Long-Term Kidney Function After the Fontan Operation: JACC Review Topic of the Week.

The Fontan procedure has improved survival and quality of life for patients with single ventricle physiology. Along with extended life expectancy comes a growing population that is experiencing long-term multiorgan adverse effects. Whereas cardiorenal interactions have been extensively studied in patients with a structurally normal heart, these are less well understood in patients with a single ventricle.

Several studies have investigated the prevalence of reduced glomerular filtration rate and albuminuria in the Fontan population; however, the long-term implication of renal dysfunction is not well established in this population. This paper provides a concise review of the published reports on the pathophysiology and spectrum of Fontan-associated renal disease. It also identifies gaps in currently available evidence that can guide ongoing and future research.


Hemodynamic derangements after the Fontan operation may lead to renal dysfunction.

Creatinine-based equations overestimate the GFR in individuals with a Fontan circulation.

Cystatin C-based GFR is more reliable and may predict adverse Fontan outcomes.

Longitudinal studies are needed to understand the morbidity and mortality burden of Fontan-associated renal disease.

1. Rychik J., Atz A. M., Celermajer D. S., et al. “Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association”. Circulation 2019; 140: e234-e284.

2. Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group. “KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease”. Kidney Int Suppl 2013; 3: 1-150.

3. darlehen Opotowsky A. R., Carazo M., Singh M. N., et al. “Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: results of the Boston Adult Congenital Heart Disease Biobank”. Am Heart J 2019; 214: 142-155.

4. Opotowsky A., Baraona F., Mc Causland F., et al. “Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation”. Heart 2017; 103: 434-442.

5. Sharma S., Reubner R. L., Furth S. L., et al. “Assessment of kidney function in survivors following Fontan palliation”. Congenit Heart Dis 2016; 11: 630-636.

6. Lee D., Levin A., Kiess M., et al. “Chronic kidney damage in the adult Fontan population”. Int J Cardiol 2018; 257: 62-66.

7. Broda C., Sriraman H., Wadhwa D., et al. “Renal dysfunction is associated with higher central venous pressures in patients with Fontan circulation”. Congenit Heart Dis 2018; 13: 602-607.

8. Wilson T., d’Udekem Y., Winlaw D., et al. “Hepatic and renal end-organ damage in the Fontan circulation: a report from the Australian and New Zealand Fontan Registry”. Int J Cardiol 2018; 273: 100-107.

Deixe uma resposta

O seu endereço de e-mail não será publicado. Campos obrigatórios são marcados com *