“…Hepatic fibrosis is noted on histological examination of liver biopsy and may be progressive with passage of time [ 8 ]. The condition poses significant risks with respect to potential compromise of liver function, development of benign liver lesions, and for the risk of liver cancer, specifically hepatocellular carcinoma [ 9 ]. Fig.…”
Section: The Multidisciplinary Approach: Areas Of Focusmentioning
Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model—the “Fontan rehabilitation, wellness and resilience development” or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.
“…Hepatic fibrosis is noted on histological examination of liver biopsy and may be progressive with passage of time [ 8 ]. The condition poses significant risks with respect to potential compromise of liver function, development of benign liver lesions, and for the risk of liver cancer, specifically hepatocellular carcinoma [ 9 ]. Fig.…”
Section: The Multidisciplinary Approach: Areas Of Focusmentioning
Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model—the “Fontan rehabilitation, wellness and resilience development” or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.
“…This is a multi-institutional retrospective cohort study. We consecutively collected the clinical data of patients who underwent and survived the Fontan operation and were discharged before 2011 from nine institutions in Japan through a retrospective chart review, as reported previously 2 6. Of a total of 1260 patients identified, 1117 (89% of all patients) for whom postoperative cardiac catheterisation data were available constituted the cohort for this study (online supplemental table 1) to determine the relationship between the baseline data at Fontan operation as well as postoperative catheterisation data and the development of advanced liver disease.…”
Section: Methodsmentioning
confidence: 99%
“…LC was diagnosed based on liver biopsy (stage F4) or imaging, including ultrasonography, contrast-enhanced CT and MRI. As in previous studies, LC was defined as the presence of contour nodularity, blunt margins, right hepatic lobe atrophy, caudate or left hepatic lobe hypertrophy (excluding symmetric liver in heterotaxy syndrome), and/or extrahepatic features (varices or congestive splenomegaly) 6 9 10. LFib was defined as stage F2 or F3 on liver biopsy or based on ultrasonography findings (blunted liver edge, densely dotted high echogenicity or coarse parenchymal echotexture) or contrast-enhanced CT/MRI (blunted liver edge or parenchymal enhancement with reticular or mosaic perfusion in portal venous phase without the above features of LC) 9 11–13.…”
Section: Methodsmentioning
confidence: 99%
“…FALD is reportedly highly prevalent in post-Fontan patients; thus, it has become a major concern 3–5. We recently reported that at 30 years after the Fontan operation, only about half of patients survive without developing advanced liver disease, such as LC or HCC 6…”
ObjectiveFontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening and management are lacking. The present study aimed to identify the predictors of liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in post-Fontan patients.MethodsThis was a multi-institutional retrospective cohort study. Clinical data of all perioperative survivors of Fontan operation before 2011 who underwent postoperative catheterisation were collected through a retrospective chart review.ResultsA total of 1117 patients (538 women, 48.2%) underwent their first Fontan operation at a median age of 3.4 years. Postoperative cardiac catheterisation was conducted at a median of 1.0 year. During a median follow-up period of 10.3 years, 67 patients (6.0%) died; 181 (16.2%) were diagnosed with liver fibrosis, 67 (6.0%) with LC, 54 (4.8%) with focal nodular hyperplasia and 7 (0.6%) with HCC. On multivariable analysis, high central venous pressure (CVP) (HR, 1.28 (95% CI 1.01 to 1.63) per 3 mm Hg; p=0.042) and severe atrioventricular valve regurgitation (HR, 6.02 (95% CI 1.53 to 23.77); p=0.010) at the postoperative catheterisation were identified as independent predictors of LC/HCC.ConclusionsPatients with high CVP and/or severe atrioventricular valve regurgitation approximately 1 year after the Fontan operation are at increased risk of developing advanced liver disease in the long term. Whether therapeutic interventions to reduce CVP and atrioventricular valve regurgitation decrease the incidence of advanced liver disease requires further elucidation.
“…The liver nodules are usually hyperechoic, hypervascular, and predominantly peripheral [9]. Nii et al [10] reported that a high central venous pressure (CVP) [hazard ratio (HR), 1.1; 95% confidence interval (CI), 1.003-1.2; p = 0.04] and severe atrioventricular valve regurgitation (hazard ratio, 6.0; 95% confidence interval, 1.5-24; p = 0.01) evident in the early postoperative period were independently predictive of liver cirrhosis and HCC.…”
Aims
The incidence of hepatocellular carcinoma (HCC) in patients with Fontan-associated liver disease (i.e., FALD-HCC) has increased over time. However, the risk factors for HCC development remain unclear. Here, we compared the levels of non-invasive markers to the survival rate of FALD-HCC patients.
Methods
From 2003 to 2021, 154 patients (66 men, 42.9%) developed liver disease after undergoing Fontan procedures. HCC was diagnosed in 15 (9.7%) (8 men, 53.3%) at a median age of 34 years (range, 21–45 years). We compared FALD-HCC and non-HCC cases; we generated marker level cutoffs using receiver operating characteristic curves. We sought to identify risk factors for HCC and mortality.
Results
The incidence of HCC was 4.9% in FALD patients within 20 years after the Fontan procedure. Compared with non-HCC patients, FALD-HCC patients exhibited higher incidences of polysplenia and esophageal varices. At the time of HCC development, the hyaluronic acid (HA) level (p = 0.04) and the fibrosis-4 index (p = 0.02) were significantly higher in FALD-HCC patients than in non-HCC patients; the total bilirubin (T-BIL) level (p = 0.07) and the model for end-stage liver disease score [excluding the international normalized ratio (MELD-XI)] (p = 0.06) tended to be higher in FALD-HCC patients. Within approximately 20 years of the Fontan procedure, 10 patients died (survival rate, 96.9%). Kaplan–Meier curve analysis indicated that patients with T-BIL levels ≥ 2.2 mg/dL, HA levels ≥ 55.5 ng/mL, and MELD-XI scores ≥ 18.7 were at high risk of HCC, a generally poor prognosis, and both polysplenia and esophageal varices. Multivariate Cox regression analyses indicated that the complication of polysplenia [Hazard ratio (HR): 10.915] and a higher MELD-XI score (HR: 1.148, both p < 0.01) were independent risk factors for FALD-HCC.
Conclusions
The complication of polysplenia and a MELD-XI score may predict HCC development and mortality in FALD patients.
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