Hepatic and renal end-organ damage in the Fontan circulation: A report from the Australian and New Zealand Fontan Registry

Wilson, Thomas G.; d’Udekem, Yves; Winlaw, David S.; Cordina, Rachael; Celermajer, David S.; Wheaton, Gavin; Bullock, Andrew; Gentles, Thomas L.; Weintraub, Robert G.; Justo, Robert; Grigg, Leeanne; Radford, Dorothy J.; Hardikar, Winita; Cheung, Michael; Cain, Tim; Rao, Padma; Alexander, Stephen I.; Ayer, Julian; Verrall, Charlotte E; Plessis, Karin du; Chapman, J Milne; Rice, Kathryn; Barry, Judith; Zannino, Diana; Iyengar, Ajay J.

doi:10.1016/j.ijcard.2018.07.118

Cited by 57 publications

(38 citation statements)

References 19 publications

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“…Although the literature is limited by short follow-up durations and a preponderance of cross-sectional data, time since Fontan surgery correlates with the presence and eventual progression of liver disease and its complications [301].…”

Section: Monitoringmentioning

confidence: 99%

“…Routine eGFR is impaired (<90 mL/min/1.73 m 2 ) in 10% of individuals with a Fontan circulation. In contrast, spot urine testing identifies microalbuminuria in a third of people with a Fontan circulation [35,37,301]. The prognostic utility of spot urine testing, and how this may correlate to future glomerular filtration rate (GFR) deterioration, remains to be confirmed in the Fontan population [37,301].…”

Section: Recent Research and Future Challengesmentioning

confidence: 99%

“…The prognostic utility of spot urine testing, and how this may correlate to future glomerular filtration rate (GFR) deterioration, remains to be confirmed in the Fontan population [37,301]. Emerging urinary biomarkers, such as kidney injury molecule-1 (KIM-1) and N-acetyl-b-Dglucosaminidase (NAG), as well as more cumbersome radionucleotide assays to quantify GFR, are useful research tools, with potential future clinical utility [37,301].…”

Section: Recent Research and Future Challengesmentioning

confidence: 99%

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Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.

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Section: Monitoringmentioning

confidence: 99%

Section: Recent Research and Future Challengesmentioning

confidence: 99%

Section: Recent Research and Future Challengesmentioning

confidence: 99%

See 1 more Smart Citation

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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“…The diagnostic accuracy of the FibroScan system for hepatic fibrosis has been validated in chronic viral hepatitis and nonalcoholic fatty liver disease. However, evidence of the utility of ultrasound-based elastography for cardiac hepatopathy remains insufficient [23,93,[103][104][105][131][132][133][134][135][136][137][138][139][140]. Because ultrasound-based elastography cannot differentiate blood from fibrosis, congestion influences the liver stiffness assessed by elastography.…”

Section: Elastographymentioning

confidence: 99%

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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“…In their cohort, odds of acute kidney injury after a third surgery was 2.4 times more likely if they had an acute kidney injury after either of the first 2 surgeries . There is a high prevalence of chronic kidney disease in the adult Fontan population with over 50% of patients having reduced glomerular filtration rate or microalbuminuria . Signs of early kidney dysfunction can be seen in younger Fontan children as well.…”

Section: Discussionmentioning

confidence: 99%

Comparison of creatinine and cystatin C for estimation of glomerular filtration rate in pediatric patients after Fontan operation

Kirelik

Fisher

Maria

et al. 2019

Congenital Heart Disease

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Background There are several limitations when using creatinine to estimate glomerular filtration rate, especially in children with chronic medical conditions who are at high risk of kidney dysfunction. Cystatin C has been the recent focus of research as a replacement biomarker for creatinine. Our objective was to compare the 2 biomarkers in pediatric single‐ventricle heart disease patients who have undergone the Fontan operation. We hypothesized that there would be poor correlation and agreement between the 2 estimates of renal function. Methods This was a single center retrospective chart review of 20 patients who had previously undergone Fontan operation. Demographic and clinical data were collected from medical records. Blood samples were collected as part of routine clinical care and simultaneously measured for serum creatinine and cystatin C. Glomerular filtration rate was calculated using the creatinine‐based bedside Schwartz formula and cystatin C‐based Zapatelli equation. Spearman correlation and Bland‐Altman analysis were used to assess correlation and agreement. Results The median Schwartz‐derived estimated glomerular filtration rate was 98.94 mL/min/1.73 m2 while the median Zappitelli‐derived estimated glomerular filtration rate was 84.76 mL/min/1.73 m2. The mean difference was −19.27 suggesting poor agreement. There was weak to moderate correlation between the Schwartz and cystatin C estimated glomerular filtration rate. Conclusion The bedside Schwartz formula may be an overestimate of glomerular filtration rate in pediatric single‐ventricle heart disease patients who have undergone the Fontan operation. While larger studies are necessary, cystatin C is a promising biomarker to replace creatinine and better estimate kidney function in this population.

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Hepatic and renal end-organ damage in the Fontan circulation: A report from the Australian and New Zealand Fontan Registry

Cited by 57 publications

References 19 publications

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Liver disease secondary to congenital heart disease in children

Comparison of creatinine and cystatin C for estimation of glomerular filtration rate in pediatric patients after Fontan operation

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