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.
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