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Published on: June 10, 2025
Fontan-associated nephropathy: Predictors and outcomes.
Jacqueline Nguyen Khuong1, Thomas G Wilson1, Leeanne E Grigg2
1Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia.
Twenty percent of adults with Fontan circulation develop renal dysfunction. Fontan-associated nephropathy is well tolerated over a decade, with similar outcomes for patients with and without renal impairment.
Area of Science:
- Cardiology
- Nephrology
- Pediatric Cardiology
Background:
- Nephropathy is a recognized complication of Fontan circulation.
- Determinants and long-term outcomes of Fontan-associated nephropathy remain largely unknown.
Purpose of the Study:
- To identify determinants of nephropathy in Fontan circulation patients.
- To evaluate patient outcomes in relation to renal function.
Main Methods:
- Utilized data from the Australia and New Zealand Fontan Registry.
- Included patients >16 years old with intact Fontan circulation.
- Calculated estimated glomerular filtration rate (eGFR) using MDRD equation; defined renal dysfunction as eGFR <90 mL/min/1.72m².
Main Results:
- 20% (67/328) of patients exhibited renal dysfunction (eGFR <90 mL/min/1.72m²).
- 10-year survival and freedom from death/transplantation were similar between groups.
- 10-year freedom from Fontan failure was also comparable.
Conclusions:
- Adults with Fontan circulation have a 20% prevalence of renal dysfunction.
- Fontan-associated nephropathy appears well-tolerated over a 10-year period.
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