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 ◽  
...  

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.

2019 ◽  
Vol 29 (Supplement_4) ◽  
Author(s):  
R Ibusuki ◽  
I Shimoshikiryo ◽  
K Shimatani ◽  
D Nishimoto ◽  
S Maenohara ◽  
...  

Abstract Background Nonalcoholic fatty liver disease (NAFLD) is increasing, being prevalent at 30% of the general population worldwide. A part of NAFLD develops nonalcoholic steatohepatitis (NASH), liver cirrhosis and hepatocellular carcinoma. Hepatic fibrosis plays an important role in their pathogenesis. However, it is unclear how hepatic fibrosis is observed and advanced in NAFLD among general population. To investigate the hepatic fibrosis among general population, we prospectively observed hepatic fibrosis using serum markers. Methods The subjects were 228 women who participated as a part of the Japan Multi-Institutional Collaborative Cohort (J-MICC) study in Kagoshima, Japan, and were followed from 2005 to 2014. NAFLD was defined as fatty liver by abdominal ultrasonography; consuming ethanol < 20 g/day; and being none hepatitis B and C carriers. NAFLD were confirmed after two-time consecutive examination, because its disappearance is ambiguous in some cases. Hepatic fibrosis was evaluated using serum M2BPGi and Type IV collagen 7s. The comparison of their changed values between groups was done using the ANOVA adjusted for age. The association between their change and related factors was done using general linear regression model. Results The prevalence of NAFLD was 31.6% at baseline. In the 5-year observation, the NAFLD + => NAFLD + ( ++) group was 23.7%; − +, 2.6%; + −, 7.9%; and - - (control), 65.8%. The values of M2BPGi and Type IV collagen 7s were higher in ++, -+, +- groups than controls at baseline. The change of M2BPGi values was observed in all groups, including controls, and the changed values were higher in ++ and -+ groups. Higher creatinine levels were positively associated with change of M2BPGi values. In contrast, the change of Type IV collagen 7s was not apparent. Conclusions This study suggested hepatic fibrosis was advanced with age among general women without NAFLD, and the presence of NAFLD enhanced hepatic fibrosis more. Key messages Hepatic fibrosis may be slightly developing with age among general population, and will be enhanced with fatty liver. It is important to prevent fatty liver development to control risk factors, such as obesity and metabolic syndrome, to reduce the risk of NASH, liver cirrhosis and hepatocellular carcinoma.


Author(s):  
Krizia Pocino ◽  
Cecilia Napodano ◽  
Gabriele Ciasca ◽  
Mariapaola Marino ◽  
Nicoletta De Matthaeis ◽  
...  

Background: Hepatocellular carcinoma (HCC) is a global health problem associated with chronic liver disease. The pathogenesis of chronic liver disease varies according to the underlying etiological factor, although in most cases it develops from a liver cirrhosis. The worsening progression of liver disease is accompanied by pathological angiogenesis, which is a prerequisite that favors the development of HCC. The aim of this study is to evaluate the clinical utility of circulating angiogenic markers VEGF, Ang-1, Ang-2, the Angiopoietin receptor (Tie1/2), HGF and PECAM-1 to screen early onset patients and to follow the evolution of HCC. Materials and Methods: We enrolled 62 patients; 33 out of 62 subjects were diagnosed for HCC and 29/62 for liver cirrhosis of different etiology without signs of neoplasia. Patients underwent venous blood sampling before and after treatments for VEGF, Ang-1, Ang-2, Tie1, Tie2, HGF and PECAM-1 measurement. Results: Ang-1 and Ang-2 are detectable not only in patients already suffering from HCC but also in cirrhotic patients without signs of cancer. Patients with HCC show higher HGF concentrations than patients with cirrhosis. A significant reduction in serum levels of Ang-2, Ang-2/Ang-1 and Ca 19-9 after DAAs therapy was observed. Moreover, VEGF levels were increased after treatment of HCC. Conclusion: The preliminary study here presented confirms that the mechanism of tumor angiogenesis is very complex and involves a very large number of factors. The integration of different methodologies and multi-marker algorithms is likely to emerge for the early diagnosis of HCC and the monitoring of the risk of relapse.


Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1805
Author(s):  
Jun Sik Yoon ◽  
Dong Ho Lee ◽  
Eun Ju Cho ◽  
Mi Kyoung Song ◽  
Young Hun Choi ◽  
...  

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.


