Successful living donor kidney transplantation in a 14-year-old boy with Fontan circulation.

We report successful living donor kidney transplantation (KT) in a 14-year-old boy with Fontan circulation. He was diagnosed with complete transposition of the great arteries (type 3) at birth. After Blalock-Taussig shunt placement, he developed acute kidney injury, which recovered to stage 4 chronic kidney disease. At 2 years old, he underwent a single-stage Fontan procedure to reduce the number of surgeries. At 7 years old, he started peritoneal dialysis. At 13 years old, the feasibility of KT was evaluated using preoperative cardiac catheterization with fluid loading, revealing that adequate systemic perfusion could be maintained during KT. Following a multidisciplinary preoperative assessment, KT was successfully performed under meticulous hemodynamic monitoring. At 1-year post-KT, the patient showed good kidney function (estimated glomerular filtration rate: 78 mL/min/1.73 m²). This case demonstrates that selected patients with Fontan circulation can safely undergo KT through appropriate preoperative assessment and perioperative management.