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.
Highlights.
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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