Abstract
Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 ± 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan®). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (p < 0.05), larger hepatic and cava veins diameter (p < 0.05), worsened NYHA class (p < 0.05), abnormal lymphocytes (p < 0.01), platelet count (p < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (p < 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (p < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (p < 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.
Highlights
The porta vein mean diameter was 12.8 ± 1.9 mm, and portal flow velocity was reduced in almost half of the patients (48.1%)
Signs of hepatic venous congestion with hepatics and cava veins dilation were documented in 60% and 84.6% of cases, respectively, without gender differences (M vs. F = inferior vena cava (IVC) diameter 20.9 ± 5.3 vs. 18.7 ± 4.9 mm; p = ns)
We found that all the scores applied, especially those exploring liver function were statistically effective in defining cases without or with advanced Fontan-associated liver disease” (FALD)
Summary
Fontan’s intervention, used since the 1970s to correct the heart defects and restore the survival of most patients up to adulthood [1,2,3]. In such patients, the usual therapeutic goal is to separate the systemic and the pulmonary circulations by means of systemic venous–. Survival with a Fontan circulation has significantly improved in the current era. For this reason, adult patients with congenital heart disease (CHD) are currently facing long-term unexpected complications associated with the Fontan circuit, which affects multiple organs. The so-called “Fontan-associated liver disease” (FALD) is an arising relevant clinical entity, which is due to chronic systemic venous hypertension that causes the occurrence of liver cirrhosis and other severe complications and can increase patients’ morbidity and mortality during adult life [4,5,6]
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