Okada Satoshi, Kamei Koichi, Nishi Kentaro, Ogura Masao, Ono Hiroshi, Sakamoto Seisuke
Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-Ku, Tokyo, 157-8535, Japan.
Division of Cardiology, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Nephrol. 2025 Jun 2. doi: 10.1007/s00467-025-06826-x.
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 m). This case demonstrates that selected patients with Fontan circulation can safely undergo KT through appropriate preoperative assessment and perioperative management.
我们报告了一名患有Fontan循环的14岁男孩成功进行活体供肾移植（KT）的病例。他出生时被诊断为大动脉完全转位（3型）。在进行Blalock-Taussig分流术后，他出现了急性肾损伤，后恢复为4期慢性肾脏病。2岁时，他接受了单阶段Fontan手术以减少手术次数。7岁时，他开始进行腹膜透析。13岁时，通过术前液体负荷心脏导管检查评估了KT的可行性，结果显示在KT过程中可以维持足够的全身灌注。经过多学科术前评估后，在细致的血流动力学监测下成功进行了KT。KT术后1年，患者肾功能良好（估计肾小球滤过率：78 mL/min/1.73 m²）。该病例表明，经过适当的术前评估和围手术期管理，选定的Fontan循环患者可以安全地接受KT。
