scholarly journals Risk of Liver Cirrhosis and Hepatocellular Carcinoma after Fontan Operation: A Need for Surveillance

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1805
Author(s):  
Jun Sik Yoon ◽  
Dong Ho Lee ◽  
Eun Ju Cho ◽  
Mi Kyoung Song ◽  
Young Hun Choi ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Cumulative Incidence ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Late Complication ◽  
Elevated Serum ◽  
Arterial Phase ◽  
Portal Venous Phase

Liver cirrhosis and hepatocellular carcinoma (HCC) are serious late complications that can occur after the Fontan procedure. This study aimed to investigate the cumulative incidence of cirrhosis and HCC and to identify specific features distinguishing HCC from benign arterial-phase hyperenhancing (APHE) nodules that developed after the Fontan operation. We retrospectively enrolled 313 post-Fontan patients who had been followed for more than 5 years and had undergone ultrasound or computed tomography (CT) of the liver between January 2000 and August 2018. Cirrhosis was diagnosed radiologically. The estimated cumulative incidence rates of cirrhosis at 5, 10, 20, and 30 years after the Fontan operation were 1.3%, 9.2%, 56.6%, and 97.9%, respectively. Multiphasic CT revealed that 18 patients had APHE nodules that were ≥1 cm in size and showed washout in the portal venous phase (PVP)/delayed phase, which met current noninvasive HCC diagnosis criteria. Among them, only seven patients (38.9%, 7/18) were diagnosed with HCC. After cirrhosis developed, the annual incidence of HCC was 1.04%. The appearance of washout in the PVP (p = 0.006), long time elapsed since the initial Fontan operation (p = 0.04), large nodule size (p = 0.03), and elevated serum α-fetoprotein (AFP) level (p < 0.001) were significantly associated with HCC. In conclusion, cirrhosis is a frequent late complication after Fontan operation, especially after 10 years, and HCC is not a rare complication after cirrhosis development. Diagnosis of HCC should not be based solely on the current imaging criteria, and washout on PVP and clinical features might be helpful to differentiate HCC nodules from benign APHE nodules.

Incidence and Expected Probability of Liver Cirrhosis and Hepatocellular Carcinoma After Fontan Operation

Circulation ◽  
2021 ◽  
Vol 144 (25) ◽  
pp. 2043-2045 ◽  
Author(s):  
Masaki Nii ◽  
Ryo Inuzuka ◽  
Kei Inai ◽  
Eriko Shimada ◽  
Tokuko Shinohara ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Fontan Operation

scholarly journals Fontan-associated liver disease and hepatocellular carcinoma in adults

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Tomomi Kogiso ◽  
Katsutoshi Tokushige
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Palliative Therapy ◽  
Nationwide Survey ◽  
Venous Drainage ◽  
Cardiac Complications ◽  
Sinusoidal Dilatation

AbstractThe Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

FRI-447-Clinical characteristics of liver cirrhosis and hepatocellular carcinoma occuring after Fontan operation

2019 ◽  
Vol 70 (1) ◽  
pp. e592-e593
Author(s):  
Jun Sik Yoon ◽  
Dong Ho Lee ◽  
Eun Ju Cho ◽  
Hyo Young Lee ◽  
Sun Woong Kim ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Clinical Characteristics ◽  
Fontan Operation

scholarly journals Magnetic resonance imaging of the cirrhotic liver: diagnosis of hepatocellular carcinoma and evaluation of response to treatment - Part 1

2017 ◽  
Vol 50 (1) ◽  
pp. 38-47 ◽  
Author(s):  
Miguel Ramalho ◽  
António P. Matos ◽  
Mamdoh AlObaidy ◽  
Fernanda Velloni ◽  
Ersan Altun ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Magnetic Resonance ◽  
Cirrhotic Liver ◽  
Response To Treatment ◽  
Arterial Phase ◽  
Resonance Imaging ◽  
Regenerative Nodules ◽  
Magnetic Resonance Imaging Mri

Abstract Magnetic resonance imaging (MRI) is the modern gold standard for the noninvasive evaluation of the cirrhotic liver. The combination of arterial phase hyperenhancement and delayed wash-out allows a definitive diagnosis of hepatocellular carcinoma (HCC) in patients with liver cirrhosis or chronic liver disease, without the requirement for confirmatory biopsy. That pattern is highly specific and has been endorsed in Western and Asian diagnostic guidelines. However, the sensitivity of the combination is relatively low for small HCCs. In this two-part review paper, we will address MRI of the cirrhotic liver. In this first part, we provide a brief background on liver cirrhosis and HCC, followed by descriptions of imaging surveillance of liver cirrhosis and the diagnostic performance of the different imaging modalities used in clinical settings. We then describe some of the requirements for the basic MRI technique, as well as the standard MRI protocol, and provide a detailed description of the appearance of various types of hepatocellular nodules encountered in the setting of the carcinogenic pathway in the cirrhotic liver, ranging from regenerative nodules to HCC.