2011 ◽  
Vol 18 (02) ◽  
pp. 251-254
Author(s):  
JAVED AHMED PHULPOTO ◽  
ALTAF AHMED SHAIKH ◽  
JAVED AKHTAR SAMO ◽  
Abrar Shaikh

Objective: To determine the frequency of co/super infection of hepatitis D in patients with hepatitis B related liver disorders. Study Design: Descriptive study. Place and Duration of Study: The hepatitis Centre Ghulam Mohammad Mahar Medical College Hospitals Sukkur & Khairpur, from January 2009 to December 2009. Methodology: All patient registered for HBV associated infections were selected. Blood was drawn from 200 patients who fulfilled the inclusion criteria. Those with an incomplete test profile were excluded. All clinical conditions were investigated through liver function tests, coagulation profile, abdominal ultrasonography, upper gastrointestinal endoscopy and liver biopsy. Liver cirrhosis and hepatocellular carcinoma (HCC) were diagnosed either on the basis of histology, or on a combination of radiological, endoscopic and laboratory data. Hepatitis B virus DNA was extracted from serum by real time PCR. Descriptive statistics were used for frequency and mean determination. Results: The 129 patients finally selected for statistical analysis included 108 (84%) males and 21 (16%) females. The ages ranged from 6-68 years (mean=31.5 ± 12.39 years). There were 70 (54.2%) patients of non-cirrhotic, chronic hepatitis (CLD), 38 (29.4%) carriers, 12 (9.3%) cirrhotic and 9 (6.9%) hepatocellular carcinoma (HCC) patients. Among the 129 patients, 45 (34.9%) were positive for double infection with HDV. These included 35 CLD cases, 7 cirrhotic and 3 carriers. Conclusions: The frequency of co/super infection of hepatitis D was found to be highest in HBV cirrhosis patients compared to patients having chronic liver disease (non-cirrhotics) and carriers.


2021 ◽  
Vol 11 (1) ◽  
pp. 108-114
Author(s):  
Amal A. Mohamed ◽  
Yousry Esam-Eldin Abo-Amer ◽  
Amyan Aalkhalegy ◽  
Lamiaa Abdelfattah Fathalla ◽  
Mostafa Bedair Elmaghraby ◽  
...  

Introduction: Collagens are the most abundant proteins in the human body, accounting for one-third of total proteins. Over the last few years, accumulated evidence have indicated that some collagens are differentially expressed in cancer. The aim of the study was to assess COL1A1 gene expression as a novel marker for the progression of hepatitis B cirrhosis into hepatocellular carcinoma. Methods: This cohort study included 348 subjects and was conducted between May 2018 and June 2019. Subjects were divided into 4 groups: group1 included HBV positive hepatocellular carcinoma patients “HCC” (n= 87), group II included HBV positive patients with liver cirrhosis “LC” (n = 87), group III included chronic hepatitis B patients with neither HCC nor cirrhosis “ C-HBV” (n = 87) and group IV consisted of healthy volunteers as controls (n = 87). Fasting venous blood samples (10 ml) were collected from each participant in this study and were used for assessment of aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin, albumin and alfa-fetoprotein (AFP). Another portion of blood was collected in 2 vacutainer tubes containing EDTA, one for Complete blood count and the other for gene expression of COL1A1. Results: The gene expression of collagen was 6.9 ± 8.8 in group 1 (HBV positive hepatocellular carcinoma patients) and this was a significant increase in comparison with the other groups. In group 2 (HBV positive patients with liver cirrhosis), the gene expression (collagen) was 3.7±1.5 and it was significantly increased when compared with group 4 (healthy volunteers). Conclusion: COL1A1 gene expression can be used as an indicator of the progression of hepatitis B cirrhosis into hepatocellular carcinoma.


2018 ◽  
Vol 9 (4) ◽  
pp. 451-453 ◽  
Author(s):  
Jenny E. Zablah ◽  
Michael Ross ◽  
Neil Wilson ◽  
Brian Fonseca ◽  
Max B. Mitchell

Single ventricle patients with interrupted inferior vena cava (IVC) and azygos continuation to the superior vena cava (SVC) are typically palliated with a bidirectional cavopulmonary shunt (BCPS), known as the Kawashima operation in this setting. Because the volume of venous blood directed to the pulmonary arteries is substantially greater in the presence of interrupted IVC, Kawashima procedures are commonly delayed to older age compared to other single ventricle patients undergoing BCPS. We report two young infant single ventricle patients with interrupted IVC and azygos continuation to the SVC who underwent stage I Norwood procedures for initial palliation. In both cases, a fenestrated hemi-Fontan procedure achieved successful Kawashima circulations.


2020 ◽  
Vol 15 (4) ◽  
pp. 466-471
Author(s):  
Min-kyung Cho ◽  
Ji-Hye Kwon ◽  
Mi Sook Gwak ◽  
Jae-Won Joh ◽  
JiYun Hwang ◽  
...  

