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Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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The Fontan procedure has markedly improved the prognosis of patients with single-ventricle physiology. However, long-term survivors often develop multiorgan dysfunction caused by chronic venous congestion, known as Fontan-associated disorders. Renal dysfunction in this population, termed Fontan-associated nephropathy, is thought to result from sustained venous congestion and decreased renal perfusion. The optimal renal replacement therapy (RRT) for these patients has not been established, and hemodialysis (HD) is often limited by hemodynamic instability. We report a case of a patient with Fontan-associated end-stage kidney disease (ESKD) who was successfully transitioned to maintenance peritoneal dialysis (PD).
A 27-year-old man with hypoplastic left heart syndrome and aortic coarctation underwent a Fontan operation at 1 year and 11 months of age. Sixteen years before admission, he developed protein-losing enteropathy but maintained normal renal function (serum creatinine 1.00 mg/dL, creatinine clearance 102.5 mL/min) until nine years before presentation. Thereafter, renal function gradually declined, and despite fenestration, lymphatic embolization, and repeated concentrated ascites reinfusion therapy (CART), ascites progressively worsened.
He was admitted with worsening ascites and dyspnea. Chest radiography revealed pulmonary congestion. On admission, serum creatinine was 1.7 mg/dL and BUN 66 mg/dL. Although urine output was preserved with paracentesis and diuretics, pulmonary congestion persisted. Urine output gradually decreased, and by hospital day 14 he became anuric, with a serum creatinine of 5.54 mg/dL. Continuous hemodiafiltration (CHDF) was initiated, followed by intermittent HD; however, hypotension limited ultrafiltration, and HD was poorly tolerated.
On hospital day 48, a PD catheter was placed, and PD was initiated using 1.5% glucose solution (1,500 mL × 2 exchanges/day). PD was well tolerated and achieved effective ascites management. With improvement in renal congestion, urine output recovered to 600–700 mL/day, and he was discharged on hospital day 75.
Patients with Fontan circulation are predisposed to cardiorenal syndrome due to chronically elevated central venous pressure and reduced renal perfusion. In such cases, PD offers several advantages over HD:
1.Gentle and continuous ultrafiltration, resulting in hemodynamic stability
2.Preservation of residual renal function
3.Avoidance of abrupt preload reduction that can compromise cardiac output
Furthermore, the ability to flexibly adjust ultrafiltration volume makes PD particularly effective for managing ascites in patients with Fontan-associated ESKD. This case demonstrates that PD can be a feasible and hemodynamically stable RRT option when HD is poorly tolerated.
In a patient with Fontan-associated ESKD, peritoneal dialysis provided effective fluid management, hemodynamic stability, and preservation of residual renal function. PD should be considered a viable and safe renal replacement option for patients with Fontan circulation and chronic venous congestion.