scholarly journals Imaging features of hepatocellular carcinoma with bile duct tumor thrombus：a multicenter study

2020 ◽  
Author(s):  
Li-Ming Huang ◽  
Jun-Yi Wu ◽  
Yan-Nan Bai ◽  
Jia-Yi Wu ◽  
Yong-Gang Wei ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Bile Duct ◽  
Ct Scans ◽  
Arterial Phase ◽  
Imaging Features ◽  
Portal Venous Phase ◽  
Bile Duct Tumor ◽  
Hepatic Arterial Phase ◽  
Mri Scans ◽  
Venous Phase

Abstract Background: There are still difficult and challenging problems in diagnosis of hepatocellular carcinoma (HCC) with bile duct tumor thrombus (BDTT) before operation. This study aimed to analyze the imaging features of HCC with B1-B3 BDTT. Methods: The clinicopathological data and imaging findings of 30 HCC patients with B1-B3 BDTT from three high-volume institutions were retrospectively reviewed. Eighteen patients underwent computed tomography (CT) scans and twelve patients underwent magnetic resonance imaging (MRI) scans before operation, respectively. The diagnosis of HCC with BDTT was confirmed by postoperative pathologic examination.Results: According to Japanese classification, 5 patients were classified as B1 BDTT, 12 B2, 13 B3, and 82 B4, respectively. The HCC lesions were detected in all patients, and the localized bile duct dilation were detected in 28 (93.3%) patients. The BDTT was observed in all B3 patients and 3 B2 patients, but it was not observed in all B1 patients on CT or MRI. The BDTT showed relatively hypoattenuation on plain CT scans and T1W images, relatively hyperattenuation signals on T2W. The BDTT showed hyperattenuation at hepatic arterial phase with washout at portal venous phase. The localized biliary dilation showed no enhancement at hepatic arterial phase and no progressively delayed enhancement at portal venous phase, but it was more obvious at portal venous phase on CT.Conclusions: The HCC lesions and the localized bile duct dilatation on CT or MRI scans are imaging features of HCC with BDTT, which might facilitate the early diagnosis for B1-B3 BDTT.

scholarly journals Incidence, Predictors, and Mortality in Patients With Liver Cancer After Fontan Operation

2021 ◽  
Vol 10 (4) ◽  
Author(s):  
Hideo Ohuchi ◽  
Yohsuke Hayama ◽  
Kimiko Nakajima ◽  
Kenichi Kurosaki ◽  
Isao Shiraishi ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Liver Cancer ◽  
Hospice Care ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Late Complication ◽  
Asymptomatic Patients ◽  
Median Period ◽  
New Onset

Background Liver cancer (LC) is a serious late complication after the Fontan operation. However, the incidence, predictors, and prognosis remain unknown. The purpose of our study was to determine these clinical characteristics. Methods and Results We assessed liver function in 339 consecutive patients who had undergone the Fontan procedure from 2005 to 2019. LC was histologically diagnosed in 10 patients after a median period of 2.9 years (range: 0.3–13.8; median age: 29.9 years [range: 14.4–41.5 years]; overall median post–Fontan procedure follow‐up: 25.6 years [range: 13–32.1 years]), and the annual incidence was 0.89%. Over the entire post‐Fontan follow‐up period, the annual incidences of new‐onset LC in the second, third, and fourth decades were 0.14%, 0.43%, and 8.83%, respectively. The patients with LC had longer follow‐up periods, higher levels of AFP (α‐fetoprotein), and higher values of liver fibrosis indices ( P <0.01–0.0001). Moreover, all indices were predictive of new‐onset LC ( P <0.01–0.0001). The LC treatments were surgical resection (n=3), transarterial chemoembolization (n=3), radiofrequency ablation (n=2), and hospice care (n=2). During a median follow‐up of 9.4 months, 4 patients died; the survival rate at 1 year was 60%, and it was better among asymptomatic patients ( P <0.01). Conclusions The LC incidence rapidly increased ≥30 years after the Fontan procedure, and liver fibrosis indices and AFP were predictive of new‐onset LC. These LC‐predictive markers should be monitored closely and mandatorily for early LC detection and better prognosis.

