Fontan-associated nephropathy: Predictors and outcomes

Background: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown. Methods: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV. Results: Serum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR <90 mL/min/1.72m(2). Renal dysfunction was present in 67/328 (20%) and 3/328 (1%) patients had an eGFR <60 mL/min/1.72m(2). The 10-year survival and 10-year freedom from death and transplantation were the same, 96% (95% CI: 0.9-1) for those with renal dysfunction, and 89% (0.83-0.95; p = 0.1) and 87% (95% CI: 0.81-0.94; p = 0.05) for patients without dysfunction. The 10-year freedom from failure were also similar, 83% (95% CI: 0.70-0.97) for those without renal dysfunction vs 80% (95% CI: 0.74-0.89; p = 0.84). There was no change in mean eGFR for the renal dysfunction group over a mean of 8 +/- 5.5 years. Conclusion: By the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated. (c) 2020 Elsevier B.V. All rights reserved.