Imaging findings in Fontan-associated liver disease: Results of a single center study.

Fontan-associated liver disease (FALD) often occurs in patients with single-ventricle physiology following Fontan surgery, and ranges from liver congestion to cirrhosis. The assessment of the severity of FALD using noninvasive methods is challenging. However, transient elastography (TE) may be useful for the noninvasive evaluation of FALD and prediction of clinical outcomes. To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events. This retrospective single-center study (Hospital Universitario La Paz, Madrid), including 91 post-Fontan patients aged > 18 years old. Laboratory and ultrasound findings, and liver stiffness measurements (LSM) by TE (FibroScan®) were assessed. FALD was defined using ultrasound criteria (hepatomegaly, liver surface nodularity, parenchymal heterogeneity, hyperechoic lesions, splenomegaly, collaterals) and advanced FALD was defined according to the European Association for the Study of the Liver-European Reference Network statement (esophageal varices, portosystemic shunts, ascites, splenomegaly). Clinically relevant events included heart or heart-liver transplantation indication, hepatocellular carcinoma, and all-cause mortality. Patient characteristics were: 60.4% male; Mean age, 33.3 ± 8.2 years; Mean elapsed time since surgery, 24.3 ± 7.7 years; 89% with FALD; 73% with advanced FALD. LSM by TE was associated with FALD [odds ratio (OR) = 1.34; 95% confidence interval (95%CI): 1.10-1.64; P = 0.003] and advanced FALD (OR = 1.10; 95%CI: 1.01-1.19; P = 0.023). Areas under the curve (AUC) were 0.905 and 0.764 for FALD and advanced FALD, respectively. FALD cut-off values comprised: Optimal, 20 kPa (sensitivity: 92.3%; specificity: 80.0%); Rule-out, 15 kPa (sensitivity: 96.9%); Rule-in, 25 kPa (specificity: 100%). A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery (AUC: 0.877; sensitivity, 95.4%; specificity, 80.0%; positive predictive value, 96.9%; negative predictive value, 72.7%). LSM by TE was associated with clinically relevant events (OR = 1.07; 95%CI: 1.01-1.13; P = 0.021) and all-cause mortality (OR = 1.23; 95%CI: 1.02-1.47; P = 0.026). In adult patients post-Fontan surgery, TE is a useful noninvasive method for FALD diagnosis. The association between LSM by TE and clinically relevant events suggests a role in prognosis.

Fontan-associated liver disease.

Genomic and transcriptomic profiling of hepatocellular carcinoma in patients with Fontan-associated liver disease.

Evaluation of diagnostic criteria for mild-to-advanced stages of Fontan-associated liver disease: A nationwide epidemiological survey in Japan.

A surveillance for advanced liver disease and characterization of hyperechogenic lesions after Fontan surgery in a prospective cohort of cases with congenital univentricular heart

Most cases of hepatocellular carcinoma (HCC) are associated with cirrhosis related to chronic hepatitis B virus (HBV) or hepatitis C virus (HCV) infection. Changes in the time trends of HCC and most variations in its age-, sex-, and race-specific rates among different regions are likely to be related to differences in hepatitis viruses that are most prevalent in a population, the timing of their spread, and the ages of the individuals the viruses infect. Environmental, host genetic, and viral factors can affect the risk of HCC in individuals with HBV or HCV infection. This review summarizes the risk factors for HCC among HBV- or HCV-infected individuals, based on findings from epidemiologic studies and meta-analyses, as well as determinants of patient outcome and the HCC disease burden, globally and in the United States.

Hepatocellular carcinoma (HCC) is a serious complication of Fontan-associated liver disease (FALD). However, data on its incidence, risk factors, and outcomes in this rare population are limited. We aimed to assess the incidence and predictors of HCC and evaluate the utility of surveillance in patients with FALD. We conducted a retrospective cohort study on patients with FALD who were referred to our gastroenterology department. The cumulative incidence of HCC after FALD diagnosis was calculated, and multivariate analysis was used to identify independent risk factors. Overall survival after FALD diagnosis was compared between patients who developed HCC and those who did not. A total of 120 patients with FALD were followed up for a median period of 50.0 months. The cumulative incidence rates of HCC at 1, 2, and 3 years after FALD diagnosis were 0.9%, 2.7%, and 3.9%, respectively. Elevated alpha-fetoprotein (AFP) at FALD diagnosis was independently associated with HCC development (hazard ratio, 1.29; 95% confidence interval, 1.04-1.60; p=0.01). There was no significant difference in overall survival between patients with and without HCC in this cohort (log-rank test, p=0.50). Patients with FALD face a non-negligible risk of developing HCC, thus supporting the need for surveillance. Elevated AFP levels at the time of FALD diagnosis may aid in risk stratification, which enables the targeted monitoring and early detection of HCC. Furthermore, routine surveillance contributes to the excellent prognosis of patients with FALD who develop HCC, thus making their prognosis comparable to that of patients without HCC.

In Gaucher disease (GD) imaging of liver and spleen is part of routine follow-up of GD patients. Focal lesions in both liver and spleen are frequently reported at radiological examinations. These lesions often represent benign accumulations of Gaucher cells, so-called “gaucheroma”, but malignancies, especially hepatocellular carcinoma, are more frequently found in GD as well. We report the imaging characteristics of all focal lesions in liver and spleen in the Dutch GD cohort. Of the 95 GD1 patients, 40% had focal splenic and/or hepatic lesions, associated with more severe GD. Lesions identified as gaucheroma have variable imaging characteristics: hyper- to hypointense on MRI, hyper- or hypoechoic on US and hypodense on computed tomography (CT). Hepatic lesions were classified as simple cysts or haemangioma based upon imaging characteristics. Focal nodular hyperplasia (FNH), gaucheroma and hepatocellular carcinoma (HCC) could not be distinguished by conventional US, CT or MRI. Growth of these lesions and/or characteristics of HCC on dynamic CT or MRI and pathology was used to identify or rule out HCC. We propose a decision-making algorithm including the use of growth and dynamic CT- or MRI-scanning to characterize lesions.