scholarly journals Quantitative Analysis of Hypervascular Hepatic Metastases and Hepatocellular Carcinoma using Spectral CT Imaging

2019 ◽  
Author(s):  
Zhiqiang Yang ◽  
Xinyi Wang ◽  
Hao Shi
Keyword(s):  
Hepatocellular Carcinoma ◽  
Quantitative Analysis ◽  
Spectral Curve ◽  
Iodine Concentration ◽  
Ct Imaging ◽  
Arterial Phase ◽  
Spectral Ct ◽  
Portal Venous Phase ◽  
Significant Difference ◽  
Venous Phase

Abstract Objective The goal of this study is to evaluate the performance of spectral CT-based quantitative analysis in differential diagnosis of hypervascular hepatic metastasis (HVHM) and hepatocellular carcinoma (HCC). Methods Spectral CT scans were performed for 47 patients with hepatic malignant tumors, including 20 patients with HVHM and 27 patients with HCC, which generated the following sets of data: single energy images in the arterial phase; iodine and water maps; marginal areas of lesions that manifested apparent signal intensification; and energy spectral parameters of normal liver tissues and abdominal aorta. Subsequently, we calculated the normalized iodine concentrations (NIC), lesion-normal parenchyma iodine concentration ratio (LNR), iodine concentration difference (ICD) between the arterial phase and the venous phase, and the spectral curve slope. An independent samples t test and receiver operating characteristic (ROC) curve analysis were applied to examine these quantitative parameters. Results In the arterial phase, the HVHM and HCC groups displayed no differences in NIC, LNR, or spectral curve slope (P > 0.05). In the venous phase, the two groups displayed significant differences in NIC, LNR, and spectral curve slope; the NIC was 0.59 ± 0.08 for the HVHM group and 0.4 5 ± 0.10 for the HCC group; the LNR was 1.17 ± 0.22 and 0.92 ± 0.16, respectively; the spectral curve slope was 1.85 ± 0.49 and 1.18 ± 0.34, respectively. In addition, there was no significant difference in ICD between the HVHM group (0.54 ± 0.39 g/L) and HCC group (0.45 ± 0.39 g/L) (P > 0.05). Finally, there were no significant differences of water or iodine concentration between the arterial phase and venous phase (P > 0.05). Taken together, the spectral curve slope in the portal venous phase had the best performance in differentiating HVHM from HCC. Conclusions HVHM and HCC have apparent differences in spectral curve and concentrations of radiocontrast agents in the portal venous phase. Hence, spectral CT imaging provides a new multiparameter quantitative approach for differentiating HVHM and HCC.

scholarly journals An Emphasis on Screening to Detect Liver Cirrhosis and Hepatocellular Carcinoma in Patients Having Undergone the Fontan Procedure in Early Childhood

2020 ◽  
Vol 14 (3) ◽  
pp. 615-623
Author(s):  
Samragnyi Madala ◽  
Kira MacDougall ◽  
Abhishek Polavarapu ◽  
Dinesh Gurala ◽  
Vivek Gumaste ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Venous Blood ◽  
Liver Function Tests ◽  
Cardiovascular Complications ◽  
Young Female ◽  
Abdominal Ultrasonography ◽  
Monitoring And Surveillance

The Fontan procedure is a surgical procedure for patients with single-ventricle anatomy that results in the flow of systemic venous blood to the lungs without passing through a ventricle. Before the 1970s, most children with single-ventricle anatomy failed to survive into adulthood. With the introduction of the Fontan procedure, and its many modifications, the survival rate of these patients improved exponentially. With patients surviving longer, complications from this procedure are being documented for the first time. Cardiovascular complications are expected early on and are well studied. More serious are the non-cardiovascular complications in patients who survive into adulthood. The biggest entity is Fontan-associated liver disease (FALD) which needs thorough monitoring to screen for hepatocellular carcinoma (HCC). FALD includes chronic passive congestion, liver cirrhosis, and HCC. Once cirrhosis develops, monitoring with annual liver function tests, AFP, and abdominal ultrasonography need to occur to screen for HCC. Patients may need to be evaluated for combined heart-liver transplantation. Strict guidelines need to be developed for monitoring and surveillance of these patients to prevent late-stage complications. Herein, we report a unique case of FALD in a young female presenting two decades after the procedure with variceal bleeding.

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