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Long-Term Kidney Function After the Fontan Operation: JACC Review Topic of the Week
Faizeen Zafar1, Adam M Lubert2, David A Katz2
1Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: https://twitter.com/FaizeenZafar.
Insights
The Fontan procedure improves survival for single ventricle patients, but long-term renal dysfunction risks are unclear. Further research is needed to understand Fontan-associated renal disease and guide future care.
Area of Science:
- Cardiology
- Nephrology
- Pediatric cardiology
Background:
- The Fontan procedure enhances survival and quality of life for individuals with single ventricle physiology.
- Increased life expectancy in this population leads to a rise in long-term, multi-organ adverse effects.
- Cardiorenal interactions are well-understood in healthy hearts but less so in single ventricle patients.
Purpose of the Study:
- To review the pathophysiology and spectrum of Fontan-associated renal disease.
- To identify knowledge gaps in current evidence regarding renal dysfunction in Fontan patients.
- To guide future research directions in this field.
Main Methods:
- Systematic review of published literature.
- Analysis of studies investigating glomerular filtration rate and albuminuria in Fontan population.
- Synthesis of information on Fontan-associated renal disease.
Main Results:
- Several studies report reduced glomerular filtration rate and albuminuria in the Fontan population.
- The long-term implications of renal dysfunction in this cohort are not well-established.
- The pathophysiology and spectrum of Fontan-associated renal disease are complex.
Conclusions:
- Fontan-associated renal disease is an emerging clinical concern in the growing Fontan population.
- Further research is crucial to elucidate the long-term consequences of renal dysfunction post-Fontan.
- Identifying research gaps will facilitate targeted investigations into Fontan-associated renal disease.
Abstract:
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.
