Prevalence and Risk Factors Associated With Chronic Kidney Disease in Patients With Single Ventricle Congenital Heart Disease After Fontan Palliation

Patel, Suhag; Kwiatkowski, David M.; Andrei, Adin-Cristian; Devareddy, Ankita; Krawczeski, Catherine D.; Deal, Barbara J.; Langman, Craig B.; Marino, Bradley S.

doi:10.1016/s0735-1097(17)33975-x

Cited by 2 publications

(3 citation statements)

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“…Renal dysfunction is now known to be relatively common in Fontan patients, with an estimated glomerular filtration rate <90 ml/ min/1.73 m 2 being reported in 10-53% of long-term survivors, in whom it is associated with poor prognosis. [17][18][19][20] Furthermore, a recent systematic review of the factors associated with long-term mortality after the Fontan procedure identified renal failure as the fourth most common cause of late death. 21 This is due to elevated central venous pressure and impaired cardiac output resulting in a cascade of pathophysiological consequences in Fontan patients, including a progressive decline in renal function.…”

Section: Discussionmentioning

confidence: 99%

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

Søndergaard

Aboulhosn²,

d’Udekem

et al. 2021

Cardiol Young

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The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with “stable” Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial’s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in “stable” patients.

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Section: Discussionmentioning

confidence: 99%

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

Søndergaard

Aboulhosn²,

d’Udekem

et al. 2021

Cardiol Young

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“…The presence of acute kidney injury following the Fontan procedure is associated with greatly increased morbidity, including prolonged mechanical ventilation and increased length of hospital stay . Fontan survivors are also at increased risk for developing chronic kidney disease later on in life with a prevalence of up to 50% after 10 years …”

Section: Introductionmentioning

confidence: 99%

“…2 Fontan survivors are also at increased risk for developing chronic kidney disease later on in life with a prevalence of up to 50% after 10 years. 3 Exogenous agents such as inulin, Ethylenediaminetetraacetic acid, and iohexol are the gold standard for assessing glomerular filtration rate. These molecules are freely filtered through the glomerulus and are neither secreted nor reabsorbed.…”

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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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Prevalence and Risk Factors Associated With Chronic Kidney Disease in Patients With Single Ventricle Congenital Heart Disease After Fontan Palliation

Cited by 2 publications

References 0 publications

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

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

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