Fontan‐Associated Liver Disease: Spectrum of Disease in Children and Adolescents

Rathgeber, Steven L.; Guttman, Orlee R.; Lee, Anna F.; Voss, Christine; Hemphill, Nicole M.; Schreiber, Richard A.; Harris, Kevin C.

doi:10.1161/jaha.119.012529

Cited by 47 publications

(43 citation statements)

References 26 publications

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“…Assessment of liver stiffness by transient elastography (TE), acoustic radiation force impulse (ARFI) elastography, and magnetic resonance elastography (MRE) enables evaluation of hepatic fibrosis 29 – 31 . However, it was not correlated with the histopathologic findings at a single time point 32 . ARFI and TE might be useful for monitoring liver stiffness in patients with Fontan physiology.…”

Section: Introductionmentioning

confidence: 83%

Fontan-associated liver disease and hepatocellular carcinoma in adults

Kogiso

Tokushige

2020

Sci Rep

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The 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.

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Section: Introductionmentioning

confidence: 83%

Fontan-associated liver disease and hepatocellular carcinoma in adults

Kogiso

Tokushige

2020

Sci Rep

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“…In a large series of Fontan patients from the Mayo Clinic, 10-, 20-, and 30-year freedom from cirrhosis was 99%, 94%, and 57% [ 16 ]. Liver disease is already present in pediatric patients with Fontan physiology [ 17 ]. The pathophysiology is multifactorial and includes factors like central venous hypertension/passive congestion, as well as hypoxia due to systemic ventricular dysfunction and collaterals [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…The pathophysiology is multifactorial and includes factors like central venous hypertension/passive congestion, as well as hypoxia due to systemic ventricular dysfunction and collaterals [ 18 ]. Routine liver health surveillance in this population should be implemented, especially considering the risk of developing hepatocellular carcinoma, but treatment options remain limited [ 17 , 19 ]. Liver transplantation or combined heart–liver transplantation could be an option in selected cases.…”

Section: Discussionmentioning

confidence: 99%

Patients with Single-Ventricle Physiology over the Age of 40 Years

Pujol

Schiele

Maurer

et al. 2020

JCM

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Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, SVP patients under active follow-up at our center who were ≥40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality. Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 ± 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8–8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction (n = 27, 55.1%) and renal disease (n = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5–106.3, p = 0.021) remained as an independent predictor. Conclusions: SVP patients ≥40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.

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“…Such liver alterations are usually more pronounced in adults than in children. Therefore, after cavopulmonary shunts, liver congestion is a major problem and seems to be present in many children with Fontan circulation (46,47). In addition, hypoxemia of the liver parenchyma may occur in low cardiac output, which is typical for Fontan-hemodynamics, as well as by a right-left shunt (e.g., in fenestrated Fontan).…”

Section: Liver Changes After Fontan-operation For Univentricular Heartsmentioning

confidence: 99%

Congenital heart disease-associated liver disease: a narrative review

Reiter¹,

Hadjamu²,

Nagdyman³

et al. 2021

Cardiovasc Diagn Ther

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Congenital heart diseases (CHD) can be associated with liver dysfunction. The cause for liver impairment can result out of a wide spectrum of different causes, including liver congestion, hypoxemia or low cardiac output. Fortunately, most CHD show a good long-term outcome from a cardiac perspective, but great attention should be paid on non-cardiac health problems that develop frequently in patients suffering from CHD. The treatment of liver dysfunction in CHD requires a close multidisciplinary management in a vulnerable patient collective. Unfortunately, structured recommendations on the management of liver dysfunction in patients with CHD are scarce. The objective of this review is to provide insights on the pathophysiology and etiologies of liver dysfunction as one of the most relevant non-cardiac problems related to CHD. Furthermore, we advise here on the management of liver disease in CHD with special attention on assessment of liver dysfunction, management of portal hypertension as well as on surveillance and management of hepatocellular carcinoma (HCC). A multidisciplinary perspective may help to optimize morbidity and mortality in the long-term course in these patients. However, as evidence is low in many aspects, we encourage the scientific community to perform prospective studies to gain more insights in the treatment of liver dysfunction in patients with CHD.

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Fontan‐Associated Liver Disease: Spectrum of Disease in Children and Adolescents

Cited by 47 publications

References 26 publications

Fontan-associated liver disease and hepatocellular carcinoma in adults

Fontan-associated liver disease and hepatocellular carcinoma in adults

Patients with Single-Ventricle Physiology over the Age of 40 Years

Congenital heart disease-associated liver disease: a narrative review

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