The calculated Fibrotest® score was converted into liver fibrosis stages according to METAVIR histological classification for liver biopsies ( 11 ). The calculation of the FALD score has previously been described in detail ( 7 ). Briefly, scoring points assigned for each hepatic abnormality detected in the diagnostics mentioned above were summed up for the final FALD score ( 7 ).…”

Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction

“…This value appears higher than the conventional one established to define cirrhosis in cases with viral or metabolic etiology (>12 kPa), for the concurrent sinusoidal venous congestion that the liver undergoes post-Fontan. This assumption is supported by results of the literature obtained in dog and human experimental and clinic studies [8], which documented a significant increase in LS values by TE, only after 4 months since Fontan operation and that were predictive of advanced FALD with values above 20 kPa [10,23]. For this reason, it seems evident that the hemodynamic changes in the venous district after Fontan can determine organ damage, particularly with progressive liver fibrosis and cirrhosis over the years.…”

Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors

“…We have also concluded that if extracardiac and lateral tunnel connection Fontans are not comparable, then a single composite index, similarly constructed, may not be equally applicable to all. Recently, Schleiger et al 42 developing a composite index to test correlation with hepatic fibrosis scores. Rather than utilizing a variety of laboratory and testing values, we based our approach upon anatomical features that likely affect passive total cavopulmonary flow, specifically in those with extracardiac connections, the current eras most frequently performed connection.…”