Recognizing the significant prevalence of hepatocellular carcinoma (HCC) in Saudi Arabia, and the difficulties often faced in early and accurate diagnoses, evidence-based management, and the need for appropriate referral of HCC patients, the Saudi Association for the Study of Liver diseases and Transplantation (SASLT) formed a multi-disciplinary task force to evaluate and update the previously published guidelines by the Saudi Gastroenterology Association. These guidelines were later reviewed, adopted and endorsed by the Saudi Oncology Society (SOS) as its official HCC guidelines as well. The committee assigned to revise the Saudi HCC guidelines was composed of hepatologists, oncologists, liver surgeons, transplant surgeons, and interventional radiologists. Two members of the task force served as guidelines editors. A wide based search on all published reports on all aspects of the epidemiology, natural history, risk factors, diagnosis, and management of HCC was performed. All available literature was critically examined and available evidence was then classified according to its strength. The whole document and the recommendations were then discussed in details by members and consensus was obtained. All recommendations in these guidelines were based on the best available evidence, but were tailored to the patients treated in Saudi Arabia. We hope that these guidelines will improve HCC patient care and enhance the multidisciplinary care needed for these patients.

2092035 How Useful Is Contrast Enhanced Ultrasound in the evaluation of Focal Liver Lesions. A Monocentric Experience

Hepatocellular carcinoma after sustained response to interferon in non-cirrhotic hepatitis C: flaws in the cure, or a clue to the flaws?

Contrast Enhanced Ultrasound, The First Step in the Evaluation of Focal Liver Lesions

Fontan-Associated Liver Disease (FALD) is a dramatically emerging problem even if not precisely defined in term of debated diagnosis and surveillance protocols. We analyze FALD prevalence, clinical impact and implications in a European cohort of patients. It’s a retrospective observational multicenter study including Fontan patients operated between 1990 and 2022. Anatomical, clinical, surgical and liver-related data were collected, defining FALD as a spectrum of time-related structural–functional liver modifications due to congestive hepatopathy (from mild liver fibrosis to liver cirrhosis and hepatocellular carcinoma) diagnosed through multiparametric evaluations. 14 centers routinely conducted liver assessment after Fontan completion. Out of 2141 patients, 343 (16%) were diagnosed with FALD (M/F = 198/145; median age 18 years, IQR 15–26) with a median follow-up time of 14 years (IQR 9–20) from Fontan surgery. Among these, there were 19 (5.5%) deaths, 5 (26.3%) of whom related to advanced liver disease/cancer. FALD showed no significant association with gender (p = 0.4, adjusted p-value = 0.5), dominant ventricular morphology (p = 0.060, adjusted p-value = 0.086) nor surgery type (p = 0.3, adjusted p-value = 0.4). Significant association emerged between FALD and fenestration absence (p < 0.001, adjusted p-value < 0.001), systemic ventricular (p < 0.001, adjusted p-value < 0.001) and atrio-ventricular valve (p < 0.001) dysfunction, III-IV NYHA classes (p < 0.001, adjusted p-value < 0.001), tachyarrhythmias (p < 0.001) and liver stiffness ≥ 22 kPa on transient elastography (p < 0.001, adjusted p-value < 0.001). The analysis demonstrated no significant association between FALD and abnormal liver function tests (p = 0.2), heart transplantation (p = 0.6, adjusted p-value = 0.6), worse survival (p = 0.38). This study shows significant mortality related to FALD, which is also associated to clinical signs of failing Fontan circulation, stressing the pressing need of universally shared diagnostic criteria and surveillance protocols, to prevent and/or early-identify FALD and its more lethal complications.

Purpose To assess the accuracy of magnetic resonance (MR) imaging-based differential diagnosis of hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC) in a cohort of patients with focal liver lesions and cirrhosis. Materials and Methods This study was institutional review board approved, and the requirement for informed consent was waived. Between January 2009 and December 2014, a cohort of cirrhotic patients, including 71 with ICC and 612 with HCC, underwent unenhanced T1- and T2-weighted MR imaging, gadolinium-based contrast material-enhanced dynamic phase imaging, and partial hepatectomy. Results of pathologic examinations were obtained for all patients. Clinical, radiologic, and pathologic results in these patients were analyzed and compared comprehensively. Differences in signal intensity on baseline and contrast material-enhanced images and dynamic enhancement patterns were evaluated and compared by using the χ(2) test or the Fisher exact test. Results The preoperative diagnoses of 517 of 683 lesions were confirmed pathologically. Five ICCs and 481 HCCs displayed contrast material washout in delayed phases (P < .001). Thirty-six ICCs and 23 HCCs displayed a progressive enhancement pattern (P < .001). Twenty-six ICCs and 63 HCCs displayed a stable enhancement pattern (P < .001). ICCs displayed significantly different enhancement patterns according to size (P = .022). Conclusion The accurate discrimination of small ICCs from HCCs in the setting of cirrhosis at MR imaging is difficult, as substantial proportions of ICCs and HCCs have similar enhancement patterns. Absence of contrast material washout at MR imaging is not a factor that prevents the misdiagnosis of HCC. (©) RSNA, 2016 Online supplemental material is available for this article.

Similar performance of liver stiffness measurement and liver surface nodularity for the detection of portal hypertension in patients with hepatocellular carcinoma.