Non‐invasive assessment of liver fibrosis in patients after the Fontan operation

Furukawa, T.; Akimoto, Katsumi; Ohtsuki, Masahiro; Sato, Keiko; Suzuki, Mitsuyoshi; Takahashi, Ken; Kishiro, Masahiko; Shimizu, Toshiaki; Kawasaki, Shiori

doi:10.1111/j.1442-200x.2011.03497.x

Cited by 27 publications

(8 citation statements)

References 28 publications

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“…Furukawa et al 16 also found that most routine liver laboratory values on serial evaluation were within normal ranges in children after Fontan, even in patients with elevated serum markers of cirrhosis. This is contrary to reports of liver laboratory abnormalities in a substantial number of Fontan patient cohorts in previous studies.…”

Section: Discussionmentioning

confidence: 91%

Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

Simpson

Esmaeeli

Khanna

et al. 2014

The Journal of Heart and Lung Transplantation

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Background Liver cirrhosis is recognized with long-term follow-up of patients after the Fontan procedure. The effect of liver cirrhosis on the use of heart transplant (HT) and on post-HT outcomes is unknown. Methods We reviewed Fontan patients evaluated for HT from 2004 to 2012 with hepatic computed tomography (CT) imaging, classified as normal, non-cirrhotic changes, or cirrhosis. The primary outcome was 1-year all-cause mortality, and the secondary outcome was differences in serial post-HT liver evaluation. Results CT imaging in 32 Fontan patients evaluated for HT revealed 20 (63%) with evidence of liver disease, including 13 (41%) with cirrhosis. Twenty underwent HT, including 5 non-cirrhotic and 7 cirrhosis patients. Characteristics at listing between normal or non-cirrhotic (n = 13) and cirrhosis (n = 7) groups were similar, except cirrhosis patients were older (median 17.6 vs 9.6 years, p = 0.002) and further from Fontan (median 180 vs 50 months, p < 0.05). Serial liver evaluation was similar, including aspartate aminotransferase, alanine aminotransferase, bilirubin, albumin, and tacrolimus dose at 1, 3, 6, 9, and 12 months. Overall patient survival was 80% at 1 year, with no difference between cirrhosis and non-cirrhosis patients (86% vs 77%, p = 0.681). Liver biopsies were performed in 7 patients before HT, and all specimens showed architectural changes with bridging fibrosis. Conclusions Most patients evaluated for HT had abnormal liver findings by CT, with cirrhosis in 41%. One-year mortality and serial liver evaluation were similar between groups after HT. Liver cirrhosis identified by CT imaging may not be an absolute contraindication to HT alone in this population.

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Section: Discussionmentioning

confidence: 91%

Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

Simpson

Esmaeeli

Khanna

et al. 2014

The Journal of Heart and Lung Transplantation

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“…A low cardiac index and reduced heart rate were significantly related to liver dysfunction in a Fontan patient series [91]. The IVC pressure showed a significant association with type IV collagen (a surrogate marker for hepatic fibrosis) in Fontan patients [145]. The CVP was a significant predictor of liver stiffness measured by MRE in a Fontan population [93].…”

Section: Hemodynamicsmentioning

confidence: 97%

Liver disease secondary to congenital heart disease in children

Komatsu

Inui

Kishiki

et al. 2019

Expert Review of Gastroenterology & Hepatology

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Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation. Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed. Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.

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“…39 Many of the biomarkers used to assess fibrosis in viral hepatitis have been extrapolated to FALD. 30,37,[40][41][42][43][44] The best characterized is the FibroTest (FibroSURE in the USA), which utilizes α 2 -macroglobulin, haptoglobin, GGT, bilirubin, apolipoprotein A1 and a proprietary algorithm to calculate a score predictive of fibrosis stage. [45][46][47] Most Fontan patients have significantly elevated FibroTest scores (range 0.33 to 0.82, normal r0.21), but there is poor correlation with time post-Fontan, other investigations or biopsy.…”

Section: Laboratory Testing For Faldmentioning

confidence: 99%

Fontan-associated liver disease: Implications for heart transplantation

Greenway

Crossland

Hudson

et al. 2016

The Journal of Heart and Lung Transplantation

106

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Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury. Pathology studies have generally shown that FALD worsens as time post-Fontan increases, but the prevalence of FALD is not well defined because the majority of Fontan patients, even those with significant hepatic fibrosis, appear to be asymptomatic and biochemical or functional hepatic abnormalities are usually subtle or absent. Alternate non-invasive investigations, derived from the study of other chronic liver diseases, have been tested in small series of pediatric and adult Fontan patients, but they have been confounded by congestion and do not correlate well with liver biopsy findings. Liver disease can complicate Fontan circulatory failure and may even be significant enough to be considered a contraindication to heart transplantation or require combined heart-liver transplantation. The search for the optimal management strategy continues in the setting of increasing numbers of Fontan patients surviving to adulthood and being referred for heart transplantation. Thus, in this review we attempt to define the scope and significance of FALD and address transplant-related assessment and management of this challenging disorder.

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Non‐invasive assessment of liver fibrosis in patients after the Fontan operation

Abstract: No specific parameter that reflects the progress in liver fibrosis was identified in this study. The possibility exists that type IV collagen reflects the degree of hepatic congestion.

Cited by 27 publications

References 28 publications

Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function

Liver disease secondary to congenital heart disease in children

Fontan-associated liver disease: Implications for heart transplantation

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