Background: Fontan-associated liver disease (FALD) is a hepatic disorder caused by hemodynamic changes and systemic venous congestion following the Fontan procedure. FALD includes liver cirrhosis and hepatocellular carcinoma (HCC), both of which may require liver transplantation (LT). However, the Fontan circulation, characterized by elevated central venous pressure and reduced cardiac output, is a challenging issue for surgeons and anesthesiologists.Case: We report a living-donor LT for the treatment of HCC. The patient was a 24-year-old male who underwent the Fontan procedure for pulmonary atresia and right ventricle hypoplasia. We focused on maintaining enough blood volume for cardiac output without causing pulmonary edema, as the patient is not well adapted to changes in volume. Owing to a multidisciplinary approach, the surgery was successfully performed without fatal adverse events.Conclusions: To our knowledge, this is the first case of isolated LT in a recipient who became an adult after having undergone the Fontan procedure.


Author(s):  
Santiago A. Ortiz Galindo ◽  
Philipp K. Haber ◽  
Christian Benzing ◽  
Felix Krenzien ◽  
Anna Riddermann ◽  
...  

Abstract Purpose The aim of this study was to analyze the impact of minimally invasive intermittent Pringle maneuver (IPM) on postoperative outcomes in patients with hepatocellular carcinoma (HCC) and liver cirrhosis. Methods In this retrospective cohort study, we evaluated the safety of IPM in patients with HCC who underwent minimally invasive liver resection during five years at our center. Factors influencing the use of IPM were examined in univariate and multivariate regression analysis. Cases with use of IPM (IPM) and those without use of IPM (no IPM) were then compared regarding intraoperative and postoperative outcomes after propensity score matching (PSM) for surgical difficulty. Results One hundred fifty-one patients underwent liver resection for HCC at our center and met inclusion criteria. Of these, 73 patients (48%) received IPM with a median duration of 18 min (5–78). One hundred patients (66%) had confirmed liver cirrhosis. In multivariate analysis, patients with large tumors (≥ 3 cm) and difficult tumor locations (segments VII or VIII) were more likely to undergo IPM (OR 1.176, p = 0.043, and OR 3.243, p = 0.001, respectively). After PSM, there were no differences in intraoperative blood transfusion or postoperative complication rates between the IPM and no IPM groups. Neither did we observe any differences in the subgroup analysis for cirrhotic patients. Postoperative serum liver function tests were not affected by the use of IPM. Conclusions Based on our findings, we conclude that the use of IPM in minimally invasive liver resection is safe and feasible for patients with HCC, including those with compensated liver cirrhosis.


Cancers ◽  
2021 ◽  
Vol 14 (1) ◽  
pp. 11
Author(s):  
Krizia Pocino ◽  
Cecilia Napodano ◽  
Mariapaola Marino ◽  
Riccardo Di Santo ◽  
Luca Miele ◽  
...  

Background: Hepatocellular carcinoma (HCC) is a global health problem associated with chronic liver disease. Its pathogenesis varies according to the underlying etiological factors, although in most cases it develops from liver cirrhosis. The disease progression is accompanied by pathological angiogenesis, which is a prerequisite that favors the development of HCC. Aims: This study aims at contributing to our understanding of the role of angiogenic factors in the progression of liver disease. For this purpose, we evaluate the clinical significance of serum angiogenic markers (VEGF, Ang-1, Ang-2, the angiopoietin receptor Tie1/2, HGF, and PECAM-1) first in cirrhotic and HCC patients separately, and then comparing cirrhotic patients with and without HCC. Materials and Methods: We enrolled 62 patients, out of whom 33 were diagnosed with HCC and 29 with liver cirrhosis without signs of neoplasia. Patients underwent venous blood sampling before and after receiving treatments for the diagnosed disease. Serum markers were evaluated using ELISA assays for Tie1 and the Bio-Plex Multiplex system for the remaining ones. Biomarker levels were investigated as a function of clinical scores for disease staging (MELD and Fibrosis Index, FI). Results: In cirrhotic patients, Ang-1 and Ang-2 correlate with MELD (ρAng-1 = −0.73, p = 2E−5) and FI (ρAng-1 = −0.52, p = 7E−3, ρAng-2 = 0.53, p = 3E−3). A reduction of Ang-2 levels (p = 0.047) and of the Ang-2/Ang-1 ratio (p = 0.031) is observed in cirrhotic patients diagnosed with viral hepatitis after antiviral treatments. In HCC patients, Ang-1 negatively correlates with FI (ρ = −0.63, p = 1E−4), and PECAM-1 positively correlates with MELD (ρ = 0.44, p = 0.01). A significant Ang-1 reduction was observed in deceased patients during the study compared to ones who survived (p = 0.01). In HCC patients, VEGF levels were increased after tumor treatment (p = 0.037). Notably, HGF levels in cirrhotic patients with HCC are significantly raised (p = 0.017) compared to that in those without HCC. Conclusions: Our results suggest that serum angiogenic markers, with emphasis on Ang-1/2, can contribute to the development of quantitative tools for liver disease staging and therapy monitoring. The comparison between cirrhotic patients with and without HCC suggests that HGF levels are potentially useful for monitoring the insurgence of HCC after a cirrhosis diagnosis. High Ang-1 levels in HCC patients appear to have a protective role as well as prognostic significance.


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