Huang, Szu-WeiSzu-WeiHuangHENG-WEN CHOUSHU-CHIEN HUANGNAI-KUAN CHOUCHIH-HSIEN WANGNAI-HSIN CHIHSI-YU YURON-BIN HSUCHING-CHIA WANGWEI-CHIEH TSENGYIH-SHARNG CHENCHUN-AN CHENSHUENN-NAN CHIUEN-TING WUCHUN-WEI LUJOU-KOU WANGMING-TAI LINMEI-HWAN WU2025-03-242025-03-242025-05https://scholars.lib.ntu.edu.tw/handle/123456789/726029Background: The Fontan operation is widely regarded as a highly successful and effective palliative measure for many congenital heart diseases. However, among patients who undergo Fontan procedures, Fontan failure inevitably occurs in some cases, giving rise to various complications, including protein-losing enteropathy, plastic bronchitis, refractory arrhythmia, and coagulopathy. When Fontan fails, heart transplantation emerges as one of the treatment options. Methods: This retrospective study examines the outcomes of heart transplantation in 10 patients experiencing Fontan failure over a span of 23 years at a single institution. Results: The statistical analysis reveals survival rates of 80.0%, 60.0%, and 33.3% at 1, 3, and 5 years posttransplantation, respectively. Moderate or severe atrioventricular valve regurgitation (AVVR) emerges as a risk factor for posttransplantation mortality. Additionally, pretransplant atrial tachycardia is identified as a risk factor for early mortality following heart transplantation. Conclusions: Moderate or severe AVVR is a risk factor for posttransplantation mortality in patients with Fontan circulation failure, while pretransplant atrial tachycardia is a risk factor for early mortality after cardiac transplantation. © 2025 Wiley Periodicals LLC.encardiac transplantationpediatric heart transplantsurvivaljournal article10.1111/petr.7005140033557